Right heart catheterization (RHC) remains a significant risk in pediatric patients with pulmonary arterial hypertension (PAH), particularly those with right ventricular dilation and who require sedation, according to a new study published in Pulmonary Circulation.

Li and colleagues collaborated in a retrospective single-center study that included 163 consecutive procedures from 147 pediatric patients with PAH who underwent diagnostic RHC at Beijing Anzhen Hospital in China between January 2007 and December 2020. On admission, 33 (20.2%) patients were highly symptomatic in New York Heart Association (NYHA) functional class 3 or 4). B-type natriuretic peptide (BNP) was available in 149 patients and turned out to be significantly increased (>300pg/ml) in 29.5% and mildly increased (100-300pg/ml) in 20.8%.

A total of 19 pulmonary hypertensive crises (PHC) occurred in the cardiac catheterization laboratory, 4 of them after anesthesia induction, 12 during catheter manipulation, and 2 after pulmonary angiography. Among all PHC events, 16 happened in patients who required sedation or general anesthesia during catheterization, of which 5 (3.1%) underwent cardiac compressions and 1 died (0.6%).


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“Patients with PHC were more likely to be younger (6.6±3.7 years vs 9.3±4.7years, p=0.019) and require some form of sedation (89.5% vs 59.7%, p=0.011) than those without PHC. After adjusting for age, sedation remained significantly associated with PHC (OR5.733 [95% CI 1.276-25.757], p=0.023),” the authors wrote.

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RHC plays an essential role in establishing the diagnosis of PAH and assessing the severity of hemodynamic impairment, as well as the response to medical management. This procedure most often requires general anesthesia, which reflects a risk in pediatric patients with PAH to develop PHC, cardiac arrest, and even death during RHC.

“Patients with NYHA class III-IV and elevated BNP over 500pg/ml are likely to have a greater risk during RHC and should receive PAH treatment prior to RHC with careful observation,” the authors said. “We observed a decreased incidence of PHC in patients with NYHA class III-IV over the past five years, which is likely due to the increasing use of advanced PAH therapies in this patient population.”

PAH is a progressive pulmonary vascular disease characterized by elevated mean pulmonary arterial pressure and vascular resistance, eventually leading to right heart failure and death. Children with PAH have a different distribution of etiologies compared with adults, including a greater incidence of both idiopathic and congenital heart disease PAH.

Because of accurate diagnosis and advanced therapies, the prognosis of pediatric patients has improved over the past decades. Nevertheless, children with PAH still face great challenges in early diagnosis, risk stratification, and efficient management. Additionally, given the risk surrounding RHC, comprehensive evaluation, close monitoring, and appropriate treatment before and during the procedure are essential for reducing mortality.

Reference

Li Q, Zhang C, Wang R, Keller B, Gu H. Pulmonary hypertensive crisis in children with pulmonary arterial hypertension undergoing cardiac catheterization. Pulm Circ. Published online March 26, 2022. doi:10.1002/pul2.12067