Pulmonary arterial hypertension (PAH) is a devastating disease. The natural progression of the disease is characterized by sustained increase in pulmonary artery pressure resulting in right ventricular failure(RHF). The mortality in acutely decompenated right heart failure patients is high. Significant progress has been made in the treatment of patients with PAH with improved survival. However management of acutely decompensated patients remains a challenge. The management usually entails intravenous diuretics which may not be tolerated because of systemic hypotension and renal insufficiency, IV epoprostenol which is not easy to prescribe at short notice.
Case series of 7 patients with newly diagnosed severe pulmonary arterial hypertension and decompensated right heart failure.All patients had evidence of RHF and acute respiratory failure and failed to improve with diuresis and were treated with oral sildenafil starting at 12.mg and then increased to 25mg every 8 hrs and 5omg every eight hours as tolerated.
All 7 patients had severe PAH with mean pulmonary artery pressure was 61 + 15.6 mmHg (mean + SD) and Pulmonary vascular resistance 13 + 5.4 woods units CI 2 + .63 L /min/sq meter.All patients demonstrated dramatic improvement with Sildenafil.(table 1)There were no adverse events.
We conclude that sildenafil is safe and effective in decompensated right heart failure.
Sildenafil should be considered in these critically ill patients .
Namita Sood, None.PatientmRAmPACIPVROutcome110471.716Extubated and off all pressors 72 hrs later2115838.7Resolution RHF discharged 72 hrs later312411.2212.4PaO2 58mm Hg on 100% NRB to off O2 72 hrs later426752.49.5Resolved RHF and improved saturations with activity discharged 72 hrs later57862.321PaO2 70 mmHg on 100%NRB to PaO2 64mmHg on 6L NC620651.58Resolution of right heart failure discharged 72hrs later719552.520PaO2 80mm Hg on 100% NRB to 88mmHg on 6L 72hr later