Chest
Clinical Investigations: Pulmonary VasculatureDistinctive Clinical Features of Portopulmonary 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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Cited by (0)
Supported by a National Institutes of Health Vascular Disease Academic Award (Dr. Rubin).