Coarctation of the aorta: management in a non-surgical centre and evaluation of aortic stiffness & cardiac function

Rafiq, Isma (2021) Coarctation of the aorta: management in a non-surgical centre and evaluation of aortic stiffness & cardiac function. Doctoral thesis, University of East Anglia.

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Abstract

Background: Adult Congenital Heart Disease (ACHD) is growing rapidly as patients with corrected congenital heart disease are living longer. Coarctation of the aorta is a common congenital heart disease characterised by stenosis of the proximal descending aorta. Despite advances in management, long-term complications remain common.

Methods: The first part analyses data from a prospective clinical register of ACHD patients conducted in the only non-surgical specialist centre with a focus on coarctation patients using cross sectional and longitudinal observational methods. Second part consists of a prospective observational study using cardiac magnetic resonance (CMR) imaging to evaluate the associations of pulse wave velocity (PWV, a measure of arterial stiffness), systemic vascular resistance (SVR) and cardiac function in patients with corrected coarctation.

Results: The NORPAP database was set up in 1993 and in January 2015, had 2322 ACHD patients (mean age 42 years, 52% female, 62%). Coarctation (n=223, 9.6%), 90% had a corrective procedure and a third required additional procedures during follow-up. Older patients at registration and those diagnosed after age 1 year had a greater likelihood of hypertension, death and recoarctation. Forty-five repaired patients were included in the CMR study: mean age 33 years left ventricular ejection fraction 64%, baseline PWV 5.8m/s and SVR 1345 dynes.cm-5. There were significant univariate associations between PWV and SVR with advancing age, female gender and hypertension, although only age was retained in the multivariate model.

Conclusions: Coarctation of the aorta comprised approximately 10% of the ACHD population, with a low risk of long-term adverse outcomes. Patients who were not diagnosed in the 1st year of life had higher risk of hypertension, death and recoarctation. Abnormalities in vascular function were found in CMR in patients with corrected coarctation, and arteriolar resistance may adversely influence myocardial function. These observations could help coarctation patients at risk of future complications.

Item Type:	Thesis (Doctoral)
Faculty \ School:	Faculty of Medicine and Health Sciences > Norwich Medical School
Depositing User:	Chris White
Date Deposited:	08 Feb 2022 15:43
Last Modified:	08 Feb 2022 15:46
URI:	https://ueaeprints.uea.ac.uk/id/eprint/83347
DOI:

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