Distinctive clinical features of portopulmonary hypertension

Authors

Paul C. Kuo, University of Maryland Medical System
Jeffrey S. Plotkin, University of Maryland Medical System
Lynt B. Johnson, University of Maryland Medical System
Charles D. Howell, University of Maryland Medical System
Jacqueline M. Laurin, University of Maryland Medical System
Stephen T. Bartlett, University of Maryland Medical System
Lewis J. Rubin, University of Maryland Medical System

Document Type

Journal Article

Publication Date

1-1-1997

Journal

Chest

Volume

112

Issue

4

DOI

10.1378/chest.112.4.980

Keywords

Alkalosis; Cirrhosis; Gas exchange; Liver; Liver transplantation; Pulmonary hypertension

Abstract

Study objective: To differentiate the cardiopulmonary profile of portopulmonary hypertension (PPHTN) from that of primary pulmonary hypertension and chronic liver disease. Design: Retrospective survey. Setting: Tertiary care center. Patients: Thirty patients with cardiac catheterization-proven PPHTN were compared to 30 randomly selected patients with primary pulmonary hypertension alone and 30 patients with chronic liver disease alone necessitating consideration of liver transplantation (L-CONT). Interventions: All patients underwent right heart catheterization, echocardiography, ECG, chest radiography, pulmonary function tests, ventilation-perfusion scanning, and room air arterial blood gas measurements. Results: Patients with PPHTN exhibited elevated pulmonary pressures (mean pulmonary pressure, 48.6±2.1 mm Hg) and pulmonary vascular resistance (11.6±1.6 mm Hg/L/min/m2) with simultaneous elevation in the cardiac index (3.8±0.3 L/min/m2) and depression of systemic vascular resistance (24.9± 1.7 mm Hg/L/min/m2). Arterial blood gas measurements indicate that PPHTN exhibits a significant accentuation of the chronic respiratory alkalosis (PCO2, 28.7±0.5 mm Hg) usually seen with chronic liver disease and pulmonary hypertension. In addition, patients with PPHTN have an increased alveolar-arterial gradient (27.0±2.7 mm Hg) when compared to patients with L-CONT, suggesting impaired gas exchange. Conclusions: PPHTN is associated with a unique clinical profile that possesses characteristics common to and exclusive of liver disease and primary pulmonary hypertension.

APA Citation

Kuo, P., Plotkin, J., Johnson, L., Howell, C., Laurin, J., Bartlett, S., & Rubin, L. (1997). Distinctive clinical features of portopulmonary hypertension. Chest, 112 (4). http://dx.doi.org/10.1378/chest.112.4.980