The prognostic value of pulmonary arterial wall distensibility in children with pulmonary vascular disease – a retrospective cohort study.

Brokelman, J. (Jody) (2015) The prognostic value of pulmonary arterial wall distensibility in children with pulmonary vascular disease – a retrospective cohort study. thesis, Medicine.

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Abstract

Background Children with congenital heart disease (CHD) with an increased pulmonary blood flow, are at high risk for the development of pulmonary vascular disease (PVD). PVD is an important cause of morbidity and mortality in children with CHD. Diagnostic tools in order to monitor disease progression in these children are rare. Based on results of previous studies, pulmonary arterial wall distensibility, a measure for pulmonary artery stiffness, is expected to be related to disease course and prognosis. The aim of this study was to investigate if pulmonary arterial wall distensibility can be of prognostic value in children with CHD-associated PVD. Methods 47 patients with CHD-associated PVD, who underwent Intravascular Ultrasound (IVUS) imaging between 1994 and 1997, were included in this cohort. We obtained almost 20 years of follow-up information of these patients. Echocardiography and heart catheterizations were used as screening tools to determine whether a patient was characterised by irreversible PVD at follow-up. Survival statuses were gathered from the national civil register. Results Pulmonary arterial wall distensibility, determined by IVUS imaging, was associated with irreversible PVD and PVD-related mortality. This study demonstrated that patients with irreversible PVD were characterised by a lower distensibility compared to patients with reversed PVD (p=0.001). In a binary logistic regression model, pulmonary arterial wall distensibility was associated with irreversible PVD (p=0.0089), also after adjusting for sex and age (p=0.0052). Likewise in a Cox proportional hazards model, pulmonary arterial wall distensibility correlated with PVD-related mortality (p=0.0129; adjusted for age and sex p=0.0056). This study found a low distensibility in patients with early PVD at baseline who had irreversible PVD at follow-up. Conclusion In conclusion, this study showed that pulmonary arterial wall distensibility is a predictor for irreversible PVD and PVD-related mortality in children with CHD-associated PVD. In the early stages of the disease, a low distensibility seems to predict irreversible PVD and therefore be of additional value. For these reasons, pulmonary arterial wall distensibility might play a role in monitoring disease progression and assessing prognosis in children with PVD. To confirm this finding, further studies are needed.

Item Type:	Thesis (Thesis)
Supervisor name:	Berger, Prof. dr. R.M.F. and Ploegstra, Drs. M.
Faculty:	Medical Sciences
Date Deposited:	25 Jun 2020 10:39
Last Modified:	25 Jun 2020 10:39
URI:	https://umcg.studenttheses.ub.rug.nl/id/eprint/89

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