Ann Thorac Surg 2008;85:1087-1089. doi:10.1016/j.athoracsur.2007.09.016
© 2008 The Society of Thoracic Surgeons
Case Reports
Tricuspidization of the Quadricuspid Aortic Valve
Kathrin I. Schmidt, MD,
Michael Jeserich, MD,
Diana Aicher, MD,
Hans-Joachim Schäfers, MD, PhD*
Department of Thoracic and Cardiovascular Surgery, University Hospitals of Saarland, Homburg/Saar, Germany
Accepted for publication September 11, 2007.
* Address correspondence to Dr Schäfers, Department of Thoracic and Cardiovascular Surgery, University Hospitals of Saarland, Homburg/Saar, D-66421, Germany (Email: h-j.schaefers{at}uniklinikum-saarland.de).
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Abstract
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Quadricuspid aortic valves represent an uncommon congenital abnormality. Patients may have aortic regurgitation and become symptomatic at a younger age than patients with other types of aortic valve anatomy. We report 3 patients who underwent successful repair by tricuspidization of the aortic valve.
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Introduction
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Congenital quadricuspid aortic valve is an uncommon developmental abnormality usually diagnosed incidentally at autopsies or during cardiac surgery [1]. With advances in imaging techniques, this malformation has been identified more frequently by echocardiography or magnetic resonance imaging. The most common hemodynamic abnormality associated with this finding is aortic regurgitation [2]. If cardiac surgery is required, the valve is usually replaced. Current valve substitutes, however, are not ideal for young patients. We report 3 cases of patients with quadricuspid aortic valve who underwent aortic valve repair.
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Case Reports
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Patient 1
Patient 1 was a 45-year-old woman with aortic regurgitation known since the age of 20 years who presented with increasing exertional dyspnea (New York Heart Association II). Transthoracic echocardiography demonstrated moderate aortic regurgitation and mild mitral regurgitation with a left ventricular end-diastolic diameter of 60 mm. Transesophageal echocardiography revealed a quadricuspid aortic valve. The patient was operated on through a median sternotomy. The aorta was opened through a transverse aortotomy, and the quadricuspid nature of the aortic valve was confirmed. The dimensions of noncoronary and right coronary cusps were near normal. In addition to a smaller left coronary cusp, we found a rudimentary accessory cusp, and both were separated by a commissure of subnormal height (Fig 1A). The mechanism of regurgitation appeared to be cusp restriction, particularly in the area of left and accessory cusps. To correct restriction, a decision was made to convert the valve into a tricuspid one. After detaching the rudimentary commissure, the accessory and the left coronary cusps were sutured together to form one common cusp. The free margin of the neocusp was several millimeters lower in its central part compared with the two other cusps. A triangular patch of pericardium was inserted to correct this difference (Fig 1B). The aortic root was stabilized by subcommissural plicating sutures. Intraoperative transesophageal echocardiography revealed only minimal regurgitation. The patient recovered well and has been asymptomatic since. Transthoracic echocardiography 2 and a half years later confirmed the stability of the intraoperative result.
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Fig 1. (A) Quadricuspid aortic valve with a rudimentary accessory cusp (arrow) in addition to the left coronary one, separated by a commissure of subnormal height. (B) The two left coronary cusps have been connected to one new cusp after detachment of the rudimentary commissure. The neocusp was augmented by a pericardial patch to achieve sufficient cusp height in its central part.
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Patient 2
Patient 2, an asymptomatic 45-year-old man with moderate to severe aortic regurgitation was referred because of an increase in left ventricular end-diastolic diameter to 65 mm. Intraoperatively, the valve was found quadricuspid with four commissures of normal height. Left and noncoronary cusps were normal in size. The right coronary cusp consisted of two separate cusps with partial fusion (Fig 2A). There were fenestrations of cusp tissue in this area of fusion, and prolapse of the two right coronary cusps appeared as the most likely mechanism of regurgitation. The accessory commissure was detached, and a triangular resection was performed in the fused area between the two cusps. The valve was converted into a tricuspid one by adapting the right coronary and the accessory cusp tissue (Fig 2B). The aortic root was plicated below the two commissures of the right coronary cusp. Intraoperative transesophageal echocardiography demonstrated only minimal regurgitation. Two years postoperatively, the patient is doing well with minimal aortic regurgitation and a left ventricular end-diastolic diameter of 53 mm.
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Fig 2. (A) Quadricuspid aortic valve with two partially fused cusps in the location of the right coronary cusp. (B) Tricuspidization of the valve by detaching the accessory commissure and a triangular resection of cusp tissue. The two cusps are adapted by interrupted sutures.
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Patient 3
Another quadricuspid aortic valve with regurgitation was found in a 45-year-old woman with mild dyspnea on exertion (New York Heart Association II). Aortic regurgitation was the only cardiopulmonary abnormality. Transesophageal echocardiography revealed regurgitation grade III and a quadricuspid aortic valve with a left ventricular end-diastolic diameter of 55 mm. Intraoperatively, two separate cusps instead of one right coronary cusp were seen. As in patient 1, restriction and thickening of cusp tissue were the most probable mechanisms of regurgitation. The rudimentary commissure was detached, and two cusps were adapted. To augment the tissue, a triangular pericardial patch was inserted, resulting in three cusps of identical height. Intraoperative transesophageal echocardiography revealed only minimal regurgitation. The postoperative course was uneventful, and transthoracic echocardiography before discharge and 6 months after surgery confirmed the intraoperative findings.
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Comment
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Quadricuspid morphology represents a rare but possible cause of aortic regurgitation. Patients with this abnormality usually become symptomatic at a relatively young age [1]. Replacement is not an optimal solution for these young patients, because they are exposed to valve-related risks such as thromboembolism, prosthetic valve degeneration or endocarditis, and bleeding. Repair of the aortic valve could be a promising option, if a reproducible technique could reliably address the mechanism of aortic regurgitation. In our patients, we observed apparent restriction and cusp prolapse as underlying causes. It is unclear whether these pathologic components are inherent to the quadricuspid morphology or develop secondarily.
Valve repair has been performed in quadricuspid valves of truncus arteriosus [3–6]. So far, different approaches have been used, and no data have been published regarding durability of repair. Our experience with bicuspid and tricuspid aortic valves has shown that valve competence was more easily obtained with fewer coaptation lines [7]. We therefore reduced the number of coaptation lines by converting the quadricuspid valve to a tricuspid one. Along this concept, we detached one commissure and created one new cusp out of two, thus also alleviating restriction. If cusp tissue of the joined cusp is insufficient to reach the free margins of the two other cusps, the neocusp may be augmented by autologous pericardium. Prolapse, if present, can easily be corrected by shortening of the cusp margin [7].
Since we have used the identical approach in the repair of two truncus arteriosus valves with similar result, we disagree with Van Son and colleagues [8], who suggested that aortic valves with more or less than three cusps are less amenable to repair. In conclusion, tricuspidization of a quadricuspid aortic valve is a reproducible reconstructive approach.
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References
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- Van Son JAM, Reddy VM, Black, MD, Rajasinghe H, Haas GS, Hanley FL. Morphologic determinants favoring surgical aortic valvuloplasty versus pulmonary autograft aortic valve replacement in children J Thorac Cardiovasc Surg 1996;111:1149-1157.[Abstract/Free Full Text]
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Abstract
Introduction
