Unicuspid aortic valve concomitant with aortic insufficiency presenting with infectious endocarditis: a case report

Abstract

Background A unicuspid aortic valve is a rare congenital cardiac abnormality. Despite its uncommon finding on an initial presentation, aortic insufficiency is accompanied with unicuspid aortic valve and this might reflect the natural history of progression in the morphology of unicuspid aortic valve. Case presentation We describe a 65-year-old Japanese man who was evaluated for endocarditis and found to have a unicuspid aortic valve concomitant with moderate aortic insufficiency, which was, owing to the lack of evidence of valve membrane destruction, independent of underlying infectious endocarditis. In addition, aortic insufficiency was progressed because of nonbacterial thrombotic endocarditis on the ventricular side, in areas of high turbulence around the heart valve. Conclusions Our case is unusual given the unicuspid aortic valve concomitant with aortic insufficiency, which was presumably independent of underlying infectious endocarditis because of the location of the vegetation and the lack of evidence of valve destruction. Therefore, attention should be paid to a variety of complications in the setting of unicuspid aortic valve.

Unicuspid and quadricuspid aortic valves: two case reports and literature review

“…22 In addition, infective endocarditis has been reported in both unicuspid and quadricuspid aortic valves. 23,24 While aortic valve disease was the only complication in the patient with quadricuspid aortic valve; aortic valve disease was accompanied by mitral valve prolapse and mild mitral valve regurgitation in the patient with the unicuspid aortic valve.…”

Unicuspid aortic valve: Case series and review

“…In the acommissural type, the valve is composed of a single membrane-like leaflet with a central circular orifice. 6 A useful differentiating characteristic is the presence or absence of a lateral attachment of the valve to the aortic wall, which is seen in the unicommissural type and is far more common. 7 All three of our patients demonstrated the unicommissural appearance.…”

“…8 Unicuspid aortic valve is associated with several congenital malformations: patent ductus arteriosus, coarctation of the aorta, anomalous coronary artery, ventricular septal defects, and aortopathies. 5,6,9 Commonly, AS alters the flow dynamics of fluid from the aortic valve leading to unequal shearing of the aorta, which may accelerate aortic dilatation and increase the risk of dissection. 9 The most common associations in our patients were mixed AS and regurgitation, with aortic dilatation noted in two of the patients.…”

“…Severe AS or mixed with regurgitation are the predominant valvular lesions, with left ventricular remodeling often present at the time of diagnosis 8 . Unicuspid aortic valve is associated with several congenital malformations: patent ductus arteriosus, coarctation of the aorta, anomalous coronary artery, ventricular septal defects, and aortopathies 5,6,9 . Commonly, AS alters the flow dynamics of fluid from the aortic valve leading to unequal shearing of the aorta, which may accelerate aortic dilatation and increase the risk of dissection 9 .…”