Bicuspid aortic valve with aortic coarctation

Bicuspid aortic valve (BAV) is the most common congenital heart defect with a prevalence of 1–2% and most commonly BAV is found in males with a rate of 1:2 varying to 1:4 (1–5). BAV is most commonly the result of fusion of the left and right coronary cusp (LCC and RCC) in over 70% of patients and not so common of fusion of the RCC with the non-coronary cusp (NCC) 10–20% and least frequent due to fusion of the LCC with NCC in 5–10% (1,3).

Coarctation of the aorta (CoA) occurs often as a discrete stenosis or a longer, hypoplastic segment of the ascending aorta. Typically, CoA occurs where the ductus arteriosus is located and only rarely ectopically in the ascending, descending or abdominal aorta (1–3). Most often reported is a prevalence in relation to all congenital heart disease (CHD) of 5–8% and a prevalence of 3 in 10,000 live births for the isolated form of CoA (1,2).

Both BAV and CoA as CHD are commonly associated in 85% of cases and can be present together with subvalvular, valvular or supravalvular aortic stenosis and malformation of the mitral valve with mitral valve stenosis. The combination of aortic stenosis at all three levels with a parachute mitral valve is called Shone complex (1). However, CoA can as well often be found in complex and genetic lesions with Turner or Williams-Beuren Syndrome.

An important associated lesion in both above-mentioned diseases is the aortopathy, in both types of CHD. Aortopathy could be a potential factor responsible for the increased morbidity and mortality in both diseases. A specific diagnostic work-up has been reported to consider the need for simultaneous aortic surgery to decrease the risk of potential lethal aortic dissection when aortic valve surgery is required (1–3,6).

This focused mini-review describes the current diagnostic and treatment algorithm for both diseases with a special focus regarding aortopathy. Further, new aspects regarding aortic valve surgery in both diseases will be addressed as these should be considered when making the surgical decision in mostly young patients to achieve the best long-term result.