Portopulmonary Hypertension: An Updated Review

Erick A. Jasso-Baltazar, MD; Gonzalo A. Peña-Arellano, MD; Jonathan Aguirre-Valadez, MD; Isaac Ruiz, MD; Bruno Papacristofilou-Riebeling, MD; Jose Victor Jimenez, MD; Cristian J. García-Carrera, MD; Fabián E. Rivera-López, MD; Jesús Rodriguez-Andoney, MD; Francisco C. Lima-Lopez, MD; José Luis Hernández-Oropeza, MD; Juan A. Torres Díaz, MD; Eric Kauffman-Ortega, MD; Jesus Ruiz-Manriquez, MD; Pablo Hernández-Reyes, MD; Jorge Zamudio-Bautista, MD; Carlos A. Rodriguez-Osorio, MD; Tomás Pulido, MD; Sergio Muñoz-Martínez, MD; Ignacio García-Juárez, MD

doi:10.1097/TXD.0000000000001517

Transplantation Direct (Aug 2023)

Portopulmonary Hypertension: An Updated Review

Erick A. Jasso-Baltazar, MD,
Gonzalo A. Peña-Arellano, MD,
Jonathan Aguirre-Valadez, MD,
Isaac Ruiz, MD,
Bruno Papacristofilou-Riebeling, MD,
Jose Victor Jimenez, MD,
Cristian J. García-Carrera, MD,
Fabián E. Rivera-López, MD,
Jesús Rodriguez-Andoney, MD,
Francisco C. Lima-Lopez, MD,
José Luis Hernández-Oropeza, MD,
Juan A. Torres Díaz, MD,
Eric Kauffman-Ortega, MD,
Jesus Ruiz-Manriquez, MD,
Pablo Hernández-Reyes, MD,
Jorge Zamudio-Bautista, MD,
Carlos A. Rodriguez-Osorio, MD,
Tomás Pulido, MD,
Sergio Muñoz-Martínez, MD,
Ignacio García-Juárez, MD

Affiliations

Erick A. Jasso-Baltazar, MD: 1 Department of Gastroenterology and Liver Transplant Unit, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Gonzalo A. Peña-Arellano, MD: 2 Department of Gastroenterology, Instituto de Seguridad Social del Estado de México y Municipios, Mexico State, Mexico.
Jonathan Aguirre-Valadez, MD: 3 Department of Gastroenterology, Hospital Angeles Pedregal, Mexico City, Mexico.
Isaac Ruiz, MD: 4 Departament of Hepatology and Liver Trasplantation, Centre Hospitalier de I´Universite of Montréal, Montreal, Canada.
Bruno Papacristofilou-Riebeling, MD: 1 Department of Gastroenterology and Liver Transplant Unit, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Jose Victor Jimenez, MD: 5 Department of Medicine, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
Cristian J. García-Carrera, MD: 1 Department of Gastroenterology and Liver Transplant Unit, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Fabián E. Rivera-López, MD: 1 Department of Gastroenterology and Liver Transplant Unit, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Jesús Rodriguez-Andoney, MD: 6 Pulmonary Circulation Unit, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Francisco C. Lima-Lopez, MD: 7 Cardiology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
José Luis Hernández-Oropeza, MD: 6 Pulmonary Circulation Unit, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Juan A. Torres Díaz, MD: 1 Department of Gastroenterology and Liver Transplant Unit, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Eric Kauffman-Ortega, MD: 1 Department of Gastroenterology and Liver Transplant Unit, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Jesus Ruiz-Manriquez, MD: 1 Department of Gastroenterology and Liver Transplant Unit, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Pablo Hernández-Reyes, MD: 7 Cardiology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Jorge Zamudio-Bautista, MD: 8 Department of Anesthesiology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Carlos A. Rodriguez-Osorio, MD: 9 Department of Critical Care Medicine, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Tomás Pulido, MD: 10 Cardiopulmonary Department, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico.
Sergio Muñoz-Martínez, MD: 11 University of Barcelona, Spain.
Ignacio García-Juárez, MD: 1 Department of Gastroenterology and Liver Transplant Unit, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

DOI: https://doi.org/10.1097/TXD.0000000000001517
Journal volume & issue: Vol. 9, no. 8
p. e1517

Abstract

Read online

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.

Published in Transplantation Direct

ISSN: 2373-8731 (Online)
Publisher: Wolters Kluwer
Country of publisher: United States
LCC subjects: Medicine: Surgery
Website: http://journals.lww.com/transplantationdirect/Pages/default.aspx

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