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The Management of Portopulmonary Hypertension

Michael J Krowka, MD

The era of liver transplantation (LT) has refocused attention on the clinical entity of pulmonary arterial hypertension (PAH) associated with portal hypertension from any cause, also known as portopulmonary hypertension (PPH) [1]. Once thought to be caused by pulmonary emboli emanating from blood clots in portocaval pathways, the pathophysiology of PPH now appears to be more complex [2]. Importantly, the implications for patients with PPH considered for LT are evolving, with new pharmacological options and outcomes that suggest that, in highly selected patients, total resolution of this pulmonary vascular abnormality can occur, at least as characterized by right heart catheterization (RHC) and transthoracic echocardiographic function of the right ventricle (RV) [1]. The American Association for the Study of Liver Disease (AASLD) has endorsed formal practice guidelines to screen for PPH in all LT candidates [3].

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