Asian Cardiovasc Thorac Ann 2008;16:e40-e41
© 2008 Asia Publishing EXchange Ltd
Congenital Quadricuspid Aortic Valve Associated With Severe Regurgitation
Yujiro Kawanishi, MD,
Hiroshi Tanaka, MD,
Keitaro Nakagiri, MD,
Teruo Yamashita, MD,
Kenji Okada, MD,
Yutaka Okita, MD
Division of Cardiovascular, Thoracic, and Pediatric Surgery, Kobe University Graduate School of Medicine, Kobe City, Japan
For reprint information contact: Yujiro Kawanishi, MD, Tel: 81 78 382 5942, Fax: 81 78 382 5959, Email: yujirokawanishi{at}hotmail.com, 7-5-2, Kusunoki-cho, Chuo-ku, Kobe City, Hyogo 650-0017, Japan.
 |
ABSTRACT
|
|---|
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.
|
INTRODUCTION
|
|---|
Quadricuspid aortic valve is a rare congenital cardiac anomaly, and an uncommon cause of aortic regurgitation. We describe the case of a patient who had severe aortic valve regurgitation with a quadricuspid aortic valve.
|
CASE REPORT
|
|---|
A 56-year-old man was referred because of severe aortic regurgitation without symptoms. Transthoracic echocardiography showed severe aortic regurgitation and a quadricuspid aortic valve. Transesophageal echocardiography confirmed quadricuspid aortic valve with an accessory cusp between the right coronary and noncoronary cusps (Figure 1
). Cardiac catheterization and aortography also showed severe aortic regurgitation. There was no other cardiac abnormality. Aortic valve replacement was performed under cardiopulmonary bypass. The excised aortic valve was found to have 3 cusps of equal size and one small accessory cusp between the right coronary and noncoronary cusps. The valve leaflets were thickened with some calcification and fenestration. The accessory cusp was prolapsed and surgical repair appeared to be difficult. The ostium of the left coronary artery was normal, but that of the right coronary artery was deviated toward the accessory cusp. The quadricuspid valve was resected and replaced with a 23-mm Carpentier-Edwards bioprosthesis (Baxter Healthcare Corp., Irvine, CA, USA) in a supra-annular position with rotation in a clockwise direction so as not to occlude the right coronary ostium with the strut. Meticulous attention was paid to preventing heart block by putting sutures into the aortic annulus itself, because the border between the aortic annulus and membranous septum was unclear. Histological examination of the cusps showed fibrotic thickening with calcification, and no evidence of any inflammatory or rheumatic disease (Figure 2). The postoperative course was uneventful, and the patient was discharged after 16 days.
View larger version (84K):
[in this window]
[in a new window]
|
Figure 1. Quadricuspid aortic valve demonstrated by transesophageal echocardiography. The accessory cusp (white arrow) is between the right coronary cusp and the noncoronary cusp. LA = left atrium, LCC = left coronary cusp, NCC = noncoronary cusp RA = right atrium, RCC = right coronary cusp.
|
|
View larger version (118K):
[in this window]
[in a new window]
|
Figure 2. The resected aortic valve with 4 cusps: 3 equal cusps and 1 smaller cusp. Acc = accessory cusp, LCC = left coronary cusp, NCC = noncoronary cusp, RCC = right coronary cusp.
|
|
|
DISCUSSION
|
|---|
Quadricuspid aortic valve is a rare congenital cardiovascular anomaly and an uncommon cause of aortic regurgitation. Autopsy series reported the incidence to be between 0.008% and 0.033%.1 Quadricuspid aortic valve is usually diagnosed at autopsy or detected accidentally during surgery, but accurate preoperative diagnosis has been possible recently with the advent of echocardiography, especially transesophageal echocardiography. In this case, transesophageal echocardiography provided the correct number and size of the aortic cusps.
Embryologically, the semilunar valves are derived from mesenchymal swellings in the aortic and pulmonary trunks following partitioning of the truncus arteriosus. It is speculated that supernumerary aortic cusp formation occurs during the early stages of truncal septation, resulting from either abnormal aortopulmonary septation or abnormal proliferation in the common trunk.1,2 Hurwitz and Roberts1 classified quadricuspid valves into 7 types depending on the relative size of the valve leaflets. Over 85% of reported cases were classified as type A, B, or C; which are valves with 4 equal cusps, 3 equal and 1 smaller cusp, or 2 equally large and 2 equally small cusps, respectively.3 The most common positions of the accessory cusp are between the right and noncoronary cusps, or between the left and right coronary cusps.4 Valvular dysfunction does not develop in childhood, but aortic regurgitation progresses later because of fibrous thickening with incomplete coaptation.1,2 Aortic stenosis sometimes coexists, but this is rare. A type A valve is considered more likely to have normal function, but there appears to be no correlation between valve function and the valve type according to leaflet size, as mentioned above, or the accessory cusp location.3,4 Quadricuspid aortic valves have been described in association with other cardiac abnormalities, such as coronary arterial abnormality, ventricular septal defect, and patent ductus arteriosus.1,3 Displacement of the coronary ostia has been reported in approximately 10% of cases, and it is important to be aware of this to prevent coronary ostial occlusion at the time of valve replacement.3 Downward displacement of the annulus has also been reported, and valve suture placement was recommended to be positioned anteriorly, high within the membranous septum.5
Aortic valve replacement is usually performed for quadricuspid aortic valve, and surgical repair has been reported in few cases. Naito and colleagues6 reported a case of successful surgical repair, converting a quadricuspid valve into a tricuspid valve by suturing the right coronary and supernumerary cusp free edges. Surgical repair with annuloplasty or valvuloplasty has resulted in failure, with conversion to valve replacement during or after the operation.7 Because of the pathological changes such as fibrous thickening and calcification in a valve with 4 cusps, valve replacement is the optimal treatment for most cases of quadricuspid aortic valve with severe regurgitation.
|
REFERENCES
|
|---|
- Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol 1973;31:623–6.[Medline]
- Davia JE, Fenoglio JJ, DeCastro CM, McAllister HA Jr, Cheitlin MD. Quadricuspid semilunar valves. Chest 1977;72:186–9.[Medline]
- Timperley J, Milner R, Marshall AJ, Gilbert TJ. Quadricuspid aortic valve. Clin Cardiol 2002;25:548–52.[Medline]
- Nakamura Y, Taniguchi I, Saiki M, Morimoto K, Yamaga T. Quadricuspid aortic valve associated with aortic stenosis and regurgitation. Jpn J Thorac Cardiovasc Surg 2001;49:714–6.[Medline]
- Pirundini PA, Balaguer JM, Lilly KJ, Gorsuch WB, Taft MB, Cohn LH, et al. Replacement of the quadricuspid aortic valve: strategy to avoid complete heart block. Ann Thorac Surg 2006;81:2306–8.[Abstract/Free Full Text]
- Naito K, Ohteki H, Yunoki J, Hisajima K, Sato H, Narita Y. Aortic valve repair for quadricuspid aortic valve associated with aortic regurgitation and ascending aortic aneurysm. J Thorc Cardiovasc Surg 2004;128:759–60.[Free Full Text]
- Yotsumoto G, Iguro Y, Kinjo T, Matsumoto H, Masuda H, Sakata R. Congenital quadricuspid aortic valve: report of nine surgical cases. Ann Thorac Cardiovasc Surg 2003;9:134–7.[Medline]
TOP
ABSTRACT
INTRODUCTION
