Authors of a new study have developed a simple echocardiographic risk stratification system for pediatric patients with pulmonary arterial hypertension (PAH).

The study, published in the Journal of the American Heart Association, found that right atrial area, left ventricular eccentricity index, and tricuspid annular plane systolic excursion (TAPSE) were particularly predictive of prognosis in these children.

The research team conducted a retrospective study on 63 pediatric patients with PAH and biventricular cardiac anatomy who underwent standardized transthoracic echocardiographic testing at a single center in London between 2005 and 2013. The patients had no shunt lesions.

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“While various noninvasive assessment tools exist, transthoracic echocardiography remains the most important modality in the routine regular assessment of patients, including 6‐minute walk test distance and natriuretic peptide levels,” the authors wrote. “The aim of the current study was . . . to evaluate the value of echocardiographic parameters for estimating transplant‐free survival and mortality in children with PAH managed in this specialized, centralized service.”

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The children were followed from echocardiography until either death or lung transplantation, with the aim of correlating echocardiographic parameters with clinical outcomes.

The results suggest that PAH has multiple effects on the heart, which can then inform the prognosis. Specifically, the combination of right atrial size and left ventricular eccentricity index can be a strong prognostic marker of transplant-free survival in young patients. In addition, TAPSE, which is a measure of longitudinal right ventricular function, complements these parameters by predicting mortality alone.

The authors caution that the current study was focused on echocardiographic markers rather than additional data related to hemodynamics, such as pulmonary vascular resistance, which could also inform the prognosis. They recommend further multicenter studies incorporating these parameters, as well as demographic, functional, and imaging data to create a more comprehensive pediatric PAH risk score.


Lammers AE, Marek J, Diller GP, Haworth SG, Moledina S. Prognostic value of transthoracic echocardiography in children with pulmonary arterial hypertension. J Am Heart Assoc. 2023;12(6):e023118. doi:10.1161/JAHA.121.023118