Quadricuspid aortic valve is a rare congenital heart defect and has been an incidental finding at open heart surgery or at autopsy. More than half of the patients with this abnormality will develop aortic insufficiency in adulthood. Quadricuspid aortic valve surgical repair the journal of. The ratio of quadricuspid pulmonic valve to quadricuspid aortic valve was 5. Surgical repair of regurgitant quadricuspid aortic valve. Comprehensive assessment of a quadricuspid aortic valve. The quadricuspid aortic valve ankush sachdeva, biswajit paul, ak omar fig. Unlike a bicuspid pulmonary valve, it rarely presents with clinical complications, such as valvular insufficiency or stenosis, and thus, it is most often a postmortem finding 1,3,4. Quadricuspid aortic valve qav is a rare congenital anomaly with an incidence of 0. Quadricuspid aortic valve is a rare variant of abnormal development of semilunar valves. Quadricuspid aortic valve qav is a rare congenital cardiac defect with an estimated frequency of 60 000 individual patient examinations. Severe aortic insufficiency in the background of a quadricuspid aortic valve was seen on the echocardiography figs 1 and 2.
Quadricuspid aortic valve qav is a rare congenital valve defect. 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. None of the seven patients with quadricuspid aortic valve had associated congenital cardiac. 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. Clinical course, diagnosis, echocardiography, quadricuspid aortic valve. Transesophageal echo showing zoomed color compare image of quadricuspid aortic valve type gstar marks showing four unequal cusps and aortic regurgitation jet arising from central area of noncoaptation. In infective endocarditis setting, their presence should prompt urgent surgery as patients can deteriorate rapidly. So far, approximately 200 cases have been described. Quadricuspid aortic valve qav is a rare congenital heart disease.
Qav is typed according to the size of the four cusps. Quadricuspid aortic valve is a rare congenital heart disease with an incidence between 0. Many cases are incidentally diagnosed in aortic surgeries or autopsies and it usually appears as an isolated anomaly. The cusps appeared of equal size type a, hurwitz and roberts classification, and the leaflets were thin and mobile. Aortic regurgitation due to quadricuspid aortic valve. The functional status of qav is predominantly a pure aortic regurgitation. Quadricuspid aortic valve associated with aortic stenosis. Quadricuspid aortic valve surgical repair rebeca lorca, md,a ruben alvarezcabo, md, b juan calvo, md, phd,c and jesus mar. Echocardiography was performed and showed a quadricuspid aortic valve with a central defect of coaptation fig 1a, video 1.
With the advent of echocardiography and other imaging diagnostic. Here, we present two cases of quadricuspid aortic valve. We report two unusual cases of this valvular pathology associated with a dilatatio. With advances in echocardiography, including harmonic imaging, and also the advent of. Quadricuspid aortic valve qav is a rare cardiac valvular anomaly where the aortic valve has four cusps, instead of the usual three. Repair of quadricuspid aortic valve by bicuspidization. Most cases have been discovered as incidental finding at surgery or autopsy. The quadricuspid aortic valve article pdf available in the journal of the association of physicians of india 62july. Quadricuspid aortic valve with sinus of valsalva aneurysm. Pdf quadricuspid aortic valve qav is a rare congenital heart disease. The infrequency with which quadricuspid semilunar valves are encountered is reflected in the statistics from autopsies. The high variability of the anatomy and the lack of standardized surgical techniques could explain this low percentage of valve repair. Quadricuspid aortic valve repair mastrobuoni annals of.
A 68yearold woman presented with almost a years history of increasing dyspnoea on exertion. Quadricuspid pulmonic valve qpv is a rare congenital anomaly, occurring in approximately 1 in 400 to 1 in 2,000 people, with a 2. Echocardiographic diagnosis of quadricuspid aortic valve. Get a printable copy pdf file of the complete article 9k.
Quadricuspid aortic valve not discovered by transthoracic. Although rare, the picture above shows an actual aortic quadricuspid heart valve. The 1st known case report was written by balington in 1862. 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. Quadricuspid aortic heart valves patient information. Quadricuspid aortic valve a case report and literature. Quadricuspid aortic valve is a rare congenital anomaly that may be found unexpectedly at surgery or diagnosed preoperatively by means of echocardiography or aortography. Quadricuspid aortic valve is an extremely rare appearance of congenital valvular malformation with an estimated incidence of quadricuspid aortic valve qav is a rare cardiac malformation. Most cases are encountered incidentally during aortic valve surgery or autopsy. Most cases are encountered incidentally during aortic valve. Quadricuspid aortic valve qav is a rare congenital heart disease with an incidence of 0. Quadricuspid pulmonic valve, pulmonary artery aneurysm. Quadricuspid aortic valve qav is a rare congenital cardiac disorder, with a reported incidence between 0.
Pulmonary quadricuspid valve is nine times more frequently found than quadricuspid aortic valve. Type a quadricuspid aortic valve infective endocarditis. If you look close enough, you can see four, distinct leaflets within the valve. Here is another picture that shows a heart valve with four tissue flaps. Get a printable copy pdf file of the complete article. Quadricuspid aortic valves, clinical cardiology 10. Quadricuspid aortic valve is a rare congenital heart defect. 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 complicated with severe aortic.
We report the case of a 78yearold female patient with the first ever reported quadricuspid aortic valve infective. Incidence, description and functional assessment of isolated quadricuspid aortic valves. We present the case of a 49yearold man with an abnormal aortic valve formed by four cusps three equal large cusps and one smaller cusp. Quadricuspid aortic valve associated with aortic stenosis and. 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. Previously, these were diagnosed at the time of surgery or postmortem examination. Quadricuspid aortic valve europe pmc article europe pmc. The patient underwent a repair of the defect with a tricuspidalization of the aortic valve, restoring the normal. Less well known is qavs association with coronary anomalies in 10. The general principles of aortic valve repair can also be applied to quadricuspid aortic valves. Since first reported in 1862, quadricuspid aortic valve qav remains rare with a reported incidence of 0. Quadricuspid pulmonic valve found on well exam military. Quadricuspid aortic valve qav was first described in 1862 and is the rarest malformation of the aortic valve av, with an incidence of 0. The aortic valve and root are then assessed to confirm the indication for valve repair or valve sparing aortic root replacement in patients with dilated root diameter 45 mm.
Quadricuspid aortic valve radiology reference article. Congenital quadricuspid aortic valve is a rare cardiac anomaly. Quadricuspid aortic valve qav is a congenital anomaly rarely seen in clinical practice with incidence rates ranging from 0. Pdf quadricuspid aortic valve is rare, with an estimated incidence of 0. Epidemiology the estimated incidence on necropsy at 1 in 8,000. Congenital quadricuspid aortic valve associated with. Incidence, description and functional assessment of isolated quadricuspid aortic. Since then there have been 110 reported cases of qav and we report 4 more. Midterm results in patients having tricuspidization of. Pdf rare case of quadricuspid aortic valve dusko nezic. A quadricuspid aortic valve qav is a very rare congenital morphological and functional evaluation of quadricuspid aortic valves using cardiac computed tomography inyoung song, md1, jung ah park, md1, bo hwa choi, md1, sung min ko, md1, je kyoun shin. Its diagnosis is often missed even with the use of transthoracic echocardiogram. Quadricuspid aortic valve is a rare cause of aortic insufficiency. We investigate the midterm results of aortic valve reconstruction by tricuspidization in patients with qav.
Clear delineation of the anatomy of the qav is difficult but of great clinical importance as these patients may present with. Quadricuspid aortic valve qav is a rare congenital anomaly. With advances in cardiac imaging, more cases are being. Quadricuspid pulmonic valve is a rare congenital abnormality, and because of its difficult noninvasive assessment, it is usually discovered in autopsies reported prevalence in postmortem specimens ranges from 1 in 400 to 1 in.
Quadricuspid aortic valves qav are a rare but well recognized cause of significant aortic regurgitation. There was severe functional regurgitation through the 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. Morphological and functional evaluation of quadricuspid. However, surgical records demonstrate that repair of quadricuspid valves is attempted in only 23% of patients 3. The functional status of qav is predominantly a pure aortic. Quadricuspid aortic valve is a rare congenital malformation of the 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. We analyzed the outcome of eight consecutive patients who underwent aortic valve reconstruction surgery avrs with pericardial leaflets with symptomatic quadricuspid aortic valve qav disease between december 2007 and may. Aortocardiac fistulae acf are exceptionally due to infective endocarditis. An isolated quadricuspid aortic valve is a rare clinical finding. Quadricuspid aortic valve is rare, with an estimated incidence of 0. Feldman bj, khandheria bk, warnes ca, seward jb, taylor cl, tajik aj.
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