Bicuspid aortic valve and the domino effect Severe aortic insufficiency and acute heart failure.

Abstract

Introduction: Bicuspid aortic valve (BAV) is a common congenital heart defect in adults, with a prevalence of 1-2% in the general population and a higher occurrence in males. The abnormal morphology of BAV disrupts laminar flow in the aorta, leading to increased wall stress, progressive valve dysfunction, and left ventricular remodeling. The most serious complication is secondary severe aortic regurgitation (IAS), which causes chronic volume overload of the heart. This overload leads to myocardial dilation and dysfunction, potentially resulting in acute heart failure if not treated promptly.

Clinical case: A 33- year- old man, previously healthy, presented with progressively worsening shortness of breath, palpitations, and reduced exercise tolerance. Physical examination revealed a grade III/VI aortic diastolic murmur, suggestive of aortic regurgitation. Echocardiography showed a bicuspid aortic valve (VAB) with severe aortic regurgitation. Despite a preserved ejection fraction (54%), he already had dilation and eccentric hypertrophy of the left ventricle. Cardiac catheterization confirmed a significant hemodynamic impact, with elevated pulmonary pressures (PSAP 55 mmHg) and a low cardiac index (1.89).

Intervention: Based on the ESC/EACTS 2025 guidelines, aortic valve replacement with a mechanical prosthesis was recommended due to severe aortic regurgitation and ventricular structural damage. Postoperatively, he developed hemopericardium with cardiac tamponade, leading to acute heart failure- a terminal event of the "domino effect" process of ABV. He was successfully treated with a pericardial window and drainage. Follow-up included drainage of a retrosternal hematoma, with a favorable outcome, including recovery of systolic function and hemodynamic stability.

Conclusion: Asymptomatic congenital valvular disease (VAB) can silently progress to acute hemodynamic collapse. Early surgical intervention, even with an LVEF of 54% at the critical threshold, combined with multidisciplinary management of complications, was essential to interrupt the pathophysiological cascade and promote a favorable prognosis, consistent with the best long-term survival outcomes reported in the literature.