Pulmonary Hypertension in Cirrhosis, Study of 22 Autopsy Cases.
Nahal Boroumand, Gregory White, Erica Gregonis, Alexander Duarte, Abida Haque. University of Texas Medical Branch, Galveston; Methodist Hospital, Houston
Background: Portopulmonary hypertension (POPH) is a form of pulmonary arterial hypertension associated with portal hypertension with or without underlying liver disease. Previous autopsy studies have reported a prevalence of pulmonary hypertension in a population with cirrhosis or portal hypertension to vary between 0.25% and 0.73%. However, prospective hemodynamic studies indicate the prevalence of POPH to be 2-4 % in patients with severe liver disease and 5-10 % in patients being evaluated for liver transplantation. The discrepancy in these prevalence rates is unclear. The aim of this study was to assess the prevalence of POPH in a group of subjects with cirrhosis undergoing a post mortem examination.
Design: The electronic autopsy data base at the University of Texas Medical Branch was examined from 1996-2009 for subjects with post mortem findings of cirrhosis. We selected those subjects with the following gross anatomical findings right ventricular hypertrophy, right ventricular dilation, and pulmonary artery atheromas or microscopic histology indicative of pulmonary vascular disease. Subsequently, those subjects with cirrhosis and pulmonary hypertension were identified and the microscopic histology of liver and lung examined by two pulmonary pathologists.
Results: We identified 256 subjects with cirrhosis. From this cohort, 36 subjects with cirrhosis and right ventricular pathology or pulmonary vascular disease were identified. However, 14 patients were excluded from further analysis due to missing pathology (n=8), absent clinical history (3) or a clinical history of left ventricular systolic dysfunction (n=3) resulting in a final cohort of 22 subjects. The mean age of the final cohort was 50 ± 8.3 years with 17 males and 5 females. Cirrhosis was recognized 5.9 ± 5.5 yrs before death. Eight subjects were known to have right ventricular hypertrophy or pulmonary hypertension before death for a mean time of 0.8 ± 0.8 yrs. Mean cardiac weight was 518 ± 237 grams and mean liver weight was 1366 ± 372 grams. Cirrhosis was identified in all hepatic histology sections. The pulmonary vascular histology revealed intimal hyperplasia (91 %), precapillary vessel dilation (59 %), plexiform lesions (77 %) and vascular necrosis (14 %).
Conclusions: In the current autopsy study, we observed the prevalence of POPH 8.9 % to be greater than prior autopsy series and similar to prospective hemodynamic studies.
Wednesday, March 2, 2011 9:30 AM
Poster Session V # 273, Wednesday Morning