Ann Thorac Surg 2005;80:1110-1111
© 2005 The Society of Thoracic Surgeons
Case report
Ross Procedure in a Quadricuspid Aortic Valve
Martin Misfeld, MD
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,
Felix Christiansen, MD,
Hans-H. Sievers, MD
Clinic of Cardiac Surgery, University Clinic of Schleswig-Holstein, Campus Luebeck, Luebeck, Germany
Accepted for publication February 18, 2004.
* Address reprint requests to Dr Misfeld, Clinic of Cardiac Surgery, University Clinic of Schleswig-Holstein, Campus Luebeck, Ratzeburger Allee 160, 23538Luebeck, Germany; (Email: martinmisfeld{at}yahoo.com).
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Abstract
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A quadricuspid aortic valve is a rare congenital cardiovascular abnormality, and when present, it is associated with aortic valve regurgitation. If aortic valve replacement is required, mechanical or biological prostheses are used. We report the case of a patient with a severely regurgitant quadricuspid aortic valve in whom a Ross procedure was performed.
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Introduction
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In contrast to bicuspid aortic valve abnormality, which occurs in 1% to 2% of the population and is the most common congenital heart defect [1], quadricuspid aortic valves have been reported to have an incidence rate of 0.008% at autopsy [2]. In most of the cases, these valves were associated with valve regurgitation and to a minor extent with valve stenosis [3, 4]. In addition, coronary ostia displacement has been reported to be associated with these valves in 8.8% of cases [5]. Therefore, aortic valve replacement in patients with quadricuspid aortic valves requires specific surgical considerations, especially if a Ross procedure is to be performed.
A 47-year-old woman was referred to the University Clinic of Schleswig-Holstein with aortic regurgitation grade III to IV, although clinically the patient remained asymptomatic during normal life. On auscultation, a diastolic decrescendo and a systolic crescendo-decrescendo murmur was evident. A chest roentgenogram showed the presence of left-sided heart cardiomegaly without signs of pulmonary congestion. Echocardiography demonstrated mild left ventricular dilatation with an end-diastolic diameter of 59 mm (35 to 56 mm), an end-systolic diameter of 30 mm (26 to 42 mm), a left ventricular end-diastolic volume of 112 mL/m2 (62 mL/m2) and an end-systolic volume of 33 mL/m2 (27.3 mL/m2). The diameter of the left ventricular outflow tract was 23.9 mm, and the dimensions of the pulmonary valve at different levels were annulus, 25.6 mm; sinus, 33.4 mm; and sinotubular junction, 24.3 mm. The ejection fraction was calculated to be 71% (45% to 90%). The aortic valve leaflets showed degenerative abnormalities without calcification. All other heart valves were normal. Left-sided heart catheterization confirmed the clinical and echocardiographic diagnosis. The coronary arteries were normal, and the ostia did not show any abnormalities with regard to their location.
Intraoperatively, four aortic valve leaflets could be identified; two were larger and two smaller. The larger ones represented the right coronary and the noncoronary leaflet. The two smaller leaflets together had the size of a normal left coronary leaflet. The additional commissure was located in the position of the left coronary ostium. Interestingly, this commissure was partly divided and attached to the wall of the aortic root on each side of the left coronary ostium at its upper part, giving the impression of almost five commissures in total. All aortic valve leaflets were thickened. There was no evidence of a perforation, calcium deposition, arteriosclerotic ulceration, or sequelae of previous valve inflammation. The coronary ostia were in a normal location and beneath the sinotubular junction. No other associated cardiac abnormality was observed.
After the leaflets were carefully excised, with special care to the area of the left coronary ostium, we used a subcoronary technique to perform a Ross procedure. The commissures A, B, and C (Fig 1) were used as targets for the commissures of the pulmonary autograft. This allowed an even scalloped shape at the proximal suture line, which was performed with interrupted 4-0 Cardiofil sutures (Tyco, Melsungen, Germany). Commissure D (Fig 1) was carefully excised and covered completely by the sinus wall of the autograft. The distal suture line was performed with 5-0 Prolene continuous sutures (Ethicon, Norderstedt, Germany), running close to the bottom edge of the left coronary ostium. Early postoperative echocardiography revealed mild regurgitation of the pulmonary autograft in the aortic position and trivial regurgitation of the homograft in the pulmonary position. The postoperative course was uneventful.
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Fig 1. Intraoperative view on a quadricuspid aortic valve. Two larger leaflets represent the right and the noncoronary leaflet. The two smaller leaflets together represent the left coronary leaflet. The commissures A, B and C are in the position of the commissures of the pulmonary autograft. The additional commissure D is located close to the left coronary ostium as indicated by an arrow.
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Comment
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Quadricuspid aortic valves are uncommon but a well-recognized congenital abnormality. Most of these valves, if clinically apparent, are associated with aortic regurgitation [3–7]. This regurgitation however, does not seem to be automatically associated with these valves, because quadricuspid aortic valves seem to be competent in childhood but develop incompetence later in life [4]. It can be speculated that abnormal valve motion and stress distribution may lead to early fibrotic changes and therefore to later valve dysfunction.
In 1973, Hurwitz and Roberts classified quadricuspid aortic valves according to leaflet size [4]. Using their classification, we determined our patient had a type C quadricuspid aortic valve with two larger and two equally smaller leaflets. The coronary ostia were not displaced. If the ostia had been displaced, aortic valve replacement, especially with the Ross procedure, would have been demanding because of the repositioning of the coronary ostia within the autograft. Challenging though, in our case, was that the left coronary ostium was enclosed in the commissure, which was divided.
Reconstruction of the valve was considered but rejected because of the thickened coaptating edge of the leaflets. The decision was made to perform a Ross procedure. As it is the policy of the institution, we used the subcoronary technique to decrease the risk of aortic root dilatation. It has been suggested that in patients with bicuspid aortic valves, histologic abnormalities of the ascending aorta may explain root and ascending aortic dilatation following the Ross procedure using the full root technique [8]. As patients with quadricuspid aortic valves also have an abnormality of the valve, it can be speculated that their risk of dilatation may also be increased following the full root technique, which further supports the strategy of a subcoronary technique. In addition, the subcoronary technique further enables the precise attachment of the partly trimmed sinus of the pulmonary valve, allowing for complete covering of the rough surface of the excised commissures in the area of the left coronary ostium.
Patients with a quadricuspid aortic valve generally have a mechanical or biological valve implanted. However, a Ross procedure can be performed safely in these patients, if specific abnormalities that have been associated with these valve types are not present to contraindicate the technique. We advocate the use of the subcoronary technique to decrease the risk of aortic root dilatation, since histologic changes may be associated with this valve abnormality in a similar way to that which has been described in bicuspid aortic valves.
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References
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- Ward C. Clinical significance of the bicuspid aortic valve Heart 2000;83:81-85.[Free Full Text]
- Simonds JP. Congenital malformations of the aortic and pulmonary valves Am J Med Sci 1923;166:584-595.
- Davia JE, Fenoglio JJ, De Castro CM, McAllister HA, Cheitlin MD. Quadricuspid semilunar valves Chest 1977;72:186-189.[Abstract/Free Full Text]
- Hurwitz LE, Roberts WC. Quadricuspid semilunar valve Am J Cardiol 1973;31:623-626.[Medline]
- Timperley J, Milner R, Marshall AJ, Gilbert TJ. Quadricuspid aortic valves Clin Cardiol 2002;25:548-552.[Medline]
- Sievers HH, Regensburger D, Bernhard A. Quadricuspid aortic valve with significant insufficiency Thorac Cardiovasc Surg 1982;30:44-45.[Medline]
- Yotsumoto G, Iguro Y, Kinjo T, Matsumoto H, Masuda H, Sakata R. Congenital quadricuspid aortic valvereport of nine surgical cases. Ann Thorac Cardiovasc Surg 2003;9:134-137.[Medline]
- de Sa M, Moshkovitz Y, Butany J, David TE. Histologic abnormalities of the ascending aorta and pulmonary trunk in patients with bicuspid aortic valve diseaseclinical relevance to the Ross procedure. J Thorac Cardiovasc Surg 1999;118:588-596.[Abstract/Free Full Text]
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Abstract
Introduction
