Portopulmonary Hypertension: An Updated Review

被引：11

作者：

Jasso-Baltazar, Erick A. A. ^{[1
,2
]}

Pena-Arellano, Gonzalo A. A.

Aguirre-Valadez, Jonathan ^{[3
]}

Ruiz, Isaac ^{[4
]}

Papacristofilou-Riebeling, Bruno ^{[1
,2
]}

Jimenez, Jose Victor ^{[5
]}

Garcia-Carrera, Cristian J. ^{[1
,2
]}

Rivera-Lopez, Fabian E. ^{[1
,2
]}

Rodriguez-Andoney, Jesus ^{[6
]}

Lima-Lopez, Francisco C. ^{[7
]}

Hernandez-Oropeza, Jose Luis ^{[6
]}

Diaz, Juan A. Torres ^{[1
,2
]}

Kauffman-Ortega, Eric ^{[1
,2
]}

Ruiz-Manriquez, Jesus ^{[1
,2
]}

Hernandez-Reyes, Pablo ^{[7
]}

Zamudio-Bautista, Jorge ^{[8
]}

Rodriguez-Osorio, Carlos A. ^{[9
]}

Pulido, Tomas ^{[10
]}

Munoz-Martinez, Sergio

Garcia-Juarez, Ignacio ^{[1
,2
,11
]}

机构：

[1] Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Dept Gastroenterol, Mexico City, Mexico

[2] Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Liver Transplant Unit, Mexico City, Mexico

[3] Hosp Angeles Pedregal, Dept Gastroenterol, Mexico City, Mexico

[4] Ctr Hosp Univ Montreal, Dept Hepatol & Liver Trasplantat, Montreal, PQ, Canada

[5] Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Dept Med, Mexico City, Mexico

[6] Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Pulm Circulat Unit, Mexico City, Mexico

[7] Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Cardiol Dept, Mexico City, Mexico

[8] Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Dept Anesthesiol, Mexico City, Mexico

[9] Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Dept Crit Care Med, Mexico City, Mexico

[10] Inst Nacl Cardiol Ignacio Chavez, Cardiopulm Dept, Mexico City, Mexico

[11] Av Vasco de Quiroga 15,Colonia Belisario Dominguez, Mexico City 14080, Mexico

来源：

TRANSPLANTATION DIRECT | 2023年 / 9卷 / 08期

关键词：

PULMONARY ARTERIAL-HYPERTENSION; LIVER-TRANSPLANTATION; HEPATOPULMONARY SYNDROME; PORTOSYSTEMIC SHUNTS; GERMLINE MUTATIONS; SURVIVAL; RECEPTOR; HEMODYNAMICS; MANAGEMENT; BOSENTAN;

D O I：

10.1097/TXD.0000000000001517

中图分类号：

R3 [基础医学]; R4 [临床医学];

学科分类号：

1001 ; 1002 ; 100602 ;

摘要：

Portal hypertension may have major consequences on the pulmonary vasculature due to the complex pathophysiological interactions between the liver and lungs. Portopulmonary hypertension (PoPH), a subset of group 1 pulmonary hypertension (PH), is a serious pulmonary vascular disease secondary to portal hypertension, and is the fourth most common subtype of pulmonary arterial hypertension. It is most commonly observed in cirrhotic patients; however, patients with noncirrhotic portal hypertension can also develop it. On suspicion of PoPH, the initial evaluation is by a transthoracic echocardiogram in which, if elevated pulmonary pressures are shown, patients should undergo right heart catheterization to confirm the diagnosis. The prognosis is extremely poor in untreated patients; therefore, management includes pulmonary arterial hypertension therapies with the aim of improving pulmonary hemodynamics and moving patients to orthotopic liver transplantation (OLT). In this article, we review in detail the epidemiology, pathophysiology, process for diagnosis, and most current treatments including OLT and prognosis in patients with PoPH. In addition, we present a diagnostic algorithm that includes the current criteria to properly select patients with PoPH who are candidates for OLT.

引用

页数：11

共 81 条

[1] Treatment Barriers in Portopulmonary Hypertension [J].