Quadricuspid aortic valve—Report of a case associated with severe aortic regurgitation and review of the literature—

A case of a 59-year-old woman who had a quadricuspid aortic valve associated with severe aortic regurgitation is reported herein. The anomaly was revealed by aortography, after which the incompetent valve was excised and replaced by a St. Jude Medical prosthesis. A quadricuspid aortic valve must be considered as a malformation which leads to severe valve failure in later life.     

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 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.     

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 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.     

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.     

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.     

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 complicated with infective endocarditis: report of a case

Congenital quadricuspid aortic valve is a rare cardiac malformation with an unknown risk of infective endocarditis. We report a case of quadricuspid aortic valve complicated with infective endocarditis. A 53-year-old Japanese woman was hospitalized with leg edema and a fever of unknown origin. Corynebacterium striatum was detected in the blood culture. Echocardiography demonstrated a quadricuspid aortic valve with vegetation and severe functional regurgitation. The condition was diagnosed as a quadricuspid aortic valve with infective endocarditis, for which surgery was performed. The quadricuspid aortic valve had three equal-sized cusps and one smaller cusp (type B according to Hurwitz classification). We dissected the vegetation and infectious focus and implanted a mechanical valve. Following the case report, we review the literature.     

Valve replacement via upper partial sternotomy for quadricuspid aortic valve

A 52-year-old man was referred to our clinic because of chronic heart failure. A Levine 3/6 diastolic heart murmur was audible at the apex. Chest radiography showed an enlarged left ventricle. Transthoracic echocardiography showed moderately severe aortic regurgitation. Left ventricular end-diastolic/systolic diameter and ejection fraction were 75/59 mm and 41 %, respectively. Preoperative transesophageal 3-dimensional echocardiography revealed a quadricuspid aortic valve whose cusps were of almost equal size. Aortic valve replacement was performed via upper partial sternotomy.     

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.     

Quadricuspid aortic valve--report of a case associated with severe aortic regurgitation and review of the literature

A case of a 59-year-old woman who had a quadricuspid aortic valve associated with severe aortic regurgitation is reported herein. The anomaly was revealed by aortography, after which the incompetent valve was excised and replaced by a St. Jude Medical prosthesis. A quadricuspid aortic valve must be considered as a malformation which leads to severe valve failure in later life.     

A case of tuberculous endocarditis with acute aortic valve insufficiency and annular subvalvular left ventricular aneurysm

Tuberculous endocarditis is an exceptionally rare disease, and its surgical operation has been reported in only one case. This is a successful surgical report of acute aortic insufficiency caused by tuberculous endocarditis associated with annular subvalvular left ventricular aneurysm (ASLVA) beneath the aortic valve. This patient had acute left ventricular failure and cardiorespiratory arrest and suffered from multiple organ dysfunction and coagulopathy disorder. Urgent surgery was performed to replace the aortic valve with the approximation of ASLVA. Histopathological findings of the excised aortic cusps gave the diagnosis of tuberculous endocarditis, and antituberculous drug administration started on the first postoperative day. Postoperative recovery has been uneventful without relapse of tuberculosis for 7 postoperative years.     

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).     

Severely calcified bicuspid aortic valve stenosis after valve-sparing root replacement: A word of caution

A 58-year-old man was admitted for reoperation for severe aortic stenosis in a previously preserved bicuspid aortic valve (BAV). He had undergone valve-sparing root replacement (VSSR) for dilated aortic root 6 years ago. Transesophageal echocardiography following VSSR showed good valve function with no aortic incompetence. However, the BAV became stenotic causing shortness of breath. At reoperation, the preserved BAV was noted to be fibrotic and calcified and had a fixed rigid small orifice. It was replaced with a biological valve plus root enlargement. Macroscopic finding showed thickening of the cusps and nodular calcification. Microscopic examination revealed severe nodular calcification.     

Quadricuspid aortic valve

A case of quadricuspid aortic valve is described. The anomaly was unexpectedly found during operation for aortic valve replacement in a 70-year-old woman. Macroscopic and histological examinations of the valve showed no sign of previous inflammatory disease. Quadricuspid aortic valve must be considered, therefore, a malformation capable of leading to severe valve failure in later life.     

Ascending aortic aneurysm associated with a quadricuspid aortic valve

We report here a rare case of ascending aortic aneurysm associated with a tricuspitalized quadricuspid aortic valve. A 45-year-old man had a fusiform ascending aortic aneurysm with aortic valve regurgitation. Transthoracic echocardiography revealed grade III aortic regurgitation. Chest computed tomography showed an ascending aortic aneurysm with a diameter of 48 mm. Surgery revealed that the aortic valve was a tricuspitalized quadricuspid aortic valve with an accessory cusp between the right coronary cusp and left coronary cusp.     

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.     

Acute occlusion of left coronary ostium associated with congenital quadricuspid aortic valve

A 10-year-old girl suffered from chest discomfort during exercise. She experienced an acute myocardial infarction accompanied by an obstruction in an orifice to the left coronary artery. An emergency operation revealed that the aortic valve was quadricuspid, and a left-sided small cusp had adhered to the aortic wall resulting in an unusual sac with a tiny slit. Resection of the sac disclosed the ostium to left coronary artery. Blood toward the left coronary artery passed through the tiny slit. The mechanisms of obstruction in the coronary artery were uncertain. She recovered well after the aortic valve replacement with resection of the aortic cusps.     

Technical modification of aortic valve replacement in aortitis syndrome —Report of a case—

In patients who have aortitis syndrome associated with aortic regurgitation, aortic valve replacement (AVR) is frequently complicated by perivalvular leakage (PVL). A 47-year-old male with aortitis syndrome and severe aortic regurgitation was treated by AVR, modified in such a way as to prevent PVL. The aortic cusps were not resected and were placed between Teflon pledgets and the aortic annulus. We believe that this technique is useful for preventing PVL in patients with aortitis syndrome in which the aortic annulus is severely inflammed.