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COMPARISON OF INVASIVELY AND NONINVASIVELY MEASURED RIGHT VENTRICULAR-VASCULAR COUPLING RATIO IN PEDIATRIC PULMONARY ARTERIAL HYPERTENSION: A MULTI-CENTER STUDY

(2016) Breeman, K. (Karel)

Introduction: In pediatric pulmonary arterial hypertension (PAH), RV failure is the main cause of death. Right ventricular-vascular coupling ratio (VVCR) is gaining interest as it encloses both contractility and afterload. Conventional determination of VVCR by catheterization (VVCRs) is invasive and frequently requires anesthesia. Therefore, the goal of this study was to compare the usefulness of noninvasively determined VVCR by cardiac magnetic resonance (CMR) (VVCRm) to VVCRs in pediatric PAH.
Hypothesis: We assessed two hypotheses: 1) VVCRm is a good estimate of VVCRs; and 2) both serve as good measures of disease severity and outcome.
Methods: Retrospectively, PAH patients who had catheterization and CMR within 90 days were included from two specialized PH centers. VVCR was defined as the end-systolic elastance/effective arterial elastance ratio. VVCRm as stroke volume/end-systolic volume ratio and VVCRs by single-beat method were compared using regression analysis and Bland-Altman plots. Both were correlated to disease severity (PVRi, mRAP, CI) and adverse outcome (death, lung transplantation, atrial septostomy and intravenous medication). The area under the receiver operating characteristic curve (AU-ROC), Kaplan-Meier curves and hazard ratios (HR) from Cox regression determined their value in predicting adverse outcome.
Results: In the 31 patients included (17 from CHC and 14 from UMCG), median age was 14 years (0.3 – 23) and median PVRi was 7.6 WU × m2 (2.1 – 32). VVCRm and VVCRs were strongly correlated (r = 0.78, p < 0.001) with a mean difference of 0.2 and 95% of the differences between -0.3 and 0.7. Both had comparable significant correlations with disease severity and adverse outcome. Also, both VVCRm and VVCRs were shown to be of good prognostic value with AU-ROCs of respectively 0.84 and 0.90 and HRs (95%-CI) of 0.82 (0.70 – 0.96) and 0.69 (0.53 – 0.90).
Conclusion: In line with previous research, the results of this study indicate that VVCRm and VVCRs are comparable in pediatric PAH and are both good predictors of outcome. The characteristic disease progression is clearly seen in VVCRm, but its clinical use has to be defined by further research.





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