What is the clinical evidence from this study?

Study design: Observational. Population: Thoracic aortopathy (n=90). Intervention: Marfan syndrome (MFS) vs. Tricuspid aortic valve (TAV). Primary outcome: Obstructive coronary sclerosis (CAGE ≥ 50) (OR 0.64, 95% CI 0.41-0.99, p=0.049).

January 12, 2023Open Access

Risk for acquired coronary artery disease in genetic vs. congenital thoracic aortopathy

ODOnur B. DolmaciOLVG TATugay AyyildizAmsterdam University Medical Centers RPRobert E. PoelmannPediatric Cardiology

Resultado clave

Patients with Marfan syndrome had a significantly lower risk of obstructive coronary sclerosis compared to patients with tricuspid aortic valves (OR 0.64).

Diseño del estudio

Tipo

Observational (n=90)

Multicéntrico

Sí

PICO estructurado

Do patients with Marfan syndrome have a lower risk of coronary artery disease compared to patients with bicuspid or tricuspid aortic valves?

Población

90 adults (mean age 53, 86.7% male) surgically treated for aortic root dilatation or aortic regurgitation, matched by age and sex into three groups: Marfan syndrome (n=30), bicuspid aortic valve (n=30), and tricuspid aortic valve (n=30). Excluded: <18 years, active endocarditis, aortic dissection.

Intervención

Marfan syndrome (MFS)

Comparador

Bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV)

Resultado

Severity of coronary artery sclerosis determined by CAGE score on preoperative coronary angiography or computed tomographysurrogate

Patients with Marfan syndrome and bicuspid aortic valves have a significantly lower prevalence of coronary artery disease compared to those with tricuspid aortic valves, despite similar age and sex.

Resultado numérico

Estimación del efecto: OR 0.64 (95% CI 0.41-0.99)

valor p: p=0.049

Limitaciones

Small sample size of 30 patients per group due to the rarity of the syndromes.
Potential selection bias as MFS and BAV patients generally develop aortopathy at a younger age than TAV patients.
A small portion of the MFS patients (16.7%) had a BAV morphology, which could influence the results.

Resumen

Objective Patients with Marfan syndrome (MFS) and patients with a bicuspid aortic valve (BAV) have a significantly increased risk to develop thoracic aortopathy. Both conditions share many pathophysiological mechanisms leading to aortic complications. Bicuspidy is known to have a low risk for acquired coronary artery sclerosis. The aim of this study is to determine the risk of coronary sclerosis in MFS patients. Methods Marfan syndrome patients with an aortic root dilatation, which were surgically treated between 1999 and 2017, were included and matched with BAV and tricuspid aortic valves (TAV) patients based on sex and age. Cardiovascular risk profiles were determined in all three groups. Coronary sclerosis was graded in all patients on coronary imaging (coronary angiography or computed tomography) using a coronary artery scoring method, which divides the coronaries in 28 segments and scores non-obstructive (20–49% sclerosis) and obstructive coronary sclerosis (49% sclerosis) in each segment. Results A total of 90 matched patients (30 within each group) were included. MFS patients showed less cardiovascular risk factors compared to BAV and TAV patients. TAV patients had higher amounts of obstructive coronary sclerosis as compared to BAV patients ( p = 0.039) and MFS patients ( p = 0.032). No difference in non- and obstructive coronary artery disease (CAD) was found between the MFS and BAV population. Conclusion Marfan syndrome and bicuspid aortic valve patients have a significantly lower risk for, and prevalence of CAD as compared to TAV individuals.

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