TEE short axis view of the aortic valve shows a non-stenotic 4-leaflet valve that fails to coapt in the center.
Quadricuspid aortic valve presenting as severe aortic insufficiency
42-year-old female presented with shortness of breath and substernal chest pain. An aortic regurgitation murmur was found at age 32. Until recently she was asymptomatic . Progression of symptoms unrelieved by ACE inhibitors and diuretic therapy lead to surgery.
Exam
Cardiac auscultation: II/VI systolic and III/VI diastolic murmur at right upper sternal border. Carotid pulses were exaggerated, lungs were clear and there was no pretibial edema. EKG showed LVH.
Transthoracic echo: LV dilatation, normal wall thickness and severe aortic regurgitation.
Cardiac cath: LV dilatation, ejection fraction = 47%. Cardiac output: 2.81 L/min. Right heart pressures and coronary anatomy were normal; no gradient across the aortic valve. Aortography demonstrated 3-4+ aortic insufficiency and a normal appearing ascending aorta.
Surgery
At surgery, short axis TEE showed an X-shaped aortic valve commissure, diagnostic of a quadricuspid valve (QAV). Inspection in situ showed three normal-size cusps with a smaller accessory cusp. There was no inflammation, fibrosis or calcification of the leaflets. Coronary ostia were normal. A 21-mm St. Jude prosthetic valve was placed. The patient recovered without complications.
NOTES on QAV:
Discussion
QAV is rare with estimated incidence of 0.0125 to 0.043%. In the past, diagnosis was made at autopsy or incidentally at surgery. More recently, 2D echo, particularly TEE, offers non-invasive diagnosis. The most common abnormality of QAV is insufficiency and is present in 50% of cases with mean age at presentation in the 4th or 5th decade. In a path review of 225 aortic valves resected for insufficiency, QAVs were discovered in two cases (0.89%).
QAV usually occurs as an isolated anomaly, but presence of an accessory cusp obscures normal landmarks for the coronary ostia and frequently leads to slight displacement of either orifice. Confirmation of ostial position is needed during valve replacement or any surgical intervention of the ascending aorta. To the extent that a "pseudo- quadricuspid" valve may result from endocarditis secondary to fibrosis, inflammation, it is not clear whether QAV is more susceptible to bacterial invasion. However, association between QAV and valvular insufficiency suggests that endocarditis prophylaxis may be appropriate in managing high-risk patients.
TEE short axis view of the aortic valve shows a non-stenotic 4-leaflet valve that fails to coapt in the center.
Echo
Color doppler TEE long axis view of the aortic valve shows severe aortic regurgitation without stenosis.