Symptomatic unicuspid aortic valve

Abstract

SUMMARYA 22-year-old man with typical angina was seeking medical attention at primary health clinics for a couple of months. Owing to his young age and the absence of coronary artery disease risk factors, he was assured of no serious problem. Proper examination at a referral centre revealed weak peripheral pulses with diminished and delayed carotid upstroke. A normal S1 with a soft S2 were audible. A 3/6 late peaking systolic murmur was best heard in the aortic area radiating to the neck. Symptomatic bicuspid aortic valve disease was suspected. Diagnosis of unicuspid aortic valve was established by transoesophageal and three-dimensional echocardiography. The valve was successfully replaced with a mechanical prosthesis. The patient remains asymptomatic at 1 year follow-up. BACKGROUND

Unicuspid aortic valve: Case series and review

“…Unicuspid aortic valve results from the abnormal fusion of the three tubercles that are converted into thin valve cusps and sinuses of valsalva through excavation during embryonic life, and present in two phenotypes. 4,5 The unicommissural type has a single leaflet originating from the aortic wall and attaching circumferentially around the annulus. The valve orifice is eccentric and elliptical in shape.…”

“…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.…”

“…Typically, patients with unicommissural valves remain asymptomatic up until the third to fifth decade, while acommissural valves require intervention in youth; this is most likely related to a smaller orifice area and a more restricted and diseased valve in the acommissural type. 5,10 Transthoracic and transesophageal echocardiography are the main modalities used to establish diagnosis, and cardiac computed tomography or magnetic resonance imaging is important in identifying extracardiac associations such as aortic dilatation. 5 Preoperative diagnosis of unicuspid aortic valve is challenging thoracic aorta surgery is a diameter ≥5.5 cm; it is also reasonable to proceed with surgery in patients with high-risk features or if undergoing other cardiac surgery in the setting of an aortic diameter ≥5.0 cm (class II).…”

“…5,10 Transthoracic and transesophageal echocardiography are the main modalities used to establish diagnosis, and cardiac computed tomography or magnetic resonance imaging is important in identifying extracardiac associations such as aortic dilatation. 5 Preoperative diagnosis of unicuspid aortic valve is challenging thoracic aorta surgery is a diameter ≥5.5 cm; it is also reasonable to proceed with surgery in patients with high-risk features or if undergoing other cardiac surgery in the setting of an aortic diameter ≥5.0 cm (class II). 10,13,14 Unicuspid aortic valve is a rare clinical condition that is often difficult to diagnose at initial presentation.…”

“…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 .…”