Portopulmonary hypertension: An update

Zeenat Safdar, Sonja Bartolome, Norman Sussman

Research output: Contribution to journalArticle

59 Citations (Scopus)

Abstract

Portopulmonary hypertension (POPH) is a serious complication of cirrhosis that is associated with mortality beyond that predicted by the Model for End-Stage Liver Disease (MELD) score. Increased pulmonary vascular resistance (PVR) may be initiated by pulmonary vasoconstriction, altered levels of circulating mediators, or shear stress, and can eventually lead to the classic vascular remodeling (plexiform lesion) that characterizes POPH. Portal hypertension is a prerequisite for the diagnosis of POPH, although the severity of pulmonary hypertension is unrelated to the severity of portal hypertension or the nature or severity of liver disease. POPH precludes liver transplantation (LT) unless the mean pulmonary artery pressure (MPAP) can be reduced to a safe level. The concept of an acceptable pressure has changed: we now consider both MPAP and PVR in the diagnosis, and we include the transpulmonary pressure gradient so that we can factor in fluid overload and left ventricular failure. Pulmonary vasodilator therapy includes oral, inhaled, and parenteral agents, and one or more of these agents may significantly lower pulmonary artery pressures to the point that LT becomes possible. The United Network for Organ Sharing recommends MELD exception points for patients with medically controlled POPH, but this varies by region. Patients who undergo LT need specialized intraoperative and postoperative management, which includes the availability of intraoperative transesophageal echocardiography for assessing right ventricular function, and rapidly acting vasodilators (eg, inhaled nitric oxide and/or epoprostenol). Published case series suggest excellent outcomes after LT for patients who respond to medical therapy.

Original languageEnglish (US)
Pages (from-to)881-891
Number of pages11
JournalLiver Transplantation
Volume18
Issue number8
DOIs
StatePublished - Aug 2012

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Liver Transplantation
Hypertension
Pressure
Pulmonary Artery
End Stage Liver Disease
Portal Hypertension
Vasodilator Agents
Vascular Resistance
Right Ventricular Function
Lung
Transesophageal Echocardiography
Epoprostenol
Vasoconstriction
Pulmonary Hypertension
Liver Diseases
Nitric Oxide
Fibrosis
Mortality
Therapeutics

ASJC Scopus subject areas

  • Surgery
  • Transplantation
  • Hepatology

Cite this

Portopulmonary hypertension : An update. / Safdar, Zeenat; Bartolome, Sonja; Sussman, Norman.

In: Liver Transplantation, Vol. 18, No. 8, 08.2012, p. 881-891.

Research output: Contribution to journalArticle

Safdar, Zeenat ; Bartolome, Sonja ; Sussman, Norman. / Portopulmonary hypertension : An update. In: Liver Transplantation. 2012 ; Vol. 18, No. 8. pp. 881-891.
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