Calcium channel blockade and epoprostenol for pediatric idiopathic pulmonary arterial hypertension resulted in 10-year survival rates of 81% (acute responders) and 61% (nonresponders), respectively.
Cohort (n=77)
What are the long-term survival and treatment success rates for children with idiopathic pulmonary arterial hypertension treated with calcium channel blockers or epoprostenol?
Survival for children with idiopathic pulmonary arterial hypertension has improved with CCB and epoprostenol, though decreases in survival after 5 years suggest a need for early transplant evaluation.
BACKGROUND: Treatment for idiopathic pulmonary arterial hypertension in children includes calcium channel blockade (CCB) for acute responders with vasodilator testing and chronic epoprostenol for nonresponders. We sought to determine parameters associated with survival and treatment success. METHODS AND RESULTS: A previously identified cohort of 77 children diagnosed between 1982 and 1995 with idiopathic pulmonary arterial hypertension was followed up through 2002. For acute responders treated with CCB (n=31), survival at 1, 5, and 10 years was 97%, 97%, and 81%, respectively; treatment success was 84%, 68%, and 47%, respectively. Survival for all children treated with epoprostenol (n=35) at 1, 5, and 10 years was 94%, 81%, and 61%, respectively; treatment success was 83%, 57%, and 37%, respectively. Because of the inconsistent availability of epoprostenol before 1995, we defined a "recent medical era" subset by excluding children from the total 77 patient cohort for whom epoprostenol was recommended but was unavailable. Survival in the recent medical era (n=44) at 1, 5, and 10 years was 97%, 97%, and 78%; treatment success was 93%, 86%, and 60%, respectively. Treatment success on CCB decreased significantly when acute responders became nonresponders. Age at diagnosis predicted treatment success in the recent medical era. CONCLUSIONS: Survival for children with idiopathic pulmonary arterial hypertension has significantly improved with CCB and epoprostenol. Children who are acute responders are treated with CCB; they are treated with epoprostenol if they become nonresponders. The decrease in survival and in treatment success after 5 years in all children supports the role for transplant evaluation before treatment failure.
Yung et al. (Tue,) conducted a cohort in Idiopathic pulmonary arterial hypertension (n=77). Calcium channel blockade (CCB) and epoprostenol was evaluated on Survival and treatment success. Calcium channel blockade and epoprostenol for pediatric idiopathic pulmonary arterial hypertension resulted in 10-year survival rates of 81% (acute responders) and 61% (nonresponders), respectively.