Objective: Childhood pulmonary hypertension is associated with varied aetiologies and outcomes. Existing therapies are largely based on studies of adult patients with idiopathic pulmonary arterial hypertension (IPAH). We performed a retrospective review of demographic characteristics and outcomes of all patients referred for ongoing long-term management of PAH to a single, statewide paediatric service.
Methods: Records of 90 patients with PAH who were managed treated between July 1998 and July 2008 were reviewed. Survival analysis was undertaken in patients who fulfilled the following criteria: age > 3 months of age at referral, PAH persisting for at least 6 months after surgery for congenital heart disease (CHD), treatment with a PAH-specific therapy for >14 days, and a mean PA pressure >25mmHg or a TR velocity >3.5m/s. Patients were grouped into one of 4 principal diagnostic categories: IPAH, CHD, chronic lung disease (CLD) and repaired diaphragmatic hernia.
Results: IPAH was the principal diagnosis in 17 of 90 patients, CLD in 14, CHD in 43, and repaired diaphragmatic hernia in 8. The accompanying Table shows freedom from death or lung transplantation at specified time intervals after diagnosis for each group.
Conclusions: The majority of children referred for management of PAG have associated heart or lung disease. The period of greatest risk for children with CLD and repaired diaphragmatic hernia is <12 months after diagnosis, whereas the risk for children with IPAH is ongoing.