Chest
Volume 112, Issue 4, October 1997, Pages 980-986
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Clinical Investigations: Pulmonary Vasculature
Distinctive Clinical Features of Portopulmonary Hypertension

https://doi.org/10.1378/chest.112.4.980Get rights and content

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.

Section snippets

Materials and Methods

PPHTN was defined as the presence of precapillary pulmonary hypertension (mean pulmonary artery pressure ≥;25 mm Hg at rest with a pulmonary capillary wedge pressure <15 mm Hg, the absence of congenital heart disease, clinically demonstrable thromboembolic disease, and other major cardiopulmonary dysfunction) accompanied by signs and symptoms of hepatic dysfunction and/or portal hypertension. The presence of hepatic dysfunction or portal hypertension was assessed by the following: (1) the

Results

The demographic and functional characteristics of the three patient groups, PPHTN, L-CONT, and P-CONT, are listed in Table 1. The majority (20/30) of patients in the three groups were female. The mean age in the three groups was approximately 47 years. As stratified by the NYHA classification system, cardiopulmonary functional status was statistically equivalent between the PPHTN and P-CONT groups, indicating that these patients developed symptoms with prolonged usual activity and were able to

Discussion

PPHTN is a poorly characterized syndrome loosely defined as the association of primary pulmonary hypertension with portal hypertension. While the literature abounds with case reports, there have been few reports examining PPHTN patients in larger numbers.4, 5, 6, 7, 8, 9, 10, 11, 12 As a result, attempts to better study this entity, develop screening strategies, and implement potential therapies have been hampered. In addition, assumptions concerning PPHTN have been largely derived from the

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Supported by a National Institutes of Health Vascular Disease Academic Award (Dr. Rubin).

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