Abstract
Bicuspid aortic valve (BAV) is a common congenital heart diagnosis and is associated with aortopathy. Current guidelines for aortic resection have been validated but are based on aortic diameter, which is insufficient to predict acute aortic events. Clinical and translational collaboration is necessary to identify biomarkers that can individualize the timing of prophylactic surgery for BAV aortopathy. We describe our multidisciplinary BAV program, including research protocols aimed at biomarker discovery and results from our longitudinal clinical registry. From 2012–2018, 887 patients enrolled in our clinical BAV registry with the option to undergo four dimensional flow cardiovascular magnetic resonance imaging (4D flow CMR) and donate serum plasma or tissue samples. Of 887 patients, 388 (44%) had an elective BAV-related procedure after initial presentation, while 499 (56%) continued with medical management. Of medical patients, 44 (9%) had elective surgery after 2.3 ± 1.4 years. Surgery patients’ biobank donations include 198 (46%) aorta, 374 (86%) aortic valve, and 314 (73%) plasma samples. The 4D flow CMR was completed for 215 (50%) surgery patients and 243 (49%) medical patients. Patients with BAV aortopathy can be safely followed by a multidisciplinary team to detect indications for surgery. Paired tissue and hemodynamic analysis holds opportunity for biomarker development in BAV aortopathy.
Highlights
Bicuspid aortic valve (BAV) is a common congenital diagnosis involving both the aortic valve and aorta, and is often categorized with connective tissue disorders such as Marfan and Turner syndromes
From 2012–2018, 887 patients with BAV enrolled in our single-center registry after presenting to our referral cardiology or cardiac surgery clinic
A comparison of overall survivorship since surgery among the surgical aortic valve replacement (SAVR), SAVR with aneurysm resection (SAVR + AN) and transcatheter aortic valve replacement (TAVR) groups was based on the log-rank test
Summary
Bicuspid aortic valve (BAV) is a common congenital diagnosis involving both the aortic valve and aorta, and is often categorized with connective tissue disorders such as Marfan and Turner syndromes. BAV typically presents as progressive valve deterioration through aortic stenosis (AS), insufficiency (AI), or infective endocarditis (IE) These valvular complications are accompanied by aortopathy in up to 42% of cases, including dilatation of the aortic root, ascending aorta, arch, or coarctation [1]. We developed a specialty BAV program with the goals to enhance clinical continuity for patients and families, and to integrate a longitudinal clinical registry with translational research efforts aimed at developing additional biomarkers for BAV aortopathy This multidisciplinary approach is similar to the ‘Heart Team’ clinical model for severe aortic stenosis, complex coronary artery disease, and advanced heart failure management [7,8,9,10]. We aim to share our experience by describing patients’ characteristics, treatment courses, and volume of translational specimens collected to date
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