Sildenafil Therapy for Pulmonary Hypertension Prior to Trial Closure of ASD with Severe Pulmonary Hypertension
Objective:
To assess the symptoms and ability of patients with Atrial Septal Defect (ASD) and severe pulmonary hypertension (PHT) to undergo closure after oral Sildenafil.
Method:
Patients more than 16 years of age with ASD and severe PHT (mean PA:Ao ratio > 0.5 and pulmonary vascular resistance (PVR) > 3 woods units) were enrolled from January 2005 to April 2007. All were given oral Sildenafil (100-150 mg daily) for at least 6 months. The NYHA functional class, 6 minute walk, cardiopulmonary test and cardiac catheterization were done at baseline and at 6 months follow-up. Patients with PVR < 8 woods unit were subjected to closure of the ASD either by trial closure with device (initial part of study) or surgical closure with a fenestrated patch. Patients were followed up with echocardiogram, exercise test and repeat cardiac catheterization.
Results:
12 patients with mean age of 31.6 years were enrolled. The mean PA/Ao pressure was 0.56 and PVR 9 woods units. 5 patients underwent closure of the ASD (mean PA: Ao ratio 0.56, PVR 7.3). 4 out of 5 are currently well at a mean of 27 months post procedure with a mean estimated PA pressure of 24 mmHg. One patient died secondary to accelerated pulmonary vascular disease a year following closure. All patients had improvement in symptoms, 6 minute walk test and cardiopulmonary test.
Conclusion:
Oral Sildenafil improved functional class status in patients with severe PHT. It had shown potential use as treatment prior to closure of ASD.