%0 Journal Article
%T Effect of PAH Specific Therapy on Pulmonary Hemodynamics and Six-Minute Walk Distance in Portopulmonary Hypertension: A Systematic Review and Meta-Analysis
%A Muhammad Faisal
%A Furqan Siddiqi
%A Ahmad Alkaddour
%A Abubakr A. Bajwa
%A Adil Shujaat
%J Pulmonary Medicine
%D 2014
%I Hindawi Publishing Corporation
%R 10.1155/2014/528783
%X Background. Little is known about the effect of pulmonary arterial hypertension (PAH) specific therapy on pulmonary hemodynamics and exercise capacity in patients with portopulmonary hypertension (PoPH) because such patients are usually excluded from randomized clinical trials (RCT) of such therapy. Methods. We searched PUBMED using the terms ¡°(Therapy/Broad (filter)) AND (portopulmonary hypertension).¡± We included studies that met the following criteria: ¡Ý5 patients, AND PoPH confirmed by right heart catheterization (RHC), AND follow-up RHC data, AND/OR baseline and follow-up 6MWD available. Results. 12 studies met our inclusion criteria. None was a RCT. The baseline mPAP was 48.6 ¡À 4.4£¿mmHg, cardiac output (CO) 5.6 ¡À 0.9£¿L/min, and pulmonary vascular resistance (PVR) 668.6 ¡À 219.1£¿dynes.sec/cm5. The baseline 6MWD was 348.2 ¡À 35.6 meters. The use of PAH specific therapy improved mPAP by 7.54£¿mmHg (95% CI 10.2 to 4.9), CO by 1.77£¿L/min (95% CI 1.1 to 2.4), and PVR by 253£¿dynes.sec/cm5 (95% CI 291.4 to 214.6) () and 6MWD by 61.8 meters (95% CI 47.5 to 76) (). Conclusions. The use of PAH specific therapy in PoPH results in significant improvement in both pulmonary hemodynamics and 6MWD. 1. Introduction Portopulmonary hypertension (PoPH) refers to pulmonary arterial hypertension (PAH) associated with portal hypertension with or without cirrhosis [1]. The reported incidence ranges from 2 to 9% [2]. PoPH falls under group I of the WHO classification of pulmonary hypertension as it is histopathologically indistinguishable from idiopathic PAH [3]. It is the third most common cause of PAH after idiopathic PAH and PAH associated with connective tissue disease. Pulmonary arterial hypertension specific therapy has been shown to result in significant improvement in pulmonary hemodynamics and exercise capacity in patients with idiopathic PAH and PAH associated with connective tissue disease [4]. However, little is known about the effectiveness of PAH specific therapy in patients with PoPH since such patients are usually excluded from randomized clinical trials (RCTs) of such therapy because of overall poor survival as well as concerns about adverse drug effects. The use of PAH specific therapy in patients with PoPH may, by causing systemic vasodilatation, potentially exacerbate the hyperdynamic circulatory state [5]. There is a concern that epoprostenol may worsen splenomegaly and cause hypersplenism [6, 7], and endothelin receptor antagonists (ERA) may worsen liver function [8]. The effect of PAH specific therapy on pulmonary hemodynamics is particularly important
%U http://www.hindawi.com/journals/pm/2014/528783/