Moderate-Term Effect of Epoprostenol on Severe Portopulmonary Hypertension

Kato, Hitoshi; Katori, Tatsuo; Nakamura, Yuzuru; Kawarasaki, Hideo

doi:10.1007/s00246-002-0120-9

Cited by 13 publications

(8 citation statements)

References 8 publications

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“…Only small retrospective studies have reported positive effects on functional status and hemodynamics in those patients. [34][35][36][37][38] The major limiting factor of epoprostenol is its mode of administration which requires continuous intravenous infusion through a tunneled central venous catheter, exposing patients to important complications, including bleeding at the time of catheter insertion, and a perpetual risk of local and/or bloodstream infections.…”

Section: Prostanoidsmentioning

confidence: 99%

Portopulmonary Hypertension

Watherald

Sitbon

Savale

2017

Semin Respir Crit Care Med

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Portal hypertension may have major consequences on the pulmonary vasculature due to complex pathophysiological interactions between liver and lungs. Portopulmonary hypertension (PoPH) is characterized by the association of portal hypertension and pulmonary arterial hypertension (PAH). As progressive elevation of right ventricular afterload can lead to right heart failure, PoPH is a serious complication of portal hypertension, affecting functional status and prognosis of patients. Early detection by transthoracic echocardiography must be performed in symptomatic patients and in candidates for liver transplantation (LT). Right heart catheterization remains mandatory to confirm the diagnosis and exclude all other causes of elevated pulmonary pressures. The management of PoPH includes PAH-targeted therapies although few studies have evaluated these medications in this specific indication. The impact of LT on PoPH outcome remains complex and must be specified by future collaborative investigation. Although uncontrolled PoPH is associated with higher risk of postoperative right heart failure and death, stabilization, improvement, or normalization of pulmonary hemodynamics after LT seem to be achievable goals in selected patients with PoPH.

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Section: Prostanoidsmentioning

confidence: 99%

Portopulmonary Hypertension

Watherald

Sitbon

Savale

2017

Semin Respir Crit Care Med

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“…Portopulmonary hypertension is a more severe and less common pulmonary complication that has been observed in children with biliary atresia 73–84. Most of the reports of portopulmonary hypertension are anecdotal and are often associated with a fatal outcome.…”

Section: Portopulmonary Hypertensionmentioning

confidence: 99%

Biliary atresia: A transplant perspective

Shneider

Mazariegos

2007

Liver Transpl

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“…This relative hypoxia may complicate an already diseased pulmonary vasculature, accelerating the appearance of symptoms. However, hypoxia is not a required stimulus for the development of PPHTN in children, because PPHTN has been described at sea level, 29 and 1 of our 7 patients developed PPHTN while living at sea level.…”

Section: Discussionmentioning

confidence: 76%

Portopulmonary Hypertension in Pediatric Patients

Condino

Ivy

OʼConnor

et al. 2005

The Journal of Pediatrics

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Objectives-To investigate the clinical presentation, manifestations, and response to therapy of portopulmonary hypertension (PPHTN) in pediatric patients.Study design-This study was a retrospective chart review describing the evaluation and course of 7 patients with PPHTN.Results-Causes of portal hypertension (HTN) included biliary atresia (3 cases), cavernous transformation of the portal vein (2 cases), and primary sclerosing cholangitis and cryptogenic cirrhosis (1 case each). The median interval from the diagnosis of portal HTN to PPHTN was 12.1 years. Four patients presented with a new heart murmur, 4 presented with syncope, and 3 presented with dyspnea. Although electrocardiograms (ECGs) and chest x-rays were normal in 3 and 2 patients, respectively, echocardiograms diagnosed pulmonary HTN in all 7 patients. Five patients had cardiac catheterizations; the average mean pulmonary artery pressure was 65 ± 20 mm Hg. Response to therapy was variable, and 4 patients died. Postmortem lung tissue examination revealed plexiform lesions and pulmonary arteriopathy.Conclusions-Because symptoms are subtle and may be overlooked, pediatric patients with portal HTN who develop a new heart murmur, dyspnea, syncope, or who are being evaluated for liver transplantation require evaluation for PPHTN. ECG and chest x-ray are insensitive screens for PPHTN. An echocardiogram and cardiology evaluation is essential for the diagnosis.Portopulmonary hypertension (PPHTN) is one of the pulmonary vascular disorders complicating chronic liver disease. 1 In 1998 the World Health Organization P(WHO) classified PPHTN as pulmonary arterial hypertension (HTN) associated with liver disease or portal HTN. 2 PPHTN is defined by elevated mean pulmonary arterial pressure (PAP) (> 25 mm Hg at rest), increased pulmonary vascular resistance (PVR) (>3 Wood units · 3 m 2 ), and normal pulmonary capillary wedge pressure (<15 mm Hg) in the presence of portal HTN. 3 Both hepatic and extrahepatic causes of portal HTN may lead to PPHTN. The prevalence of PPHTN in adult patients with cirrhosis is 0.25% to 0.73% based on autopsy series 1,4 and 3.5% to 8.5% in liver transplant candidates. 1,5 The diagnosis of PPHTN is usually made 4 to 7 years after the diagnosis of portal HTN in adults. 1,6 Copyright © 2005 Elsevier Inc. All rights reserved. METHODSA retrospective review of patient records in the Pediatric Liver Center, Children's Hospital identified 7 pediatric patients with PPHTN diagnosed between 1995 and 2004 ( Table I). All of these children had been previously enrolled in an institutional review board-approved protocol for prospective longitudinal evaluation of childhood pulmonary HTN, for which written informed consent was obtained from the parents or guardians. Data obtained from the medical record included age at diagnosis of portal HTN and PPHTN, cause of portal HTN, symptoms and/or signs precipitating the evaluation for pulmonary HTN, and additional review of chest x-ray, electrocardiogram (ECG), Doppler echocardiogram, right heart catheter...

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Moderate-Term Effect of Epoprostenol on Severe Portopulmonary Hypertension

Cited by 13 publications

References 8 publications

Portopulmonary Hypertension

Portopulmonary Hypertension

Biliary atresia: A transplant perspective

Portopulmonary Hypertension in Pediatric Patients

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