Ann Thorac Surg 2005;80:1111-1113
© 2005 The Society of Thoracic Surgeons
Case report
Cleft Mitral Valve in Association With Anomalous Left Coronary Artery Arising From Pulmonary Artery
Sridhar Rathinam, FRCS,
Oliver Stümper, MD, PhD,
William J. Brawn, FRCS, FRACS,
David J. Barron, MD, FRCS (C Th)
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Department of Pediatric Cardiac Surgery and Pediatric Cardiology, Diana Princess of Wales Hospital for Children, Birmingham, United Kingdom
Accepted for publication February 17, 2004.
* Address reprint requests to Dr Barron, Department of Cardiothoracic Surgery, Diana Princess of Wales Hospital for Children, Steel House Ln, Birmingham B4 6NH, UK (Email: david.barron{at}bch.nhs.uk).
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Abstract
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An anomalous origin of the left coronary artery from the pulmonary artery is commonly associated with mitral valve insufficiency. Usually this is secondary to left ventricular dysfunction or papillary muscle ischemia. We describe the association of an anomalous left coronary artery from the pulmonary artery with structural abnormalities of the mitral valve (cleft mitral leaflet) in 2 children, both of whom were being investigated for primary mitral valve disease. Both underwent successful operations for coronary transfer and mitral valve repair.
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Introduction
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Origin of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA) is a rare but serious congenital anomaly [1]. Mortality without treatment in the first year of life is 90% due to myocardial ischemia, infarction, and congestive cardiac failure [2]. Occasionally diagnosis is made in later childhood or adulthood with more insidious signs of congestive cardiac failure or ischemia. Mitral regurgitation (MR) is commonly seen in association with ALCAPA and is due to papillary muscle ischemia, regional myocardial dysfunction, or is secondary to left ventricular dilatation.
Anomalous left coronary artery arising from the pulmonary artery is usually an isolated cardiac anomaly, but in rare cases it is associated with ventricular septal defect, patent ductus arteriosus, tetralogy of Fallot, and coarctation of the aorta. It has not been previously associated with structural mitral valve abnormalities.
We report two cases in which older children being investigated for primary MR were subsequently found to have ALCAPA and were treated by coronary transfer and mitral valve repair.
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Case Reports
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Patient 1
A 3-year-old boy was under follow-up for MR. He was symptom free with good exercise tolerance and had been commenced on an ACE inhibitor. His electrocardiogram was noted to have ST-segment elevation inversion in the anterior leads and bi-ventricular hypertrophy. Echocardiogram demonstrated severe MR with a dysplastic and prolapsing anterior mitral leaflet, dilated left atrium, and a normal ejection fraction, but reduced contractility of the lateral wall. The papillary muscle position and function appeared normal. Detailed examination suggested reversed flow in diastole in the proximal part of the pulmonary artery suggestive of ALCAPA. In view of the electrocardiogram and echocardiographic findings, the patient underwent urgent cardiac catheterization that confirmed the presence of ALCAPA.
At surgery the left coronary artery was arising posteriorly from the main pulmonary artery in the middle of the sinus. Coronary transfer was performed into the aorta and the defect in the pulmonary artery was repaired using autologous pericardial patch. The mitral valve was approached through Waterson’s groove and was found to have a complete cleft in the anterior mitral valve leaflet, which was closed with a series of interrupted 5-0 Prolene sutures (Ethicon, Somerville, NJ). An intraoperative transesophageal echocardiogram demonstrated laminar mitral inflow, trivial MR, and good left ventricular function. His postoperative course was uneventful. Echocardiography at 3 months revealed no MR and normal ventricular function. The electrocardiogram showed regression in the ventricular hypertrophy and the t-wave inversion had completely resolved.
Patient 2
A 6-year-old boy presented with tiredness and atypical chest pain. The echocardiogram demonstrated moderate MR due to a prolapsing anterior mitral valve leaflet with a suggestion of two separate mitral valve orifices, dilated left atrium, and dilated left ventricle with good contractility and no regional wall motion abnormalities. His electrocardiogram showed ST-segment elevation changes in the anterior leads. There was a suspicion of a coronary artery fistula due to the large size of the right coronary artery. Cardiac catheterization revealed a markedly dilated right coronary artery with retrograde filling of the left coronary system draining into the pulmonary artery (Fig 1).
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Fig 1. (Left) Angiogram in patient 2 showing mitral regurgitation, (center) origin of the right coronary artery from the aorta, and (right) delayed retrograde filling of the left coronary system and pulmonary artery.
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At surgery the large left coronary artery was arising from the posterior sinus of the pulmonary artery and was transferred into the aorta. The defect in the pulmonary artery was repaired with autologous pericardium. The mitral valve was explored through the atrial septum and was found to have normal chordal attachments but an incomplete cleft in the central portion of the anterior leaflet. This was closed with two 5-0 Prolene sutures (Ethicon), and a De Vega annuloplasty was performed. Intraoperative epicardial echocardiogram demonstrated laminar mitral inflow with no residual MR and good ventricular function. Echocardiogram before discharge was unchanged. The electrocardiogram showed sinus rhythm with resolution of the ST-segment elevation changes. There was no MR at his 6-month follow-up.
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Comment
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The age at presentation for ALCAPA is variable. The majority of cases present between 4 and 12 weeks of age as the pulmonary artery resistance progressively decreases, leading to increased coronary steal and chronic myocardial ischemia. It is very unusual for these patients to present with isolated MR. Rather they present with chest pain (such as an inconsolable baby, typically during feeding) or congestive cardiac failure or with sudden death. Any degree of MR that is seen in these patients is generally secondary to papillary muscle ischemia or due to left ventricular dilatation secondary to ischemia [2].
However, a small group of patients (5% to 10%) with ALCAPA do not present as infants and can remain reasonably symptom free even into adulthood [2]. These patients have a better developed collateral network within the myocardium and have very high right coronary artery flow to compensate. Some may have a degree of left coronary ostial stenosis that helps to reduce the extent of the "steal" phenomenon into the pulmonary circulation.
In the present report, the two cases of ALCAPA are unusual because they only presented due to the investigations into separate structural mitral valve anomalies. There was no evidence to suggest that ischemia was the cause of the MR, although the limited coronary flow reserve in the myocardium with ALCAPA may have contributed to the children becoming symptomatic at an earlier age than with a normal coronary supply.
A possible link between these anomalies may be that the incompetent mitral valve helps to reduce afterload during the period of relative ischemia of the left ventricle and allows time for collateralization to occur without acute decompensation.
There is still controversy regarding the approach to the management of ALCAPA with associated mitral valve regurgitation. Some authors have the view that the MR will improve after restoration of blood flow to the myocardium, however, other authors suggest that the mitral valve should be repaired during the first operation [2, 3]]. The echocardiographic findings in these cases suggested structural mitral valve defects and warranted inspection with a view to repair.
Isolated MR is very rare in children and a cleft anterior leaflet of the mitral valve without an ostium primum defect is also unusual. It has been described in association with ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, tricuspid atresia, and double-inlet left ventricle [4]. Isolated mitral valve clefts have not been described in association with ALCAPA. Cleft mitral valves with no associated MR may not require surgery, but if MR is present, then valve repair is indicated because it has a good outcome with a low risk of needing valve replacement [5, 6].
In conclusion, in the setting of ALCAPA with MR it should not be presumed that the MR is functional, and any suggestion of a structural defect in the mitral valve at least warrants inspection of the mitral valve.
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References
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- Michielon G, Di Carlo D, Brancaccio G, et al. Anomalous coronary artery origin from the pulmonary arterycorrelation between surgical timing and left ventricular function recovery. Ann Thorac Surg 2003;76:581-588.[Abstract/Free Full Text]
- Dodge-Khatami A, Mavroudis C, Backer CL. Anomalous origin of the left coronary artery from the pulmonary arterycollective review of surgical therapy. Ann Thorac Surg 2002;74:946-955.[Abstract/Free Full Text]
- Cochrane AD, Coleman DM, Davis AM, et al. Excellent long-term functional outcome after an operation for anomalous left coronary artery from the pulmonary artery J Thorac Cardiovasc Surg 1999;117(2):332-342.[Abstract/Free Full Text]
- Van Praagh S, Porras D, Oppido G, et al. Cleft mitral valve without ostium primum defectanatomic data and surgical considerations based on 41 cases. Ann Thorac Surg 2003;75:1752-1762.[Abstract/Free Full Text]
- Tamura M, Menahem S, Brizard C. Clinical features and management of isolated cleft mitral valve in childhood J Am Coll Cardiol 2000;35(3):764-770.[Abstract/Free Full Text]
- Fraisse A, Massih TA, Kreitmann B, et al. Characteristics and management of cleft mitral valve J Am Coll Cardiol 2003;42(11):1988-1993.[Abstract/Free Full Text]
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Abstract
Introduction
