Bicuspid aortic valve and aortic coarctation in congenital heart disease—important aspects for treatment with focus on aortic vasculopathy

Abstract

Prevalence of congenital heart disease (CHD) is constantly increasing during the last decades in line with the treatment options for patients ranging from the surgical as well to the interventional spectrum. This mini-review addresses two of the most common defects with bicuspid aortic valve (BAV) and coarctation of the aorta (CoA). Both diseases are connected to aortic vasculopathy which is one of the most common reasons for morbidity and mortality in young patients with CHD. The review will focus as well on other aspects like medication and treatment of pregnant patients with BAV and CoA. New treatment aspects will be as well reviewed as currently there are additional treatment options to treat aortic regurgitation or aortic aneurysm especially in patients with valvular involvement and a congenital BAV thus avoiding replacement of the aortic valve and potentially improving the future therapy course of the patients.

Marfan Syndrome Versus Bicuspid Aortic Valve Disease: Comparative Analysis of Obstetric Outcome and Pregnancy-Associated Immediate and Long-Term Aortic Complications

“…Previous investigations confirmed that the anatomic site of major aortic pathology was distinct in both entities and showed that the maximal aortic dilatation occurred at the ascending aorta in individuals with bicuspid aortic valve disease [37,38]. In accordance with the literature, we found coarctation of the aorta only with bicuspid aortic valve disease but not with Marfan syndrome [39], and identified mitral valve prolapse only with Marfan syndrome but not with bicuspid aortic valve disease [40].…”

CT—Guided Endovascular Exclusion of Pseudoaneurysmal Subclavian Bypass After Early-Age Surgical Correction of Complex Aortic Coarctation

“…The etiology of aortic dilation, dissection, and pseudo/aneurysm may be related to a generalized arteriopathy because intracerebral aneurysms are seen in up to 10% of patients and early development of coronary artery disease has been described ( 2 , 4 , 5 ). Hypotheses point to deficiency of microfibrillar proteins, hyperactivity of matrix metalloproteinases and tunica medial degeneration, mutations in genes such as NOTCH1, and developmental abnormalities of neural crest tissue that give rise to muscular arteries of the heart, aortic arch, and cervicocephalic arteries as potential contributors ( 4 , 5 ). Additionally, 70% to 75% of patients with coarctation have concomitant BAV ( 4 ), and incidence of aortic valve disease in cases of associated coarctation is higher than that of isolated BAV ( 2 ).…”

Changes in inflammation and oxidative stress signalling pathways in coarcted aorta triggered by bicuspid aortic valve and growth in young children

“…One of the most common congenital cardiac abnormalities is coarctation of the aorta (CoA) ( 1 ). Around 85% of CoA patients also have a bicuspid aortic valve (BAV) ( 2 ). Following CoA repair, patients are at increased risk of developing cardiovascular complications including hypertension, impaired left ventricular function, aortic aneurysms and aortic dissection ( 3-5 ).…”