A quadricuspid aortic valve discovered incidentally during surgery consisted of 4 of different sizes and a supernumerary cusp between the right and noncoronary cusps. Congenital quadricuspid aortic valve anomaly associated with hypertrophic nonobstructive cardiomyopathy. Congenital quadricuspid aortic valve is a rare cardiac anomaly. Quadricuspid aortic valve anomaly is seen generally isolated but in rare cases it may accompany coronary artery anomaly. A 75yearold man with moderate aortic stenosis and regurgitation admitted due to heart failure underwent uneventful aortic valve replacement with a carpentieredwards pericardial bioprosthesis valve. The recognition of quadricuspid aortic valve has clinical significance as it causes aortic valve dysfunction, and is often associated with other congenital cardiac abnormalities. Quadricuspid aortic valve not discovered by transthoracic. Endocarditis was diagnosed in a 69yearold previously healthy man.
None of the seven patients with quadricuspid aortic valve had associated congenital cardiac. Quadricuspid aortic valve in transposition of the great. More than half of the patients with this abnormality will develop aortic insufficiency in adulthood. The pulmonary artery and valve were normal no truncus. The aortic valve and root are then assessed to confirm the indication for valve repair or valvesparing aortic root replacement in patients with dilated root diameter 45 mm. We proceeded with transesophageal echocardiography that confirmed a quadricuspid aortic valve with severe aortic incompetence in both patients. Quadricuspid aortic valve is a rare congenital heart defect. Quadricuspid aortic valve associated with aortic stenosis. Quadricuspid aortic valve is a rare congenital heart disease with an incidence between 0.
Vegetations, related to the left coronary cusp, and root abscess were confirmed. A great majority of quadricuspid aortic valves do not have a normal function and often necessitate surgical treatment. The most common congenital anomaly of the usual trileaflet aortic valve is the bicuspid structure, which is seen in approximately 2% of the general population. If patients present clinically, it is typically in the fourth or fifth decade of life with aortic valve insufficiency. With advances in cardiac imaging, more cases are being. Bicuspid aortic valve is the most common anomaly of this kind with unicuspid valve being the second most common. Here, we present two cases of quadricuspid aortic valve. At this point, it is of paramount importance to carefully and systematically asses the valve leaflets with particular attention to the quality of tissue, cusp mobility. Quadricuspid aortic valve qav is a rare cardiac condition, with an incidence from 0. Quadricuspid aortic valve repair mastrobuoni annals of. Several different anatomical variations of a quadricuspid aortic valve has been described and aortic regurgitation is the predominant valvular dysfunction associated with quadricuspid aortic valve. Natural correspondence history progresses to aortic regurgitation. Imaging revealed a quadricuspid aortic valve with aortic regurgitation.
Quadricuspid aortic valve is a rare congenital heart defect and has been an incidental finding at open heart surgery or at autopsy. Previously this anomaly was detected accidentally during surgery or. Quadricuspid aortic valve is rare, leading mostly to aortic regurgitation. Female adolescent with quadricuspid aortic valve the. Bicuspid aortic valve is the most common with an incidence of 2%. Unfortunately, both mechanical and biologic valve replacements present prosthesisrelated complications thromboembolism, valve thrombosis, and hemorrhages related to the mandatory anticoagulation1 in mechanical valves, or valve degeneration in. Endocarditis in a quadricuspid aortic valve the annals. A 68yearold woman presented with almost a years history of increasing dyspnoea on exertion. Echocardiography revealed severe aortic regurgitation ar, a normally dimensioned left ventricle, large vegetations, and aortic root abscess.
The quadricuspid aortic valve is a very rare congenital defect that may be an isolated entity or associated with a multitude of cardiovascular abnormalities. Quadricuspid aortic valve a case report and literature. Reported incidence varies between 1 in to 1 in 10 000. Significant valvulopathy can lead to left ventricular lv dysfunction. Congenital malformations of the aortic valve are not uncommon. However, quadricuspid aortic malformations are rarely seen in the clinical practice with incidence of approximately 0. Isolated cases usually manifest late in life with the complication of aortic regurgitation, which can be delayed by early valve replacement. It is vital that patients with quadricuspid aortic valve who present with progressive aortic regurgitation undergo valve replacement or repair at appropriate time. Quadricuspid aortic valve is a rare congenital anomaly that may be found unexpectedly at surgery or diagnosed preoperatively by means of echocardiography or aortography.
Congenital quadricuspid aortic valve associated with. Association between transcatheter aortic valve replacement. Quadricuspid aortic valve with ruptured sinus of valsalva. Even rarer is its clinical presentation with aortic stenosis. In the general populations the prevalence of a quadricuspid aortic valve was reported to be in the range of 0. Tricuspidization of the quadricuspid aortic valve the. Quadricuspid aortic valves timperley 2002 clinical.
It is a defect that occurs during embryological development of the aortic trunk during gestation. Given its low estimated frequency qav characteristics, natural history, and longterm outcomes have remained poorly defined. There is an increased risk of developing postnatal aortic regurgitations and other heartrelated diseases. There was no evidence of any other congenital anomaly. Integrated transthoracic and transesophageal echocardiography enables identification and characterization of a quadricuspid aortic valve anomaly. A totally asymptomatic 40yearold white man was referred to our division of cardiology after accidental finding of a heart murmur. Bedside transthoracic doppler echocardiography revealed preserved left ventricular systolic function and possible quadricuspid aortic valve with severe aortic incompetence in both patients. We showed the important role of multimodality imaging in diagnosing a quadricuspid aortic valve associated with ruptured sinus of valsalva. Transesophageal echocardiography detected a quadricuspid aortic valve characterized by four cusps. A quadricuspid aortic valve in an asymptomatic 40yearold. Echocardiography is the imaging modality of choice, with computerized tomography or cardiac magnetic resonance imaging being utilized as adjunctive imaging modalities in certain cases.
A 47 year old female without a significant past medical history presented with a. Diagnosis of qav could be challenging but is of great importance as patients often present with progressive aortic regurgitation. Patients may have aortic regurgitation and become symptomatic at a younger age than patients with other types of aortic valve anatomy. A quadricuspid aortic valve is a rare congenital heart disease occurring in 0. Quadricuspid aortic heart valves patient information. Transesophageal echocardiography was unremarkable except for the incidental finding of a quadricuspid aortic valve figure. Although the function of quadricuspid pulmonary valve is nearly always normal, quadricuspid aortic valves frequently function abnormally. A quadricuspid aortic valve qav is an exceedingly rare congenital heart anomaly with around 200 cases reported in the literature since the first case was reported in 1862. Quadricuspid aortic valve qav is a rare congenital heart disease with pure aortic regurgitation ar as its predominant clinical manifestation. Shota hasegawa, hiroshi tanaka and yutaka okita, valvesparing aortic root replacement combined with bicuspidization for a quadricuspid aortic valve, european journal of cardiothoracic surgery, 10. We report 3 patients who underwent successful repair by tricuspidization of the aortic valve.
Quadricuspid aortic valve is a rare cardiac malformation, with an estimated incidence of 0. Pulmonary quadricuspid valve is nine times more frequently found than quadricuspid aortic valve. We report a case of a 29yearold female with newonset palpitations, diagnosed with qav and mild aortic regurgitation during. Quadricuspid semilunar valves are rare and far less common than bicuspid or even unicuspid valves. Although rare, the picture above shows an actual aortic quadricuspid heart valve. Its physical manifestations vary, and it may be associated with other cardiac lesions. Quadricuspid aortic valve qav is an anatomic valvular variant, with a prevalence of 0. Valvular insufficiency is the most common hemodynamic abnormality that is not observed in the early years of life. If you look close enough, you can see four, distinct leaflets within the valve. A quadricuspid aortic valve qav is a rare congenital heart defect characterized by the presence of four cusps, instead of the usual three found normally in the aortic valve. The right coronary artery arises from the right coronary cusp arrow and the left main artery arises from the left coronary cusp arrowhead. A quadricuspid aortic valve is an uncommon valve lesion. The quadricuspid aortic valve article pdf available in the journal of the association of physicians of india 62july.
Quadricuspid aortic valves represent an uncommon congenital abnormality. Quadricuspid morphology of the aortic valve sciencedirect. Quadricuspid aortic valve is a rare anomaly with a frequency of 0. The ratio of quadricuspid pulmonic valve to quadricuspid aortic valve was 5. Quadricuspid aortic valve qav is a rare congenital disorder. Quadricuspid aortic valve is a rare variant of abnormal development of semilunar valves. Among the 35 patients with quadricuspid pulmonic valves, there were ten patients with clinical and pathologic evidence of coexisting congenital cardiac defects, eight of which resulted in severe cyanotic heart disease in infancy. An emergency operation was performed and a true quadricuspid aortic valve qav was identified fig 1. Congenital heart defect exists from birth but is usually detected in a later stage of life, as in case of quadricuspid aortic valve. A quadricuspid aortic valve with mixed stenosis and insufficiency was identified. Here is another picture that shows a heart valve with four tissue flaps. Most cases are encountered incidentally during aortic valve surgery or autopsy.
Quadricuspid aortic valve qav is a rare congenital anomaly with an incidence of 0. Quadricuspid aortic valve europe pmc article europe pmc. Quadricuspid aortic valve is rare, with an estimated incidence of 0. Quadricuspid aortic valve qav is a rare congenital cardiac defect characterized by the presence of four aortic valve leaflets of equal or varying sizes. The patient underwent successful aortic valve replacement and was discharged home. With the advent of echocardiography and other imaging diagnostic. Bicuspid aortic valve was estimated to have a prevalence of 1% in 2004 1,2 and was more prone to early degeneration and accounted for up to 50% of patients requiring surgery in the younger population. To the best of our knowledge, there has not been any case of qav associated with pregnancy. Quadricuspid aortic valve qav is a rare congenital cardiac defect in which an aortic valve is made up of 4 aortic valve cusps instead of 3 semilunar cusps as observed in a normal aortic valve. The most common hemodynamic abnormality associated with this anomaly is aortic insufficiency. So far, approximately 200 cases have been described. Quadricuspid aortic valve surgical repair the journal of.
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