Successful valve repair for congenital bicuspid aortic valve and degenerated prolapsed mitral valve

A 54-year-old man with congenital bicuspid aortic valve underwent simultaneous valve repair for aortic and mitral regurgitation. Surgical technique consisted of plication of redundant aortic valve repair and mitral annuloplasty with chordal replacement. One-year follow-up transthoracic echocardiography showed no valve regurgitation. Valve repair for both bicuspid aortic valve and mitral valve regurgitation should be the first option in this subset of patients.     

A case report of double orifice mitral valve (DOMV) associated with incomplete endocardial cushion defect (ECD)

A case of DOMV associated with incomplete ECD in a 17-year old girl is presented. Preoperatively, the mitral valve was suspected to have two orifice divided by the bridging tissue with sector echocardiography. DOMV is very rare congenital malformation, and 25 cases of DOMV are reported in Japan. Two of them were diagnosed as DOMV preoperatively with sector echocardiography. Sector echocardiography is useful to diagnose DOMV and to know the shape of it preoperatively.     

Valve replacement for congenital mitral stenosis associated with double orifice mitral valve: a case report and a review of Japanese literatures

A 3-year-old girl with congenital mitral stenosis (CMS) associated with double mitral orifice valve (DOMV) underwent successfully mitral valve replacement (MVR). DOMV is a rare form of congenital cardiovascular anomaly. To our knowledge, our patient is the first infant case of CMS due to DOMV in Japan. We present an infant case of CMS associated with DOMV who needed MVR, and discuss our patient with a review of Japanese literatures.     

Diagnosis and management of the double orifice mitral valve: three case reports

Double orifice mitral valve (DOMV) is an uncommon congenital heart defect. The isolated occurrence of this anomaly is very rare and, more often, is associated with another congenital malformation, dominated by atrioventricular canal defects (AVCD). Mitral insufficiency and/or stenosis may complicate this malformation. Treatment may be summarized as abstention, surgical valve repair, or valve replacement. In here, we report three cases with DOMV. The first patient was an 18-month-old boy who was operated for severe mitral valve stenosis and mild insufficiency (in 1980s), the second was a 47-year-old female, who was operated recently for mitral valve replacement (MVR) under cardiopulmonary bypass due to severe mitral valve insufficiency. This patient had been operated due to the secundum-type atrial septal defect in our institution previously. The last one, a 3-year-old boy, with DOMV and three papillary muscles, was on clinical follow-up because he had no symptoms.     

Ascending aorta dissection associated with bicuspid aortic valve

Aortic dissection etiology involve many factors that are difficult to identify clearly. We report a 47-year-old man who underwent a Bentall operation with reattachment of bypass grafts for a dissecting aneurysm (DeBakey type II) 4 years after combined triple coronary artery bypass grafting and mitral valve replacement. This case appeared to be associated with factors leading to dissecting aneurysm although it remains unclear which was more influential congenital bicuspid aortic valve or proximal anastomosis of venous grafts or both. This case suggests the need to consider appropriate timing in surgical intervention for cases of congenital bicuspid aortic valves and the selection of additional aortic valve replacement in initial surgery.     

Outcomes of aortic valve repair according to valve morphology and surgical techniques

OBJECTIVES The aim of this study was to assess the impact of aortic valve morphology and different surgical aortic valve repair techniques on long-term clinical outcomes. METHODS Between February 2003 and May 2010, 216 patients with aortic insufficiency underwent aortic valve repair in our institution. Ages ranged between 26 and 82 years (mean 53?±?15 years). Aortic valve dysfunctions, according to functional classification, were: type I in 55 patients (25.5%), type II in 126 (58.3%) and type III in 35 (16.2%). Sixty-six patients (27.7%) had a bicuspid valve. Aortic valve repair techniques included sub-commissural plasty in 138 patients, plication in 84, free-edge reinforcement in 80, resection of raphe plus re-suturing in 40 and the chordae technique in 52. Concomitant surgical procedures were CABG in 22 (10%) patients, mitral valve repair in 12 (5.5%), aortic valve-sparing re-implantation in 78 (36%) and ascending aorta replacement in 69 (32%). Mean follow-up was 42?±?16 months and was 100% complete. RESULTS There were six early deaths (2.7%). Overall late survival was 91.5% (18 late deaths). There were 15 (6.9%) late cardiac-related deaths. NYHA functional class was ≤II in all patients. At follow-up, 28 (14.5%) patients had recurrent aortic insufficiency?≥?grade II. The freedom from valve-related events was significantly different between bicuspid and tricuspid valve implantation (P?相似文献   

Pure aortic regurgitation due to congenital bicuspid valve: a case report

A rare case of pure severe aortic regurgitation (AR) accompanied with congenital bicuspid aortic valve is presented. A 36-year-old female was admitted with rapid development of congestive heart failure. Examinations clarified severe AR with congenital bicuspid valve. Either calcification or vegetation on the valve was completely denied. At operation, both leaflets were apart from each other with a distance of 7 mm at the anterior commissura. A mechanical valve (Bj?rk-Shiley 23A) was sutured and she has been well. Pathological study of the resected leaflets revealed neither calcium deposit nor finding of infection but collagenous fibrous tissue with focal mucinous degeneration. A few cases of AR with bicuspid aortic valve have been reported in patients of annuloaortic ectasia. The partial annular ectasia was suspected as the cause of solitary aortic regurgitation with bicuspid aortic valve in our case.     

A case of ascending aortic dissection after aortic valve replacement in congenital bicuspid aortic valve]

Congenital bicuspid aortic valve is a risk factor of aortic dissection, but the case is rare in Japan. Several reports described ascending aortic aneurysm after aortic valve replacement. In these reports, most of aneurysms were false aneurysm, but the cases of ascending aortic dissection were rare. In this case, dissecting aneurysm of the ascending aorta occurred 4 years after aortic valve replacement, which was performed with mechanical prosthesis because of infective endocarditis, and it was repaired successfully by the modified Cabrol's method. This case was congenital bicuspid aortic valve, and had already been complicated with moderate aortic dilatation in the ascending aorta. In patients of congenital bicuspid aortic valve with aortic dilatation, consideration of complete replacement of the ascending aorta with aortic valve replacement is important.     

A case report of aortic valve replacement following ruptured aneurysm of the sinus of valsalva with bicuspid valve

Only three cases of the combination of bicuspid aortic valve and ruptured aneurysm of the sinus of Valsalva, associated with previously repaired coarctation of aorta, have been reported. A twenty-year-old man with a sudden onset of CHF due to ruptured aneurysm of the sinus of Valsalva underwent intracardiac repair by direct closure of the sinus Valsalva in combination with patch closure of a subarterial VSD. Although, no AR was detected preoperatively, massive reguratitation occurred after the repair due to subsequent failure of aortic valve coaptation in the present of the bicuspid aortic valve, which was not diagnosed preoperatively. Aortic valve replacement with SJM 25 mm was successfully performed.     

Anomalous origin of the right coronary artery from the pulmonary artery combined with aortic valve stenosis

We report the case of a 41-year-old patient presenting with anomalous of origin of the right coronary artery from the pulmonary artery (ARCAPA) and severe aortic valve stenosis. Surgical correction was performed by partial (T-shaped) sternotomy. After replacing the bicuspid aortic valve, the right coronary artery ostium was directly reimplanted into the aortic root. The main pulmonary artery was reconstructed using an autologous pericardial patch. Postoperative recovery was uneventful. Four cases involving an association of the bicuspid aortic valve and ARCAPA have been reported in the literature. This potential relationship should be considered whenever diagnosing a bicuspid aortic valve.     

Valve-sparing replacement of the noncoronary sinus of Valsalva in patients with a bicuspid aortic valve

Progressive dilatation of the aortic root and ascending aorta is frequent in patients with bicuspid aortic valve, and isolated dilatation of the noncoronary sinus has been reported. We describe our technique for the selective replacement of the noncoronary sinus of Valsalva in patients with bicuspid aortic valve.     

Bicuspid aortic valve.

The bicuspid aortic valve is a common congenital cardiac anomaly, having an incidence in the general population of 0.9% to 2.0% and a frequency of 54% in all patients aged >15 years with valvular aortic stenosis. In most cases it remains undetected until infective endocarditis or calcification supervenes. The bicuspid aortic valve may function normally throughout life, may develop progressive calcification and stenosis or may develop regurgitation with or without infection. The association of the bicuspid aortic valve with dissection of the aorta is also common. The recognition of the bicuspid valve in patients with aortic valve disease remains an important challenge to the clinician, whereas preoperative knowledge of valve morphology would be helpful in planning the surgery. Antibiotic prophylaxis is also recommended in such patients, since these valves are likely to become the most important intrinsic cardiac predisposition for infective endocarditis with the virtual disappearance of rheumatic fever in developed countries.     

Surgical treatment of active native valve endocarditis

A report is presented of 24 patients (23 male), mean age 38 years, who underwent surgery for active native valve endocarditis of the left heart in 1975-1988. The aortic valve was affected in all patients, and also the mitral valve in five. Pre-existing aortic valve disorder was present in 17 cases (13 congenitally bicuspid 4 rheumatic affection). There were five hospital deaths (20.8%). Staphylococci as causal organism and extensive infection predicted the highest mortality and morbidity. The mean follow-up time was 39.7 (range 2-114) months. Two reoperations because of prosthetic valve dehiscence revealed endocarditis of the implanted valve. Strong correlation was found between favourable postoperative course and rapid normalization of C-reactive protein levels, which did not fall in patients with persistent infection. Early surgery is recommended if the course of bacterial endocarditis is severely complicated.     

Ruptured mitral valve aneurysm in a patient with quadricuspid aortic valve

Mitral aneurysm is commonly associated with infectious endocarditis of the aortic valve. In rare instances, it is associated with other underlying inflammatory or metabolic disorders. A 62-year-old man with mitral valve insufficiency with moderate aortic valve insufficiency underwent operation. Operative findings were the ruptured aneurysm of the mitral valve in association with the calcified quadricuspid aortic valve. There was no evidence of infection in the mitral valve and the aortic valve. The mitral valve was repaired, the aortic valve replaced, and Cox Maze procedure added. A rare combination of mitral and aortic valve lesions without endocarditis suggested that mechanical stress alone may induce mitral valve aneurysm.     

Surgery for aortic dissection associated with congenital bicuspid aortic valve

Congenital bicuspid aortic valve is a relatively rare malformation. It is reported that the presence of this anomaly predisposes the patient to the development of dissecting aortic aneurysms. Between 1981 and October 1997, 7 patients with aortic dissection associated with congenital bicuspid aortic valve underwent surgical treatment at our institution. The patients consisted of six males and one female. The age of the patients ranged from 54 to 74 years (mean 61 years). The classification of dissecting aortic aneurysms was DeBakey type I dissection in 4 patients, type II dissection in 2 patients and type IIIb dissection in 1 patient. These 7 patients constituted 2.0% (7/356) of all cases of surgical operation for dissecting thoracic aneurysm. Aortic valve dysfunction was noted in 5 patients, 4 of whom had previously undergone aortic valve replacement. We performed graft replacement of the ascending aorta in 4 patients, graft replacement of the ascending aorta and aortic arch in 2 patients, and graft replacement of the descending aorta in 1 patient. There were no hospital deaths in any of the 7 patients. Pathological examination of surgical specimens of the aortic wall showed cystic medial necrosis in 2 patients and mucoid degeneration in 4 patients. In addition to complication by valve dysfunction, patients with congenital bicuspid aortic valve are at risk for the development of aortic dissection.     

Morphometric analysis of aortic media in patients with bicuspid and tricuspid aortic valve

BACKGROUND: Patients with bicuspid aortic valves tend to develop dilatation of the ascending aorta. The aim of this study was to analyze whether or not there is any histologic difference in the aortic media of patients with a bicuspid aortic valve or a tricuspid aortic valve. METHODS: A morphometric analysis of the wall of the ascending aorta was performed in 107 patients with bicuspid aortic valves undergoing aortic valve operations. The thickness of the elastic lamellae of the aortic media and the distances between the elastic lamellae were measured with the use of an image analysis system. The histologic specimens of the ascending aorta from 61 surgical patients with tricuspid aortic valve disease served as a control. RESULTS: The patients with bicuspid aortic valves had thinner elastic lamellae of the aortic media (2.71 +/- 0.23 microm) of the ascending aortic wall than the patients with tricuspid aortic valve disease (2.83 +/- 0.23 microm) (p = 0.006). The patients with bicuspid aortic valves also had greater distances between the elastic lamellae (27.21 +/- 8.69 microm) of the ascending aortic wall in comparison with the patients with tricuspid aortic valve disease (24.34 +/- 5.32 microm) (p = 0.033). There was no difference in the total thickness of the aortic media between the groups (p = 0.62). CONCLUSIONS: Patients with a bicuspid aortic valve had thinner elastic lamellae of the aortic media and greater distances between the elastic lamellae than patients with a tricuspid aortic valve.     

Aortic shear stress in patients with bicuspid aortic valve with stenosis and insufficiency

Objectives

Bicuspid aortic valve, characterized by valve malformation and risk for aortopathy, displays profound alteration in systolic aortic outflow and wall shear stress distribution. The present study performed 4-dimensional flow magnetic resonance imaging in patients with bicuspid aortic valve with right-left cusp fusion, focusing on the impact of valve function on hemodynamic status within the ascending aorta.

Unexpected durability of Smeloff-Cutter aortic ball valve prosthesis

We report a case in which replacement of a Smeloff-Cutter aortic ball prosthesis was required 28 years after initial implantation. A 57-year-old woman underwent aortic valve replacement with a 21-mm Smeloff-Cutter ball prosthesis and open mitral commissurotomy for aortic stenosis, aortic regurgitation, and mitral stenosis in 1973. Severe aortic regurgitation occurred in April 2001, and aortic valve reoperation combined with mitral valve replacement was successfully performed. The patient's aortic ball valve was nearly intact with perivalvular leakage probably causing the aortic regurgitation. Our experience documents longer durability for the Smeloff-Cutter prosthesis than has been reported to date.     

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.     

Surgical treatment of traumatic rupture of the bicuspid aortic valve; report of a case

A 27-year-old man was injured during a motocross game. He was suffered from dyspnea, orthopnea, and hemoptysis. The to-and-fro murmur was noticed 3 days after the accident and then the patient was admitted to our hospital. Echocardiography revealed severe aortic regurgitation. Computerized tomography also showed severe pulmonary contusions. Seventeen days after the accident the aortic valve replacement was performed. The aortic valve was anterior-posterior type bicuspid valve (fusion of right coronary cusp and light coronary cusp) and the tear was detected in the anterior cusp. The postoperative course was uneventful. Rupture of the aortic valve due to a blunt chest trauma is rare and reported in 20 cases previously in Japan. This case is the second report of traumatic rupture of the bicuspid aortic valve.