Clinical InvestigationEchocardiography in ChildrenRight Atrial Conduit Phase Emptying Predicts Risk of Adverse Events in Pediatric Pulmonary Arterial Hypertension
Section snippets
Study Population
This study was a single-center, prospective cohort analysis of consecutive patients with known idiopathic or heritable PAH seen in the pulmonary hypertension clinic at Children's Hospital Colorado. Physicians specializing in pulmonary hypertension followed each patient clinically and confirmed the diagnosis. Subjects were enrolled at the time of their first clinical echocardiogram during the study period from January 2014 to March 2019. We did not exclude subjects based on date of diagnosis or
Study Population
Forty-two consecutive pediatric PAH patients with idiopathic or heritable pulmonary hypertension were prospectively enrolled (median age, 11.9 years; IQR, 7.8-15.6 years). No subject had a history of congenital heart disease. One study candidate was retrospectively excluded due to lack of follow-up data. Forty subjects had either no or a small interatrial communication, while one had an iatrogenic moderate-size atrial-level shunt at the time of enrollment. Thirty-six subjects had either trivial
Discussion
In this prospective study we found that (1) children with idiopathic and heritable PAH had decreased RA conduit emptying compared to age- and sex-matched controls; (2) RA cF% had a significant negative correlation with PAH severity by catheterization; (3) PAH subjects with an adverse clinical event had decreased RA cF%, larger RAA, and higher right heart pressures by catheterization compared to those without an event; and (4) decreased RA cF% and increased RVSP% were most prognostic for
Conclusion
Right atrial conduit phase emptying, as measured by noninvasive echocardiography, is significantly altered in pediatric PAH. We report the finding that PAH patients with benign clinical course have a mild decrease in conduit emptying compared with controls, while those with a severe course have worse conduit emptying. In addition, RA cF% correlates with invasive markers of disease severity and is highly prognostic for risk of a clinical adverse event within the pediatric PAH population.
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Cited by (9)
Low Pulmonary Artery Pulsatility Index by Echocardiography Is Associated With Increased Mortality in Pulmonary Hypertension
2023, Journal of the American Society of EchocardiographyCitation Excerpt :However, it is not the PH per se that conveys the risk but rather the inability of the RV to appropriately adapt to the elevated afterload and hence predispose to right heart failure.3-11,16 Thus, the key factors that are best associated with outcomes in patients with PH are those that characterize the state of the right heart.3-11 It has long been recognized that the factors on right heart catheterization that are most strongly associated with outcome in PH patients are not the degree of pulmonary pressure elevation but rather measures of right heart function or integrative measures that reflect RV adaptation to load, such as pulmonary vascular resistance and PA compliance.3,4,10,11,16-18
The Prognostic Value of Right Atrial Strain Imaging in Patients with Precapillary Pulmonary Hypertension
2021, Journal of the American Society of EchocardiographyLongitudinal assessment of right atrial conduit fraction provides additional insight to predict adverse events in pediatric pulmonary hypertension
2021, International Journal of CardiologyCitation Excerpt :In patients with diastolic dysfunction, impaired active ventricular relaxation leads to increased reliance on atrial contraction during late diastole to maintain RV preload [10]. Our findings support prior reports from prospective and retrospective cohorts in children and adults showing the value of RA cF% to predict adverse clinical events in the PAH population [6]. Although cross-sectional studies have provided evidence for the prognostic value of a variety of imaging and functional biomarkers in children with PAH, longitudinal studies to validate these metrics as potential treatment targets are limited [1,11]. [
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Conflicts of Interest: The authors have no conflicts of interest to disclose.