PURPOSE: Pulmonary arterial hypertension (PAH) in the setting of portal hypertension, or Porto-pulmonary Hypertension (PPHTN), has an estimated prevalence of 3.5% to 12.5% in patients referred for liver transplantation. Complications of cirrhosis such as encephalopathy, high cardiac output state, and thrombocytopenia have made treatment with prostanoids challenging. Furthermore, Bosentan is contraindicated in chronic liver disease and severe pulmonary hypertension is a contraindication to liver transplantation. We report our experience with oral Sildenafil monotherapy in PPHTN.
METHODS: This is a case series of three patients with PPHTN treated with off-label Sildenafll. Secondary causes of pulmonary hypertension were excluded. Laboratory and hemodynamic data, New York Heart Association functional class, and six-minute walk distance (6MWD) were obtained at baseline and after treatment for an extended period with Sildenafil.
RESULTS: All three patients had improvements in their hemodynamics and 6MWD. Functional improvement was evident in all three patients. During the 31 patient-months of follow-up, there were no directly attributable side effects or complications of Sildenafil.
CONCLUSION: Sildenafil is a safe and effective pulmonary vasodilator for patients with PPHTN.
CLINICAL IMPLICATIONS: Because conventional PAH therapies are potentially problematic in the setting of cirrhosis, sildenafil should be considered as a chronic therapeutic choice in PPHTN and may serve as a "bridge" to liver transplantation.
DISCLOSURE: Shiraz Daud, None.
Shiraz A. Daud MD * Murali M. Chakinala MD Washington University in Saint Louis, St Louis, MO
COPYRIGHT 2005 American College of Chest Physicians
COPYRIGHT 2005 Gale Group
Home
A
Arthritis
