Congenital quadricuspid aortic valve associated with severe regurgitation

A 56-year-old man was referred because of severe aortic regurgitation. He had a quadricuspid aortic valve with a small accessory cusp between the right coronary and noncoronary cusps. The ostium of the right coronary artery was deviated toward the accessory cusp commissure. Aortic valve replacement was performed with a bioprosthesis. The resected cusps showed fibrotic thickening with calcification and fenestration.     

Asymptomatic chronic traumatic aortic valve perforation with severe aortic regurgitation

Here, we present a young asymptomatic male patient incidentally diagnosed to have aortic regurgitation (AR). The patient had a history of a blunt trauma to the thorax two years back but did never have any symptoms. Transthoracic echocardiography showed a moderately dilated left ventricle with normal systolic function and severe AR with normal nondilated aortic root and tri-leaflet aortic valve. To diagnose the etiology of the AR, a transesophageal echocardiogram (TEE) was done, which revealed a perforation in the nonadjacent leaflet (NAL) and confirmed severe AR with two AR jets being clearly visualized, one through the point of incomplete coaptation and other one through the perforated area in the NAL. The patient was treated with aortic valve replacement and was doing well on follow-up.     

Quadricuspid aortic valve with centrally fenestrated leaflets causing aortic insufficiency

The occurrence of a quadricuspid aortic valve with single, central leaflet fenestrations causing aortic insufficiency has not previously been reported. We recently encountered these features during aortic valve replacement for severe aortic regurgitation in a 20-year-old man who had had a history of aortic insufficiency since age 2 years. The patient's late increase in symptoms was probably due to the fact that 1 of the 4 leaflets was somewhat flail, allowing incomplete coaptation and producing regurgitation over and above that caused by the central fenestrations.     

Premature opening of aortic valve in severe aortic regurgitation.

Premature opening of the aortic valve in late diastole has been demonstrated by echocardiography in a patient with severe aortic regurgitation. Valve opening coincided with the end of the diastolic murmur and equalization of aortic and left ventricular diastolic pressures. Echocardiography of the aortic valve in severe aortic reflux may, therefore, provide valuable information about the underlying haemodynamic disturbance.     

Premature opening of aortic valve in severe aortic regurgitation.

Premature opening of the aortic valve in late diastole has been demonstrated by echocardiography in a patient with severe aortic regurgitation. Valve opening coincided with the end of the diastolic murmur and equalization of aortic and left ventricular diastolic pressures. Echocardiography of the aortic valve in severe aortic reflux may, therefore, provide valuable information about the underlying haemodynamic disturbance.     

Quadricuspid aortic valve associated with severe aortic regurgitation

A 57-year old man with severe aortic regurgitation was found to have a quadricuspid aortic valve. Aortic valve replacement was performed successfully with a St. Jude Medical prosthetic valve. On histological examination, the resected cusps showed fibrotic thickening with calcification.     

Quadricuspid aortic valve as a cause of severe aortic regurgitation.

Quadricuspid aortic valves (QAVs) constitute a rare congenital malformation, with an incidence ranging from 0.008 to 0.048%. We report a case of severe aortic regurgitation associated with a QAV, which was diagnosed intraoperatively using transesophageal echocardiography. Since the first case described in 1862, 186 QAVs have been reported. In most cases, QAVs are associated with valve regurgitation, with a concurrent stenosis in some patients, while only a small number of QAVs are functionally normal. Once the diagnosis has been made, echocardiographic follow-up is recommended, as progression to severe valve regurgitation is common. Antibiotic prophylaxis is advisable for dental, and "dirty" surgical procedures, to minimize the risk of infective endocarditis.     

Quadricuspid aortic valve: a rare etiology of aortic regurgitation.

Quadricuspid aortic valve is a rare cause of aortic insufficiency. We report two unusual cases of this valvular pathology associated with a dilatation of the aortic root. The mechanism leading to this valve incompetence is incompletely understood and is discussed in regard to these cases.     

Quadricuspid aortic valve

We reported a case of a 45 year-old woman who had a quadricuspid aortic valve associated with moderate aortic regurgitation. The valve abnormality was detected by transthoracic echocardiography. Transesophageal echocardiography showed mild thickening of 4 symmetric aortic valve cusps, a small rectangular central regurgitant orifice, and moderate aortic insufficiency. In addition visualised this anomaly in 3D transesophageal echocardiography too.