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Semin Respir Crit Care Med 2017; 38(05): 651-661
DOI: 10.1055/s-0037-1606251
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Portopulmonary Hypertension

Laurent Savale
1   Faculté de Médecine, Univ. Paris–Sud, Université Paris-Saclay, Le Kremlin Bicêtre, France
2   AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre, France
3   INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
,
Jason Watherald
1   Faculté de Médecine, Univ. Paris–Sud, Université Paris-Saclay, Le Kremlin Bicêtre, France
2   AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre, France
3   INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
4   Division of Respirology, Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada
,
Olivier Sitbon
1   Faculté de Médecine, Univ. Paris–Sud, Université Paris-Saclay, Le Kremlin Bicêtre, France
2   AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre, France
3   INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
› Author Affiliations
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Publication History

Publication Date:
17 October 2017 (online)

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Abstract

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.

Keywords

hypertension - pulmonary - pulmonary arterial hypertension - portal hypertension - cirrhosis - liver transplantation - survival
 

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