Combination Therapy of Prostacyclin for Pulmonary Hypertension in Congenital Heart Disease
Kritvikrom Durongpisitkul MD*, Decho Jakrapanichakul MD**, Duangmanee Laohaprasitiporn MD*, Jarupim Soongswang MD*, Prakul Chanthong MD*, Apichart Nana MD*
Affiliation : *Department of Pediatrics, ** Department of Internal Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
Background : Pulmonary arterial hypertension (PAH) is a recognized complication of congenital heart
disease. Despite differences in etiology and pathophysiology, successful therapy for idiopathic PAH may
benefit in patients with congenital heart disease. We theorized that combination of oral and aerosolization
prostacyclin will benefit this group of patients in long term.
Materials and Methods : The study design was single group and open label study with intention to treat for
patients with congenital heart disease with pulmonary artery (PA pressure) more than 50% of systemic
pressure. All patients were given a combination of orally given beraprost sodium and inhalation of iloprost
for 12 months. Data were collected prospectively consisting of functional class, O2 saturation, 6-minute walk
test and right ventricular systolic pressure (RVSP).
Results : There were 23 patients with an average right ventricular systolic pressure (+ SD) of 94.8 + 14.5
mmHg and with average age of 27.8 + 14.9 years (2.5 to 50 years). The average oxygen saturation
was 87.9 + 7 %. There were 12 patients with post surgical repair or cardiac catheterization interventional
procedure and 11 with and Eisenmengerûs syndrome. There were significant improvement of 6-minute-walk
test from an average of 268 + 70 meters to 308 + 57 meters at the end of 12 months. The functional class
of patients was also improving. However, there were no significant different in oxygen saturation.
Conclusion : Combination therapy of oral and inhalation of aerosolized vasodilators is a fascinating concept
in the therapy of pulmonary hypertension. Treated patients showed an improvement in exercise capacity and
right ventricular systolic pressure without a worsening in oxygen saturation.
Keywords : Pulmonary hypertension, Congenital heart disease
