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J Thorac Cardiovasc Surg 2002;123:348-349
© 2002 The American Association for Thoracic Surgery
Brief Communications |
From the Departments of Cardiothoracic Surgery and Anatomy & Embryology, Leiden University Medical Centre, Leiden, The Netherlands.
Received for publication March 15, 2001. Accepted for publication April 27, 2001. Address for reprints: M. G. Hazekamp, MD, PhD, Department of Cardiothoracic Surgery, D6-S, Leiden University Medical Centre, PO Box 9600, 2300 RC, Leiden, The Netherlands (E-mail: M.G.Hazekamp{at}lumc.nl).
Quadricuspid semilunar valves are rare and far less common than bicuspid or even unicuspid valves.
1 The most common anomaly associated with quadricuspid semilunar valve is truncus arteriosus, in which the frequency of the truncal valve being quadricuspid is reported to be around 20% to 25%. 2 In nontruncus hearts, quadricuspid semilunar valves are extremely uncommon. Quadricuspid semilunar valves are more often pulmonary than aortic valves. 3 All reported quadricuspid aortic valves have been found in hearts with ventriculoarterial concordance. To the best of our knowledge, this is the first report of a quadricuspid aortic valve in a heart with ventriculoarterial discordance.
Description of specimen
In the Leiden collection of heart specimens, we encountered a specimen with a quadricuspid aortic valve in the presence of ventriculoarterial discordance and ventricular septal defect. The anatomy of this heart is as follows:
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Discussion
Semilunar valves with an abnormal number of leaflets may cause clinical symptoms because they may be or may become stenotic or incompetent. 4,5 Furthermore, abnormal semilunar valve anatomy leads to earlier and accelerated calcific leaflet degeneration. In the era of surgical techniques such as correction of truncus arteriosus or the Ross and the Nikaidoh procedures for complex congenital heart disease (involving translocation of semilunar valves), a correct diagnosis of semilunar valve anatomy is of great importance. Insufficient quadricuspid valves in truncus arteriosus must be repaired, and a quadricuspid pulmonary valve is a contraindication to the Ross procedure. Repair of a quadricuspid valve may involve tailoring or removal of the smaller accessory cusp.
Quadricuspid valves are very uncommon and have been reported to occur 9 times more frequently in the pulmonary than in the aortic position. 3 Necropsy series showed an incidence of quadricuspid valves of around 0.003% to 0.008%. 6 Aortic regurgitation was the most prevalent hemodynamic abnormality (56%) according to the review of Janssens, Klues, and Hanrath 1 with 70 hearts revised from 1923 to 1994.
The question may arise whether we are dealing with a true quadricuspid aortic valve. Knowledge of the developmental background of valve formation, such as factors that regulate commissure and leaflet formation, is insufficient to answer this question. Moreover, a coronary artery may arise from a pulmonary sinus. On the basis of the embryonic background of the great arteries, the aorta being defined by its connection to the fourth pharyngeal arch arteries and the pulmonary trunk by its connection to the sixth arch arteries, we have to conclude that we are dealing with a true quadricuspid aortic valve.
Although a quadricuspid valve is usually an incidental autopsy or intraoperative finding, it may be diagnosed by means of noninvasive techniques such as 2-dimensional echocardiography or magnetic resonance imaging. Echocardiographic identification is easier in the presence of 4 cusps of equal size (X-shaped semilunar valve pattern on short axis) and is more difficult in cases with a small accessory cusp between the right and noncoronary cusps, which is the most frequent morphologic pattern. 6 This pattern is more likely to result in valvular insufficiency or endocarditis because of unequal distribution of stress and abnormal leaflet coaptation, according to Feldman and associates. 5
Only a few reports have been published concerning the association of quadricuspid valves with other cardiac defects. Of these, anomalies of the coronary ostia and coronary arteries are the most frequent. In the review by Janssens, Klues, and Hanrath, 1 only 6 of 70 hearts were associated with abnormalities other than coronary anomalies: 2 with ventricular septal defect, 1 with persistent ductus arteriosus, 1 with fibromuscular subaortic stenosis, 1 with anterior mitral leaflet anomaly, and 1 with hypertrophic nonobstructive cardiomyopathy.
In conclusion, we want to add one more major congenital defect—transposition of the great arteries—to those associated with quadricuspid valves to emphasize the rarity of this condition. Even more unusual, in this case the quadricuspid aortic valve is associated with a unicuspid pulmonary valve in the setting of polyvalvular disease.
References
This article has been cited by other articles:
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  M. Hazekamp, F. Portela, and M. Bartelings The optimal procedure for the great arteries and left ventricular outflow tract obstruction.: An anatomical study Eur. J. Cardiothorac. Surg., May 1, 2007; 31(5): 879 - 887. [Abstract] [Full Text] [PDF]
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 M. Ricci, G. A. Cohen, E. Kocyildirim, S. Khambadkone, and M. J. Elliott Transposition of the Great Arteries and Quadricuspid Pulmonary Valve: An Unusual Combination Ann. Thorac. Surg., April 1, 2005; 79(4): 1428 - 1430. [Abstract] [Full Text] [PDF]
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