Quadricuspid Aortic Valve with Ruptured Sinus of Valsalva Aneurysm: a Case Report

Abstract Quadricuspid aortic valve (QAV) and sinus of Valsalva aneurysm (SVA) are rare congenital anomalies. We report an elderly patient with QAV associated with a ruptured SVA to the right atrium. Transthoracic echocardiographic and computed tomographic images are presented. We emphasize the important role of computed tomography angiography in establishing and confirming the diagnosis and facilitating treatment planning. The patient was successfully operated by a minimally invasive approach.

Estenosis aórtica severa con válvula tetracúspide: Un trébol de cuatro hojas

La válvula aórtica cuadricúspide es una anomalía congénita poco frecuente, que generalmente se presenta como una malformación aislada causando regurgitación aórtica más frecuentemente en la quinta o en la sexta década de la vida. Los primeros casos notificados se encontraron en autopsias o se hicieron evidentes en hallazgos quirúrgicos. Actualmente, la ecocardiografía y otros métodos diagnósticos por imágenes permiten un diagnóstico más temprano, incluso en pacientes asintomáticos. Se presenta el caso de un hombre de 53 años con válvula aórtica cuadricúspide diagnosticada mediante ecocardiografía y complicado con el desarrollo de estenosis valvular severa. Se incluye una breve discusión de la enfermedad.

The quadricuspid aortic valve is a rare congenital anomaly, which usually presents as an isolated malformation causing aortic regurgitation more frequently in the fifth or sixth decade of life. The first reported cases were found at autopsies or became evident in surgical findings. Currently, echocardiography and other diagnostic imaging methods allow earlier diagnosis, even in asymptomatic patients. The case of a 53-year-old man with quadricuspid aortic valve diagnosed by echocardiography and complicated with the development of severe valve stenosis is presented. A brief discussion of the disease is included.

A valva aórtica quadricúspide é uma anomalia congênita rara, que geralmente se apresenta como uma malformação isolada, causando regurgitação aórtica com maior frequência na quinta ou sexta década de vida. Os primeiros casos relatados foram encontrados em autópsias ou evidenciados em achados cirúrgicos. Atualmente, a ecocardiografia e outros métodos de diagnóstico por imagem permitem o diagnóstico precoce, mesmo em pacientes assintomáticos. O caso de um homem de 53 anos com valva aórtica quadricúspide diagnosticada por ecocardiograma e complicado com o desenvolvimento de estenose valvar grave é apresentado. Uma breve discussão sobre a doença está incluída.

A Case of Quadricuspid Aortic Valve with Multiple Fenestrations / 日本心臓血管外科学会雑誌

A 73-year-old man diagnosed with severe aortic regurgitation by transthoracic echocardiography was referred to our hospital. Quadricuspid aortic valve was diagnosed on preoperative transesophageal echocardiography. We performed aortic valve replacement with a bioprosthetic valve. In the operation, the accessory cusp was found to be located between the right coronary and noncoronary cusps, and the cusps had multiple fenestrations near the commissures.

Quadricuspid aortic valve: A rare intraoperative diagnosis by transesophageal echocardiography

Quadricuspid aortic valve (QAV) is a rare congenital anomaly frequently associated with other anomalies particularly coronary anomalies. It may be detected on transthoracic or transesophageal echocardiography. We present here a case report of a 27-year-old male patient with a QAV, the valve being regurgitant and requiring aortic valve replacement. It has been reported as isolated case reports in the literature and various theories exist to the development of QAV. The diagnosis requires a high degree of suspicion and a detailed assessment, and if asymptomatic, then patients need to be carefully followed up for the development of aortic regurgitation.

Quadricuspid Aortic Valve Complicated with Severe Aortic Regurgitation and Left-Sided Inferior Vena Cava

No abstract available.

Válvula aórtica cuadricúspide: caso clínico y discusión / Ouadricuspid aortic valve

The quadricuspid aortic valve is a rare congenital anomaly, usually presenting as an isolated malformation causing aortic regurgitation in the faith or sixth decades of life. The first reported cases were found at autopsy or became evident by surgical findings. However, the emergence of new imaging modalities currently allows an earlier diagnosis, including in asymptomatic patients. Hereby the case of a 60-year-old woman with quadricuspid aortic valve diagnosed by echocardiography is presented. A brief discussion of the disease is included.

Two Cases of Quadricuspid Aortic Valve with Aortic Regurgitation / 日本心臓血管外科学会雑誌

Congenital quadricuspid aortic valve is a very rare malformation. We report two cases with severe aortic regurgitation due to isolated quadricuspid aortic valve. It consisted of three equal cusps and one smaller cusp, which was identified at the time of valve replacement surgery for severe aortic regurgitation.

Quadricuspid Aortic Valve Associated with Mitral Regurgitation and Tricuspid Regurgitation / 日本心臓血管外科学会雑誌

Congenital quadricuspid aortic valve is a very rare malformation. Most cases have been discovered as an incidental finding at aortic valve surgery or at autopsy. It frequently evolves to aortic regurgitation, which can manifest in adulthood and may require surgical treatment. A 66-year-old man was admitted because of dysprea. Echocardiogram revealed aortic regurgitation, mitral regurgitation, and tricuspid regurgitation. We performed aortic valve replacement, mitral annuloplasty, and tricuspid annuloplasty successfully. The aortic valve showed one large, two intermediate and one smaller cusp, which were classified as typed by Hurwitz's classification. An accessory cusp was situated between the right and left coronary cusps. No coronary abnormality was involved. The postoperative course was uneventful and he is doing well 6 months after operation.

Aortic Root Replacement with a Valve Sparing Technique for Quadricuspid Aortic Valve / 日本心臓血管外科学会雑誌

A 67-year-old man with ascending aortic aneurysm was referred to our hospital. Transthoracic echocardiography showed severe aortic regurgitation with annuloaortic ectasia and transesophageal echocardiography revealed a quadricuspid aortic valve. This patient underwent aortic root replacement with a valve sparing technique. Under deep hypothermic circulatory arrest with retrograde cerebral perfusion, replacement of the ascending aorta was successfully performed. The postoperative course was uneventful. This patient is doing well 6 months after surgery without recurrence of aortic regurgitation.

A Case of Quadricuspid Aortic Valve Complicated by Infectious Endocarditis / 日本心臓血管外科学会雑誌

We report a case of quadricuspid aortic valve concomitant with infective endocarditis. A 73-year-old woman was admitted to our hospital because of general fatigue, loss of body weight and high fever. Transthoracic echocardiography showed moderate aortic regurgitation and left ventricle-right atrium fistulae with vegetation. Infectious endocarditis was diagnosed. Since her fever and hemolytic anemia were not controlled by antibiotics, we operated and the aortic valve had four cusps and there were vegetations on the aortic valve and left ventricle outflow tract. Perforation of the membranous septum was observed. Complete debridement and aortic valve replacement with patch repair of a left ventricle-right atrium (LV-RA) fistula was performed. Although she needed a permanent pacemaker due to complete AV block, her postoperative course was uneventful.

Two Cases of Quadricuspid Aortic Valve Associated with Aortic Regurgitation and Infective Endocarditis

Quadricuspid aortic valves (QAVs) is a rare congenital malformation. We report two cases of QAV associated with aortic regurgitation. The one is incidentally diagnosed and uncomplicated, and the other is symptomatic and associated with infective endocarditis. In most cases, QAVs are associated with valve regurgitation, with a concurrent stenosis in some patients, while only a small number of QAVs are functionally normal. Once the diagnosis has been made, echocardiographic follow-up is recommended, because of progression to severe valve regurgitation and the risk of infective endocarditis.

A Case of 51 Year Old Woman with Quadricuspid Aortic Valve Associated with Regurgitation

Quadricuspid aortic valve is a rare congenital malformation of the aortic valve. A case is reported of a 51 year old woman with quadricuspid aortic valve associated with regurgitation. In the past, quadricuspid aortic valve was recognised at surgery or necropsy, but now transthoracic and transesophageal echocardiography play a pivotal role in diagnosing this rare valve malformation. On occasion the transthoracic echocardiogram cannot show the quadricuspid nature of the aortic valve, and transesophageal echocardiography must be performed.

Quadricuspid Aortic Valve: Report of Two Cases

Quadricuspid aortic valve is an uncommon congenital anomaly and cause of aortic regurgitation. We report two cases of quadricuspid aortic valve with aortic regurgitation. Case 1, a 51-year-old woman was admitted to our hospital for fatigue and chest tightness. She had to and fro murmur along the left sternal border. Transthoracic echocardiography and transeso- phageal echocardiography showed grade 2-3 aortic regurgitation with quadricuspid aortic valve. The aortic valve consisted of four cusps of equivalent size(Hurwitz type a). Case 2, a 35-year-old man was admitted to our hospital for exertional dyspnea and chest tightness. He had grade 4/6 to and fro murmur along the left sternal border. Transthoracic echocardiography showed grade 4 aortic regurgitation with suggested quadricuspid aortic valve. The aortic valve consisted of two equal larger cusps and two equal smaller cusps and a supernumerary cusp located between the right and noncoronary cusps(Hurwitz type c). He was performed aortic valve replacement with a 21 mm On-X valve.

Quadricuspid Aortic Valve : Report of Three Cases and Review of the Literature

Three cases of patients with quadricuspid aortic valve diagnosed with transthoracic echocardiography are presented. Two dimensional and color Doppler echocardiography are very useful for the diagnosis of quadricuspid aortic valve. According to the types of quadricuspid aortic valve classified by the size of four cusps identified by parasternal short axis view, therapeutic plan and prognosis of different types of quadricuspid valve will be varied. Echocardiographic demonstration of quadricuspid aortic valve widens the spectrum of aortic valve diseases especially if the cause of aortic regurgitation will not be defined clearly.