Cardiac Magnetic Resonance Evaluation in Paediatric Pulmonary Arterial Hypertension :Correlations with disease severity and prognosis

Wigger, J.F.A. (2015) Cardiac Magnetic Resonance Evaluation in Paediatric Pulmonary Arterial Hypertension :Correlations with disease severity and prognosis. thesis, Medicine.

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Abstract

Background: Pulmonary arterial hypertension (PAH) is a progressive disease characterized by elevated arterial pressure in the pulmonary circulation. The main clinical manifestations are the result of cardiac failure. Recently, cardiac magnetic resonance (CMR) derived variables have been associated with disease severity and prognosis in adults with PAH. Whether CMR derived variables are valuable in paediatric PAH, is insufficiently studied. The main purpose of this study was to evaluate the value of CMR in the assessment of disease severity and prognosis in paediatric PAH. Methods: This retrospective cohort study included all children diagnosed with PAH, that were followed in the Dutch referral centre for paediatric PAH, and who received a CMR. Disease severity was defined using the following variables: World Health Organization-functional class (WHO-FC), NTproBNP, 6-minute walk test and pulmonary vascular resistance, indexed for body surface area, (PVRi). Variables for disease severity were obtained within six months of the CMR. CMR derived measurements included ventricular volumetry, eccentricity index and mass, pulmonary arterial characteristics (e.g. distensibility and elastic modulus) and right ventricular (RV) arterial coupling. These CMR measurements were correlated with disease severity and also with clinical outcome, which was defined as transplant-free survival. Results: The current study included 35 CMR studies in 22 patients. Their mean age was 13.5 ±2.4 years and 36% were male. Mean pulmonary arterial pressure was 54±20 mmHg. RV ejection fraction (RVEF) and RV-arterial coupling correlated significantly with disease severity (RVEF with WHO-FC (r= -0.491), NTproBNP (r= -0.747) and PVRi (r= -0.650), respectively; for RV arterial coupling (r= -0.491, r= -0.747 and r= -0.687, respectively). The eccentricity index correlated with NTproBNP and PVRi (r=0.527, r=0.455). Pulmonary artery distensibility and elastic modulus correlated with both NTproBNP (r=-0.574 and r=0.574) and PVRi (r=-0.694 and r=0.694, respectively). Only RVEF and RV-arterial coupling were significantly correlated with transplant-free survival (p=0.046 and p=0.016, respectively). The correlation between eccentricity index and outcome only just failed to reach statistical significance (p= 0.055). Conclusion: This study demonstrated strong correlations between CMR derived measurements and disease severity in paediatric PAH. In addition, two non-invasive CMR derived measurements appeared predictors of outcome in paediatric PAH. These findings indicate that CMR is a non-invasive tool that may be of value in management decisions in children with PAH and has the potential to substitute invasive cardiac catheterizations during the follow-up of paediatric PAH patients.

Item Type:	Thesis (Thesis)
Supervisor name:	1st supervisor: and Berger, R.M.F. MD and 2nd supervisor: and Gorter, T.M. MD and Research institution: University Medical Centre Groningen (U and Research department: Department of Paediatric Cardiology
Faculty:	Medical Sciences
Date Deposited:	25 Jun 2020 10:57
Last Modified:	25 Jun 2020 10:57
URI:	https://umcg.studenttheses.ub.rug.nl/id/eprint/1727

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