The introduction of prostanoid therapy has revolutionized the treatment of pulmonary arterial hypertension (PAH). However, continuous intravenous prostacyclin infusion poses significant risks and challenges, particularly in children. Inhaled treprostinil has been shown to be safe and efficacious in adults. This study describes the safety and efficacy of inhaled treprostinil in children with PAH. A retrospective analysis of 29 children, treated with inhaled treprostinil for ≥ 6 weeks was performed. Effects of inhaled treprostinil on exercise capacity, functional class, echocardiographic, and hemodynamic data were evaluated. Adverse events were documented. Patients received 3 - 9 breaths (6ucg/breath) of inhaled treprostinil 4 times daily. All were receiving background PH therapy; 12 had previously received parenteral prostanoid. Inhaled treprostinil was discontinued in 4 patients because of symptoms including cough and bronchospasm (3) and progression of PAH (1). Mild side effects including cough (9), sore throat(6) did not require discontinuation of therapy. The WHO functional class improved in 19 and was unchanged in 10; exercise capacity significantly improved, with the six minute walk distance (6MWD) improving on follow-up from 455.7+/-71.5 to 498+/-70 meters, (p=0.01) and peak oxygen consumption (pVO2) increasing from 25.5+/-10.2 to 27.4+/-10 (p=0.04). In conclusion, inhaled treprostinil was associated with improvement in exercise capacity and WHO functional class when added to background targeted PAH therapy in children, and had an acceptable safety profile. Based on these early data, further study of inhaled treprostinil appears warranted in pediatric PAH patients.

Cardiac catheterization is important for the management of patients with pulmonary arterial hypertension (PAH). It is used for diagnosis, assessment, and monitoring of PAH patients, as well as to perform interventions such as balloon atrial septostomy and coil embolization of collateral vessels. Although reports on the risks of catheterization in PAH patients are scarce, many centers hesitate to perform these procedures in such fragile patients. We performed a retrospective chart review of all cardiac catheterizations performed in PAH patients over 10 years at our pulmonary hypertension center. Demographic, hemodynamic, and outcome data were collected. Complication rates were determined, and multivariate proportional hazards modeling was performed to identify predictors of catheterization-related complications. There were 1,637 catheterizations performed in 607 patients over 10 years. Pediatric patients accounted for 50% of these cases, 48% were performed in patients with idiopathic PAH, and 49% were performed under general anesthesia. While the overall complication rate was 5.7%, the rate of major complications was only 1.2% (n = 20). Although there were 8 deaths during the admission following catheterization, only 4 of these were related to the procedure, yielding a catheterization-related mortality of 0.2%. In conclusion, when performed at a pulmonary hypertension center with expertise in the care of PAH patients, cardiac catheterization is associated with low complication rates and mortality, and it should remain an important tool in the management of these patients.

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