Takehisa Ueno1, Ryuta Saka2, Yuichi Takama2, Hiroaki Yamanaka2, Yuko Tazuke2, Kazuhiko Bessho3, Hiroomi Okuyama2. 1. Department of Pediatric Surgery, Osaka University of Graduation School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan. ueno@pedsurg.med.osaka-u.ac.jp. 2. Department of Pediatric Surgery, Osaka University of Graduation School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan. 3. Department of Pediatrics, Osaka University of Graduation School of Medicine, Suita, Japan.
Abstract
PURPOSE: Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are extrahepatic complications of biliary atresia (BA). Their detection is sometimes delayed, which may result in missed opportunities for liver transplantation. The aim of this study was to determine the onset ages of HPS and PoPH in BA patients. METHODS: BA patients followed at our institution were identified. Patients visited our clinic for routine blood work, as well as regular electrocardiography, chest X-rays, and arterial blood gas tests. Lung perfusion scintigraphy and cardiac ultrasound were performed to diagnose HPS. Cardiac catheterization was conducted to diagnose PoPH. RESULTS: The study population consisted of 88 BA patients. The median follow-up duration was 11.6 years (range 0.8-26.0 years). Six patients (6.8%) developed HPS and three patients (3.4%) developed PoPH. The median age of onset of HPS was significantly younger than that of PoPH (HPS: 4 years, PoPH: 15 years, P < 0.019). Two patients (66%) with PoPH died, while all patients with HPS survivied. CONCLUSION: The onset of HPS was significantly earlier than that of PoPH. The mortality rate was high in patients with PoPH. Teenagers with BA should receive routine cardiac echocardiograms to detect PH in its early stages.
PURPOSE:Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are extrahepatic complications of biliary atresia (BA). Their detection is sometimes delayed, which may result in missed opportunities for liver transplantation. The aim of this study was to determine the onset ages of HPS and PoPH in BA patients. METHODS: BA patients followed at our institution were identified. Patients visited our clinic for routine blood work, as well as regular electrocardiography, chest X-rays, and arterial blood gas tests. Lung perfusion scintigraphy and cardiac ultrasound were performed to diagnose HPS. Cardiac catheterization was conducted to diagnose PoPH. RESULTS: The study population consisted of 88 BA patients. The median follow-up duration was 11.6 years (range 0.8-26.0 years). Six patients (6.8%) developed HPS and three patients (3.4%) developed PoPH. The median age of onset of HPS was significantly younger than that of PoPH (HPS: 4 years, PoPH: 15 years, P < 0.019). Two patients (66%) with PoPH died, while all patients with HPS survivied. CONCLUSION: The onset of HPS was significantly earlier than that of PoPH. The mortality rate was high in patients with PoPH. Teenagers with BA should receive routine cardiac echocardiograms to detect PH in its early stages.
Authors: Y Urashima; T Tojimbara; I Nakajima; S Sato; M Nakamura; T Kawase; K Kai; S Fuchinoue; S Teraoka Journal: Transplant Proc Date: 2004-10 Impact factor: 1.066
Authors: H Soh; T Hasegawa; T Sasaki; T Azuma; A Okada; S Mushiake; S Kogaki; T Matsushita; T Harada Journal: J Pediatr Surg Date: 1999-12 Impact factor: 2.545