Ross procedure in a quadricuspid aortic valve

A quadricuspid aortic valve is a rare congenital cardiovascular abnormality, and when present, it is associated with aortic valve regurgitation. If aortic valve replacement is required, mechanical or biological prostheses are used. We report the case of a patient with a severely regurgitant quadricuspid aortic valve in whom a Ross procedure was performed.     

Congenital quadricuspid aortic valve: report of nine surgical cases.

We report the clinicopathologic characteristics of the congenital quadricuspid aortic valve necessitating surgery. Among 616 patients for whom we performed an aortic valve operation over the past 20 years, nine patients (1.46%) (five men and four women, mean age 60 years) with quadricuspid aortic valve were encountered. All had aortic regurgitation (AR) except one with aortic stenosis and mild regurgitation (ASr). All were free of cardiac anomaly including that of the coronary arterial system. Macroscopically, severe calcification of the valve was seen in the one case of ASr. Fenestration of the cusp was seen in five cases of AR. Infective endocarditis was not seen. Histological study disclosed fibrous thickening and myxoid degeneration in the AR cases. In accordance with the Hurwitz and Roberts classification, four valves were type b (three equal-sized cusps and one smaller cusp), two valves were type a (four equal-sized cusps), two valves were type d (one large, two intermediate, and one small cusp), and one valve was type g (four unequal-sized cusps). Valve repair failed in one patient and was converted to valve replacement during the operation. All patients underwent successful aortic valve replacement (AVR).     

Quadricuspid aortic valve associated with rapidly progressive aortic stenosis during chronic hemodialysis; report of a case

A 70-year-old man was referred to our department for surgical treatment for aortic valve stenosis. He was diagnosed with aortic regurgitation 30 years ago, but he was asymptomatic at that time. Ten years ago, chronic hemodialysis was instituted for diabetic nephropathy. In recent years, he became aware of nocturnal dyspnea and echocardiography revealed moderate aortic stenosis. After that, the symptom was getting worse and surgical treatment was indicated. We performed aortic valve replacement. Intraoperatively, we noticed his aortic valve was quadricuspid. The postoperative course was uneventful and he was discharged without complications. Quadricuspid aortic valve is rare congenital anomaly and patients are often operated on for aortic regurgitation. Our case is indicated for valve replacement because of aortic stenosis. In addition, there are a few reports of quadricuspid aortic valve associated with hemodialysis. We consider hemodialysis is one of the cause of rapid progression of aortic stenosis in this patient.     

Quadricuspid aortic valve: case reports

Two cases of quadricuspid aortic valve with aortic regurgitation are reported. Case 1, a 66-year-old woman was operated on because of aortic regurgitation, and an aortic valve replacement with a bioprosthesis was performed. When the valve was exposed during the operation, it showed four cusps, three of which were of equal size and one smaller cusp which was interposed between the right and left coronary cusp. Case 2, a 46-year-old man was diagnosed, using echocardiography and aortography before surgery, as suffering from aortic regurgitation because of a quadricuspid aortic valve. During surgery, two larger cusps and two smaller cusps and a displacement of the right coronary artery ostium, (which was placed in a lower position and close to the commissure between the right coronary and the right posterior cusps) were found. The four cusps were excised and replaced by a tilting disc prosthesis. In both cases, the postoperative recovery was uneventful. Using the 24 cases from the literature and two of our own cases, the correlation between the size or the position of the accessory cusp and the occurrence of aortic regurgitation was analyzed. The larger the accessory cusp was, the higher the incidence of aortic regurgitation occurred (p less than 0.05).     

Quadricuspid aortic valve with ascending aortic aneurysm: a case report and histopathological investigation

We describe the case of a 69 year-old woman with a dilated ascending aorta, who presented with aortic valve regurgitation due to a quadricuspid aortic valve (QAV). There are only a few reports in the literature describing aortic replacement and subsequent aortic valve replacement for a malfunctioning QAV. We discuss the pathogenesis of the dilated ascending aorta in this patient and the indication for ascending aorta replacement in such cases.     

A case of minimal invasive aortic valve replacement for quadricuspid aortic valve with aortic regurgitation]

Congenital quadricuspid aortic valve is rare, which may be a cause of sever aortic regurgitation. We report a case of a 55-year-old man who had severe aortic regurgitation with congenital quadricuspid aortic valve. Preoperative aortography showed severe aortic regurgitation. Preoperative trans-esophageal echocardiography revealed abnormal quadricuspid aortic valve. We performed minimal invasive aortic valve replacement with SJM # 21 mm HP prosthetic valve through the limited upper sternotomy. Skin incision was 9 cm. Aortic cross-clamping time was 92 min, cardiopulmonary bypass time was 108 min. At the operation, a quadricuspid valve with three equal cusps and one small cusp was noted. The postoperative course was excellent and he discharged 8 days after the operation.     

A case of concomitant repair of aortic regurgitation due to congenital aortic quadricuspid valve and coronary artery occlusive disease

A case of rare congenital anomaly of quadricuspid aortic valve and coexisting coronary artery occlusive disease of a 60-year-old female was presented. She was admitted to our hospital for heart murmur and angina pectoris. The echocardiography and aortography showed quadricuspid aortic valve and aortic valve regurgitation. The coronary arteriography revealed the presence of triple vessels disease. Successful aortic valve replacement and Coronary bypass grafting were performed simultaneously.     

Original aortic valve plasty with autologous pericardium for quadricuspid valve

Quadricuspid aortic valves represent a very rare pathology. Most cases have been discovered incidentally during heart operations or at autopsy. Patients may become symptomatic with aortic regurgitation. We encountered a symptomatic patient with aortic regurgitation and a quadricuspid aortic valve. Successful aortic valve plasty was done with our original technique of tricuspid replacement by glutaraldehyde-treated autologous pericardium.     

A case of quadricuspid aortic valve with aortic regurgitation

A 67-year-old man with grade 3 aortic valve regurgitation was found to have a quadricuspid aortic valve. The aortic valve consisted of 1 large, 2 intermediate and 1 small sized cusp. An accessory cusp located between the right and noncoronary cusps, and shaped like a hammock which sling by the fibrous strings originating from the both commissures to the aortic wall. Aortic valve replacement was successfully performed with a 23 mm St. Jude Medical prosthetic valve, and the patient is asymptomatic five months post-operatively. Histological examination of the resected cusps showed fibrous thickening and no rheumatic valvulitis or infective endocarditis.     

Congenital quadricuspid aortic valve with tetralogy of Fallot and pulmonary atresia

We report the case of a 4-year-old girl who had quadricuspid aortic valve regurgitation with tetralogy of Fallot and pulmonary atresia. This combination is very uncommon. Aortic valve replacement was performed successfully due to aortic regurgitation which had progressed one year after the total repair. The dilated aortic annulus plus quadricuspid aortic valve may result in progressive aortic regurgitation for a short period.     

A case of quadricuspid aortic valve associated with coronary artery disease

A 65-year-old man with aortic regurgitation was found to have a quadricuspid aortic valve. Surgical correction consisted of aortic valve replacement with a 21 mm Hyper dynamic plus St. Jude Medical valve and coronary revascularization of LAD and the first diagonal branch. During the operation, a quadricuspid aortic valve with 1 smaller and 3 larger cusps and normal coronary orifice were noticed. Accurate information by the transesophageal echo proved to be quite valuable. Our case is one of 43 cases which have been reported in Japan.     

A case of quadricuspid aortic valve associated with coronary arterial legion

A case of quadricuspid aortic valve is reported in a patient with coronary artery disease and abdominal aortic aneurysms. A 54-year-old male who had undergone aortic replacement because of abdominal aortic aneurysms three years before presentation was readmitted due to complaints of angina pectoris and palpitations. Aortography and coronary arteriography revealed severe aortic regurgitation and proximal occlusion of LAD and RCA. Surgical correction consisted of aortic valve replacement with a Bj?rk-Shilely valve and coronary revascularization of LAD. During the operation, a quadricuspid aortic valve with one smaller and three larger cusps that showed mild myxomatous degeneration without dystrophic calcification and normal coronary arterial orifices were noted. Accordingly, severe aortic regurgitation may have resulted from the dysfunction of congenital malformed cusps and acquired sclerotic coronary disease was the main cause of the chest pain.     

Surgery of aortic valvular disease with congenital bicuspid or quadricuspid aortic valve

Twelve patients (age 18 to 69 years old) with surgical treatment for congenital bicuspid aortic valve and one (age 26 years old) for quadricuspid aortic valve were reported. These 13 patients occupied 12.3% of the total number of patients with aortic valve replacement during the same period. Among the patients with bicuspid aortic valve, 10 patients were male (83%) and 2 were female (17%). Seven patients exhibited aortic stenosis and 5 showed predominant aortic regurgitation. The mean age of patients with aortic stenosis was higher than that of patients with aortic regurgitation. (63.3 years vs 32.0 years old). Infective endocarditis occurred in 2 patients (17%). Three patients had pure aortic regurgitation without evidence of endocarditis. One 26-year-old female patient who had a quadricuspid aortic valve presented with aortic regurgitation associated with PDA. All patients underwent aortic valve replacement with no early and late deaths.     

A quadricuspid aortic valve diagnosed by transesophageal echocardiography: Report of a case

A quadricuspid aortic valve is a very rare anomaly which may cause aortic regurgitation in adulthood. We describe herein the case of a 54-year-old man with aortic regurgitation in whom a quadricuspid aortic valve was diagnosed, not through transthoracic investigation, but by transesophageal echocardiography (TEE). TEE also indicated that the right coronary ostium was located in a lower position. Subsequent aortic valve replacement was successfully performed, at which time the diagnosis was confirmed. Thus, TEE played an important role in identifying the anatomy of the aortic valve and the location of the coronary ostium.     

A case report of surgical treatment of quadricuspid aortic valve associated with regurgitation.

A case of a 65-year-old woman who had a quadricuspid aortic valve associated with aortic regurgitation is reported. The patient had severe aortic regurgitation and four equally divided aortic cusps. The valve abnormality was detected by a transesophageal echo and an aortography. The incomplete aortic valve was excised and replaced by a St. Jude Medical prosthesis. Although this case had no coronary abnormality, a coronary displacement is often reported in quadricuspid aortic valve cases. In order to perform an operation safely, accurate information which is obtained by a non-invasive examination of the transesoph-ageal echo is quite valuable as it can indicate the need for further preoperative examinations of the coronary arteries.     

四叶式主动脉瓣畸形的诊断及外科治疗

目的 总结四叶式主动脉瓣畸形的超声心动图诊断并探讨外科治疗.方法 回顾分析上海市心血管病研究所2004年1月至2007年5月超声心动图资料,总结四叶式主动脉瓣膜病例的超声诊断特点.观察23例四叶式主动脉瓣四叶分布形态、回声、启闭及血流动力学情况.并总结分析手术治疗的9例病人的资料.结果 在92656例心脏超声检查中,共检出四叶式主动脉瓣膜畸形23例,检出率为0.248‰.年龄19～83岁,平均(50.3±18.8)岁;男11例,女12例.23例中22例合并不同程度主动脉瓣膜反流,仅1例主动脉瓣膜功能正常.其中9例行主动脉瓣膜置换手术,术中发现A型8例,F型1例.结论 四叶式主动脉瓣膜畸形是非常少见的先天性心脏畸形,多数由超声心动图检查发现,实时三维超声心动图对可疑病例可明确诊断.四叶式主动脉瓣膜畸形主要并发主动脉瓣膜反流,严重者应手术治疗.     

Aortic regurgitation due to quadricuspid aortic valve--case report and literature review

Quadricuspid aortic valve is a rare variant of aortic semilunar valve, often being an unexpected discovery during cardiac surgery. We present the case of a 59 years old patient, who was admitted for dyspnea on light exertion and palpitations. The transthoracic echocardiography revealed severe aortic valve regurgitation due to a quadricuspid aortic valve, the result being confirmed by the transesophageal echo examination. The patient had a first class indication for aortic valve replacement and the surgical intervention was uneventful. The operative technique and case particularities are discussed in view of the literature published so far regarding this uncommon condition.     

Quadricuspid aortic valve associated with aortic stenosis and regurgitation

A 75-year-old man with moderate aortic stenosis and regurgitation admitted due to heart failure underwent uneventful aortic valve replacement with a Carpentier-Edwards pericardial bioprosthesis valve. A quadricuspid aortic valve discovered incidentally during surgery consisted of 4 of different sizes and a supernumerary cusp between the right and noncoronary cusps. No coronary abnormality was involved. Resected cusps showed fibrotic thickening with calcification and no sign of previous inflammatory disease. Although quadricuspid aortic valve is a very rare anomaly, its potential for severe valve failure in adulthood should not be neglected.     

Aortic root replacement after operation for the ascending aorta and/or aortic valve

We reviewed ten cases who underwent aortic root replacement after operation for the ascending aorta and/or aortic valve. As initial operation, aortic valve replacement (AVR) was performed in five patients, replacement of the ascending aorta in two, original Bentall operation in two, and entry closure and suspension of the aortic valve in one. At reoperation, three patients were diagnosed as aneurysm of the ascending aorta, two were annulo-aortic ectasia, and one was acute aortic dissection, chronic dissecting aneusym, pseudoaneurysm of the ascending aorta, prosthetic valve endocarditis, and massive aortic regurgitation. Aortic root replacement was performed using mechanical valved composite graft in all cases. One patient who underwent repeat aortic root replacement for prosthetic valve endocarditis was died of septemia and ventricular fibrillation. Five patients had nine complications (two low output syndrome, respiratory failure and cerebral infarction, one gastrointestinal bleeding, septemia and ventricular fibrillation). In conclusion, aortic root replacement after operation for the ascending aorta and/or aortic valve was performed with acceptable morbidity and mortality.     

Early failure of quadricuspid aortic valve tricuspidization due to dehiscence of approximating coronary cusps

A 48-year-old woman diagnosed with aortic regurgitation showing a quadricuspid aortic valve (QAV) underwent repair with tricuspidization. Two non-coronary cusps (NCC) with fenestrations were detected intraoperatively. Tricuspidization of QAV was performed by approximating the two NCCs via interrupted sutures. The aortic annulus was plicated by subcommissural annuloplasty. The residual aortic regurgitation was mild, with a vena contracta of 2.1 mm. On postoperative day 5, echocardiography demonstrated a new regurgitation from the middle of the neo-NCC, and increased central regurgitation. Intraoperative findings showed the dehiscence of approximating sutures of the neo-NCC. Mechanical valve replacement was performed uneventfully.