Quadricuspid aortic valve: a rare cause of aortic insufficiency diagnosed by doppler echocardiography. Report of two cases and review of the literature.

Quadricuspid aortic valve is an uncommon congenital valve disease mostly occurring as isolated lesion or sometimes in association with truncal anomalies. Approximately 50% of patients with quadricuspid aortic valve have aortic regurgitation. Before the advent of echocardiography most cases were diagnosed at the time of surgery or at post-mortem examination. We describe 2 cases of patients with quadricuspid aortic valve diagnosed by echocardiography. The first case, a quadricuspid aortic valve with four equal-sized cusps (type A, according to the classification of Hurwitz and Roberts), was identified in a 26-year-old man undergoing echocardiography because of a heart murmur. The second case, a quadricuspid aortic valve with three relatively equal cusps and one smaller cusp (type B, according to the classification of Hurwitz and Roberts), was identified in a 47-year-old man with a history of murmur. The identification and periodical non-invasive evaluation of a quadricuspid aortic valve is important, because such valves are more vulnerable to infection and need adequate prophylaxis against endocarditis.     

Quadricuspid semilunar valves: report of two cases

Quadricuspid semilunar valves are uncommon congenital anomalies sometimes associated with severe valvular dysfunction. Two cases of isolated quadricuspid semilunar valve were identified. The first, a heavily calcified quadricuspid aortic valve with four equal-sized cusps, was identified in a 56-year-old man at the time of valve replacement surgery for severe aortic stenosis and regurgitation. The second valve was a quadricuspid pulmonary valve with three equal-sized cusps and one smaller cusp, identified incidentally at autopsy in a 79-year-old woman with a ruptured abdominal aortic aneurysm. Review of the literature found that while quadricuspid pulmonary valves are infrequently associated with serious clinical complications, the majority of quadricuspid aortic valves are associated with clinically significant dysfunction, most commonly aortic insufficiency. Further, contrary to previous thinking, the frequency of aortic valvular dysfunction does not appear to be related to valve morphology.     

Infective endocarditis associated with quadricuspid aortic valve

A 26-year old Japanese woman experienced new aortic valve regurgitation associated with a preceding high fever of unknown cause. During the fever episode, although bacteremia or fungemia was not evident despite frequent blood cultures, intravenous panipenem/betamipron (PAPM/BP) gradually resulted in decline of the fever. Echocardiography and operative procedures revealed a quadricuspid aortic valve (QAV), which was composed of two equal larger cusps and two unequal smaller cusps (type f). A smaller accessory cusp was damaged but showed no active vegetation. A Medtronic Freestyle bioprosthesis was implanted using a subcoronary technique. Although the risk of endocarditis for this rare valve abnormality is not well documented, the present case may support the conventional assumption that patients with unequal small cusps are prone to endocarditis.     

Quadricuspid aortic valve perforation resulting from bacterial endocarditis--2-D echo- and angiographic diagnosis and its surgical treatment

A 75-year-old man with quadricuspid aortic valve regurgitation affected by bacterial endocarditis is reported. The aortic valve consisted of 4 equal-sized cusps (type a) and a supernumerary cusp located between the right and noncoronary cusps. A right coronary ostium was close to the accessory commissure, but there was no displacement. A few small fenestrations were found at the 4 commissures and a large perforation resulting from endocarditis was observed in the noncoronary cusp. 2-D echocardiogram and angiogram suggested these findings, and they were confirmed at surgery. Successful aortic valve replacement was achieved.     

Congenital quadricuspid aortic valve associated with severe regurgitation

A 56-year-old man was referred because of severe aortic regurgitation. He had a quadricuspid aortic valve with a small accessory cusp between the right coronary and noncoronary cusps. The ostium of the right coronary artery was deviated toward the accessory cusp commissure. Aortic valve replacement was performed with a bioprosthesis. The resected cusps showed fibrotic thickening with calcification and fenestration.     

Congenital quadricuspid aortic valve associated with aortic regurgitation.

A case of quadricuspid aortic valve is described. The aortic valve consisted of 3 equal-sized and 1 smaller cusps, and a supernumerary cusp located between the right and noncoronary cusps. A right coronary ostium was close to the accessory commissure, and in a lower position. Three fenestrations were found at the supernumerary commissure. Aortic valve replacement was performed successfully with a St. Jude Medical prosthetic valve. On histological examination, the resected cusps showed fibrotic thickening with calcification and no sign of previous inflammatory disease. Quadricuspid aortic valve must be considered a malformation capable of leading to severe valve failure in later life.     

Quadricuspid aortic valve associated with severe aortic regurgitation

A 57-year old man with severe aortic regurgitation was found to have a quadricuspid aortic valve. Aortic valve replacement was performed successfully with a St. Jude Medical prosthetic valve. On histological examination, the resected cusps showed fibrotic thickening with calcification.     

Quadricuspid Pulmonary Valve Identified by Transthoracic Echocardiography

The quadricuspid pulmonary valve is a rare congenital anomaly. It tends to be clinically quiescent. Its diagnosis by two-dimensional echocardiography could be very difficult because of the anatomical features. We report on the echocardiographic findings of a 66-year-old female patient with mitral and aortic regurgitation of rheumatic origin and severe pulmonary hypertension. There was an aneurismatic dilation of the pulmonary artery trunk allowing visualization of the short-axis view of the pulmonary valve. It showed four cusps of similar size and an important deficit of central coaptation. Very few cases of the quadricuspid pulmonary valve are documented in live patients .     

Quadricuspid aortic valve by transthoracic echocardiography

A 55-year-old woman seeks medical attention with palpitations, atypical chest pain and dyspnea upon exertion, lasting for 12 months. ECG showed left ventricular hypertrophy. Transthoracic Doppler echocardiogram confirmed hypertrophy of the left chambers and showed a quadricuspid aortic valve, with equal-sized cusps, in addition to a moderate regurgitant central flow. Upon echocardiography, the quadricuspid valve is identified by its characteristic "X" form during diastole and rectangular aspect during systole. Heart failure symptoms are presumably caused by valve insufficiency due to abnormal leaflet coaptation. Considering the possibility of progressive regurgitation, follow up on the patient was performed with periodic echocardiographic control. Symptoms responded to treatment.     

A quadricuspid aortic valve in a patient with neurofibromatosis type 1

Quadricuspid aortic valve is a rare cardiovascular abnormality. Herein is described the case of a male patient with neurofibromatosis type 1 (NF1) who was found to have a quadricuspid aortic valve causing severe aortic regurgitation. Although congenital cardiac malformations have been described in patients with NF1, to the best of the present authors' knowledge this is the first time that a quadricuspid valve in a patient with neurofibromatosis has been described.     

Three-dimensional echocardiographic evaluation of an incidental quadricuspid aortic valve

Background: Quadricuspid aortic valve is one of the rare forms of congenitalcardiac valvular disease. Its diagnosis is often missed, evenwith the transthoracic echocardiogram. Many of these patientsprogress to aortic incompetence later in life requiring surgicalintervention. In addition, quadricuspid aortic valve can beassociated with other congenital cardiac deformities. Henceearly recognition and follow-up is critical in these patients. Case presentation: We report a patient with quadricuspid aortic valve identifiedon intraoperative transesophageal 3-D echocardiography. This66-year-old male presented with the features of congestive heartfailure. The preoperative transthoracic echocardiogram (TTE)disclosed, moderately severe aortic valve insufficiency alongwith severe mitral and tricuspid regurgitation, but failed toreveal the quadricuspid anomaly of the aortic valve. Interestingly,this patient had undergone transthoracic echocardiography ontwo previous occasions during the past seven years for the evaluationof his valvular heart disease, which all failed to documentthis anomaly. Intraoperatively, transesophageal echocardiography(TEE) displayed an aortic valve composed of three medium andone small cusps. Conclusion: Our patient's case demonstrates the usefulness of transesophagealechocardiography in detection of this uncommon congenital malformation.     

Quadricuspid aortic valve

We reported a case of a 45 year-old woman who had a quadricuspid aortic valve associated with moderate aortic regurgitation. The valve abnormality was detected by transthoracic echocardiography. Transesophageal echocardiography showed mild thickening of 4 symmetric aortic valve cusps, a small rectangular central regurgitant orifice, and moderate aortic insufficiency. In addition visualised this anomaly in 3D transesophageal echocardiography too.     

Dynamic evaluation of a quadricuspid aortic valve with coronary computed tomography angiography

A 35-year-old man presented with 9 years of chronic chest pain and was found to have moderate-to-severe aortic regurgitation on echocardiography. Aortic valve morphology on the initial echocardiogram was deemed normal. Computed tomography angiography was obtained for further evaluation of the aortic root dimension and anatomy. In addition to noninvasive evaluation of the coronary arteries, the higher spatial resolution and volumetric coverage of computed tomography angiography can better define the valvular and aortic anatomy. Reconstruction of retrospectively gated cine images through the aortic valve plane revealed a quadricuspid valve with 4 equal-sized cusps. Incomplete coaptation of the aortic valve cusps was seen during diastole, explaining the marked aortic regurgitation. In addition, a dilated ascending thoracic aorta (4.7 cm) was revealed. On the basis of these findings, the patient was treated surgically with a composite valve graft replacement of his aortic root.     

Quadricuspid aortic valve: report of three cases

Quadricuspid aortic valve (QAV) is a very rare congenital malformation. We have encountered three patients with QAV, of whom one patient may be the eldest reported patient with this particular anatomical abnormality. In another of our patients, there was aortic regurgitation, aortic stenosis, and healed infective endocarditis, with adhesion of the tips of the cusps. In all three patients, the cusps were all of equal size. Until now, there has been very little documented evidence about the anatomical variations in QAV or its relationship with infective endocarditis. From the available literature, we conclude that the anatomical variations in patients with QAV are similar to those in patients with quadricuspid pulmonary valve, and infective endocarditis may not be an uncommon complication.     

Quadricuspid aortic valve: an unusual echocardiographic finding and a review of the literature

This case report presents a rare clinical finding of an isolated quadricuspid aortic valve in an otherwise healthy 24-year-old female who was referred for cardiac evaluation due to newly identified murmur. A transthoracic echocardiogram suggested an unusual case of a bicuspid aortic valve with two raphes with normal opening and mild aortic regurgitation. This case exemplified that caution should be taken when encountering circumstances like the one presented, as the aortic valve anatomy might be mistaken based on a new proposed classification for bicuspid aortic valves recently published. Further evaluation identified the presence of a quadricuspid aortic valve using computer tomography of the chest. Care should be taken in evaluating aortic valves as in this case. The literature regarding quadricuspid aortic valves is reviewed.     

Quadricuspid pulmonary valve with valvular stenosis and regurgitation identified by transthoracic echocardiography: a case report

A 74-year-old man was admitted to our hospital complaining of dyspnea. Parasternal transthoracic echocardiography showed a quadricuspid pulmonary valve above the aortic valve and a hypoplastic accessory cusp between the right and left cusps of the pulmonary valve. The pulmonary valve ring diameter was normal (26 mm) but the transvalvular peak velocity was 3.5 m/sec, suggesting a pressure gradient of 49 mmHg across the pulmonary valve. The pulmonary valve had thickening and decrease in mobility of the leaflets without complete closure during diastole, and severe pulmonary regurgitation was present. Heart failure was treated successfully with digitalis and diuretics. Quadricuspid pulmonary valve is difficult to identify using transthoracic echocardiography because of the anatomical features. In this case, the dilated main pulmonary artery caused the pulmonary valve orifice to shift anteriorly, allowing visualization of the short-axis view of the pulmonary valve.     

彩色多普勒超声诊断先天性主动脉瓣病变

目的探讨各型先天性主动脉瓣病变在彩色多普勒超声的不同表现。方法通过胸骨旁左心室长轴切面和主动脉根部短轴切面,观察主动脉瓣数目、形态、回声及运动情况;通过彩色多普勒血流显像(CDFI)观察主动脉瓣的狭窄及关闭不全程度。结果主动脉瓣单叶畸型时收缩期如圆顶状突向主动脉腔,舒张期突向左心室流出道;二叶畸型时短轴收缩期呈二"字型,舒张期呈一"字型;三叶畸型时,三个瓣叶均增厚,回声强,运动受限;四叶畸型时,舒张期左心室长轴呈"型,短轴呈十"字型。同时各种主动脉瓣病变均有不同程度的狭窄、关闭不全及明显的左心室肥厚。结论超声显象可准确诊断先天性主动脉瓣病变。     

Quadricuspid aortic valve in a patient with Turner syndrome

A quadricuspid aortic valve is an uncommon congenital anomaly that is often associated with other cardiac disorders. Most reported cases of quadricuspid aortic valves are detected incidentally during necropsy or aortic valve replacement and, therefore, the potential clinical course still remains unclear. A case of a 47-year-old woman with grade III to IV aortic insufficiency and mild left ventricular dilation with an end-diastolic diameter of 59 mm is presented. During surgery for aortic valve replacement (Ross procedure), a quadricuspid aortic valve was identified. Two years after the successful Ross procedure, a molecular genetic study of this rare anomaly was performed using karyotyping, fluorescence in situ hybridisation and polymerase chain reaction. Cytogenetic analysis detected chromosomal aberration 45,X0/46,XX, indicating a low-level X chromosome mosaicism; repeat karyotypes were normal. This is the first reported case of a quadricuspid aortic valve in a woman with Turner syndrome.     

Conservative surgery in multiple cusp involvement in tricuspid valve endocarditis.

Tricuspid and mitral valve endocarditis caused by Staphylococcus epidermidis in a 57 year old previously healthy man with no history of drug abuse presented as bi-ventricular failure and multiple episodes of pulmonary emboli. He was treated for four weeks with intravenous antibiotics and had serial echocardiographic assessment of the vegetation on the tricuspid valve. This was followed by mitral valve replacement, local excision of vegetation from all the three cusps of the tricuspid valve, and autologous pericardial reconstruction of these cusps with functional assessment by perioperative transoesophageal echocardiography. Postoperative cardiac function was excellent and serial echocardiographic assessment confirmed satisfactory tricuspid valve function. This is believed to be the first recorded case in which autologous pericardial repair was used to reconstruct all the three cusps in a tricuspid valve after excision of vegetations.     

Conservative surgery in multiple cusp involvement in tricuspid valve endocarditis

Tricuspid and mitral valve endocarditis caused by Staphylococcus epidermidis in a 57 year old previously healthy man with no history of drug abuse presented as bi-ventricular failure and multiple episodes of pulmonary emboli. He was treated for four weeks with intravenous antibiotics and had serial echocardiographic assessment of the vegetation on the tricuspid valve. This was followed by mitral valve replacement, local excision of vegetation from all the three cusps of the tricuspid valve, and autologous pericardial reconstruction of these cusps with functional assessment by perioperative transoesophageal echocardiography. Postoperative cardiac function was excellent and serial echocardiographic assessment confirmed satisfactory tricuspid valve function. This is believed to be the first recorded case in which autologous pericardial repair was used to reconstruct all the three cusps in a tricuspid valve after excision of vegetations.