Congenital quadricuspid aortic valve, a rare cause of aortic. Quadricuspid aortic valve circulation aha journals. 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 surgical repair rebeca lorca, md,a ruben alvarezcabo, md, b juan calvo, md, phd,c and jesus mar. If you look close enough, you can see four, distinct leaflets within the valve. Quadricuspid aortic valve qav is a rare congenital anomaly. Quadricuspid aortic valve qav is a rare cardiac valvular anomaly where the aortic valve has four cusps, instead of the usual three. A 59year old lady underwent aortic valve replacement for aortic regurgitation. Earlier, the diagnosis was mostly during postmortem or intraoperative, but now with the advent of better imaging. Imaging revealed a quadricuspid aortic valve with aortic regurgitation. Quadricuspid aortic valves, clinical cardiology 10. 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.
With advances in echocardiography, including harmonic imaging, and also the advent of. A 60 polypropylene is passed through the middle of free edge of the accessory cusp and the smaller cusp. Qav is most commonly associated with aortic insufficiency ai, which is found in almost 75% of cases 5. Quadricuspid aortic valve associated with fibromuscular. In this case, the aortic valve is composed of four equalsized cusps type a qav. It is a defect that occurs during embryological development of the aortic trunk during gestation. Quadricuspid aortic valve is a rare congenital cardiac malformation, usually found as an isolated anomaly, but it may be associated with coronary artery anomalies, ventricular septal defects. The condition frequently progresses to aortic regurgitation, which can manifest in adulthood and may require surgical treatment. Tricuspid valve stenosis is most commonly caused by inflammation from rheumatic fever, a disease that is related to strep infections.
Congenital quadricuspid aortic valve is a rare cardiac anomaly. Dec 16, 2016 quadricuspid aortic valve qav is rare congenital malformation of the aortic valve with estimated prevalence of 0. Previously, these were diagnosed at the time of surgery or postmortem examination. The quadricuspid aortic valve is a rare congenital heart defect. We investigate the midterm results of aortic valve reconstruction by tricuspidization in patients with qav. Many of these patients progress to aortic incompetence later in life, hence requiring surgical intervention. 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. Its diagnosis is often missed even with the use of transthoracic echocardiogram. Quadricuspid aortic valve with ruptured sinus of valsalva. 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.
Links to pubmed are also available for selected references. Quadricuspid aortic valve 10year case series and literature. 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, md2, hyun keun chee, md2, jun seok kim, md2. Quadricuspid aortic valve surgical repair the journal of.
Quadricuspid aortic valve is a rare congenital malformation of the aortic valve. Since first reported in 1862, quadricuspid aortic valve qav remains rare with a reported incidence of 0. Repair of quadricuspid aortic valve by bicuspidization. Mar 01, 2010 quadricuspid aortic valve,aneurysm of the right sinus of valsalva and vsd. Congenital quadricuspid aortic valve associated with. Apr 15, 20 congenital quadricuspid aortic valve is a rare cardiac anomaly. Quadricuspid aortic valve is rare, leading mostly to aortic regurgitation. We showed the important role of multimodality imaging in diagnosing a quadricuspid aortic valve associated with ruptured sinus of valsalva. Since then there have been 110 reported cases of qav and we report 4 more.
Quadricuspid aortic valve is a rare malformation, with an estimated incidence of 0. Full text is available as a scanned copy of the original print version. Congenital heart defect exists from birth but is usually detected in a later stage of life, as in case of quadricuspid aortic valve. The valve was excised and replaced with an edwards perimount magna valve. Quadricuspid aortic valve qav is a rare congenital heart disease with an incidence of 0. A quadricuspid aortic valve discovered incidentally during surgery consisted of 4 of different sizes and a supernumerary cusp between the right and noncoronary cusps. With the diagnosis of aortic valve insufficiency and subvalvular fibromuscular stenosis, the patient was operated upon.
To our knowledge, this is the first report of quadricuspid aortic valve associated with idiopathic dilated cardiomyopathy. Epidemiology the estimated incidence on necropsy at 1 in 8,000. It is commonly associated with premature aortic stenosis. Most cases are encountered incidentally during aortic valve surgery or autopsy. Quadricuspid aortic valve is a rare congenital heart disease with an incidence between 0. This valvular lesion has a light predominance in the male population 2. Dec 01, 2002 quadricuspid aortic valves qav are a rare but well recognized cause of significant aortic regurgitation. 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. 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 valve associated with aortic stenosis and. 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. The most common hemodynamic abnormality associated with this anomaly is aortic insufficiency. Quadricuspid aortic valve radiology reference article.
More than half of the patients with this abnormality will develop aortic insufficiency in adulthood. The quadricuspid aortic valve ankush sachdeva, biswajit paul, ak omar fig. Quadricuspid aortic valve is a rare congenital malformation of the heart leading to significant aortic regurgitation or stenosis. Morphological and functional evaluation of quadricuspid. Pdf quadricuspid aortic valve is rare, with an estimated incidence of 0.
Quadricuspid aortic valve qav is a rare congenital lesion, generally manifesting with valve regurgitation. Here is another picture that shows a heart valve with four tissue flaps. Given its low estimated frequency quadricuspid aortic valve qav there is an accessory cusp next to a cusp left coronary cusp in our case that is smaller than the other two. All patients had aortic regurgitation ar except for one with aortic stenosis and mild regurgitation asr. The functional status of qav is predominantly a pure aortic regurgitation. Quadricuspid aortic valve anomaly is seen generally isolated but in rare cases it may accompany coronary artery anomaly. Quadricuspid aortic valve associated with idiopathic dilated. A quadricuspid aortic valve is often associated with other cardiac disorders, such as patent ductus, mitral valve malformation, hypertrophic. Quadricuspid aortic valve qav is a rare congenital cardiac defect characterized by the presence of four aortic valve leaflets of equal or varying sizes. Quadricuspid aortic valve is a rare congenital heart defect and has been an incidental finding at open heart surgery or at autopsy. Mar 30, 2016 quadricuspid aortic valve qav is a rare congenital cardiac anomaly causing aortic regurgitation usually in the fifth to sixth decade of life. Quadricuspid aortic valve qav is a rare congenital heart disease. Tricuspid valve regurgitation is caused by destruction of. Quadricuspid aortic valve qav is a rare cardiac condition, with an incidence from 0.
Aortic dilatation was present in 29% of the patients, and 26% had moderate or severe aortic valve regurgitation at the index diagnosis. Quadricuspid aortic valve, wiener klinische wochenschrift. It is vital that patients with quadricuspid aortic valve who present with progressive aortic regurgitation undergo valve replacement or repair at appropriate time. All patients were free of other cardiac anomalies including. 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. Read quadricuspid aortic valve, wiener klinische wochenschrift on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at. Quadricuspid aortic valve is rare, with an estimated incidence of 0. Midterm results in patients having tricuspidization of the. Of the 114 cases reported, 46 had the aortic valve replaced, most commonly in the 5th and 6th decade of life. Quadricuspid aortic valve is a rare congenital heart defect. So far, approximately 200 cases have been described.
A great majority of quadricuspid aortic valves do not have a normal function and often necessitate surgical treatment. Its diagnosis by transthoracic echocardiography is difficult. Most cases are encountered incidentally during aortic valve. A quadricuspid aortic valve is usually isolated but can be occasionally associated with other cardiac anomalies, such as. Quadricuspid aortic valve is a rare variant of abnormal development of semilunar valves.
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 the biologic. The functional status of qav is predominantly a pure aortic. Clear delineation of the anatomy of the qav is difficult but of great clinical importance as these patients may present with. Quadricuspid aortic valve europe pmc article europe pmc. In the case described in this report, a 61yearold woman is presented with the features of congestive heart failure. Female adolescent with quadricuspid aortic valve the. The authors present a case of this anomaly in a young, asymptomatic adolescent who presented to the echocardiography laboratory for further evaluation of a cardiac murmur detected on routine physical examination.
Quadricuspid pulmonic valve found on well exam military. Standard treatment involves valve replacement, though anecdotal cases of successful repair by means of valve tricuspidization have been reported. Apr 23, 2015 quadricuspid pulmonic valve qpv is a rare congenital anomaly, occurring in approximately 1 in 400 to 1 in 2,000 people, with a 2. Jul 04, 2009 although rare, the picture above shows an actual aortic quadricuspid heart valve. Problems with the tricuspid valve may develop over time acquired. Quadricuspid aortic valve a case report and literature. Congenital quadricuspid aortic valve associated with aortic. When the aortic valve was exposed at operation, it showed a quadricuspid valve with three cusps of equal size and a small fourth cusp situated between the right and the noncoronary cusp fig 1. However, quadricuspid aortic malformations are rarely seen in the clinical practice with incidence of approximately 0.
Where the normal aortic valve is constructed of 3 cusps or leaflets, it is more common to find an abnormal bicuspid valve. Natural correspondence history progresses to aortic regurgitation. Tricuspid valve disease the patient guide to heart, lung. Aortic regurgitation due to quadricuspid aortic valve case. Get a printable copy pdf file of the complete article 9k, or click on a page image below to browse page by page. Her preoperative transthoracic echocardiogram showed a quadricuspid aortic valve fig. 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. Pdf quadricuspid aortic valve qav is a rare congenital heart disease.
Here, we present two cases of quadricuspid aortic valve. Quadricuspid aortic valve qav is a rare congenital cardiac defect characterized by the presence of four aortic valve leaflets of equal or varying. Quadricuspid aortic valve is a rare congenital anomaly that may be found unexpectedly at surgery or diagnosed preoperatively by means of echocardiography or aortography. Hurwitz and roberts classified quadricuspid valves. Quadricuspid aortic heart valves patient information. Quadricuspid aortic valve european journal of cardio. This case demonstrates a patient with quadricuspid aortic valve qav, which is a rare congenital valvular anomaly wherein there are four cusps, instead of the usual three, in the aortic valve. Pulmonary quadricuspid valve is nine times more frequently found than quadricuspid aortic 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.
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