PURPOSE: This study aimed to clarify surgical complications associated with open surgery for congenital diaphragmatic hernia (CDH). METHODS: We performed an exploratory data analysis of the clinical characteristics of surgical complications of neonates with CDH who underwent laparotomy or thoracotomy between 2006 and 2021. Data of these patients were obtained from the database of the Japanese CDH Study Group. RESULTS: Among 1,111 neonates with left or right CDH, 852 underwent open surgery (laparotomy or thoracotomy). Of these 852 neonates, 51 had the following surgical complications: organ injury (n = 48; 6% of open surgeries); circulatory failure caused by changes in the organ location (n = 2); and skin burns (n = 1). Injured organs included the spleen (n = 30; 62% of organ injuries), liver (n = 7), lungs (n = 4), intestine (n = 4), adrenal gland (n = 2), and thoracic wall (n = 2). Fourteen of the patients who experienced organ injury required a blood transfusion (2% of open surgeries). The adjusted odds ratio of splenic injury for patients with non-direct closure of the diaphragm was 2.2 (95% confidence interval, 1.1-4.9). CONCLUSION: Of the patients who underwent open surgery for CDH, 2% experienced organ injury that required a blood transfusion. Non-direct closure of the diaphragmatic defect was a risk factor for splenic injury.

BACKGROUND: Although the fetal sphericity index (SI) and fractional shortening (FS) of 24 transverse segments have been previously reported after the 20th gestational week, there have been no reports during the first and early second trimester. OBJECTIVE: This study aimed to clarify the SI and FS of 24 transverse segments in normal fetuses before the 20th gestational week. METHODS: A total of 101 normal fetuses aged between 12 and 20 gestational weeks were examined. The displacement of the ventricular endocardium during the cardiac cycle was computed using speckle-tracking software (GE Healthcare, Milwaukee, WI). We analyzed the length of 24 end-diastolic lateral segments and the end-diastolic basal (seg1-6)- middle (Seg7-15)-apical (Seg16-24) distribution from the base to the apex of each ventricle, according to the method described by DeVore et al. The SI and FS were computed for each of the 24 segments by dividing the mid-basal-apical length by the transverse size. RESULTS: The SI for each segment was independent of the gestational age. The SI of the right ventricle was significantly lower than that of the left ventricle for segments 1-14, suggesting that the right ventricle was more spherical than the left ventricle in the basal segment only. The FS of the right ventricle was significantly lower than that of the left ventricle in segments 1 to 2 and 13 to 24. CONCLUSION: The morphology of the ventricles before 20 weeks of gestation differs from that between 20 and 40 weeks of gestation. This difference may be related to myocardial densification or performance.

症例は30代男性.幼少期に完全大血管転位III型に対してFontan手術が施行され,近医に定期的に通院していた.20XX年7月腹部USで多発肝腫瘤を指摘され当院紹介受診となった.造影CTにて最大13cmの多発肝細胞癌と判明した(BCLC stage B).画像上は門脈圧亢進所見や明らかな肝形態異常を認めなかったが,肝生検でCongestive Hepatic Fibrosis Score 3であり,実際には線維化の進展を認めていた.肝内多発のため外科手術やRFAの適応外であった.また最大径の腫瘍は肝外に突出しており,腹腔内破裂の危険性もあることから,まずTACEを施行した.再発に応じて各種抗癌剤治療を行い,生存中である.画像上は肝線維化を示唆する所見はなかったが,Fontan術後の特殊な循環動態では,肝線維化が進展している可能性があり,本症例を通して肝癌サーベイランスの重要性を再考する.(著者抄録)

OBJECTIVE: To assess the reliability of pulmonary artery (PA) parameters as a prognostic marker in neonates with isolated left-sided congenital diaphragmatic hernia (IL-CDH). STUDY DESIGN: A retrospective cohort study conducted by the Japanese CDH Study Group (JCDHSG). RESULTS: 323 IL-CDH patients registered with the JCDHSG were included. 272 patients survived to 90 days of age. Right PA (RPA) and left PA (LPA) diameters and pulmonary artery index (PAIndex) at birth were significantly larger in survivors. The cutoff values of RPA and LPA diameters and PAIndex for survival up to 90 days were 3.2 mm, 2.8 mm and 83.7, respectively, and logistic regression analysis showed that these were significantly related to survival. Multiple logistic regression analysis showed that both the PA parameters and liver herniation were significantly related to survival. CONCLUSIONS: The three PA parameters at birth can predict clinical outcomes and are considered as independent risk factors of liver herniation.

OBJECTIVES: To evaluate the survival and intact-survival rates among preterm infants with congenital diaphragm hernia (CDH). STUDY DESIGN: Multicenter retrospective cohort study of 849 infants born between 2006 and 2020 at 15 Japanese CDH study group facilities. Multivariate logistic regression analysis adjusted using inverse probability treatment weighting (IPTW) method was used. We also compare trends of intact-survival rates among term and preterm infants with CDH. RESULTS: After adjusting using the IPTW method for CDH severity, sex, APGAR score at 5 min, and cesarean delivery, gestational age and survival rates have a significantly positive correlation [coefficient of determination (COEF) 3.40, 95% confidence interval (CI), 1.58-5.21, p value <0.001] and higher intact-survival rate [COEF 2.39, 95% CI, 1.73-4.06, p value 0.005]. Trends of intact-survival rates for both preterm and term infants had significantly changed, but improvement in preterm infants was much smaller than in term infants. CONCLUSION: Prematurity was a significant risk factor for survival and intact-survival among infants with CDH, regardless of adjustment for CDH severity.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a severe congenital disease. Some CDH infants suffer from gastro-esophageal reflux disease (GERD), even after surgical correction of gastric position. A transpyloric tube (TPT) is inserted into CDH patients under direct observation intraoperatively in some hospitals in Japan to establish early enteral feeding. This strategy avoids gastric expansion to maintain a better respiratory condition. However, it is unclear whether the strategy has a secure effect for patient prognosis. This study aimed to evaluate the effectiveness of intraoperative TPT insertion on enteral feeding and postoperative weight gain. METHODS: The Japanese CDH Study Group database was used to identify infants with CDH born between 2011 and 2016, who were then divided into two groups: the TPT group and gastric tube (GT) group. In the TPT group, infants underwent intraoperative TPT insertion; postoperative insertion/extraction of TPT was irrelevant to the analysis. Weight growth velocity (WGV) was calculated using the exponential model. Subgroup analysis was performed using Kitano's gastric position classification. RESULTS: We analyzed 204 infants, of which 99 and 105 were in the TPT and GT groups, respectively. Enteral nutrition (EN) in the TPT and GT groups was 52 ± 39 and 44 ± 41 kcal/kg/day (p = 0.17) at age 14 days (EN14), respectively, and 83 ± 40 and 78 ± 45 kcal/kg/day (p = 0.46) at age 21 days (EN21), respectively. WGV30 (WGV from day 0 to day 30) in the TPT and GT groups was 2.3 ± 3.0 and 2.8 ± 3.8 g/kg/day (p = 0.30), respectively, and WGV60 (WGV from day 0 to day 60) was 5.1 ± 2.3 and 6.0 ± 2.5 g/kg/day (p = 0.03), respectively. In infants with Kitano's Grade 2 + 3, EN14 in the TPT and GT groups was 38 ± 35 and 29 ± 35 kcal/kg/day (p = 0.24), respectively, EN21 was 73 ± 40 and 58 ± 45 kcal/kg/day (p = 0.13), respectively, WGV30 was 2.3 ± 3.2 and 2.0 ± 4.3 g/kg/day (p = 0.76), respectively, and WGV60 was 4.6 ± 2.3 and 5.2 ± 2.3 g/kg/day (p = 0.30), respectively. CONCLUSION: Intraoperative TPT insertion did not improve nutritional intake and WGV30. WGV60 in TPT was less than that in GT. In Grade 2 + 3 subgroup analysis, TPT also had no advantage. We could not recommend routine TPT insertion at surgery. LEVEL OF EVIDENCE: III.

We encountered two consecutive cases of adult-onset Kawasaki disease (AKD) that were difficult to diagnose. In both cases, Kawasaki disease was not considered as a differential diagnosis in the early stages. However, it was possible to make a diagnosis by citing the disease as a differential diagnosis and introducing the patients to the pediatrics department. AKD has a minimal incidence rate and may have a clinical course different from that of childhood-onset Kawasaki disease. Therefore, it is necessary to incorporate Kawasaki disease into the differentiation of an adult fever and to consult with a pediatrician for its diagnosis.

Coronary aneurysm secondary to coronary fistula is a rare condition, with no existing report on its pathological examination. We report the case of a patient diagnosed with a right coronary artery fistula with coronary aneurysm during the fetal period. During follow-up after delivery, the aneurysm became larger, even though the shunt size decreased. We were afraid the aneurysm would rupture and therefore, planned elective catheter embolization. At the age of 4 years, the patient underwent surgery, which involved closing the fistula and making the lumen of the aneurysm smaller. However, the surgery was not catheter embolization as planned because segment 3 branched off from the largest aneurysm where we planned to embolize. Pathologically, the structure of the coronary artery differed from that of a healthy one, with thickened intima and media, fewer scattered smooth muscle cells, widely distributed elastic fibers, and mucoid degeneration in the media. The structure of the coronary artery suggested that the vessel wall was weak and that the aneurysm would rupture if not treated. Postoperative coronary angiography showed that segment 2 was obstructed, while the collaterals from the left coronary artery perfused the area. We could have treated the fistula with a catheter as scheduled.

PURPOSE: To develop a predictive score for small diaphragmatic defects in infants with congenital diaphragmatic hernia (CDH) for determining thoracoscopic surgery indication. METHODS: The Japanese CDH Study Group cohort was randomly divided into derivation (n = 397) and validation (n = 396) datasets. Using logistic regression, a prediction model and weighted scoring system for small diaphragmatic defects were created from derivation dataset and validated with validation dataset. RESULTS: Six weighted variables were selected: no hydramnios, 1 point; 1 min Apgar score of 5-10, 1 point; apex type of the lung (left lung is detected radiographically in apex area), 1 point; oxygenation index < 8, 1 point; abdominal nasogastric tube (tip of the nasogastric tube is detected radiographically in the abdominal area), 2 points; no right-to-left flow of ductus arteriosus, 1 point. In validation dataset, rates of small diaphragmatic defects for Possible (0-3 points), Probable (4-5 points), and Definite (6-7 points) groups were 36%, 81%, and 94%, respectively (p < 0.001). Additionally, sensitivity, specificity, positive predictive value, and C statistics were 0.78, 0.79, 0.88, 0.76, and 0.45, 0.94, 0.94, 0.70 for Probable and Definite groups, respectively. CONCLUSION: Our scoring system effectively predicted small diaphragmatic defects in infants with CDH.

BACKGROUND: Mortality prediction of congenital diaphragmatic hernia (CDH) is essential for developing treatment strategies, including fetal therapy. Several researchers have reported prognostic factors for this rare but life-threatening condition; however, the optimal combination of prognostic factors remains to be elucidated. OBJECTIVES: This study aimed to develop the most discriminative prenatal and postnatal models to predict the mortality of infants with an isolated left-sided CDH. METHODS: This multi-institutional retrospective cohort study included infants with CDH born at 15 tertiary hospitals of the Japanese CDH Study Group between 2011 and 2016. We developed multivariable logistic models with every possible combination of predictors and identified models with the highest cross-validated area under the receiver operating characteristic curve (AUC) for prenatal and postnatal predictions. RESULTS: Among 302 eligible infants, 44 died before discharge. The prenatal mortality prediction model was based on the observed/expected lung area to head circumference ratio (O/E LHR), liver herniation, and stomach herniation (AUC, 0.830). The postnatal mortality prediction model was based on O/E LHR, liver herniation, and the lowest oxygenation index (AUC, 0.944). CONCLUSION: Our models can facilitate the prenatal and postnatal mortality prediction of infants with isolated left-sided CDH.

PURPOSE: The prenatal diagnosis of the stomach position in congenital diaphragmatic hernia (CDH) has been a reliable prognostic factor, but few studies have focused on the postnatal position. We therefore evaluated the significance of the nasogastric (NG) tube position just after birth. METHODS: The Japanese CDH Study Group database enrolled 1037 CDH neonates over 15 years. In our multicenter retrospective study, 464 cases of left-sided isolated CDH with prenatal diagnoses were divided into two groups: NG tube below the diaphragm (BD; n = 190) or above the diaphragm (AD; n = 274). The primary outcome was the 90-day survival rate, and the secondary outcomes were mechanical ventilation duration, hospitalization duration, and recurrence rate. RESULTS: The BD group had a significantly higher 90-day survival rate (98.4 vs. 89.4%, p < 0.001), shorter mechanical ventilation (11 vs. 19 days, p < 0.001), shorter hospitalization (38 vs. 59 days, p < 0.001), and lower recurrence rate (p = 0.002) than the AD group. A multivariate analysis showed that BD (adjusted odds ratio, 3.68; 95% confidence interval 1.02-13.30) was a favorable prognostic factor for the 90-day survival. CONCLUSION: The assessment of the NG tube position revealed it to be a reliable prognostic factor of left-sided isolated CDH. Therefore, it should be included as a routine assessment.

PURPOSE: This study aimed to evaluate prenatal predictors of mortality in fetuses with congenital diaphragmatic hernia (CDH). METHODS: A systematic literature search was performed to identify relevant observational studies that evaluated the ability of lung-to-head ratio (LHR), observed-to-expected LHR (o/e-LHR), observed-to-expected total fetal lung volume (o/e-TFLV), lung-to-thorax transverse area ratio (L/T ratio), intrathoracic herniation of the liver and the stomach, and side of diaphragmatic hernia, using a threshold for the prediction of mortality in fetuses with CDH. Study quality was assessed using the QUADAS-2 tool. Hierarchical summary receiver operating characteristic curves were constructed. RESULTS: A total of 50 articles were included in this meta-analysis. The QUADAS-2 tool identified a high risk of bias in more than one domain scored in all parameters. Among those parameters, the diagnostic odds ratio of mortality with o/e-LHR < 25%, o/e-TFLV < 25%, and L/T ratio < 0.08 were 11.98 [95% confidence interval (CI) 4.65-30.89], 11.14 (95% CI 5.19-23.89), and 10.28 (95% CI 3.38-31.31), respectively. The predictive values for mortality were similar between the presence of liver herniation and retrocardiac fetal stomach position. CONCLUSIONS: This systematic review suggests that o/e-LHR, o/e-TFLV, and L/T ratio are equally good predictors of neonatal mortality in fetuses with isolated CDH.

PURPOSE: The objective of our study was to compare and consider reference values of fetal atrioventricular (AV) intervals as measured by four different pulsed Doppler wave techniques (left ventricular inflow/outflow [LV in/out], pulmonary vein/pulmonary artery [PV/PA], innominate vein/ascending aorta [InnV/AA], and supra vena cava/ascending aorta [SVC/AA]) in pregnant women with anti-SSA/Ro antibodies. METHODS: Between March 2014 and September 2020, 52 pregnant women with anti-SSA antibodies were enrolled. No bradyarrhythmia was observed in the group. A pulsed Doppler examination of the fetal heart was performed to obtain measurements of the mechanical Doppler AV interval. Doppler measurements were performed using four methods: LV in/out, PV/PA, InnV/AA, and SVC/AA. A statistical analysis was performed to examine the mean, standard deviation, significant difference, and correlation of the four methods. The detection rate of each method was also calculated. RESULTS: There was no significant difference in the AV intervals between any of the four methods. There was also a positive correlation in the AV intervals of each of the four methods. The fetal heart rate and AV interval showed no correlation. The gestational age and AV interval also showed no correlation. The detection rate was highest for LV in/out (62.6%, 95% confidence interval: 56.5-68.4). CONCLUSION: All four pulsed Doppler methods are useful for measuring AV intervals. The most practical method is LV in/out.

BACKGROUND: Treatment modalities for neonates with congenital diaphragmatic hernia (CDH) have greatly improved in recent years, with a concomitant increase in survival. However, long-term outcomes restrict the identification of optimal care pathways for CDH survivors in adolescence and adulthood. Therefore, we evaluated the long-term outcomes within the Japanese CDH Study Group (JCDHSG). METHODS: Participants were born with CDH between 2006 and 2018 according to the JCDHSG. Participants were enrolled in the database at 1.5, 3, 6, and 12 years old. Follow-up items included long-term complications, operations for long-term complication, and home medical care. RESULTS: A total of 747 patients were included in this study, with 626 survivors (83.8%) and 121 non-survivors (16.2%). At 1.5, 3, 6, and 12 years old, 45.4%, 36.5%, 34.8%, and 43.6% developed complications, and 20.1%, 14.7%, 11.5%, and 5.1% of participants required home care, respectively. Recurrence, pneumonia, pneumothorax, gastroesophageal reflux disease, and intestinal obstruction decreased with age, and thoracic deformity increased with age. CONCLUSIONS: As CDH survival rates improve, there is a need for continued research and fine-tuning of long-term care to optimize appropriate surveillance and long-term follow-up.

We report the case of a 4-year-old boy with coarctation of the aorta resulting from persistent fifth aortic arch, a rare abnormality, along with an interrupted fourth aortic arch. When he visited a general practitioner with an upper respiratory infection, a heart murmur was noted. Computed tomography findings led to the diagnosis of persistent fifth aortic arch with an interrupted fourth aortic arch. He underwent aortic arch repair, excision of the fifth aortic arch, and anastomosis of the original arch with the descending aorta. Pathologically, the tissue of the fifth aortic arch was different from that of the normal aortic arch.

PURPOSE: Predicting lethal pulmonary hypoplasia in infants with congenital diaphragmatic hernia (CDH) before extracorporeal membrane oxygenation (ECMO) initiation is difficult. This study aimed to predict lethal pulmonary hypoplasia in patients with CDH prior to ECMO. METHODS: This was a multicenter cohort study involving neonates prenatally diagnosed with isolated unilateral CDH (born 2006-2020). Patients who required ECMO due to respiratory insufficiency were included in this study. Patients who underwent ECMO due to transient disorders were excluded from analysis. Blood gas analysis data within 24 h of birth were compared between survivors and non-survivors. Predictive abilities were assessed for factors with significant differences. RESULTS: Overall, 34 patients were included (18 survivors and 16 non-survivors). The best pre-ductal PaO2 was significantly lower in non-survivors than in survivors (50.4 [IQR 30.3-64.5] vs. 67.5 [IQR 52.4-103.2] mmHg, respectively; p = 0.047). A cutoff PaO2 of 42.9 mmHg had a sensitivity, specificity, and positive predictive value of 50.0%, 94.4%, and 88.9%, respectively, to predict mortality. CONCLUSION: The best PaO2 within 24 h after birth predicted mortality following ECMO initiation. This should be shared to families and caregivers to optimize the best interests of the infants with CDH.

Absent pulmonary valve syndrome (APVS) with tricuspid atresia is very rare. We report a case of APVS in which the fetus died due to severe hydrops fetalis. In addition to weakened cardiac contraction due to left ventricular noncompaction cardiomyopathy, a circular shunt through a ventricular septal defect exacerbated heart failure. Although APVS with tricuspid valve atresia is not supposed to cause a circular shunt, we herein present the echocardiographic demonstration of a case of circular shunt.

PURPOSE: To date, there is no compelling evidence of the optimal timing of surgery for congenital diaphragmatic hernia (CDH). This study aimed to establish the optimal timing of surgery in neonates with isolated left-sided CDH. METHODS: This multicenter cohort study enrolled 276 patients with isolated left-sided CDH at 15 institutions. Based on the timing of surgery, the patients were classified into four groups [< 24 h (G1), 24-47 h (G2), 48-71 h (G3), and ≥ 72 h (G4)]. The clinical outcomes were compared among the groups using a logistic regression model, after adjusting for potential confounders, such as disease severity. RESULTS: Multivariate analyses showed that G2 also had a lower mortality rate than the other groups. In mild and severe cases, there were no significant differences in mortality across the four groups. In moderate cases, G2 resulted in significantly increased survival rates, compared with G1. CONCLUSION: The study results suggest that surgery within 24 h of birth is not recommended for patients with moderate severity CDH, that there is no benefit in the delay of surgery for more than 72 h in mild severity CDH patients, and that there is no definite optimal time to perform surgery in severe cases of CDH.

We diagnosed two cases of double aortic arch (DAA) remotely. In both cases, the right aortic arch was suspected, and a remote diagnosis was requested. We performed a remote diagnosis by the spatio-temporal image correlation (STIC) method. DAA was diagnosed by detecting the left aortic arch in an axial transverse section in the three-vessel trachea view. Since the STIC method can make multiple cross sections, making a diagnosis by the STIC method was useful for the remote diagnosis of these patients. In particular, HD flow render mode clearly showed left aortic arch, so it was possible to diagnose DAA.

PURPOSE: We compared the efficacy of thoracoscopic repair (TR) with that of open repair (OR) for neonatal congenital diaphragmatic hernia (CDH). METHODS: The subjects of this multicenter retrospective cohort study were 524 infants with left-sided isolated CDH, diagnosed prenatally, and treated at one of 15 participating hospitals in Japan between 2006 and 2018. The outcomes of infants who underwent TR and those who underwent OR were compared, applying propensity score matching. RESULTS: During the study period, 57 infants underwent TR and 467 underwent OR. Ten of the infants who underwent TR required conversion to OR for technical difficulties and these patients were excluded from the analysis. The survival rate at 180 days was similar in both groups (TR 98%; OR 93%). Recurrence developed after TR in 3 patients and after OR in 15 patients (TR 7%, OR 3%, p = 0.40). The propensity score was calculated using the following factors related to relevance of the surgical procedure: prematurity (p = 0.1), liver up (p < 0.01), stomach position (p < 0.01), and RL shunt (p = 0.045). After propensity score matching, the multivariate analysis adjusted for severity classification and age at surgical treatment revealed a significantly shorter hospital stay (odds ratio 0.50) and a lower incidence of chronic lung disease (odds ratio 0.39) in the TR group than in the OR group. CONCLUSIONS: TR can be performed safely for selected CDH neonates with potentially better outcomes than OR.

OBJECTIVE: To determine the appropriate initial ventilatory mode for neonatal congenital diaphragmatic hernia (CDH) by comparing patient prognosis following conventional mechanical ventilation (CMV) versus high-frequency oscillatory ventilation (HFO). STUDY DESIGN: This multicenter retrospective cohort study was performed at 15 participating hospitals in Japan between 2011 and 2016. The 328 eligible CDH infants were classified into CMV (n = 78) and HFO groups (n = 250) to compare mortality and incidence of bronchopulmonary dysplasia (BPD). Propensity score matching was applied to reduce confounding by indication. RESULT: While crude mortality was significantly higher in the HFO than the CMV group, adjusted odds ratio (OR) did not show significant difference in mortality between groups (OR of HFO group: 0.98, 95% confidence interval (CI): 0.57-1.67). Adjusted OR of BPD incidence showed no significant difference between groups (OR of HFO group: 1.66, 95%CI: 0.50-5.49). CONCLUSION: Initial ventilatory mode in CDH patients, whether CMV or HFO, does not affect prognosis.

We prospectively performed remote fetal cardiac screening using the spatio-temporal image correlation (STIC), and examined the usefulness and problems of remote screening. We performed heart screening for all pregnant women at four obstetrics clinics over the three years from 2009 to 2014. The STIC data from 15,404 examinations in normal pregnancies (16-27 weeks, median 25 weeks) were analyzed. Obstetricians and sonographer collected STIC data from four-chamber view images. Eight pediatric cardiologists analyzed the images offline. A normal heart was diagnosed in 14,002 cases (90.9%), an abnormal heart was diagnosed in 457 cases (3.0%), and poor images were obtained in 945 cases (6.1%). 138 cases had congenital heart disease (CHD) after birth, and severe CHD necessitating hospitalization occurred in 36 cases. We were not able to detect CHD by screening in 12 cases. The sensitivity and specificity of STIC in CHD screening was 50% and 99.5%, respectively. The sensitivity and specificity of STIC in screening for severe CHD was 82% and 99.9%, respectively. The STIC method was useful in fetal remote screening for CHD. However, the fact that > 10% of images that could not be analyzed by this method was a problem.

PURPOSE: This study aimed to elucidate the clinical characteristics of neonates with congenital diaphragmatic hernia (CDH) associated with pneumothorax and evaluate the risk factors for the development of pneumothorax. METHODS: A retrospective cohort study was conducted in the 15 institutions participating in the Japanese CDH Study Group. A total of 495 neonates with isolated CDH who were born between 2011 and 2018 were analyzed in this study. RESULTS: Among the 495 neonates with isolated CDH, 52 (10.5%) developed pneumothorax. Eighteen (34.6%) patients developed pneumothorax before surgery, while 34 (65.4%) developed pneumothorax after surgery. The log-rank test showed that the cumulative survival rate was significantly lower in patients with pneumothorax than in those without pneumothorax. Univariate analysis revealed significant differences between patients with pneumothorax and those without pneumothorax with regard to the best oxygenation index within 24 h after birth, mean airway pressure (MAP) higher than 16 cmH2O, diaphragmatic defect size, and need for patch closure. Multiple logistic regression analysis indicated that only the MAP was associated with an increased risk of pneumothorax. CONCLUSIONS: The cumulative survival rate was significantly lower in isolated CDH patients with pneumothorax than in those without pneumothorax. A higher MAP was a risk factor for pneumothorax in CDH patients.

INTRODUCTION:  We aimed to establish and validate a risk score for fetuses with congenital diaphragmatic hernia (CDH) using only prenatal ultrasound findings. MATERIAL AND METHODS:  Derivation (2011-2016, n = 350) and validation (2006-2010, n = 270) cohorts were obtained from a Japanese CDH study group database. Using a logistic regression analysis, we created a prediction model and weighted scoring system from the derivation dataset and calculated the odds ratio of an unsatisfactory prognosis (death within 90 days of life or hospitalization duration exceeding 180 days). Five adverse prognostic factors obtained using prenatal ultrasound, including an observed/expected lung area-to-head circumference ratio (o/eLHR) <25%, liver herniation occupying more than one-third of the thoracic space, thoracic stomach, right-side CDH, and severe malformations, were used as predictors. The obtained model was validated using the validation cohort. RESULTS:  The unsatisfactory prognosis prediction model was obtained based on the adjusted odds ratios. The C statistics of the model were 0.83 and 0.80 in the derivation and validation datasets, respectively. The five variables were weighted proportionally to their adjusted odds ratios for an unsatisfactory prognosis (o/eLHR <25%, 1 point; liver herniation occupying more than one-third of the thoracic space, 1 point; thoracic stomach, 1 point; right-side CDH, 2 points; and severe malformations, 3 points). Unsatisfactory prognosis rates for the low- (0-2 points), intermediate- (3-5 points), and high-risk (6-8 points) groups were 17, 46, and 100%, respectively (p < 0.001), in the validation cohort. CONCLUSION:  Our simple risk score effectively predicted the prognosis of fetuses with CDH.

Transposition of the great arteries type I is a severe congenital heart disease that induces serious cyanosis immediately after birth and death within 24 hours, unless proper treatment is administered. A few cases have presented with reversed differential cyanosis, manifesting as separated cyanosis with high SpO 2 values in the lower limbs. However, there have been few reports of survivors of transposition of the great arteries type I presenting with reversed differential cyanosis. We experienced a case of transposition of the great arteries type I presenting with reversed differential cyanosis immediately after birth. The infant was urgently transported because of postnatal SpO 2 of 40% in the upper limbs and 90% in the lower limbs. The echocardiographic diagnosis was transposition of the great arteries type I with a narrow foramen ovale. We immediately performed balloon atrioseptostomy, and the reversed differential cyanosis was improved. The infant seems to have presented with reversed differential cyanosis because of the foramen ovale narrowing and complicating pulmonary hypertension due to fetal circulation and characteristic of transposition of the great arteries type I. Congenital heart disease, presenting with reversed differential cyanosis, is a clinical condition requiring emergency management.

Objectives: To investigate the clinical course of fetal tachycardia and analyze the impact of intrauterine treatment on the postnatal treatment and patient outcomes. Study design: This was a retrospective review of cases of fetal tachycardia that occurred from 2004 to 2006. Data were collected from questionnaires that were sent to all 750 secondary or tertiary perinatal care centers in Japan. Results: Eighty-two cases (14 with fetal hydrops) were analyzed (supraventricular tachycardia [SVT], n = 52 atrial flutter [AFL], n = 23 and ventricular tachycardia, n = 7). The overall mortality was 3.7%. Intrauterine treatment was performed for 41 fetuses (50.0%). Digoxin, flecainide and sotalol were mainly used for SVT and AFL. Fetal tachycardia resolved in 90.0% (27/30) of the cases without fetal hydrops and 90.9% (10/11) of the cases with fetal hydrops. Intrauterine treatment significantly reduced the incidence of cesarean delivery (29.3 vs. 70.7%, p < .01), preterm birth (12.2 vs. 41.5%, p =.02) and neonatal arrhythmias (48.8 vs. 78.0%, p =.01) in comparison to untreated fetuses. Conclusions: This nationwide survey revealed that intrauterine treatment was performed for approximately half of the cases of fetal tachycardia and was associated with lower rates of cesarean delivery, premature birth and neonatal arrhythmias in comparison to untreated fetuses.

Purpose: To validate the relationship between retrograde blood flow in the aortic isthmus (AoI-R) by color Doppler in fetal echocardiography and postnatal coarctation of the aorta (CoA) diagnosed as isthmus narrowing. Methods: This was a retrospective study of 22 cases of prenatally suspected simple CoA or CoA with small ventricular septum defect based on fetal echocardiography performed by pediatric cardiologists in our hospital. Gestational age at the first detection of AoI-R and the optimal cut-off value for the prediction of postnatal CoA were mainly evaluated according to the postnatal diagnosis of CoA. Results: All 22 cases had AoI-R prenatally, and nine of them (40.9%) had isthmus narrowing and were diagnosed as having CoA immediately after birth. The gestational age at the first detection of AoI-R was significantly lower in cases with postnatal CoA than in those without (average 34.4 weeks P = 0.034). The cut-off value for the prediction of postnatal CoA was 35.5 weeks of gestation, with a sensitivity and specificity of 77.8 and 69.2%, respectively. Conclusion: AoI-R determined by color Doppler echocardiography can become a useful tool in the screening of fetal CoA, especially at <  35 weeks of gestation.

BACKGROUND: At our institution, we perform bilateral pulmonary artery banding (BPAB) as the first-stage palliation for interrupted aortic arch (IAA) with low birth weight or severe subaortic stenosis (SAS). The present study aimed to identify factors that may influence the decision regarding the type of second-stage operation, that is, univentricular palliation or biventricular repair, in these patients. METHODS: Cardiac catheterization and angiographic data of nine patients with IAA who underwent initial BPAB and subsequent univentricular or biventricular repair were retrospectively analyzed. RESULTS: Between 2004 and 2014, of nine patients with IAA who underwent initial BPAB, biventricular repair was subsequently performed in six patients (group B) and univentricular repair in three patients (group U). All patients survived. There was no significant intergroup difference in IAA classification, location of ventricular septal defect, presence of 22q11.2 deletion, presence of aberrant right subclavian artery, band diameter, or post-BPAB pulmonary artery pressure and index. Timing of BPAB and the body weight at the time of BPAB, however, differed significantly between the groups ( P = .02). Catheter data before BPAB were not significantly different between the groups, with the exception of the degree of subaortic stenosis (or hypoplasia of the left ventricular outflow tract) expressed as percentage of the normal end-systolic aortic valve annular diameter for patient body surface area. This metric (%SAS before BPAB) was significantly higher in group B (60%-68%) than in group U (47%-60%; P = .04). Among patients for whom baseline %SAS was < 60%, the %SAS did not increase after BPAB. CONCLUSION: The most important factor that allowed biventricular repair was not the pulmonary artery pressure or diameter but the degree of SAS. Patients who initially had more severe SAS ultimately underwent univentricular repair due to lack of substantial improvement in dimensions of the left ventricular outflow tract after BPAB.

Cardiac resychronization therapy (CRT) was performed via transvenous approach in a 2-year-old boy with a tetralogy of Fallot and postoperative severe heart failure, and complete atrioventricular block treated with a dual-chamber pacemaker. Epicardial leads were unavailable because of mediastinitis and the presence of severe bilateral pleural effusions requiring continuous drainage. There were no procedural complications. Biventricular pacing was significantly effective and both mediastinitis and pleural effusions recurred. The transvenous CRT was exchanged for an epicardial CRT after 4 months because of the possibility of a venous obstruction.

胎児心エコーにおけるカラーDoppler法で観察される大動脈峡部の逆行性血流が、大動脈縮窄症の指標となるか検討した。当院で胎児心エコー検査におけるカラーDoppler法により、3-vessel tracheal view(3VTV)で逆行性血流を有し、単純型大動脈縮窄症もしくは心室中隔欠損合併で大動脈縮窄症疑いであった22例を対象患児とし、後向きに評価した。その結果、出生直後の検査で大動脈縮窄症と診断された患児は9例(40.9%)に認められ、大動脈縮窄群では、逆行性血流が最初に検出された胎齢が非縮窄群よりも有意に早期であり、ROC曲線から、逆行性血流が最初に検出された胎齢が、出生後大動脈縮窄症と関連することが明らかにされた。また、出生後の大動脈縮窄推定に関するカットオフ値は胎齢35.5週で、感度77.8%、特異度69.2%、PPV 63.6%、NPV 81.8%、正確度72.7%であったことから、特に在胎35週未満の場合には、カラーDoppler法により、逆行性血流を評価することは大動脈縮窄症の出生前スクリーニングとして有用と判断された。

Introduction Several retrospective or single-centre studies demonstrated the efficacy of transplacental treatment of fetal tachyarrhythmias. Our retrospective nationwide survey showed that the fetal therapy will be successful at an overall rate of 90%. For fetuses with hydrops, the treatment success rate will be 80%. However, standard protocol has not been established. The objective of this study is to evaluate the efficacy and safety of the protocol-defined transplacental treatment of fetal tachyarrhythmias. Participant recruitment began in October 2010. Methods and analysis The current study is a multicentre, single-arm interventional study. A total of 50 fetuses will be enrolled from 15 Japanese institutions. The protocol-defined transplacental treatment is performed for singletons with sustained fetal tachyarrhythmia >= 180 bpm, with a diagnosis of supraventricular tachycardia or atrial flutter. Digoxin, sotalol, flecainide or a combination is used for transplacental treatment. The primary endpoint is disappearance of fetal tachyarrhythmias. The secondary endpoints are fetal death related to tachyarrhythmia, proportion of preterm birth, rate of caesarean section attributable to fetal arrhythmia, improvement in fetal hydrops, neonatal arrhythmia, neonatal central nervous system disorders and neonatal survival. Maternal, fetal and neonatal adverse events are evaluated at 1 month after birth. Growth and development are also evaluated at 18 and 36 months of corrected age. Ethics and dissemination The Institutional Review Board of the National Cerebral and Cardiovascular Center of Japan has approved this study. Our findings will be widely disseminated through conference presentations and peerreviewed publications.

歌舞伎症候群37例のうち心疾患を合併した18例(男10例、女8例)を、大動脈狭窄などの左室閉塞性疾患(LOL+)12例とそれ以外の心疾患(LOL-)6例に分けて、後方視的に検討した。初診時年齢はLOL-の1例を除き全例生後0日であり、LOL+のうち7例は出生前に心疾患が診断されていた。合併症は、全例に発達遅滞を認め、口腔外科疾患83.3%、腎疾患61.1%、消化器疾患55.5%、てんかん5.5%であった。LOL+の合併症はLOL-と比較して消化器疾患が多かった。心臓手術はLOL-の6例中1例、LOL+の12例中9例で行われた。死亡例は両群に1例ずつ認め、いずれも感染症が契機であった。

OBJECTIVE: To establish a simple risk stratification system for patients with congenital diaphragmatic hernia (CDH) based on postnatal information within 24 h after birth. STUDY DESIGN: A multi-institutional retrospective cohort study was conducted including 348 neonates who had isolated CDH born between 2006 and 2010. Based on the two most powerful variables for 90-day survival selected by multivariate analyses, a risk stratification system was established. RESULTS: Multiple logistic regression analysis identified two adverse prognostic factors: an Apgar score at 1 min (Ap1) of 0-4 (odds ratio (OR) 3.3, P = 0.004), and a best oxygenation index (OI) >= 8.0 (OR 11.4, P < 0.001). Based on a combinations of these two factors, patients were classified into three risk categories. The 90-day survival rates in categories 1-3 were 100, 88 and 52%, respectively (P < 0.001). CONCLUSION: Our simple risk stratification system based on Ap1 and best OI was capable of predicting mortality well.

Background: A number of case reports show various outcomes of premature closure of the ductus arteriosus in utero, including persistent pulmonary hypertension of the newborn and fetal or neonatal death; however, no study clarifies the clinical observations that are related to their prognoses. We aimed to clarify the prognostic factors of intrauterine ductal closure by a systematic literature review. Data sources We searched PubMed database (1975-2014) to identify case reports and studies on intrauterine closure of the ductus arteriosus, including maternal, fetal, and neonatal clinical information and their prognoses. Results: We analysed the data of 116 patients from 39 articles. Of these, 12 (10.3%) died after birth or in utero. Fetal or neonatal death was significantly correlated with fetal hydrops (odds ratio = 39.6, 95% confidence interval = 4.6-47.8) and complete closure of the ductus arteriosus (odds ratio = 5.5, 95% confidence interval = 1.2-15.1). Persistent pulmonary hypertension was observed in 33 cases (28.4%), and was also correlated with fetal hydrops (odds ratio = 4.2, 95% confidence interval = 1.3-4.6) and complete closure of the ductus arteriosus (odds ratio = 5.5, 95% confidence interval = 1.6-6.0). Interestingly, maternal drug administration was not correlated with the risk of death and persistent pulmonary hypertension. Conclusions: Fetal hydrops and complete ductal closure are significant risk factors for both death and persistent pulmonary hypertension. Cardiac or neurological prognoses could be favourable if the patients overcome right heart failure during the perinatal period.

OBJECTIVE: To seek a simple approach for prenatally classifying congenital diaphragmatic hernia (CDH) severity using fetal magnetic resonance imaging (MRI) markers. STUDY DESIGN: A retrospective, multicenter study using questionnaires to investigate fetal MRI findings. We included fetuses prenatally diagnosed with isolated left-sided CDH and delivered after 36 weeks of gestation. We focused on three fetal MRI morphological signs: incomplete pulmonary baseline (IPB), liver up (LU) and retrocardiac stomach (RCS). We also evaluated the fetal MRI score defined as the total number of positive signs; the primary outcome was survival at discharge. RESULTS: In 256 patients (from 56 institutions), IPB, LU and RCS findings correlated with lower survival: odds ratio (95% confidence interval), 0.16 (0.08 to 0.33); 0.24 (0.12 to 0.51); and 0.14 (0.07 to 0.28); respectively. Patients with higher fetal MRI scores had a higher mortality rate. CONCLUSION: IPB, LU and RCS on fetal MRI are related to CDH severity.

症例は在胎25週に胎児水腫と心拡大を指摘され、当院へ紹介された。胎児心エコー検査で重症大動脈弁狭窄症(critical aortic stenosis:cAS)と診断した。左室機能は極度に低下し、重度の僧帽弁逆流と卵円孔の狭小化を認めた。胎児死亡の可能性のある最重症例であり、小児循環器科、産科、新生児科、心臓血管外科、麻酔科、集中治療科が分娩の時期と方法、出生直後に予測される緊急対応について何度も協議を行った。33週3日に左室機能が更に低下し、帝王切開で分娩となった。体重2,650gで全身浮腫を認めた。出生後、直ちに人工呼吸管理が始まったが、卵円孔の狭小化が強く、酸素飽和度70%台と酸素化不良のため、生後2時間で心房中隔欠損作成術と両側肺動脈絞扼術を施行した。生後2日に経皮的バルーン大動脈弁拡大術を施行した。左室駆出率は34%から44%に改善した。その後、段階的に経皮的バルーン大動脈弁拡大術を施行し、生後51日に大動脈弓峡部の順行性血流を初めて認め、左室駆出率は45%に改善した。生後4ヵ月で2心室修復に到達できた。胎児水腫を伴う致死的疾患な病態のcritical ASに対してチーム医療を行い救命するだけでなく、左室の発育を待ち2心室修復に到達できた。関連各科が医療情報を共有することで迅速な対応が可能であった。(著者抄録)

Background/purpose: The purpose of this study was to investigate echocardiographic parameters in relation to the outcomes of isolated left-sided congenital diaphragmatic hernia (CDH). Methods: This multicenter, retrospective, observational study was conducted among patients with CDH born between 2006 and 2010. Patients in this study did not have severe cardiac malformations or chromosomal aberrations. Patients with incomplete echocardiographic examinations were excluded. In total, 84 patients with left-sided isolated CDH were included in this study. The prognostic parameters were obtained from postnatal echocardiographic images within 24 h after birth. Results: Eight patients died before 90 days of birth. Univariate analysis showed that the presence of continuous right to left shunt at the ductus, left pulmonary artery diameter of <2.7 mm, right pulmonary artery diameter of <3.3 mm, and left ventricular diastolic diameter of <10.8 mm, were the predictors of poor prognosis. Multivariate logistic regression analysis showed that right pulmonary artery diameter of <3.3 mm (adjusted OR 10.28, 95% C.I.: 1.15-249.19) and left ventricular diastolic diameter of <10.8 mm (adjusted OR 7.86, 95% C.I.: 1.01-82.82) were predictors of poor prognosis. Conclusions: This study revealed that the predictors of poor prognosis associated with CDH include smaller right pulmonary artery and left ventricular diastolic diameters. Retrospective Study-Level II. (C) 2016 Published by Elsevier Inc.

We experienced a case of severe congenital diaphragmatic hernia (CDH) with severe deleterious hypoxemia after birth in which we succeeded in saving the patient with radical surgery. Observation of the patient's detailed hemodynamics was possible for the entire perioperative period. The central venous pressure (CVP) was high according to the pressure in the liver. The CVP subsequently decreased after the operation, and the oxygen saturation increased. Therefore, the cardiac diastolic disorder improved. This case suggests that the diastolic dysfunction in severe CDH patients is worsen by compression from herniated visceral organs.

In this study, we present the case of an extremely low birth weight infant with severe coarctation of the aorta after ductus ligation. We treated the patient with balloon angioplasty using the descending aorta as a new access route. This method reduced many complications typically observed during the intervention for extremely low birth weight infants, which may expand the available treatment options for extremely low birth weight infants.

Left pulmonary artery sling (LPAS) is a rare vascular anomaly. The left pulmonary artery arises distally from the right pulmonary artery on the right side of the trachea and passes between the trachea and esophagus towards the left lung, compressing the lower trachea. LPAS is associated with congenital tracheal stenosis, which frequently requires early surgical intervention and has a poor prognosis due to severe airway obstruction after birth. Therefore, LPAS should be prenatally diagnosed to prepare for surgical intervention for tracheal stenosis. To the best of our knowledge, there are few reports on prenatal echocardiographic findings in LPAS. We report three prenatal cases of LPAS, which resulted in respiratory symptoms. We discuss fetal ultrasound findings and highlight the abnormal rotation of the fetal cardiac axis to the right as a useful sign in the prenatal screening of LPAS.

Purpose The optimal surgical approach for neonatal congenital diaphragmatic hernia (CDH) remains unclear. We conducted a systematic review and meta-analysis of the effectiveness of endoscopic surgery (ES) for neonatal CDH. Methods A systematic literature search was conducted using MEDLINE and the Cochrane Library. Studies that compared surgical approaches for neonatal CDH were selected. Mortality and recurrence of herniation were analyzed as primary endpoints. Each study was evaluated following the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system. Results Eight observational studies comparing ES and open surgery (OS) met the criteria. As compared with the OS group, the ES group showed both a significantly lower mortality rate [risk ratio (RR) 0.18, 95 % confidence interval (CI) 0.09-0.38, p < 0.0001] and a significantly higher recurrence rate (RR 3.10, 95 % CI 1.95-4.88, p < 0.00001). However, serious selection bias was seen in seven of the eight studies-because the indication of ES had been determined intentionally, the ES groups may have included less severe cases. Conclusion Although the evidence was insufficient, ES was clearly associated with more recurrence than was OS. Therefore, ES should not be the routine treatment for every neonate. It is crucially important to select suitable cases for ES.

Objective The measurement of diastolic wall strain (DWS), a new method of evaluating cardiac diastolic function, was employed to evaluate ventricular diastolic function in patients with congenital diaphragmatic hernia (CDH). Materials and methods Eighteen neonates with a CDH who were born and treated in our hospital between September 2009 and January 2013 were studied. The left ventricular posterior wall thickness during the systolic phase (PWs) and diastolic (PWd) phase was measured using M-mode imaging, and the DWS was calculated as (PWs-PWd)/PWs. The Tei index, the isovolumic relaxation time (IRT), and the fraction shortening (FS) were measured as indices of cardiac function in 14, 15, and 18 cases, respectively. Cardiac function was measured before and after surgery. Statistical analyses were performed using the paired t test. Results The pre- and postoperative DWS, Tei index, IRT and FS values were 0.19 +/- A 0.06 and 0.26 +/- A 0.11 (P < 0.01), 0.40 +/- A 0.12 and 0.31 +/- A 0.11 (P < 0.05), 48 +/- A 14 and 39 +/- A 5.0 ms (P < 0.05), 30 +/- A 7.7 and 34 +/- A 7.4 % (P < 0.05), respectively. Conclusion The diastolic and systolic functions were not only measured by the Tei index, IRT and FS values, but also by the DWS value, which improved after surgery. The measurement of DWS is an easy and useful method for evaluating the diastolic function of CDH patients.

We recently diagnosed two cases of isolated unilateral absence of the pulmonary artery just after birth. Through the therapy, we could not prevent obstruction of the pulmonary artery and lead to complications. There have been no symptoms attributed to isolated unilateral absence of the pulmonary artery so far. We should carefully consider the strategy for therapeutic intervention for asymptomatic cases.

BackgroundThe aim of this study was to clarify how extracorporeal membrane oxygenation (ECMO) is used to treat congenital diaphragmatic hernia (CDH) in Japan. MethodsWe completed a nationwide survey of CDH involving 614 infants. The subjects included 43 patients who underwent ECMO. We compared the clinical data of the patients who did and did not survive 90 days, and analyzed the 24h blood gas data in isolated CDH cases in both groups. ResultsOf the 43 CDH patients, non-isolated CDH associated with other life-threatening or chromosomal anomalies was diagnosed in six patients. Only one of these six patients was able to discontinue ECMO and survived, and the other five died shortly after birth. The other 37 patients all had isolated CDH. The reason for initiating ECMO in 31 of these patients was persistent pulmonary hypertension of the newborn (PPHN). In the 37 patients with isolated CDH, ECMO was initiated within 24h after birth. Sixteen patients (37%) survived 90 days, and intact discharge was possible in eight cases. Among the isolated CDH patients, on ROC analysis of the lowest oxygenation index (OI) to predict 90 day survival, the cut-off was 15. ConclusionsECMO is used to treat PPHN starting from an early period after birth, but the mortality and morbidity are not favorable. For lowest OI, the index used to predict survival following ECMO, the cut-off was 15.

BackgroundTetralogy of Fallot with absent pulmonary valve (TF/APV) is a rare and severe congenital heart disease with high mortality. The aim of this study was to assess whether TF/APV prognosis is related to fetal and postnatal clinical course and pulmonary artery (PA) configuration. MethodsThe fetal and postnatal echocardiograms and clinical outcomes of 13 patients with TF/APV (diagnosed antenatally in 9 patients and postnatally in 4) were reviewed, and divided into two groups: group A (n = 6), alive; and group D (n = 7), dead. ResultsFetal period: group A, polyhydramnios n = 0, hydrops fetalis (HF) n = 0, patent ductus arteriosus (PDA) n = 2; group D, polyhydramnios n = 3, HFn = 2, PDAn = 0. Postnatal period: group A, five patients underwent intracardiac repair, including one requiring artificial ventilation (AV). A further AV patient required three operations before extubation. Postoperative courses were all good. Group D, excluding the two intrauterine fetal deaths, four patients required AV (three of whom died neonatally or in early childhood) and one underwent intracardiac repair. PA configuration: all group A patients had bulbous expansion of left and right PA (clover type). Three patients in group D had bulbous expansion of main PA (balloon type). ConclusionsPDA was a factor associated with good prognosis. Hydramnion and HF were factors associated with poor prognosis. Given that there was a higher rate of postnatal AV and poorer prognosis in balloon type than in clover type PA (P < 0.05), PA configuration is also considered an important factor to predict postnatal outcome in TF/APV.

Background: Because there is limited information on fetal bradyarrhythmia associated with congenital heart defects (CHD), we investigated its prognosis and risk factors. Methods and Results: In our previous nationwide survey of fetal bradyarrhythmia from 2002 to 2008, 38 fetuses had associated CHD. Detailed clinical data were collected from secondary questionnaires on 29 fetuses from 18 institutions, and were analyzed. The 29 fetuses included 22 with isomerism, 4 with corrected transposition of the great arteries (TGA) and 3 with critical pulmonary stenosis; 14 had complete atrioventricular block (AVB), 8 had second-degree AVB, and 16 had sick sinus syndrome; 5 died before birth, and 10 died after birth (5 in the neonatal period). Neonatal and overall survival rates for fetal bradyarrhythmia with CHD were 66% and 48%, respectively. Pacemaker implantation was needed in 17 cases (89%). Beta-sympathomimetics were administered in utero in 13 cases and were effective in 6, but were not associated with prognosis. All cases of corrected TGA or ventricular rate >= 70 beats/min survived. A ventricular rate <55 beats/min had significant effects on fetal myocardial dysfunction (P=0.02) and fetal hydrops (P=0.04), resulting in high mortality. Conclusions: The prognosis of fetal bradyarrhythmia with CHD is still poor. The type of CHD, fetal myocardial dysfunction, and fetal hydrops were associated with a poor prognosis, depending on the ventricular rate.

Objectives To review the fetal echocardiograms of patients with total anomalous pulmonary venous connection (TAPVC) in order to determine whether the distance between the left atrium and the descending aorta would be useful in the prenatal diagnosis of fetal TAPVC. Methods We reviewed the fetal echocardiograms of eight cases of TAPVC (five supracardiac type and three infracardiac type) with no other cardiac malformations. We evaluated the ratio of the left atrium-descending aorta distance to the diameter of the descending aorta ('post-LA space index') in 101 normal and eight TAPVC fetuses, and compared the values between groups. In addition, we examined the tricuspid valve/mitral valve diameter ratio (TVD/MVD) and the right ventricular end-diastolic diameter/left ventricular end-diastolic diameter ratio (RVDd/LVDd). Results The echocardiograms for fetuses with TAPVC and normal fetuses were performed at mean gestational ages of 27.5weeks and 29.6 weeks, respectively. There were no significant differences in the TVD/MVD and RVDd/LVDd ratios between the groups. However, the post-LA space index was significantly higher in the TAPVC cases (mean, 1.51) than it was in the normal fetuses (mean, 0.71 +/- 0.23) (P < 0.0001). On an analysis of the receiver-operating characteristics curve, a post-LA space index cut-off of 1.27 was found to be optimal for distinguishing between TAPVC and normal hearts, with a sensitivity of 100% and specificity of 99%. Conclusions The novel post-LA space index could potentially be used for the prenatal diagnosis of TAPVC. A diagnosis of TAPVC is very likely in cases with a post-LA space index of >1.27. Copyright (c) 2014 ISUOG. Published by John Wiley & Sons Ltd.

Bilateral pulmonary artery banding is considered as 'first-stage' palliation for neonates who have hypoplastic left heart syndrome. This study aimed to identify risk factors that influence outcome before the bidirectional Glenn operation. This retrospective evaluation involved 30 consecutive patients with hypoplastic left heart syndrome, or a variant, who underwent bilateral pulmonary artery banding between August 2005 and December 2011 at our institution. Clinical echocardiographic, operative and catheter examination data were reviewed. This study included 9 patients with hypoplastic left heart syndrome and 21 patients with variants. Bilateral pulmonary artery banding was performed at a median age of 7 days. Finally, 19 patients had the bidirectional Glenn operation performed (Group A), and the remaining 11 patients died before the bidirectional Glenn procedure (Group NA). Catheter evaluations before the bidirectional Glenn procedure were carried out at 97 +/- 34 days. The mean pulmonary venous wedge pressure was significantly lower (Group A: 13.1 +/- 3.1 mmHg vs Group NA: 22.9 +/- 3.7 mmHg, P < 0.01), systemic ventricular ejection fraction was higher (54.4 +/- 10.7 vs 41.7 +/- 9.9%, P < 0.05), systemic ventricular end-diastolic pressure was lower (6.1 +/- 2.4 vs 10.5 +/- 3.6 mmHg, P < 0.05) and the rate of patients with more than mild systemic atrioventricular valve regurgitation was lower in Group A than in Group NA (15.7 vs 62.5%, P < 0.05). Multivariate logistic regression analysis showed that mean pulmonary venous wedge pressure was the most significant predictor of attaining the bidirectional Glenn anastomosis (odds ratio: 2.35, P < 0.01). Postoperative atrioventricular valve regurgitation, cardiac function and mean pulmonary venous wedge pressure are closely correlated with mortality after bilateral pulmonary artery banding. Additional treatments, including operations, are considered to maintain cardiac function and not to raise pulmonary venous wedge pressure before the bidirectional Glenn procedure.

The purpose of this study is to evaluate the outcome of our therapeutic strategy for antenatally diagnosed congenital diaphragmatic hernia (ADCDH). We treated 61 cases of ADCDH according to our strategy. Prostaglandin E1 was required to be maintained the patency of the ductus arteriosus (PDA) in 39 cases (Group I) while it was not administered in 22 cases (Group II). Left ventricular end-diastolic dimension (LVDD) and Tei index were measured with echocardiography on days 0, 2, and 7 after birth. Radical surgery was performed on all cases by day 2. On day 0, Group I showed smaller LVDD and Tei index than those in Group II. Between day 0 and day 2, these parameters increased significantly in Group I, but not in Group II. On day 7, no significant difference in these parameters was observed between the two groups. Five patients died of cardiac and respiratory failure, resulting in a survival rate of 92 %. Our therapeutic strategy improves the clinical outcome of ADCDH. This can be attributed to two factors: earlier surgery resulting in improved LV function. The latter attenuates pulmonary hypertension and maintains PDA with a consequent decrease in right ventricular afterload to compensate for the low cardiac output resulting from PDA.

BackgroundThe aim of this study was to clarify the outcome of patients with cardiovascular malformation (CVM) among those with congenital diaphragmatic hernia (CDH) who are indicated for the Fontan procedure. MethodsThe subjects included 76 CDH patients with CVM recruited from a national survey of 614 CDH patients. The outcomes were evaluated between two groups divided according to indication for the Fontan procedure. Patients with functional univentricular disease were considered to be candidates for the Fontan procedure. ResultsSixteen (21.1%) of the 76 patients were candidates for the Fontan procedure, accounting for 2.6% of all 614 patients with CDH. None of these patients, however, underwent the Fontan procedure. Among the 16 patients, the absence of obstruction of the left ventricular outflow tract (LVOTO) was significantly associated with better 90day survival (71.4%, 5/7, for those without LVOTO vs 0.0%, 0/9, for those with LVOTO, P = 0.0007). After excluding 22 patients with chromosomal and/or genetic abnormalities or syndromes, the 90day survival rate was significantly better in neonates without than with indication for the Fontan procedure (62.5%, 25/40 vs 28.6%, 4/14, P = 0.0271). ConclusionsPatients with indications for the Fontan procedure are rare, and the outcome of patients with LVOTO among those with CDH is especially poor.

Background/Purpose: The purpose of this study was to clarify the relationship between the lung to thorax transverse area ratio (L/T ratio) and the observed to expected lung area to head circumference ratio (O/E LHR), based on the results of a nationwide Japanese survey conducted in 2011, and to evaluate the compatibility of these prognostic predictors of fetal CDH. Methods: Two hundred and forty-two prenatally diagnosed isolated CDH patients born between 2006 and 2010 were included in the present analysis. A regression analysis was conducted to investigate the relationship between the L/T ratio and the O/E LHR based on 191 simultaneous measurements of these parameters in 120 patients. Results: The linear regression equation between the L/T ratio and the O/E LHR was: L/T ratio = 0.0233 + (0.00222 x O/E LHR), (R = 0.847, p < 0.0001). According to this equation, 25% of the O/E LHR, the cut-off value used in the fetal intervention for CDH, was equivalent to an L/T ratio of 0.08, a commonly accepted cut-off value for identifying the most severe cases of fetal CDH. Conclusions: As there is a positive correlation between the L/T ratio and the O/E LHR, these two parameters proved to be used interchangeably according to the linear regression equation. (C) 2014 Elsevier Inc. All rights reserved.

IntroductionPneumothorax remains a life-threatening complication that occurs in congenital diaphragmatic hernia (CDH), even under respiratory management with gentle ventilation. The aim of this study was to evaluate the prevalence of pneumothoraces as a fatal complication during the management of CDH based on the results of a nationwide Japanese survey conducted in the era of gentle ventilation. Materials and MethodsA retrospective cohort study was performed as part of a nationwide Japanese survey of CDH. A total of 510 neonates with isolated CDH born between 2006 and 2010 were included in this study. The patients were divided into four groups according to operative findings related to the diaphragmatic defect size and operability, which represents the disease severity: defects less than 25%, defects more than 25% but less than 75%, defects more than 75%, and a patient group that was unable to undergo surgery. The prevalence of pneumothorax and the survival rate were compared with respect to each disease severity group. Each case was evaluated to determine whether the development of a pneumothorax was the primary cause of death. ResultsOf the 510 neonates with isolated CDH, 69 patients developed a pneumothorax before and/or after surgical intervention. Of the 69 patients 38 patients died, and only 26 patients were discharged from the hospital without any major morbidity that requires home treatment (intact discharge). The prevalence of pneumothorax increased and the survival rate and intact discharge rate decreased as the severity of the disease worsened. The number of patients whose pneumothorax was presumed to be the primary cause of death also increased as the severity of the disease worsened. The survival rate of the patients with pneumothorax was significantly lower than that of the patients without pneumothorax among the groups with 25 to 75% defects and 75% or more defects. ConclusionsPneumothoraces was found to more likely occur in neonates with CDH associated with a large defect of the diaphragm. The survival rate and intact discharge rate decreased as the severity of the disease worsened, especially among the patients who developed pneumothorax accompanied by large diaphragmatic defects. No other risk factors related to pneumothorax occurrence were found, except for the severity of the disease itself, thus suggesting that pneumothorax was associated with a lethal outcome in neonates with CDH associated with a large defect of the diaphragm.

We report a case of severe congenital diaphragmatic hernia accompanied by hydrops fetalis in a fetus conceived by a 33-year-old woman. Fetal magnetic resonance imaging performed at 27 weeks of gestation revealed herniation of the stomach, bowel, and left liver into the thoracic cavity, confirming severe left-sided CDH. Presence of hydrops fetalis at 32 weeks of gestation and its progression over the next 2 weeks was confirmed by fetal echography. Subsequently, fetal echocardiography revealed severe tricuspid regurgitation and reverse flow in the inferior vena cava at 33 weeks, with abnormal left and right ventricular Tei indices, extended left ventricular isovolumetric relaxation time, and increased preload index. Maternal hyperoxygenation (MHO) therapy was initiated at 35 weeks of gestation to increase pulmonary blood flow and promote venous return to the left ventricle. The hydrops was attenuated by MHO for 2 weeks, and fetal echocardiography demonstrated improved cardiac performance. A female infant was born at 37 weeks of gestation and underwent diaphragmatic repair shortly after birth. She survived surgery and was discharged at 4 months of age with no adverse sequelae. This case report suggests that MHO may be a potential therapy for severe congenital diaphragmatic hernia associated with hydrops fetalis. Crown Copyright © 2014 Published by Elsevier Inc.

症例は5歳女児。先天性心疾患に対し3歳時にFontan手術が行われ、術後経過は良好であった。散歩中に血液の大量嘔吐を認め、救急搬送。気道出血と診断されたが出血を繰り返すため、ヘリコプターで当センターへ緊急搬送となった。側副血管に対しコイル塞栓術を何度も行ったが大量出血が継続したため、救命的にシリコン製のEndobronchial Watanabe Spigot(EWS)による気管支充填術を施行した。難治性の気道出血であったが、側副血管へのコイル塞栓術およびEWSによる気管支充填術の併用により救命することができた。本症例は小児で初めてのEWSによる気管支充填術施行例である。術後急性期にEWSの脱落および再充填などを要したが以後は明らかな合併症なく経過しており、EWSは難治性の気道出血においてコイル塞栓術との併用も考慮すべき治療法であると考えた。今後はEWSの長期留置に伴う合併症に注意しながら経過観察の予定である。(著者抄録)

Background: Congenital diaphragmatic hernia is associated with cardiovascular malformation. Many prognostic factors have been identified for isolated congenital diaphragmatic hernia; however, reports of concurrent congenital diaphragmatic hernia and cardiovascular malformation in infants are limited. This study evaluated congenital diaphragmatic hernia associated with cardiovascular malformation in infants. Factors associated with prognosis for patients were also identified. Methods: This retrospective cohort study was based on a Japanese survey of congenital diaphragmatic hernia patients between 2006 and 2010. Frequency and outcome of cardiovascular malformation among infants with congenital diaphragmatic hernia were examined. Severity of congenital diaphragmatic hernia and cardiovascular malformation were compared as predictors of mortality and morbidity. Results: Cardiovascular malformation was identified in 76 (12.3%) of 614 infants with congenital diaphragmatic hernia. Mild cardiovascular malformation was detected in 19 (33.9%) and severe cardiovascular malformation in 37 (66.1%). Their overall survival rate at discharge was 46.4%, and the survival rate without morbidity was 23.2%. Mortality and morbidity at discharge were more strongly associated with severity of cardiovascular malformation (adjusted OR 7.69, 95% CI 1.96-30.27; adjusted OR 7.93, 95% CI 1.76-35.79, respectively) than with severity of congenital diaphragmatic hernia. Conclusions: The prognosis for infants with both congenital diaphragmatic hernia and cardiovascular malformation remains poor. Severity of cardiovascular malformation is a more important predictive factor for mortality and morbidity than severity of congenital diaphragmatic hernia.

Complete congenital heart block (CCHB) accompanied with neonatal lupus erythematosus is caused by an immune reaction between maternal anti-Ro/SSA antibodies and the fetal heart and is generally considered an irreversible process. This reaction mostly occurs before 30 weeks' gestation, especially between the 20th and 24th week. Reported here is an atypical case of neonatal lupus erythematosus with CCHB detected after 32 weeks' gestation that showed postnatal improvement in the degree of heart block after preterm delivery and immunoglobulin use. The clinical condition of the infant worsened with an increase in heart rate due to possible cardiomyopathy induced by the immune reaction. © 2013 The Authors.

主要施設における新生児横隔膜ヘルニアの治療成績と治療方針の施設間差異について検討した。対象は10例以上の症例を治療した経験のある22施設で、症例調査票を用いて各症例に対する診断、治療、転帰に関する調査を実施した。その結果、出生前診断率、横隔膜ヘルニア単独(Isolated)症例の割合とも施設間により大きな差があり、重症度も施設によって重症例の占める割合に偏りがあった。出生前診断例に対する治療方針についても施設間で異なり、全例帝王切開分娩を施行した施設は9施設あった一方、帝王切開症例の割合が50%未満の施設も4施設あった。胎児麻酔については9施設で全く行っていなかったが、1施設で全例に施行していた。呼吸・循環管理については、全施設で原則としてgentle ventilationの考え方に基づき呼吸管理を行っており、心臓超音波検査を治療方針決定の参考にしていた。一方、人工呼吸器管理上容認できるpre PaCO2の最高値と最低値には開きがあり、NO吸入療法の使用頻度やECMOについても施設間に差異があった。また一定期間待機してから手術を行う施設は11施設、一定のstabilizationの基準を満たせば手術をする施設は10施設、なるべく早期に手術をするのは1施設であったが、実際に待機した時間の中央値には明らかな差はなかった。Isolated症例において比較的早期に手術をしている施設では生存率がやや高い傾向であった。合併症については、気胸、胃食道逆流症などの発症率に施設間差異を認め、Isolated症例の生存率が低かった施設で気胸の発生率が高い傾向であった。

Background During the last decade, new supportive modalities and new therapeutic strategies to treat congenital diaphragmatic hernia (CDH) have been introduced. In Japan, the large number of hospitals prevents centralizing infants with CDH in tertiary centers. The aim of this study was to evaluate the correlations between the number of CDH patients, survival rates, and the current strategies employed to treat CDH at the individual hospitals. Methods Eighty-three hospitals with 674 CDH cases were analyzed using questionnaires. We classified the hospitals into three groups according to the number of CDH patients treated: Group 1 (G1; more than 21 patients), Group 2 (G2; 1120 patients), and Group 3 (G3; fewer than 10 patients). Results The median number of CDH patients in G1, G2, and G3 were 28, 14, and 4, respectively. The overall survival rate was 74.5%. When only the isolated CDH cases with a prenatal diagnosis were included, the overall survival rate was 79.3%. The survival rate of isolated CDH cases with a prenatal diagnosis was significantly higher in G1 than that in G2 or G3 (87.2% vs 75.2% vs 74.3%; P < 0.001). There were no differences in perinatal therapeutic strategies among the three groups. Conclusions We concluded that it might therefore be important to centralize infants with CDH, especially those with isolated CDH with a prenatal diagnosis, to tertiary centers in Japan in order to improve the survival rates.

Background/Purpose: Few nationwide surveys have been reported regarding the perinatal status, clinical course and postnatal outcome of cases with congenital diaphragmatic hernia (CDH). The aim of this study was to review the current profile and the outcomes of a large cohort of CDH cases in Japan. Methods: A nationwide retrospective cohort study was conducted on neonates diagnosed to have CDH between January 2006 and December 2010. The questionnaires were sent to 159 representative regional institutions and 109 (68.6%) institutions responded to the preliminary survey which had 674 cases. Eleven institutions which had 60 CDH neonates did not respond to the second questionnaire, and 26 institutions had no cases. Finally, 614 CDH neonates from 72 institutions had been collected and were used in the detailed survey. The perinatal status, clinical course and the postnatal outcome were reviewed. Survival was defined as infants alive at hospital discharge, at the time of transfer or still in the hospital at the time of questionnaire, which was confirmed during the period from July 2011 to November 2011 by the investigators. Results: Four hundred sixty-three (75.4%) of 614 CDH neonates survived in this study. The overall survival rate of neonates with isolated CDH was 84.0%. A total of 444 (72.0%) patients were prenatally diagnosed, and had a survival rate of 70.8%. Four hundred thirty-three (70.9%) patients were treated with high-frequency oscillatory ventilation (HFOV) as the initial ventilation, 344 (56.0%) patients received inhaled nitric oxide (iNO) and 43 (7.0%) required extracorporeal membrane oxygenation (ECMO). The overall survival rates of the CDH neonates who had been treated using HFOV, iNO and ECMO were 74.3%, 68.3% and 37.2%, respectively. Conclusions: This study demonstrated that the current status for CDH treatment in Japan and the overall survival rate were comparable to those of recent reports from other countries. (c) 2013 Elsevier Inc. All rights reserved.

We report an infantile case of Loeys-Dietz syndrome prenatally diagnosed with congenital complex heart disease - double outlet right ventricle and interruption of the aortic arch. The patient also showed prominent dilatation of the main pulmonary artery. Emergency bilateral pulmonary artery banding was performed on the 9th day. However, on the 21st day, the patient died of massive bleeding due to rupture of the right pulmonary artery. Subsequently, a mutation of the TGEBR1 gene was detected. As cardiovascular lesions of Loeys-Dietz syndrome appear early and progress rapidly, the prognosis is generally poor. Patients require periodic examination and early intervention with medical therapy such as Losartan administration and surgical therapy. Early genetic screening is thought to be useful for the prediction of complications as well as vascular disease.

Background: There are few large studies of fetal congenital bradyarrhythmia. The aim of the present study was to investigate the effects and risks of transplacental treatment for this condition. Methods and Results: Using questionnaires, 128 cases of fetal bradyarrhythmia were identified at 52 Japanese institutions from 2002 to 2008. Of the 128 fetuses, 90 had structurally normal hearts. Among these 90 fetuses, 61 had complete atrioventricular block (CAVB), 16 had second-degree AVB, 8 had sinus bradycardia, and 5 had other conditions. The 61 CAVB fetuses were divided into those who did (n=38) and those who did not (n=23) receive transplacental medication. Monotherapy with beta-sympathomimetics, steroid monotherapy, and combination therapy with these agents was given in 11, 5 and 22 cases, respectively. Beta-sympathomimetics improved bradycardia (P<0.001), but no medication could significantly improve the survival rate. Fetal hydrops was associated with a 14-fold increased risk of perinatal death (P=0.001), and myocardial dysfunction was a significant risk factor for poor prognosis (P=0.034). Many adverse effects were observed with steroid treatment, with fetal growth restriction increasing significantly after >10 weeks on steroids (P=0.043). Conclusions: Treatment with beta-sympathomimetics improved bradycardia, but survival rate did not differ significantly in fetuses with and without transplacental medication. It is recommended that steroid use should be limited to <10 weeks to avoid maternal and fetal adverse effects, especially fetal growth restriction and oligohydramnios. (Circ J 2012; 76: 469-476)

背景:扁桃肥大は小児の上気道狭窄の最多原因であり, 上気道狭窄による低酸素血症, 高二酸化炭素血症は, 肺動脈圧の上昇, 慢性肺性心を惹起させる可能性からFontan循環に悪影響を及ぼす可能性がある.
対象:1993年から2009年までの, いわゆるFontan trackにのった全207例中, 扁桃腺摘出術(以下, 扁摘)を行った9例.
方法:扁桃肥大を合併し上気道狭窄が疑われるFontan candidateおよびFontan術後患者に対する扁摘の臨床的効果を, 扁摘前後の, 経皮的動脈血酸素飽和度(SpO₂), 扁摘前後に心臓カテーテル検査を行った4例での, 動脈血二酸化炭素分圧(PaCO₂), 平均肺動脈圧(mPAP)を用いて検討した.
結果:9例中8例はFontan手術に到達し, 1例は両方向性Glenn術後Fontan待機中である. 扁摘前後のSpO₂は, 術前81±7％から術後85±6％に, 全例で上昇した. 扁摘の前後でPaCO₂は, 術前41±4 mmHgから術後37±4 mmHgに, 1例を除いて低下し, mPAPは, 術前22±12 mmHgから術後13±4 mmHgに, 全例で低下した.
結論:扁桃肥大を合併し上気道狭窄が疑われるFontan track症例に対する扁摘は, 肺血管抵抗を低下させた可能性があり, Glenn・Fontan循環を改善させることが示唆された.

症例は5歳2ヵ月女児、純型肺動脈閉鎖と胎児診断を受け、段階手術、最終的には3歳時にFontan手術を経てチアノーゼが消失した。無治療の静脈管開存症を合併していたが肝機能に異常はなく、良好な外来経過であった。5歳2ヵ月時、散歩中に突然大量の血液を吐き、救命センターに搬送され、直ちに気管挿管が行われ、気道出血と診断された。到着時、顔面蒼白、頻脈、血圧低下の出血性ショック状態であった。造影CTで右下葉S6と上葉S2を中心とした肺出血が認められた。転院後も出血が続くため、直ちに下行大動脈から右上葉に向かう側副血管にコイル塞栓術を行った。しかし、気道出血が再発し、気管支ファイバースコピーで左肺からの出血を確認したため、左内胸動脈と左鎖骨下動脈から派生する側副血管にコイル塞栓術を行った。気道出血は止まったが、4日後に再度出血を認め、3回の塞栓術でコイル塞栓が可能な側副血管は全て塞栓できたと考えられた。しかし、その後も大量の気道出血を繰り返し、循環動態も悪化するため、気管支側からの止血処置として気管支充填術を選択した。その後も気道出血が継続し、左側からの出血が疑われ、4回目のコイル塞栓術を施行したが著効せず、左下甲状腺動脈より派生する側副血管に対する5回目のコイル塞栓術を施行した。その後は出血を認めず、人工呼吸器から離脱できた。急性期の影響で低酸素性虚血性脳症を呈したが全身状態は良好で、約4ヵ月後に退院した。1年経過したが現在まで気道出血の再発はみられていない。

Background/Purpose: The aim of this study was to establish a prenatal prognostic classification system for risk-stratified management in fetuses with isolated congenital diaphragmatic hernia (CDH). Methods: Amulti-institutional retrospective cohort study of isolated CDH, diagnosed prenatally in fetuses delivered during the 2002 to 2007 period at 5 participating institutions in Japan, was conducted. The risk stratification system was formulated based on the odds ratios of prenatal parameters for mortality at 90 days. The clinical severity in CDH infants were compared among the stratified risk groups. Results: Patients were classified into the 3 risk groups: group A (n = 48) consisted of infants showing liver-down with contralateral lung-to-thorax transverse area ratio (L/T) ratio >= 0.08; group B of infants showing liver-down with L/T ratio <0.08 or liver-up with L/T ratio >= 0.08 (n = 35), and group C of infants showing liver-up with L/T ratio <0.08 (n = 20). The mortality at 90 days in groups A, B, and C were 0.0%, 20.0%, and 65.0%, respectively. The intact discharge rates were 95.8%, 60.0%, and 5.0%, respectively. This system also accurately reflected the clinical severity in CDH infants. Conclusions: Our prenatal risk stratification system, which demonstrated a significant difference in postnatal status and final outcome, would allow for accurate estimation of the severity of disease in fetuses with isolated CDH, although it needs prospective validation in a different population. (C) 2011 Elsevier Inc. All rights reserved.

Background: There are few reports describing the features of maternal anti-SSA antibody-associated congenital complete heart block (CCHB) patients developing endocardial fibroelastosis (EFE). The aim of this study was to describe the clinical features and the outcome of patients with CCHB, with or without EFE. Methods and Results: Over a 20-year period, 12 consecutive patients diagnosed with maternal anti-SSA antibody-associated CCHB were identified. The maternal anti-SSA antibody levels were measured and fetal echocardiographic findings were reviewed. The ratios of the thickness of the endocardium to that of the whole wall of the left ventricle (LE/W) and right ventricle (RE/W) were measured to investigate the degree of endocardial thickening. A total of 7 patients survived (living group) and were not diagnosed as having EFE. The remaining 5 patients died and were diagnosed with EFE during autopsy (dead group). Fetal echocardiography of the patients showed differences in the thickening and hyperintensity of the endocardium. The RE/W value was significantly higher in the dead group than in the living group. The titers of both maternal anti-52-kDa and anti-60-kDa SSA antibodies were high, but showed no significant differences between the 2 patient groups. Conclusions: EFE was the major negative prognostic factor for CCHB. Myocardial damage, predominantly in the right ventricle, was related to the outcome of CCHB associated with EFE. (Circ J 2011; 75: 1215-1221)

To review the recent Japanese experience with prenatally diagnosed congenital diaphragmatic hernia (CDH) based on a multi-institutional survey. A multicenter, retrospective cohort study was conducted on 117 patients born between 2002 and 2007 with isolated prenatally diagnosed CDH. All patients were managed by maternal transport, planned delivery, immediate resuscitation and gentle ventilation. The primary outcome measurements were the 90-day survival and intact discharge. The examined prenatal factors included gestational age (GA) at diagnosis, lung-to-head ratio (LHR), lung-to-thorax transverse area ratio (L/T) and liver position. Physical growth and motor/speech development were evaluated at 1.5 and 3 years of age. Data were expressed as the median (range). The mean GA at diagnosis was 29 (17-40) weeks. The LHR and L/T were 1.56 (0.37-4.23) and 0.11 (0.04-0.25), respectively. There were 48 patients with liver up. The mean GA at birth was 38 (28-42) weeks. The 90-day survival rate and intact discharge rate were 79 and 63%, respectively. Twelve patients had major morbidity at discharge, and 71% of these patients had physical growth or developmental retardation at 3 years of age. This multicenter study demonstrated that the 90-day survival rate of isolated prenatally diagnosed CDH was 79%, and that subsequent morbidity remained high. A new treatment strategy is needed to reduce the mortality and morbidity of severe CDH.

OBJECTIVES:
To document outcome and to explore prognostic factors in fetal left congenital diaphragmatic hernia (CDH).

METHODS:
This was a multicenter retrospective study of 109 patients with prenatally diagnosed isolated left CDH born between 2002 and 2007. The primary outcome was intact discharge, defined as discharge from hospital without major morbidities, such as a need for respiratory support including oxygen supplementation, tube feeding, parenteral nutrition or vasodilators. All patients were managed at perinatal centers with immediate resuscitation, gentle ventilation (mostly with high-frequency oscillatory ventilation) and surgery after stabilization. Prenatal data collected included liver and stomach position, lung-to-head ratio, gestational age at diagnosis and presence or absence of polyhydramnios. Stomach position was classified into four grades: Grade 0, abdominal; Grade 1, left thoracic; Grade 2, less than half of the stomach herniated into the right chest; and Grade 3, more than half of the stomach herniated into the right chest.

RESULTS:
Overall intact discharge and 90-day survival rates were 65.1% and 79.8%, respectively. Stomach herniation was classified as Grade 0 in 19.3% of cases, Grade 1 in 45.9%, Grade 2 in 13.8% and Grade 3 in 21.1%. Multivariate analysis revealed that liver position was the strongest prognostic variable for intact discharge, followed by stomach position. Based on our results, we divided patients into three groups according to liver (up vs. down) and stomach (Grade 0-2 vs. Grade 3) position. Intact discharge rates declined significantly from liver-down (Group I), to liver-up with stomach Grade 0-2 (Group II), to liver-up with stomach Grade 3 (Group III) (87.0%, 47.4% and 9.5% of cases, respectively).

CONCLUSION:
Current status and outcomes of prenatally diagnosed left CDH in Japan were surveyed. Stomach herniation into the right chest was not uncommon and its grade correlated with outcome. The combination of liver and stomach positions was useful to stratify patients into three groups (Group I-III) with different prognoses.

Purpose An accurate prenatal assessment of the patients' severity is essential for the optimal treatment of individuals with congenital diaphragmatic hernia (CDH). The purpose of this study was to clarify the reliability of the lung to thorax transverse area ratio (L/T) as a prenatal predictive parameter. Methods A multicenter retrospective cohort study was conducted on 114 isolated CDH fetuses with a prenatal diagnosis during the period between 2002 and 2007 at five participating centers in Japan. The relationship between the gestational age and the L/T was analyzed. The most powerful measurement point and accurate cutoff value of the L/T was determined by an analysis of a receiver operating characteristic curve, which was verified by comparing the patients' severity. Results There was a negative correlation between the gestational age and the L/T in the non-survivors, and no correlation in the survivors. There were significant differences in the parameters which represented the patients' severity including the respiratory and circulatory status, the surgical findings, and the final outcomes between the groups divided at 0.080 in the minimum value of the L/T during gestation. Conclusion The L/T was not strongly influenced by the gestational age, and it was found to be a reliable prenatal predictive parameter in fetuses with isolated CDH.

Objective. Prenatal constriction of the ductus arteriosus associated with maternal drug ingestion was reported several decades ago. There are fewer reports of the complete closure of the ductus arteriosus; therefore, the clinical features of the latter are poorly understood. The aim of this study is to clarify the clinical features of complete ductal closure and postnatal pulmonary hypertension by performing echocardiography of the fetus. Patients. We diagnosed four fetuses with complete ductal closure by performing fetal echocardiography and reviewed the prenatal and postnatal medical records of the mother and fetus. Results. One mother each had bronchial asthma, ulcerative colitis, and idiopathic thrombocytopenic purpura, and they had received nonsteroidal anti-inflammatory drugs and/or corticosteroids during pregnancy. The fourth mother did not have basal disease and had not ingested any drugs. Fetal diagnosis was performed at 32-38 weeks of gestation. All fetuses had right heart dilatation with tricuspid regurgitation in the absence of any cardiac defects, and Doppler echocardiography indicated that the right ventricular pressure was elevated. Two of the fetuses had fetal hydrops, which suggested severe right heart dysfunction. All fetuses were delivered by emergent cesarean delivery. After birth, all the infants developed persistent pulmonary hypertension and required oxygen inhalation. Of these, three required mechanical ventilation, and two, nitric oxide inhalation. All infants improved within 2 weeks, and they had no neurological and cardiac complications after discharge. Conclusion. Right heart dilatation and severe tricuspid regurgitation in the absence of a cardiac defect in the fetus strongly suggested ductal dysfunction. Careful evaluation of ductal patency and right ventricular function can lead to precise early diagnosis and good prognosis.

2002年1月〜2007年12月に出生した、胎児横隔膜ヘルニア(CDH)と出生前に診断された、単胎である、当該疾患以外の重篤な胎児奇形(染色体異常、致命的な心疾患)がない、出生直後からgentle ventilationによる呼吸管理を含む集中治療が行われた各調査実施5施設で出生した新生児を対象に、後方視的に検討し、治療成績を明らかにして、出生前評価から予後不良群を予測する可否を検討した。調査の結果、117例の登録が得られた。病変は左109例、右6例、両側2例で、左CDHのみを対象にした。全体の治療成績は90日生存87例(79.8%)、退院81例(74.3%)、合併症なき退院71例(65.1%)であった。胃が左胸腔から右胸腔にまで脱出している症例が34.9%もあったことは予想外で、胃泡の右胸腔内脱出が左横隔膜ヘルニアではまれでないことが判明した。約半数が帝王切開で娩出され、殆どの症例で高頻度人工換気(HFO)が使用されていた。NO吸入療法も80%の症例で使用されていた。ECMOは16例(15%)の症例で施行されていた。横隔膜の修復には約半数の症例で人口布が使用されていた。肝脱出の有無、胃の位置、LHRが有意なリスク因子であった。肝脱出のない群の90日生存率と合併症なき退院率はそれぞれ95.7%、87.0%と良好であった。肝脱出群の予後を左右する因子としては胃の位置の方がLHRより強力であることが示唆された。

2001年1月〜2007年12月に研究班参加5施設で在胎36週以降に出生した先天性横隔膜ヘルニア(CDH)110例について後方視的に検討した。また、出生直後からgentle ventilationによる呼吸管理を含む集中治療が行われた症例を分娩方針により2群に分類し、予定帝王切開(帝切)群46例と予定経腟分娩群64例に分け両群の背景、胎児超音波所見および出生前治療内容について検討した。在胎週数は予定帝切群で僅かに早い傾向を認めた。出生体重、男女比については有意差を認めなかった。胎児超音波所見では重症度の指標とされるL/T比は有意差を認めなかったが、LHR(肝臓が胸腔内に脱出する所見(liver up所見))を伴うものの頻度、胃胞が胸腔内で半分以上患側に偏位するものの頻度は予定帝切群で多かった。両群の新生児予後については、主評価項目である生後90日の生存、合併症なき退院については両群間で有意差を認めなかったが、予定帝切群でより新生児予後が不良の傾向を認めた。それ以外の副評価項目では両群間に有意差を認めなかった。予定帝切群では新生児予後がより不良との結果と同時に胎児超音波所見で重症度が高いと予想される症例が多かった。

2002年〜2007年の6年間に国立成育医療研究センター、神奈川県立こども医療センター、大阪大学医学部附属病院、大阪府立母子保健総合医療センター、九州大学病院の5施設で出生し、出生前診断された先天性横隔膜ヘルニア(CDH)117例について後方視的に検討した。最初に出生前診断された在胎週数は29(17〜40)週で、24例に羊水過多を認めた。出生週数は38週(28〜42)週、出生体重は2.78(1.04〜4.04)kgで分娩方法は経腟55例、帝王切開62例であった。高頻度人工換気(HFO)は116例(99%)、一酸化窒素(NO)は94例(80%)に、膜型人工肺体外酸循環(ECMO)は19例(16%)に使用され90日生存7例で合併症なしで退院は2例であった。平均気道内圧(MAP)最高値は14(12〜15)cmH2Oで、病変部位は左109、右6、両側2であった。全症例の生存曲線は生後90日でほぼプラトーに達し、90日生存は92例(78.6%)、そのうち4例は90日以降に入院死、2例はそれぞれ18ヵ月、24ヵ月で生存入院中である。これら6例を除く86例(73.5%)が生存退院し、うち12例は退院時に重篤な合併症を伴っていた。退院時に合併症を認めた12例中、1.5歳および3歳時に身体発育遅延または精神・運動発達遅延を認めた割合はそれぞれ80%、71%であった。一方合併症なし退院例のそれぞれの割合は44%、27%と合併症あり退院例に比べ有意に低かった。

Background-Data on the clinical presentation and genotype-phenotype correlation of patients with congenital long-QT syndrome (LQTS) diagnosed at perinatal through infantile period are limited. A nationwide survey was conducted to characterize how LQTS detected during those periods is different from that in childhood or adolescence. Methods and Results-Using questionnaires, 58 cases were registered from 33 institutions. Diagnosis (or suspicion) of LQTS was made during fetal life (n = 18), the neonatal period (n = 31, 18 of them at 0 to 2 days of life), and beyond the neonatal period (n = 9). Clinical presentation of LQTS included sinus bradycardia (n = 37), ventricular tachycardia/torsades de pointes (n = 27), atrioventricular block (n = 23), family history of LQTS (n = 21), sudden cardiac death/aborted cardiac arrest (n = 14), convulsion (n = 5), syncope (n = 5), and others. Genetic testing was available in 41 (71%) cases, and the genotype was confirmed in 29 (71%) cases, consisting of LQT1 (n = 11), LQT2 (n = 11), LQT3 (n = 6), and LQT8 (n = 1). Ventricular tachycardia/torsades de pointes and atrioventricular block were almost exclusively observed in patients with LQT2, LQT3, and LQT8, as well as in those with no known mutation. In LQT1 patients, clues to diagnosis were mostly sinus bradycardia or family history of LQTS. Sudden cardiac death/aborted cardiac arrest (n = 14) was noted in 4 cases with no known mutations as well as in 4 genotyped cases, although the remaining 6 did not undergo genotyping. Their subsequent clinical course after aborted cardiac arrest was favorable with administration of beta-blockers and mexiletine and with pacemaker implantation/implantable cardioverter-defibrillator. Conclusions-Patients with LQTS who showed life-threatening arrhythmias at perinatal periods were mostly those with LQT2, LQT3, or no known mutations. Independent of the genotype, aggressive intervention resulted in effective suppression of arrhythmias, with only 7 deaths recorded. (Circ Arrhythm Electrophysiol. 2010; 3: 10-17.)

We report the first case of prenatally diagnosed aortopulmonary window (APW) with interrupted aortic arch. The mother was referred for fetal echocardiography at 29 weeks' gestation because of suspected congenital heart disease. On the short-axis view, we clearly imaged the aortopulmo nary septal defect at the proximal pulmonary artery and type A interrupted aortic arch. The window was 4 mm and the shunt flow detected by color flow Doppler was right-to-left in systole and early diastole. All aortic arch branches originated from the aorta proximal to the interruption. The well-developed ascending aorta was not continuous with the descending aorta in the sagittal view of the aortic arch. Visualization of the direction of the shunt flow across the APW and each origin of subclavian arteries was useful for the diagnosis of APW with interrupted aortic arch. Copyright (C) 2009 S. Karger AG, Basel

母体は32歳、潰瘍性大腸炎に対しmesalazine,prednisoloneを内服していた。36週1日の健診にて、胎児の心拡大と三尖弁逆流を指摘され、翌日、胎児心エコーにて動脈管早期閉鎖と診断、緊急帝王切開となった。出生後の心エコーで肺高血圧を認め、推定右室圧は70mmHgであった。人工呼吸管理行い、一酸化窒素の投与も開始した。肺血圧は次第に低下し日齢5に一酸化窒素を中止し、日齢8に抜管できた。日齢11の心エコーでは肺高血圧を認めず退院となった。その後の心機能は問題なく精神運動発達も正常である。(著者抄録)

Background The therapeutic strategy for pulmonary atresia (PA) and severe pulmonary stenosis (PS) with intact ventricular septum is controversial. Recent improvements in prenatal detection necessitate the identification of predictors of outcome for appropriate counseling and prenatal management planning. Methods and Results Echocardiograms of 18 fetuses antenatally diagnosed with PA (n=14) and PS (n=4) were reviewed and the total cardiac dimension (TCD) and tricuspid valve diameter (TVD) were measured. The right ventricular end-diastolic volume (RVEDV) was calculated from the right ventriculogram of the neonatal period by the percentage of the predicted normal value (%RVEDV). There was a positive correlation between TVD/TCD and %RVEDV (p < 0.001). As the initial treatment, balloon atrioseptostomy was performed in 13 cases of TVD/TCD < 0.26. As the final treatment, patients with TVD/TCD < 0.17 underwent or were planned for the Fontan procedure. Patients with TVD/TCD > 0.21 underwent or were planned for biventricular repair. Patients whose TVD/TCD was between 0.17 and 0.21 underwent or were planned for 1.5 ventricular repairs. Conclusion TVD/TCD is a useful index for selecting the postnatal initial treatment for PA/PS and to predict the final status of the fetus. Prenatal detection and prediction of the future status is helpful for family counseling Furthermore, it will help to decide the postnatal management prenatally.

高度房室弁逆流を伴う多脾症候群に、2段式肺動脈絞扼術と経皮的バルーン血管形成術を行った事例(1歳2ヵ月・女児)を報告した。高度房室弁逆流に対して7ヵ月時にグレン手術、2段式肺動脈絞扼術を、1歳2ヵ月時にチアノーゼの改善と肺動静脈瘻の予防を目的に、絞扼部に対して経皮的バルーン血管形成術を行った。治療後、房室弁逆流の増加を認めず、フォンタン手術の待機中である。

We describe the clinical course and autopsy findings of a female fetus with hydrops fetalis due to a huge pericardial rhabdomyoma. Fetal echocardiography at 21 weeks gestation demonstrated a huge tumor in the left ventricle. The fetus died of hydrops fetalis due to cardiac dysfunction at 24 weeks gestation. Autopsy demonstrated that the tumor protruded from the epicardial region of the apex into the pericardial cavity and induced a hypoplastic left ventricle and lung. Microscopically, the cardiac tumor showed characteristics of rhabdomyoma. This localization of cardiac rhabdomyoma is rare, but we remain aware of the possibility of an unusual and rapid progression of cardiac rhabdomyoma.

Newborn case of maternal anti-SSA antibody-induced congenital complete heart block (CCHB) accompanying cardiomyopathy is presented. Unexpectedly, she died of ventricular tachycardia, not bradycardia, 6 days after birth. Autopsy revealed left ventricular cardiomyopathy with endocardial fibroelastosis. Thus, when evaluating fetal cardiac performance in cases of maternal anti-SSA antibody-induced CCHB, it is necessary to pay attention to myocardial attributes such as endocardial hyperplasia.

Purpose: The prognosis of antenatally diagnosed congenital diaphragmatic hernia (ADCDH) is still very poor despite of innovation of various therapeutics. The authors reviewed their new therapeutic strategy of ADCDH from a viewpoint of cardiologic function. Methods: The cardiac function in 19 cases of ADCDH was reviewed. The patients, at the age of 0 days, were divided into 2 groups, PG (+) and PG (-), according to the requirement of prostaglandin El (PGE(1)) to attenuate pulmonary hypertension. The left ventricular (LV) end-diastolic dimension (LV diastolic diameter index [LVDI]) and bilateral pulmonary arterial diameters (total pulmonary artery index [TPAI]) were measured on days 0 and 2. Result: Only I patient died of cardiac or respiratory failure, and the survivors' postoperative course was uneventful. Eleven patients needed inhalation of nitric oxide (NO), and in 9 of those, PGE(1) was administered. The LVDI and TPAI of day 0 in PG (+) were significantly smaller than those in PG (-) and the controls. The LVDI increased from postnatal day 0 to day 2 in both PG (+) and PG (-). Although the LV was too small to output enough volume, the right ventricle successfully compensated for the low output through the ductus arteriosus, kept patent by NO and PGE(1). Conclusion: For ADCDH with sever pulmonary hypertension, keeping patent ductus arteriosus with NO and PGEI plays a critical role in obtaining excellent clinical outcome. Thus, the authors proposed a new therapeutic strategy for ADCDH based on a circulatory management. (c) 2005 Elsevier Inc. All rights reserved.

Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is a rare form of congenital heart disease with a high risk of perinatal mortality, particularly when diagnosed before birth. We aimed to assess whether global left and right ventricular function in fetal TOF/APV, using the Tei index, correlate with outcome. We reviewed the fetal echocardiogram and clinical outcome of eight fetuses diagnosed with TOF/APV. Of the eight cases, four developed cardiovascular compromise, leading to intrauterine death in two fetuses and neonatal demise in two fetuses, and four fetuses survived the neonatal period. The right ventricular (RV) Tei index was significantly greater and the left ventricular (LV) Tei index tended to be greater in nonsurvivors compared with survivors with TOF/APV (RV Tel, 0.90 +/- 0.17 versus 0.30 +/- 0.28, p < 0.05; LV Tei, 0.97 +/- 0.42 versus 0.54 +/- 0.21). The global LV and RV function can be affected in TOF/APV. Furthermore, more severe pulmonary insufficiency and worse biventricular function as assessed by Tei index likely contribute to the high perinatal mortality associated with this disease.

We sought to determine whether left ventricular (LV) function is abnormal in a fetus with severe tricuspid valve disease (TVD), and whether it may contribute to the outcome. We measured the LV Tei index (including isovolumic relaxation [IRT], contraction, and ejection times) in 31 fetuses with TVD, compared the data with measurements from 32 normal fetuses, and correlated these measurements with pulmonary morphology and outcome. LV Tei index was significantly greater, ejection times shorter, and IRT longer in TVD compared with controls. The Tei index was highest in fetuses with either pulmonary insufficiency or atresia compared with those with forward flow. Finally, LV Tei index was significantly greater in fetuses with fetal (n = 5) or neonatal (n = 9) demise than in neonatal survivors (n = 7). Global LV performance as assessed by the Tei index is abnormal in TVD and likely contribute to the high mortality associated with TVD.

Transcatheter coil embolization for coronary artery to left ventricular fistula was successfully performed in a neonate. At 30 weeks' gestation, fetal echocardiography showed a hypoplastic right ventricle with intact ventricular septum, absent pulmonary valve, tricuspid atresia, and marked distension of the right coronary artery. After birth. the neonate had congestive heart failure and the electrocardiogram showed myocardial ischemic changes in the left ventricular area. Aortography showed a dilated right coronary artery arising from the ascending aorta and draining into the left ventricle. Transcatheter coil embolization was carried out on the 9th day after birth. Since the procedure, no myocardial ischemic changes have been detected. Transcatheter coil embolization is a useful therapy for coronary artery fistula associated with myocardial ischemia.

Objectives. Supraventricular arrhythmias are one of the most common and fatal sequelae of the Fontan operation. P wave triggered signal averaged electrocardiography was performed in patients undergoing the Fontan operation to evaluate the presence of atrial degeneration, and to clarify which factors affected the development of atrial arrhythmias. Methods. P wave triggered signal averaged electrocardiography was recorded in 14 patients after the Fontan-type operation (conventional atriopulmonary connection in 5 and total cavopulmonary connection in 9) and 15 healthy controls. The duration and area of the filtered P wave, and the signal magnitudes (M20, M30)at 20 Hz and 30 Hz obtained from the frequency domain analysis of the P wave(M20, M30) were evaluated and compared with the hemodynamic data. Results. The duration and area of the filtered P wave, M20 and M30 in patients after atriopulmonary connection were significantly greater than in those after total cavopulmonary connection and the control subjects (p < 0.05). M20 was significantly greater in patients after total cavopulmonary connection than in the control subjects. Right atrial volume in patients after atriopulmonary connection was significantly (p < 0.001) larger than in patients after total cavopulmonary connection (p < 0.05). There were no significant differences in other indices including atrial pressure between the two groups. Conclusions. Our results suggest that the substrate for atrial arrhythmias such as atrial myocardial degeneration and fibrosis is frequently present in patients after the Fontan operation, especially after atriopulmonary connection. Thus, the enlarged right atrium may be involved in the presence of a substrate for atrial arrhythmias. The developmental risk for late atrial arrhythmias seems to be present even in patients after total cavopulmonary connection.

Background:Operative survival after the Fontan procedure is good; however, there are some patients with disappointing results, especially those with atrial isomerism. Objectives: We tested whether the diameter of the pulmonary veins, which is reported as a useful indicator of pulmonary blood flow, predicts operative results after the Fontan operation. Patients and Methods: We evaluated 30 consecutive patients undergoing either the bidirectional Glenn anastomosis (BDG) or the Fontan operation. Age at operation ranged from 3 to 81 months (mean 30). Diagnosis was right or left isomeric heart in 15 patients, double-outlet right ventricle in 4 and various other malformations in 11. BDG was performed in 16 patients and the Fontan operation in 14 patients. The diameters of the pulmonary veins were measured proximal to the entrance into the atrium in the late phase of a pulmonary arteriogram. The pulmonary vein (PV) index (in mm(2)/m(2)) was calculated from the sum of the cross-sectional areas of these veins divided by the body surface area. Results: Of the patients undergoing BDG (+/- ancillary procedures), 12 had successful results and 4 had unsuccessful results. The PV index for hemodynamically successful patients was 361 +/- 153 and 275 +/- 60 mm2/m2 (mean +/- SD) for unsuccessful patients (p = 0.30). Of the patients who underwent the Fontan operation, 13 had successful and 1 had unsuccessful results. The PV index for successful patients was >285 mm(2)/m(2) and 137 mm(2)/m(2) for the nonsuccessful patients. The new pulmonary vascular resistance (PVR) calculated by using the PV index (mean pressure difference between the pulmonary artery and the atrium/PV index) for BDG patients with successful or unsuccessful results was 2.0 +/- 0.5 or 3.5 +/- 0.2 mmHg/mm(2) per m(2), respectively (p < 0.01). The new PVR for Fontan patients with successful results was <2.0 mmHg/mm(2) per m(2), while that for the patient with an unsuccessful result was 4.4. The new PVR completely separated patients into successful and unsuccessful groups, while conventionally calculated PVR did not (p = 0.63). Conclusions: PV index appears to be a useful morphological indicator of pulmonary blood flow and "new" PVR may improve the decision-making strategy for patients presenting with univentricular heart, especially those associated with isomeric heart.

Hepatic venous blood has been thought to play some role as a vasoactive agent in the development of pulmonary arteriovenous fistulas in patients with congenital heart disease. During the last 15 years, we have observed pulmonary arteriovenous fistulas in 3, and systemic arteriovenous fistulas in 2, patients from our 16 cases of left isomerism. During the same period, neither pulmonary nor systemic arteriovenous fistulas were detected among 50 patients with right isomerism. Pulmonary arteriovenous fistulas had developed in the absence of surgery in I of the patients. Both pulmonary and systemic fistulas were detected in an another patient, in whom the hepatic venous blood bypassed the pulmonary circulation. The level of somatostatin, which is known to reduce splanchnic blood flow was high in the systemic venous blood of this patient. Although the mechanism of development of the fistulas has yet to be clarified, we should be aware that not only pulmonary, but also systemic arteriovenous fistulas can be found in patients with left isomerism, even prior to any surgical intervention.

Objectives: This study was done to clarify which diameter, that of the pulmonary arteries (PAs) or that of the pulmonary veins (PVs), more precisely reflects pulmonary blood flow (PBF) bilaterally and unilaterally. Methods: To evaluate bilateral PBF, we studied 15 consecutive patients with Kawasaki disease as normal patients and 30 patients with tetralogy of Fallot who received cardiac catheterization. To evaluate unilateral PBF, 20 patients with various congenital heart diseases undergoing cineangiography and lung perfusion scintigraphy were studied. The diameter of PA was measured immediately proximal to the origin of the first lobar branches bilaterally, and right PA area, left PA area, PA area (mm(2)), and PA index (mm(2)/m(2)) were calculated. The diameter of PV was also measured distal to the junction with the left atrium. Right PV area, left PV area, PV area (mm(2)), and PV index (mm(2)/m(2)) were calculated from these diameters. Pulmonary blood flow (PBF) was obtained by the Pick method during catheterization. To evaluate unilateral PBF, PBF was divided into right and left PBF according to the right/left perfusion ratio measured by lung perufusion scintigraphy. Results: Evaluation of bilateral PBF was as follows: in normal patients, PA and PV areas were correlated with body surface area (r = 0.88, p = 0.0001 and r = 0.93, p = 0.0001); PA index and PV index ranged from 248 to 436 (mean = 343) mm(2)/m(2) and from 346 to 595 (mean = 466) mm(2)/m(2), respectively, and were constant irrespective of body surface area; PA and PV areas were correlated with PBF in normal patients, as well as in patients with tetralogy of Fallot. There was a better correlation between PV area and PBF than between PA area and PBF in normal patients, as well as a significantly better correlation in patients with tetralogy of Fallot. Evaluation of unilateral PBF was as follows: right PV area was correlated with right PBF (p = 0.0002), while right PA area was not; left PV area and left PA area were correlated with left PBF; right/left PV area ratio was correlated with the right/left perfusion ratio with better agreement than right/left PA area ratio. Conclusion: Our data suggest that the size of PVs in patients with congenital heart disease may be more useful than the size of PAs to indicate bilateral and unilateral PBF than the size of PAs. Differences in PV area of each lung may be a suitable indicator of discrepancy in blood flow to each lung.

The objective of the study was to examine the relation between the morphology of the common atrioventricular valve and regurgitation of the valve in patients with right atrial isomerism, We examined seven consecutive patients with documented right atrial isomerism who subsequently underwent postmortem examination during a 10-year period. The degree of regurgitation and the diameters of the common valve were evaluated via cineangiography, and the site of regurgitation was evaluated by echocardiography. The morphology of the common atrioventricular valve was assessed further at autopsy. Cineangiography revealed valve diameters ranging from 14.8 to 27.8 mm (mean 20.9 mm). Valvar regurgitation was revealed within 2 months of birth in all patients. Regurgitation abruptly worsened in three patients after placement of a Blalock-Taussig shunt or a central shunt and postintubation. Autopsies revealed that the common atrioventricular valve consisted of four leaflets in five patients, and three leaflets in two. The anterior leaflets were large and protruding in all patients, and the lateral leaflets were thickened in six, All patients had a mass consisting of the left lateral leaflets and chordae with direct attachment of the chordae to the ventricular muscle (the right lateral leaflet was attached to the ventricular muscle and immobile in one patient). The lateral leaflets clung to the ventricular wall and exhibited poor movement in six patients, Leaflets with poor mobility corresponded to the regurgitant valvar site as assessed by echocardiography in six patients; and the regurgitation in three patients with acute deterioration occurred at the valvar side with poor mobility. It is concluded that the common atrioventricular valve in patients with right atrial isomerism has morphologic characteristics that may be associated with valvar regurgitation and malignant potential for abrupt deterioration after replacement of systemic-pulmonary shunting.

Although transaortic patch repair under cardiopulmonary bypass is a suitable procedure for aortopulmonary window, another method without cardiopulmonary bypass may be the only one for an infant with extremely low birth weight. We describe a successful repair of an infant with extremely low birth weight (758 g) by closing the window with a clip through a left thoracotomy. Cardiac catheterization 7 months after the operation showed no residual shunt and no stenosis of ascending aorta, pulmonary artery, or coronary arteries in the patient, whom we believe to be one of the smallest with successful repair.

These findings suggest that PA banding may be suitable in children with congenital heart disease and excessive pulmonary flow, and that best results are obtained when the band circumference is < 90% of the standard pulmonary valve-ring circumference, as calculated from an equation derived from normal pulmonary valve dimensions. This guideline applies equally well to small infants weighing < 3 kg and to larger patients. © 1995.

Surgical treatment for a hypoplastic aortic arch associated with coarctation or interruption of the aorta is controversial. We evaluate the changes of diameter of proximal transverse aortic arch after surgery in 28 patients. Proximal transverse aortic arch in all patients was preoperatively 3.5 +/- 0.9 mm (2.5 to 7 mm), and 54 +/- 12% (36 to 84%) to the normal aortic valve dimension (n-AVD: 16.6 X BSA0.6). While postoperative proximal transverse aortic arch was 6.5 +/- 1.8 mm, and 76 +/- 12% to the n-AVD, and significantly grew more than the preoperative arch dimension (p = 0.0001). In 18 patients having two times cardiac catheterization postoperatively, proximal transverse aortic arch was 6.5 +/- 1.6 mm, and 75 +/- 13% to n-AVD on the 1st postoperative examination. On the 2nd examination, the arch was 9.9 +/- 1.9 mm, and 88 +/- 12% to n-AVD, and significantly grew with increasing years (p < or = 0.0003). We concluded that the proximal transverse aortic arch, which was more than 36% to n-AVD in diameter, if not dilated surgically, grew with increasing years after aortic arch repair.

生後31日目の総肺静脈還流異常症Darling分類1-A型症例に対する根治手術後に人工心肺からの離脱が困難な左心不全をきたした.垂直静脈を開放することにより人工心肺からの離脱および閉胸が可能となった.術後3日目に垂直静脈に通していた糸を引き上げることにより垂直静脈の経皮的二期的閉鎖を行った.閉鎖前後において左房圧,体動脈圧に変化はなく術後経過は良好であった.垂直静脈の経皮的二期的閉鎖は本症の術後左心不全に対する有用な一手段と考えられたので報告する.

Abstract Background:Operative survival after the Fontan procedure is good however, there are some patients with disappointing results, especially those with atrial isomerism. Objectives: We tested whether the diameter of the pulmonary veins, which is reported as a useful indicator of pulmonary blood flow, predicts operative results after the Fontan operation. Patients and Methods: We evaluated 30 consecutive patients undergoing either the bidirectional Glenn anastomosis (BDG) or the Fontan operation. Age at operation ranged from 3 to 81 months (mean 30). Diagnosis was right or left isomeric heart in 15 patients, double‐outlet right ventricle in 4 and various other malformations in 11. BDG was performed in 16 patients and the Fontan operation in 14 patients. The diameters of the pulmonary veins were measured proximal to the entrance into the atrium in the late phase of a pulmonary arteriogram. The pulmonary vein (PV) index (in mm2/m2) was calculated from the sum of the cross‐sectional areas of these veins divided by the body surface area. Results: Of the patients undergoing BDG (± ancillary procedures), 12 had successful results and 4 had unsuccessful results. The PV index for hemodynamically successful patients was 361 ± 153 and 275 ± 60 mm2/m2 (mean 5 SD) for unsuccessful patients (p = 0.30). Of the patients who underwent the Fontan operation, 13 had successful and 1 had unsuccessful results. The PV index for successful patients was > 285 mm2/m2 and 137 mm2/m2 for the nonsuccessful patients. The new pulmonary vascular resistance (PVR) calculated by using the PV index (mean pressure difference between the pulmonary artery and the atrium/PV index) for BDG patients with successful or unsuccessful results was 2.0 ± 0.5 or 3.5 ± 0.2 mmHg/mm2 per m2, respectively (p < 0.01). The new PVR for Fontan patients with successful results was < 2.0 mmHg/mm2 per m2, while that for the patient with an unsuccessful result was 4.4. The new PVR completely separated patients into successful and unsuccessful groups, while conventionally calculated PVR did not (p = 0.63). Conclusions: PV index appears to be a useful morphological indicator of pulmonary blood flow and “new” PVR may improve the decision‐making strategy for patients presenting with univentricular heart, especially those associated with isomeric heart. Copyright © 1985, Wiley Blackwell. All rights reserved

現代の電子診療録には視覚情報は多いが聴覚情報は少ない。本研究では昔から重要視されていた聴診所見を可視化することで客観的情報として診療録に掲載することで聴診所見を有効に活用することを目指す。方法は先天性心疾患に認める心雑音を電子聴診器でデジタル録音し、音の構成要素（最強点、信号強度、時間、広がり、周波数）を解析する。次に各構成要素と心エコー法で診断する心疾患の重症度との関係を調べ、音の構成要素をsevere, moderate, mild, trivialに序列化する。この序列化した要素をレーダーチャートに組みなおし電子診療録に音源と共に掲載できるようにする。 研究の進め方は【2022年度】心雑音の収集と解析（録音、心エコーによる重症度判定）, 【2023年度】心雑音の解析と心エコーの重症度とのマッチング、【2024年度】レーダーチャートの作成である。2023年4月まで、心雑音60名の登録を完了した。登録した内容は、収縮期駆出性雑音48例（肺動脈弁狭窄38例、大動脈弁狭窄10例）、収縮期逆流性雑音6例（僧帽弁逆流）、拡張期逆流性雑音6例（大動脈弁逆流１例、肺動脈弁逆流5例）である。 疾患の程度は、収縮期駆出性雑音がtrivial 2例、mild 23例、moderate 20例、severe3例、収縮期逆流性雑音がmild 5例、moderate 1例、拡張期逆流性雑音がmild 1例、moderate 5例であった。 症例数の多かった収縮期駆出性雑音について心雑音の解析を行い、疾患の程度と比較した。最大RMSはmild -29.9±4.3dB、moderate -30.0±3.4dB、severe -25.6±2.0dB最大周波数はmild 15.6±2.1Hz、moderate 21.9±4.9Hz、severe 28.1±2.0Hzでいずれも統計学的有意差は認めなかった。