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 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.     

Quadricuspid aortic valve with aortic insufficiency: case report and review of the literature

We report the case of a 42-year-old woman with aortic regurgitation discovered to be caused by a quadricuspid aortic valve (QAV) diagnosed by intraoperative transesophageal echocardiogram. With improvements in echocardiographic imaging, the diagnosis of QAV is likely to be made more reliably in the future and should prompt close clinical follow-up given the frequent association of this lesion with valvular insufficiency.     

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.     

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.     

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—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.     

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 valve reconstruction using self-developed aortic valve plasty system in aortic valve disease

Aortic valve disease is usually treated by prosthetic valve replacement. We have performed aortic valve plasty (AVP) using glutaraldehyde-treated autologous pericardium. AVP was performed for 88 patients from April 2007 through August 2009. Sixty-five patients had aortic stenosis, and 23 patients had aortic regurgitation (AR). Twenty-one patients showed bicuspid aortic valves, and one patient showed quadricuspid valve. There were 43 males and 45 females. Their mean age was 70.6±10.5 years old. First, diseased leaflets excised. Then, the distance between each commissure was measured. The new leaflet were trimmed with an original template from a glutaraldehyde-treated autologous pericardium sample. Finally, the annular margin of the pericardial leaflet was running sutured to each annulus. There was no operative mortality or embolic event. Postoperative echocardiography revealed a mean peak pressure gradient (PG) of 19.0±9.1 mmHg one week after surgery. Thirty-two patients had echocardiography one year after surgery. The peak PG became 12.9±5.8 mmHg. Ten patients showed no AR, 20 patients showed trivial AR, and two patients showed mild AR. Freedom from reoperation is 100% at three years follow-up.     

Surgery for aortic valve disease with congenital bicuspid aortic valve

From January 1998 to December 2002, a total of 120 patients underwent aortic valve surgery at our institution. Of these, 26 patients (22%) had congenital bicuspid aortic valve (BAV). Main valvular lesion of BAV was aortic valve stenosis (AS) in 17 and regurgitation (AR) in 9. There was no significant difference in the aortic annular size between BAV and non-BAV cases measured by echocardiography preoperatively [22.8 +/- 2.0 mm versus 22.5 +/- 2.2 mm in AS (NS), and 25.4 +/- 2.4 mm versus 23.4 +/- 2.5 mm in AR (NS)]. At operation, however, supraannular type prosthetic valve was selected more frequently in BAV than in non-BAV because of the difficulty of implanting the proper size prosthetic valve in annular position in BAV [10/25 (40%) in BAV versus 18/91 (20%) in non-BAV (p < 0.05)]. Moreover, ascending aortic dimension in BAV cases was significantly larger than in non-BAV cases [43.7 +/- 7.3 mm versus 39.6 +/- 7.3 mm (p<0.05)]. These features were more prominent in non-raphe BAV cases. These results suggested that operative procedure including prosthetic valve selection needs careful consideration in BAV especially no-raphe patients.     

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.