Key result
Idiopathic pulmonary arterial hypertension was associated with a significantly increased risk of pulmonary hypertensive crisis during right heart catheterization compared to congenital heart disease-associated PAH (OR 14.02).
Why the study?
The clinical characteristics, hemodynamics, and adverse events of pediatric PAH patients undergoing right heart catheterization were not well defined.
What is the rate of adverse events during right heart catheterization in pediatric patients with pulmonary arterial hypertension?
Cohort (n=591)
No
What is the rate of adverse events during right heart catheterization in pediatric patients with pulmonary arterial hypertension?
Odds Ratio: 14.02 (95% CI 4.49–43.85)
Absolute Event Rate: 11.5% vs 0.6%
p-value: p=<0.001
Right heart catheterization is generally low-risk in pediatric PAH, but pulmonary hypertensive crisis occurs in approximately 3% of procedures, with significantly higher risk in idiopathic PAH.
Supports heightened monitoring in pediatric idiopathic PAH during catheterization; leaves open whether targeted strategies reduce crises in prospective studies.
Aim: To define the clinical characteristics, hemodynamics, and adverse events for pediatric patients with pulmonary arterial hypertension (PAH) undergoing right heart catheterization (RHC). Methods: The large referral single center data of 591 diagnostic RHC procedures performed between 2005 and 2020 on pediatric PAH patients was retrospectively collected and analyzed. Results: A total of 591 RHC procedures performed on 469 patients with congenital heart disease (CHD)-PAH (median age 8.8 years, 7.9% New York Heart Association (NYHA) class > II, 1.5% with syncope) and 122 patients with idiopathic PAH (median age of 9.0 years, 27.0% NYHA class > II, 27.0% with syncope) were included. Of those, 373 (63.1%) procedures were performed under general anesthesia. Eighteen patients (18/591, 3.0%) suffered adverse events (mainly pulmonary hypertensive crisis, PHC, n = 17) during the RHC procedure, including 14 idiopathic pulmonary arterial hypertension (IPAH) patients and 4 CHD-PAH patients, and one IPAH patient died in hospital 63 hours after RHC. The risk of developing PHC was significantly increased in patients with IPAH (OR = 14.02, 95%CI: 4.49−43.85, p < 0.001), atrial blood gas pH < 7.35 (OR = 12.504, 95%CI: 3.545−44.102, p < 0.001) and RAP > 14 mmHg (OR = 10.636, 95%CI: 3.668−30.847, p < 0.001). Conclusions: RHC is generally a low-risk procedure in pediatric patients with PAH. However, PHC occur in approximately 3% of patients. Therefore, RHC should be performed in a large, experienced referral pediatric cardiology center, especially in pediatric patients with IPAH requiring general anesthesia.
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Xu et al. (2022) conducted a cohort in Pediatric Pulmonary Arterial Hypertension (n=591). Idiopathic Pulmonary Arterial Hypertension (IPAH) vs. Congenital heart disease-associated PAH (CHD-PAH) was evaluated on Pulmonary hypertensive crisis (PHC) during right heart catheterization (OR 14.02, 95% CI 4.49-43.85, p=<0.001). Idiopathic pulmonary arterial hypertension was associated with a significantly increased risk of pulmonary hypertensive crisis during right heart catheterization compared to congenital heart disease-associated PAH (OR 14.02).
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