Coenraad, I. (Iris) (2019) Right ventricular parameters assessed with cardiac magnetic resonance are of prognostic value in pediatric pulmonary arterial hypertension. thesis, Medicine.
Full text available on request.Abstract
Background Pulmonary arterial hypertension (PAH) is a rare progressive and often lethal disease of the pulmonary vasculature due to pathologic remodeling in the pulmonary artery leading to right ventricle failure and death. Limited literature is available about managing PAH in children. Cardio magnetic resonance (CMR) is a non-invasive technique for assessing characteristics of both ventricles and pulmonary arteries. Research in adult patients with PAH has shown promising results on the predictive value of this technique on survival. The goal of this study is to investigate the prognostic value of CMR derived variables in children with PAH. Methods This observational longitudinal cohort study with retrospective analysis used data of the National Referral Center for pediatric pulmonary hypertension in the University Medical Center Groningen. Children with PAH who underwent at least one CMR were included. Quantification of volumes and function of both ventricles were obtained using the CMR images. Also eccentricity index (EI), ventricular vascular coupling ratio (VVCR) and variables indicating stiffness of the pulmonary artery were obtained. Correlations between these variables and clinical disease severity variables in PAH were analyzed using Spearman Correlation test. Associations with transplant-free survival were analyzed using cox-regression. Results A total of 38 CMR in 38 patients with PAH were included. Median (interquartile range) age at CMR was 13.0 years (10.8-15.0) and most of the patients were female (66%). The majority of the CMR derived variables correlated with disease severity, especially with NT-proBNP. Right ventricle ejection fraction (RVEF) was significantly associated with transplant-free survival (p = 0.023), as were right ventricle end-systolic volume index (RVESVi) (p = 0.039), right ventricle mass index (RVMi) (p = 0.003), EI (p = 0.001) and VVCR (p = 0.029). Conclusions In this study specific CMR derived variables have been revealed to be associated with transplant-free survival in children with PAH. These results confirm the utility in childhood of CMR derived variables in managing pediatric PAH and should be studied further as potential treatment target.
| Item Type: | Thesis (Thesis) |
|---|---|
| Supervisor name: | Faculty Supervisor: and Berger, R.M.F. and Supervisor: and Haarman, M.G. and Department of Pediatric Cardiology, Beatrix Children's Hosp |
| Faculty: | Medical Sciences |
| Date Deposited: | 25 Jun 2020 10:48 |
| Last Modified: | 25 Jun 2020 10:48 |
| URI: | https://umcg.studenttheses.ub.rug.nl/id/eprint/976 |
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