Abstract
BACKGROUND: Right ventricular (RV) function is a major contributor to the outcome of pulmonary arterial hypertension (PAH). Adult studies demonstrated that regional and global changes in RV deformation are prognostic in PAH using 3-dimensional echocardiography (3DE). However, regional and global dynamic changes in RV mechanics have not been described in pediatric PAH. We compared 3DE RV regional and global deformation between pediatric patients who had associated PAH with congenital heart disease (APAH-CHD), pediatric patients who had idiopathic PAH (IPAH), and normal controls, and evaluated the clinical outcomes. METHODS: A total of 48 controls, 47 patients with APAH-CHD, and 45 patients with IPAH were evaluated. 3DE RV sequences were analyzed and post-processed to extract global and regional deformation (circumferential, longitudinal, and area strain). Statistical analyses compared the sub-groups on the basis of global and regional deformation, and outcome analysis was performed. RESULTS: Patients with PAH had significantl8y different global and regional deformation (p < 0.001) compared with controls. Patients with APAH-CHD and and those with IPAH significantly differed in global circumferential strain (p < 0.010), area strain (inlet septum, p = 0.041), and circumferential strain at the inlet septum (p < 0.019), apex free wall (p < 0.004), and inlet free wall (p < 0.004). Circumferential strain at the inlet free wall and circumferential, longitudinal, and area strain at the apex free wall were predictors of adverse events. CONCLUSIONS: RV regional and global strain differ between controls and pediatric patients with PAH. RV apical free-wall area strain provides insight into the mechanism of RV dysfunction in pediatric patients with PAH, with regional strain emerging as outcome predictors, suggesting that this novel measure may be considered as a future measure of RV function.
Original language | English (US) |
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Pages (from-to) | 138-148 |
Number of pages | 11 |
Journal | Journal of Heart and Lung Transplantation |
Volume | 40 |
Issue number | 2 |
DOIs | |
State | Published - Feb 2021 |
Funding
This study is supported by the Jayden DeLuca Foundation , the Leah Bult Foundation , and the Frederick and Margaret L Weyerhaeuser Foundation . This study is also supported by the National Institutes of Health / National Center for Advancing Translational Sciences Colorado Clinical and Translational Science Awards Grant Number UL1 TR002535 .
Keywords
- 3-dimensional echocardiography
- clinical outcomes
- pediatric pulmonary hypertension
- prognosis
- right ventricular area strain
- ventricular function
ASJC Scopus subject areas
- Surgery
- Pulmonary and Respiratory Medicine
- Cardiology and Cardiovascular Medicine
- Transplantation