Ann Thorac Surg 2006;81:2306-2308
© 2006 The Society of Thoracic Surgeons
Case report
Replacement of the Quadricuspid Aortic Valve: Strategy to Avoid Complete Heart Block
Paul A. Pirundini, MD
*
,
Jorge M. Balaguer, MD,
Kevin J. Lilly, CCP,
William Brian Gorsuch, PA,
Margaret Byrne Taft, MS,
Lawrence H. Cohn, MD,
Robert J. Rizzo, MD
Brigham and Women's Hospital/Cape Cod Hospital, Division of Cardiac Surgery, Hyannis, Massachusetts
Accepted for publication August 23, 2005.
* Address correspondence to Dr Pirundini, 40 Quinlan Way, Suite 204, Hyannis, MA 02601 (Email: ppirundini{at}capecodhealth.org).
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Abstract
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Quadricuspid aortic valves are rarely encountered by the cardiac surgeon during aortic valve replacement. The most common location for the supranumerary cusp is between the noncoronary and the right coronary cusp, located over the membranous septum, which can potentially increase the risk of complete heart block after valve replacement. We present three quadricuspid aortic valve replacements, one of which was complicated by complete heart block postoperatively. We suggest a strategy to possibly avoid this complication.
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Introduction
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Quadricuspid aortic valves are rarely encountered by the cardiac surgeon. Hurwitz and Roberts [1] encountered two such valves in an autopsy series of 6,000 cases for an incidence of 0.033%. During the last 13 years, 4 such cases were identified at both Brigham and Women's Hospital and Cape Cod Hospital, 3 of which required replacement of the quadricuspid aortic valve. After obtaining institutional review board approval, clinical data were obtained retrospectively through chart review. At Brigham and Women's Hospital, a total of 4,032 aortic valve replacements were performed between January 1, 1992 and April 25, 2005, 2 of which were found to be congenitally quadricuspid (0.05%). An additional quadricuspid aortic valve was identified on transthoracic echocardiogram in a patient undergoing mitral valve repair. In that case the aortic valve was hemodynamically normal.
At Cape Cod Hospital, 91 aortic valve replacements were performed between the program's inception on August 15, 2002 and April 25, 2005, 1 of which was a quadricuspid valve (1%). Also noted were 28 (31%) bicuspid valves and 1 (1%) monocuspid valve from this institution.
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Case Reports
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Patient 1
The first case involved a 72-year-old man evaluated for a history of increasing shortness of breath. The patient's preoperative transthoracic echocardiogram demonstrated critical aortic stenosis with an aortic valve area of 0.5 cm2 and a mean gradient of 72 mm Hg. The valve was initially thought to be trileaflet. A preoperative cardiac catheterization revealed significant two-vessel coronary artery disease. The patient underwent aortic valve replacement and double coronary artery bypass grafting. Intraoperative transesophageal echocardiography findings were consistent with the preoperative transesophageal echocardiography findings. On inspection a fourth aortic valve cusp was noted between the right and the noncoronary cusp (see Fig 1). After excision of the valve leaflets, the sutures in the annulus were placed with pledgets on the ventricular side of the annulus in noneverting fashion. A 23-mm Medtronic Mosaic porcine valve (Medtronic Inc, Minneapolis, MN) was utilized for a supra-annular implant. The postoperative course was complicated by the presence of complete heart block, requiring placement of a pacemaker on the second postoperative day. The patient was discharged on postoperative day 14.
Patient 2
The second case involved a 36-year-old woman with recurrent endocarditis and severe aortic insufficiency on echocardiogram. A quadricuspid aortic valve was encountered on direct inspection of the valve, with the supranumerary cusp found again between the right and noncoronary cusps. The patient underwent aortic valve replacement with a 21-mm Magna pericardial valve (Edwards LifeSciences, Irvine, CA). The valve sutures were placed in standard noneverting fashion; however the sutures placed anteriorly in the location of the supranumerary leaflet were transitioned to a supra-annular position in an effort to avoid the conduction system. The patient recovered uneventfully and was discharged to home on postoperative day 8 in sinus rhythm.
Patient 3
The third case was a 61-year-old man with a known heart murmur for more than 45 years. During the 6 months prior to surgery the patient noted increased dyspnea on exertion. A preoperative transthoracic echocardiogram demonstrated a severely calcified valve with a peak gradient of 100 mm Hg. The presence of a quadricuspid aortic valve was again not appreciated until direct visualization in the operating room during aortic valve replacement. A 23-mm St. Jude valve (St. Jude Medical Inc, St. Paul, MN) was placed by using simple interrupted sutures. The patient was discharged home on postoperative day 7 after recovering uneventfully.
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Comment
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Quadricuspid aortic valves are unusual congenital abnormalities rarely encountered by the adult cardiac surgeon. In addition to the 0.033% incidence found by Hurwitz and Roberts [1] in their autopsy series, Simonds [2], in a pooled autopsy series, found two such valves among 25,666 cases for an incidence of 0.008%.
Although not appreciated in this study, quadricuspid aortic valves are more commonly associated with insufficiency than stenosis if they become dysfunctional. In 1977, Davia and colleagues [3] reported 18 postmortem cases among which 1 (6%) was stenotic, 7 (39%) were incompetent, and 10 (58%) were described as functionally normal. Recently Naito and colleagues reported the repair of an incompetent quadricuspid aortic valve associated with an ascending aortic aneurysm by converting it to a trileaflet valve [4]. Two classification schemes have been used to describe quadricuspid valves. The first, described by Hurwitz and Roberts [1], classifies the valves based on the relative size of the supranumerary cusp relative to the remaining cusps. In this classification, the most common variant encountered were three cusps of equal size with an additional fourth smaller cusp, similar to what were encountered in the first two cases presented in this report. Nakamura and colleagues [5] designed a classification in which the focus is the position of the nondominant supranumerary cusp. The most common variation in their study was the presence of the supranumerary cusp between the right coronary and the noncoronary cusps, a variation seen in 13 of the 42 patients in their series (30.9%).
This was the anatomic variant seen in the first two operative cases presented in the present report. As previously noted, the recovery of the first patient, in whom valve sutures were all placed in standard fashion below the native annulus, was notable for the development of complete heart block postoperatively. Given the most common location of the supranumerary cusp (Fig 2), one can potentially expect an increased risk of this postoperative complication if standard valve suture techniques are used. In the case in which the valve sutures were transitioned to a supra-annular location anteriorly high within the membranous septum, no postoperative heart block was seen. The normal location of the conduction system where it traverses the lower membranous septum is between the noncoronary and the right coronary cusps, which is the most common position of the supranumerary cusp in quadricuspid aortic valves. In this situation, the annulus is displaced downward toward the muscular septum, thus increasing risk of injury to the conduction system when sutures are placed around the annulus. Valve suture placement should be transitioned to a supra-annular position at the location that corresponds to the supranumerary cusp.
Downward displacement of the annulus toward the muscular septum may also be encountered in other congenitally abnormal valves, particularly bicuspid valves fused between the right and noncoronary cusps, and similar concerns may be present.
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References
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- Hurwitz LE, Roberts WC. Quadricuspid semilunar valve Am J Cardiol 1973;31:623-626.[Medline]
- Simonds JP. Congenital malformations of the aortic and pulmonary valves Am J Med Sci 1923;166:584-595.
- Davia JE, Fenoglio JJ, DeCastro CM, McAllister HA, Cheitlin MD. Quadricuspid semilunar valves Chest 1977;72:186-189.[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 Thor and Cardiovasc Surgery 2004;128(5):759-760.
- 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;49:714-716.[Medline]
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P. A. Pirundini, K. J. Lilly, and R. J. Rizzo
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Abstract
Introduction
