Significant improvement of portopulmonary hypertension after 1-week terlipressin treatment

Sildenafil treatment for portopulmonary hypertension.

Portopulmonary hypertension (POPH) is regarded as a subtype of pulmonary arterial hypertension (PAH); however, established PAH therapies have not been evaluated for this condition. The current authors treated 14 patients (four male, 10 female; mean (range) age 55 (39-75) yrs) with moderate (n = 1) or severe (n = 13) POPH caused by alcoholic liver disease (n = 7), chronic viral hepatitis (n = 3)...

Portopulmonary hypertension *

Venous outflow obstruction and portopulmonary hypertension after orthotopic liver transplantation

PATIENT Female, 54 FINAL DIAGNOSIS: Suprahepatic inferior vena cava anastomosis stricture Symptoms: Ascites • fatigue • lower limb edema • hepatomegaly MEDICATION - Clinical Procedure: - Specialty: Transplantology • Critical Care Medicine. OBJECTIVE Unusual clinical course. BACKGROUND Suprahepatic inferior vena cava anastomosis stricture is an unusual vascular complication after orthotopi...

Aggravation of Hepatopulmonary Syndrome after Sildenafil Treatment in a Patient with Coexisting Portopulmonary Hypertension

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are complications of portal hypertension and cirrhosis. Their pathophysiological mechanisms clearly differ. HPS is characterized by a defect in arterial oxygenation induced by pulmonary vascular dilatation. In contrast, PPHTN is predominantly due to excessive pulmonary vasoconstriction and vascular remodeling, but is rarely ...

Bosentan therapy for portopulmonary hypertension.

The dual endothelin receptor antagonist bosentan has been approved in several countries for pulmonary arterial hypertension, and patients with portopulmonary hypertension (PPHTN) have not specifically been excluded. However, no data have been published on the efficacy and safety of bosentan in this patient population. Here, the first clinical experiences with bosentan in patients with Child A c...