Unusual clinical course, Challenging differential diagnosis, Unusual or unexpected effect of treatment
Guadalupe Aguirre-Avalos, Marco Antonio Covarrubias-Velasco, Antonio Gerardo Rojas-Sánchez
(Guadalajara, Jalisco , Mexico)
Am J Case Rep 2013; 14:354-358
Background: Suprahepatic inferior vena cava anastomosis stricture is an unusual vascular complication after orthotopic liver transplantation with the “piggyback” technique. Clinical manifestations are dependent upon the severity of the stenosis. Portopulmonary hypertension after orthotopic liver transplantation is a complication that carries high mortality due to cardiopulmonary dysfunction. The pathogenesis of pulmonary vascular disorders after orthotopic liver transplantation remains uncertain.
Case Report: We report a case of acute right heart pressure overload after surgical correction of the suprahepatic inferior vena cava anastomotic stricture in a 54-year-old woman who had preexisting pulmonary arterial hypertension associated with portal hypertension after orthotopic liver transplantation. Twenty months posttransplantation, she developed fatigue and progressive ascites. On admission, the patient had hepatomegaly, ascites, and lower limb edema. Symptoms in the patient developed gradually over time.
Conclusions: Recurrent portal hypertension by vascular complications is a cause of pulmonary arterial hypertension after orthotopic liver transplantation. Clinical manifestations of suprahepatic inferior vena cava anastomotic stenosis are dependent upon their severity. Sildenafil is an effective drug for treatment of pulmonary arterial hypertension after portal hypertension by vascular complications.
Keywords: Suprahepatic inferior vena cava, portopulmonary hypertension, Liver Transplantation, pulmonary arterial hypertension, acute cor pulmonale