Pulmonary Arterial Remodeling in End-Stage Liver Disease.
Gregory A Fishbein, Tisha Wang, Rajeev Saggar, Rajan Saggar, Michael Hansen, Michael C Fishbein. David Geffen School of Medicine at UCLA, Los Angeles, CA
Background: Pulmonary arterial remodeling is a well-recognized phenomenon associated with end-stage liver disease (ESLD). Two major pulmonary manifestations of ESLD exist: the hepatopulmonary syndrome and portopulmonary hypertension (POPH). Though both diseases are reported to be of low prevalence, screening for POPH is routine, as POPH is a relative contraindication to orthotopic liver transplantation. Little is known about the pathogenesis of POPH, and there are relatively few detailed quantitative studies of the pulmonary vasculature in ESLD. We reviewed autopsy specimens from 38 patients with ESLD, 11 with known POPH, in order to quantify pulmonary vascular morphology compared to normal controls.
Design: Using trichrome/elastic stained histologic sections, we performed computer-assisted morphometry of pulmonary arteries in 38 patients with ESLD and 12 patients with no known pulmonary or hepatic pathology. Eleven of the patients with ESLD had known POPH. A mean of 75 arteries per patient (range = 22-123) were analyzed, with diameters ranging from 50-1000 micrometers. Using calibrated software, tracings were made of external and internal elastic laminae, and endothelial surfaces, so as to calculate the areas occupied by the intima, media, and lumen of each vessel.
|Cases (males)||Mean Age||No. Arteries|
|POPH(-) ESLD||27 (18)||52||1907|
|POPH(+) ESLD||11 (4)||54||827|
|Media Area||Intima Area|
|Mean||Std Dev||Mean||Std Dev|
|ESLD (no POPH)||7968||5069||9500||3680|