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Outcomes in children with idiopathic pulmonary arterial hypertension
Delphine Yung1, Allison C Widlitz, Erika Berman Rosenzweig
1Department of Pediatrics, Columbia University College of Physicians & Surgeons, New York, NY 10032, USA.
Insights
Pediatric idiopathic pulmonary arterial hypertension survival improved with calcium channel blockers (CCB) and epoprostenol. Early treatment success is high, but declines over time, suggesting earlier transplant evaluation is beneficial.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Medical Research
Background:
- Idiopathic pulmonary arterial hypertension (IPAH) in children requires tailored treatment strategies.
- Current treatments include calcium channel blockade (CCB) for acute responders and epoprostenol for nonresponders.
Purpose of the Study:
- To identify factors influencing survival and treatment success in pediatric IPAH patients.
- To evaluate the long-term efficacy of CCB and epoprostenol in this population.
Main Methods:
- Retrospective analysis of 77 children diagnosed with IPAH between 1982 and 1995.
- Follow-up data collected through 2002.
- Analysis of treatment outcomes based on vasodilator response (CCB vs. epoprostenol) and a "recent medical era" subset.
Main Results:
- Survival rates at 1, 5, and 10 years were high for both CCB (97%, 97%, 81%) and epoprostenol (94%, 81%, 61%) treated groups.
- Treatment success rates declined significantly over time, particularly after 5 years.
- In the recent medical era (n=44), 10-year survival was 78% and treatment success was 60%, with age at diagnosis predicting success.
Conclusions:
- Both CCB and epoprostenol have improved survival in pediatric IPAH.
- Treatment success diminishes over time, highlighting the need for proactive management.
- Transplant evaluation should be considered earlier in the treatment course to prevent failure.
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.
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