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Individuals with coarctation of the aorta have historically had poor long-term outcomes with a mean life expectancy of 35 years. Natural history studies demonstrated 90% of individuals dying before age 50 years. Over the past several decades, improvements in prenatal diagnosis, paediatric cardiac surgery, intensive care and adult congenital care have led to a robust success story with the majority of adults living to older ages.
The Heart article by Choudhary et al1 studies the modern day adult coarctation population and defines a new trajectory for longevity. Although reintervention appears to be inevitable in many cases, with late complications including recoarctation, aneurysm development and cardiovascular death, the overall population demonstrates improved outcomes in those who reach adolescence with very good long-term survival to age 60 years.
Despite this optimistic outlook, the authors recognise that atherosclerotic cardiovascular disease (ASCVD) and cerebrovascular events comprise the majority of late morbidity and mortality. The observed deaths were primarily sequelae of acute or chronic hypertension; however, the only identified predictor of hypertension was late repair. Until we are able to better risk stratify which individuals are at the greatest risk for hypertension and late adverse events, adult and paediatric cardiologists must be vigilant with all patients including those with repaired coarctation regarding aggressive primary prevention of ASCVD.
In coarctation of the aorta, hypertension, endothelial dysfunction and increased aortic stiffness contribute to subsequent development of premature coronary artery disease and diastolic heart failure. It is likely that these factors are significantly interrelated in their aetiology and therefore …
Contributors ABB and DDY participated in the writing, editing and finalising of this editorial paper.
Competing interests None declared.
Provenance and peer review Commissioned; internally peer reviewed.
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