Abstract

Methods This retrospective study included subjects with PAH who a cardiac magnetic resonance (CMR) study within 14 days of cardiac catheterization between January 2009-August 2013. The effective elastance (Ea, index of arterial load) and right ventricular maximal end-systolic elastance (Emax, index of contractility) were determined by a combination of CMR and hemodynamic data. Ea is defined as (mean pulmonary arterial pressure minus pulmonary capillary wedge pressure)/stroke volume. Emax is defined as mean pulmonary arterial pressure/ end systolic volume. Ea/Emax ratio was derived. Additionally, a measure of non-invasive ventricular arterial coupling (assuming PWCP is insignificant, making Ea/ Emax = end systolic volume/stroke volume) was derived from only CMR. Pulmonary vascular resistance indexed (PVRi) and pulmonary vascular reactivity, as defined by Barst criteria (decrease in mean pulmonary artery pressure of > 20%, unchanged/increased cardiac index, and decreased/unchanged pulmonary to systemic vascular resistance ratio), were also determined. Pearson correlation coefficients were calculated between PVRi and Ea, Emax, and Ea/Emax. Receiving operating characteristic (ROC) curve analysis determined the diagnostic value of Ea/Emax in predicting vascular reactivity.

Highlights

  • Pulmonary arterial hypertension (PAH) remains a disease with high morbidity/mortality in pediatrics

  • Emax is defined as mean pulmonary arterial pressure/ end systolic volume

  • 1Pediatric Cardiology, Children’s Hospital Colorado, Aurora, Colorado, USA Full list of author information is available at the end of the article (ROC) curve analysis determined the diagnostic value of Ea/Emax in predicting vascular reactivity

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Summary

Background

Pulmonary arterial hypertension (PAH) remains a disease with high morbidity/mortality in pediatrics. Understanding ventricular-arterial coupling, a measure of how well matched the ventricular and vascular function is, may elucidate the pathway leading to right heart failure

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