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Int J Cardiol. 2019 Oct 15;293:211-217. doi: 10.1016/j.ijcard.2019.05.021. Epub 2019 May 10.

Right ventricular-vascular coupling ratio in pediatric pulmonary arterial hypertension: A comparison between cardiac magnetic resonance and right heart catheterization measurements.

Author information

1
Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, the Netherlands. Electronic address: karelbreeman@gmail.com.
2
Department of Bioengineering, University of Colorado, United States of America.
3
Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, the Netherlands.
4
Department of Radiology, University Medical Center Groningen, University of Groningen, the Netherlands.
5
Department of Bioengineering, University of Colorado, United States of America; Department of Paediatrics, Section of Cardiology, Children's Hospital Colorado, United States of America.
6
Department of Paediatrics, Section of Cardiology, Children's Hospital Colorado, United States of America.
7
Department of Paediatrics, Section of Cardiology, Children's Hospital of Richmond of Virginia Commonwealth University, United States of America.

Abstract

BACKGROUND:

In pulmonary arterial hypertension (PAH), right ventricular (RV) failure is the main cause of mortality. Non-invasive estimation of ventricular-vascular coupling ratio (VVCR), describing contractile response to afterload, could be a valuable tool for monitoring clinical course in children with PAH. This study aimed to test two hypotheses: VVCR by cardiac magnetic resonance (VVCRCMR) correlates with conventional VVCR by right heart catheterization (VVCRRHC) and both correlate with disease severity.

METHODS AND RESULTS:

Twenty-seven patients diagnosed with idiopathic and associated PAH without post-tricuspid shunt, who underwent RHC and CMR within 17 days at two specialized centers for pediatric PAH were retrospectively studied. Clinical functional status and hemodynamic data were collected. Median age at time of MRI was 14.3 years (IQR: 11.1-16.8), median PVRi 7.6 WU × m2 (IQR: 4.1-12.2), median mPAP 40 mm Hg (IQR: 28-55) and median WHO-FC 2 (IQR: 2-3). VVCRCMR, defined as stroke volume/end-systolic volume ratio was compared to VVCRRHC by single-beat pressure method using correlation and Bland-Altman plots. VVCRCMR and VVCRRHC showed a strong correlation (r = 0.83, p < 0.001). VVCRCMR and VVCRRHC both correlated with clinical measures of disease severity (pulmonary vascular resistance index [PVRi], mean pulmonary artery pressure [mPAP], mean right atrial pressure [mRAP], and World Health Organization functional class [WHO-FC]; all p ≤ 0.02).

CONCLUSIONS:

Non-invasively measured VVCRCMR is feasible in pediatric PAH and comparable to invasively assessed VVCRRHC. Both correlate with functional and hemodynamic measures of disease severity. The role of VVCR assessed by CMR and RHC in clinical decision-making and follow-up in pediatric PAH warrants further clinical investigation.

KEYWORDS:

Cardiac magnetic resonance; Pediatric pulmonary hypertension; Right heart catheterization; Right ventricular function; Ventricular-vascular coupling ratio

PMID:
31109778
PMCID:
PMC6710117
[Available on 2020-10-15]
DOI:
10.1016/j.ijcard.2019.05.021

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