T Sasaki; T Hasegawa; T Kimura; A Okada; S Mushiake; T Matsushita
JOURNAL OF PEDIATRIC SURGERY 35 11 1647 - 1650 2000年11月
[査読有り] Purpose: This report presents biliary atresia (BA) patients with intrapulmonary arteriovenous shunting (IPS), which was evaluated suitably by contrast-enhanced echocardiography (CEC).
Methods: Of 88 BA patients seen in the last 20 years, 8 (9.1%) had IPS at 8 months to 16 years of age. Two were associated with polysplenia syndrome, 1 had persistent jaundice after hepatic portoenterostomy, and 2 underwent splenorenal shunt. According to the comparison between microbubbles in left atrium (LA) and in right atrium (RA) detected by CEC, IFS was classified as grade I, mild (LA << RA); grade II, moderate (LA < RA); grade III, severe (LA = RA).
Results: Grade I consisted of 4 patients whereas grade II and III held 2 patients each. Clinical symptoms such as cyanosis, exertional dyspnea, or clubbing were present in 50% of grade 1 and all of grade II and ill. Mean Pao, in grade I, II, and III was 70.5, 50.4, and 35.3 mm Hg, respectively. In 1 patient with grade I, IFS spontaneously disappeared, but pulmonary hypertension developed later. One patient in grade II died of pulmonary complications, and the other is considered a candidate for liver transplantation (LTx). One patient in grade III died of liver failure, whereas the other is free of IFS after LTx.
Conclusion: IFS can lead to a life-threatening complication in postoperative BA patients, and CEC may be a convenient and useful method to evaluate the degree of IFS and determine therapeutic strategy. Copyright (C) 2000 by W.B. Saunders Company.