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.
This is a case series of three patients with PPHTN treated with off-label Sildenafil. 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.
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.
Sildenafil is a safe and effective pulmonary vasodilator for patients with PPHTN.
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.
Patient 1Patient 2Patient 3Duration of sildenafil therapy9 months7 months15 monthsDose of sildenafil50mg QID50 mg TID50 mg TIDPrePostPrePostPrePostPASP (mm)968594579085PADP (mm)402852244033mPAP (mm)605066386051mRAP (mm)203290235CO (L/min)2.07.72.74.53.54.0CI (L/min)22.214.171.124.21.82.2PCWP (mm)167N/A*3N/A*7PVR (dynes/sec/cm5)1760447N/C622N/C880PaO2 sat%3467477358686MWD (ft)375875ND#13502501500NYHA Class434141Hemoglobin (g/dL)1110.416.11712.514.6Platelets (103 /mcl)244972953948
PASP = pulmonary arterial systolic pressure, PADP = pulmonary arterial diastolic pressure, mPAP = mean pulmonary arterial pressure, mRAP = mean right atrial pressure, CO = cardiac output, CI = cardiac index, PCWP = pulmonary capillary wedge pressure, 6MWD = six minute walk distance, NYHA = New York Heart Association,Patient 1Patient 2Patient 3Age565953GenderFMMEtiology of cirrhosiscryptogenicalcoholHepatitis C, alcoholChild-Pugh classBBBComplicationsEV,GV, portal vein thrombosis, thrombocytopeniaEV, thrombocytopeniaEncephalopathy, thrombocytopeniaSymptoms at presentationProgressive breathlessness, lower extremity edema, syncopeProgressive breathlessness, syncopeExertional breathlessness, syncope
EV = esophageal varices, GV = gastric varices
Shiraz Daud, None.