Congenital quadricuspid aortic valve associated with aortic regurgitation.

A case of quadricuspid aortic valve is described. The aortic valve consisted of 3 equal-sized and 1 smaller cusps, and a supernumerary cusp located between the right and noncoronary cusps. A right coronary ostium was close to the accessory commissure, and in a lower position. Three fenestrations were found at the supernumerary commissure. Aortic valve replacement was performed successfully with a St. Jude Medical prosthetic valve. On histological examination, the resected cusps showed fibrotic thickening with calcification and no sign of previous inflammatory disease. Quadricuspid aortic valve must be considered a malformation capable of leading to severe valve failure in later life.     

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.     

Revelation of quadricuspid aortic valve at aortography in elderly patient with severe aortic regurgitation

Aortic regurgitation is associated with numerous eponymous signs. It has been reported severe aortic regurgitation also due to a quadricuspid aortic valve, a rare congenital anomaly. We present a case of revelation of quadricuspid aortic valve at left ventriculography with aortography in a 71-year-old Italian woman with severe aortic regurgitation.     

Congenital quadricuspid aortic valve associated with congenital complete heart block.

Congenital quadricuspid aortic valve is very uncommon, and is often associated with other cardiac disorders, such as patent ductus, ventricular septal defect, pulmonary stenosis, mitral valve malformation, hypertrophic cardiomyopathy and coronary abnormalities. We report a patient with congenital quadricuspid aortic valve associated with congenital complete heart block. To our knowledge, this association has not been reported so far.     

Congenital quadricuspid aortic valve in children

Quadricuspid aortic valves are rare congenital anomalies, usually diagnosed in adult patients affected by severe aortic regurgitation. We have now encountered three such valves in children undergoing transthoracic echocardiography. All children were asymptomatic. The first child possessed a valve with two larger leaflets, and smaller leaflets of equal size. In the second child, 3 leaflets were of equal size, with 1 small accessory leaflet. The third child had a valve with four leaflets of approximately equal size. In one child, the aortic valve was functioning normally, and came to attention because of mitral valvar prolapse causing mild regurgitation. In the remaining two children, central mild aortic regurgitation was detected using colour flow analysis, and associated dilation of the aortic root was revealed by measurements of the cross-sectional images.     

Infective endocarditis associated with quadricuspid aortic valve

A 26-year old Japanese woman experienced new aortic valve regurgitation associated with a preceding high fever of unknown cause. During the fever episode, although bacteremia or fungemia was not evident despite frequent blood cultures, intravenous panipenem/betamipron (PAPM/BP) gradually resulted in decline of the fever. Echocardiography and operative procedures revealed a quadricuspid aortic valve (QAV), which was composed of two equal larger cusps and two unequal smaller cusps (type f). A smaller accessory cusp was damaged but showed no active vegetation. A Medtronic Freestyle bioprosthesis was implanted using a subcoronary technique. Although the risk of endocarditis for this rare valve abnormality is not well documented, the present case may support the conventional assumption that patients with unequal small cusps are prone to endocarditis.     

Quadricuspid aortic valve with severe aortic regurgitation

Quadricuspid aortic valve is a rare congenital anomaly that usually presents with aortic regurgitation. Its importance, however, lies in its association with coronary abnormalities, which may lead to surgical catastrophe, if not diagnosed pre-operatively. This report describes a case of quadricuspid aortic valve detected incidentally during routine pre-operative transesophageal echocardiography.     

主动脉瓣四叶瓣畸形合并主动脉瓣病变的外科处理

目的 探讨主动脉瓣四叶瓣畸形对主动脉瓣功能的影响及其外科治疗.方法 2000年1月至2013年1月,我院通过经胸超声心动图和术中病理诊断主动脉瓣四叶瓣畸形13例.其中4例主动脉瓣的功能基本正常,9例合并主动脉瓣病变：重度关闭不全7例,重度关闭不全伴狭窄2例,合并二尖瓣中度关闭不全2例,三尖瓣中重度关闭不全1例.结果 13例患者中4例主动脉瓣功能正常者,继续随访中.9例合并主动脉瓣病变者,均行主动脉瓣置换术,同期行二尖瓣成形术2例、三尖瓣成形术1例.围术期无严重并发症及早期死亡,均康复出院.术后平均随访（6.34±5.17）年,心彩超提示主动脉瓣功能良好,无远期死亡.结论 主动脉瓣四叶瓣畸形是一种少见的先天性畸形,超声心动图有助于早期诊断.主动脉瓣功能正常时,可以随访;当合并主动脉瓣功能障碍时,应及时行主动脉瓣置换或修复,手术后可获得良好的远期效果.     

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.     

Unicuspid aortic valve mimicking quadricuspid valve

Unicuspid aortic valve is a rare congenital cardiac abnormality, leading to aortic stenosis or regurgitation. We report the case of a 55-year-old man with severe aortic regurgitation caused by a unicuspid valve mimicking quadricuspid valve.     

Congenital aortic regurgitation in a child with a tricuspid non-stenotic aortic valve

After follow up for seven years a 10 year old boy with congenital aortic regurgitation was found to have a tricuspid non-stenotic aortic valve at operation. The right coronary cusp was dysplastic, thickened, and contracted; the gap between its free margin and aortic wall was bridged with two fibrous bands; and the left coronary and non-coronary cusps were almost normal. The aortic valve was replaced with a prosthesis (St Jude Medical No 23), and the postoperative course was uneventful.     

Congenital aortic regurgitation in a child with a tricuspid non-stenotic aortic valve.

After follow up for seven years a 10 year old boy with congenital aortic regurgitation was found to have a tricuspid non-stenotic aortic valve at operation. The right coronary cusp was dysplastic, thickened, and contracted; the gap between its free margin and aortic wall was bridged with two fibrous bands; and the left coronary and non-coronary cusps were almost normal. The aortic valve was replaced with a prosthesis (St Jude Medical No 23), and the postoperative course was uneventful.