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.     

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.     

A quadricuspid aortic valve in a patient with neurofibromatosis type 1

Quadricuspid aortic valve is a rare cardiovascular abnormality. Herein is described the case of a male patient with neurofibromatosis type 1 (NF1) who was found to have a quadricuspid aortic valve causing severe aortic regurgitation. Although congenital cardiac malformations have been described in patients with NF1, to the best of the present authors' knowledge this is the first time that a quadricuspid valve in a patient with neurofibromatosis has been described.     

Tricuspidation of quadricuspid aortic valve: case reports

Quadricuspid aortic valve in adulthood is a rare pathology which often leads to aortic valve regurgitation that requires surgical treatment. Herein are described two patients with severe regurgitation on a quadricuspid aortic valve and with dilated left ventricle, who where successfully repaired using a technique of tricuspidation of the valve at the level of the abnormal commissure. In each patient, the repair was stabilized and leaflet coaptation increased by subcommissural annuloplasty stitches at the level of the three commissures. After one year and six months' follow up, respectively, both patients presented with trivial aortic regurgitation and good mobility of the three leaflets.     

Three-dimensional echocardiographic evaluation of an incidental quadricuspid aortic valve

Background: Quadricuspid aortic valve is one of the rare forms of congenitalcardiac valvular disease. Its diagnosis is often missed, evenwith the transthoracic echocardiogram. Many of these patientsprogress to aortic incompetence later in life requiring surgicalintervention. In addition, quadricuspid aortic valve can beassociated with other congenital cardiac deformities. Henceearly recognition and follow-up is critical in these patients. Case presentation: We report a patient with quadricuspid aortic valve identifiedon intraoperative transesophageal 3-D echocardiography. This66-year-old male presented with the features of congestive heartfailure. The preoperative transthoracic echocardiogram (TTE)disclosed, moderately severe aortic valve insufficiency alongwith severe mitral and tricuspid regurgitation, but failed toreveal the quadricuspid anomaly of the aortic valve. Interestingly,this patient had undergone transthoracic echocardiography ontwo previous occasions during the past seven years for the evaluationof his valvular heart disease, which all failed to documentthis anomaly. Intraoperatively, transesophageal echocardiography(TEE) displayed an aortic valve composed of three medium andone small cusps. Conclusion: Our patient's case demonstrates the usefulness of transesophagealechocardiography in detection of this uncommon congenital malformation.     

Quadricuspid morphology of the aortic valve

Quadricuspid aortic valve is a rare congenital heart defect. It may be isolated or associated to other cardiac anomalies. It may cause aortic valve dysfunction, commonly aortic regurgitation. Management of patients with quadricuspid aortic valve is represented by strict follow-up, because they may require aortic valve replacement in later life. We report the case of a 37-year old male patient, occasionally diagnosed to have quadricuspid aortic valve. Diagnosis and management are discussed.     

Transesophageal echocardiographic findings of quadricuspid aortic valve.

We report a case of a 40-year-old man who had a quadricuspid aortic valve associated with aortic regurgitation. The anomaly was revealed by transesophageal echocardiography (TEE), and confirmed by cardiac surgery. TEE is a useful noninvasive method for identifying the quadricuspid aortic valve.     

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.     

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.     

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.     

Quadricuspid aortic valve: a chance for repair

Quadricuspid aortic valve is an uncommon congenital anomaly that often causes aortic regurgitation in adulthood, necessitating valve replacement. A 54-year-old woman presented with symptoms and signs of aortic regurgitation, but during surgery a quadricuspid aortic valve was identified. The abnormal cusp was fibrotic, short, thick, and fenestrated. Excision of the fourth cusp was performed and valve repair carried out successfully, with only minimal regurgitation.     

Quadricuspid aortic valve: a rare cause of aortic insufficiency diagnosed by doppler echocardiography. Report of two cases and review of the literature.

Quadricuspid aortic valve is an uncommon congenital valve disease mostly occurring as isolated lesion or sometimes in association with truncal anomalies. Approximately 50% of patients with quadricuspid aortic valve have aortic regurgitation. Before the advent of echocardiography most cases were diagnosed at the time of surgery or at post-mortem examination. We describe 2 cases of patients with quadricuspid aortic valve diagnosed by echocardiography. The first case, a quadricuspid aortic valve with four equal-sized cusps (type A, according to the classification of Hurwitz and Roberts), was identified in a 26-year-old man undergoing echocardiography because of a heart murmur. The second case, a quadricuspid aortic valve with three relatively equal cusps and one smaller cusp (type B, according to the classification of Hurwitz and Roberts), was identified in a 47-year-old man with a history of murmur. The identification and periodical non-invasive evaluation of a quadricuspid aortic valve is important, because such valves are more vulnerable to infection and need adequate prophylaxis against 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.     

Quadricuspid aortic valve: an unusual echocardiographic finding and a review of the literature

This case report presents a rare clinical finding of an isolated quadricuspid aortic valve in an otherwise healthy 24-year-old female who was referred for cardiac evaluation due to newly identified murmur. A transthoracic echocardiogram suggested an unusual case of a bicuspid aortic valve with two raphes with normal opening and mild aortic regurgitation. This case exemplified that caution should be taken when encountering circumstances like the one presented, as the aortic valve anatomy might be mistaken based on a new proposed classification for bicuspid aortic valves recently published. Further evaluation identified the presence of a quadricuspid aortic valve using computer tomography of the chest. Care should be taken in evaluating aortic valves as in this case. The literature regarding quadricuspid aortic valves is reviewed.     

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.     

Quadricuspid semilunar valves: report of two cases

Quadricuspid semilunar valves are uncommon congenital anomalies sometimes associated with severe valvular dysfunction. Two cases of isolated quadricuspid semilunar valve were identified. The first, a heavily calcified quadricuspid aortic valve with four equal-sized cusps, was identified in a 56-year-old man at the time of valve replacement surgery for severe aortic stenosis and regurgitation. The second valve was a quadricuspid pulmonary valve with three equal-sized cusps and one smaller cusp, identified incidentally at autopsy in a 79-year-old woman with a ruptured abdominal aortic aneurysm. Review of the literature found that while quadricuspid pulmonary valves are infrequently associated with serious clinical complications, the majority of quadricuspid aortic valves are associated with clinically significant dysfunction, most commonly aortic insufficiency. Further, contrary to previous thinking, the frequency of aortic valvular dysfunction does not appear to be related to valve morphology.     

Congenital quadricuspid aortic valve. Apropos of a case. Review of the literature

The authors report a new case of quadricuspid aortic valve, a fortuitous finding in a 6 year old child. This rare congenital anomaly is usually isolated. The diagnosis is made by 2D echocardiography and the main risk is progression to severe aortic regurgitation in adulthood.     

Quadricuspid aortic valve associated with unruptured sinus of Valsalva aneurysm.

Quadricuspid aortic valve is a rare congenital anomaly. It may present as an isolated anomaly but is occasionally associated with aortic regurgitation. Sinus of Valsalva aneurysm (SVA) is also an infrequent congenital anomaly, typically associated with tricuspid aortic valves. There are only a few reported cases of SVA (ruptured) associated with quadricuspid aortic valves in the literature. We report the first case of the association of quadricuspid aortic valve with an unruptured SVA in an adult patient.     

Rare or unusual causes of chronic, isolated, pure aortic regurgitation

Six patients undergoing aortic valve replacement had rare or unusual causes of isolated, pure aortic regurgitation. Two patients had congenitally bicuspid aortic valves with a false commissure (raphe) displaced to the aortic wall ("tethered bicuspid aortic valve"), two had floppy aortic valves, one had a congenital quadricuspid valve, and one had radiation-induced valve damage.