Onset Ages of Hepatopulmonary Syndrome and Pulmonary Hypertension in Patients with Biliary Atresia

Overview

Journal Pediatr Surg Int

Specialties General Surgery
Pediatrics

Date 2017 Sep 6

PMID 28871319

Citations 1

Authors

Takehisa Ueno

Ryuta Saka

Yuichi Takama

Hiroaki Yamanaka

Yuko Tazuke

Kazuhiko Bessho

Hiroomi Okuyama

Affiliations

Soon will be listed here.

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.

Citing Articles

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