Ann Thorac Surg 2006;82:322-323
© 2006 The Society of Thoracic Surgeons
Case report
Supracardiac Total Anomalous Pulmonary Venous Connection With a Supramitral Ring: A Rare, Surgically Correctable Anomaly
Anand P. Iyer, MCh
*
,
Arunkumar Haridas, MS,
Sameet A. Pathak, MS,
Rajnish Duara, MS,
Prashant Y, MS,
Sooraj Pradhan, MS,
Krishnamanohar S. Rema, MCh
Sri Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India
Accepted for publication September 6, 2005.
* Address correspondence to Dr Iyer, Department of Cardiovascular and Thoracic Surgery, Sri Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, 11 Kerala, 695014 India (Email: anandcvts{at}hotmail.com).
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Abstract
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Total anomalous pulmonary venous connection is rarely associated with a supramitral ring. This condition should be suspected in any infant having total anomalous pulmonary venous connection with obstruction to the pulmonary venous return. Preoperative echocardiography is usually successful in detecting the lesion. The Shumaker and King repair for total anomalous pulmonary venous connection is most appropriate for such composite lesions as it enables the surgeon to identify the membrane and excise it easily and completely. We report one such case.
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Introduction
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The association of a supramitral ring and total anomalous pulmonary venous connection is a very rare surgically correctable anomaly. The supramitral ring is a fibrous membrane and is situated on the left atrial side of the mitral annulus [1]. The atrial appendage is proximal to the ring. The ring may be a nonobstructive incidental finding or it may protrude into the orifice, producing a variable degree of obstruction (Fig 1). When it is associated with total anomalous pulmonary venous connection, the proximal chamber of the left atrium does not contain the pulmonary veins. The pulmonary veins converge into a common chamber and join the innominate vein through the vertical vein (Fig 2). We would like to emphasize the surgical implications of this association.
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Fig 1. Echocardiograph (apical 4-chamber view) showing the supramitral ring (SMR), atrial septal defect (ASD), and the chambers of the heart. (LV = left ventricle; MV = mitral valve; RA = right atrium.)
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The patient, aged 3 months, presented with complaints of failure to thrive and repeated respiratory tract infections. Chest roentgenogram showed cardiomegaly and pulmonary congestion. Two-dimensional echocardiography showed supracardiac total anomalous pulmonary venous connection with an atrial septal defect and a supramitral ring. The patient was admitted for corrective surgery. Cardiopulmonary bypass was instituted, the ductus was ligated, and patient was cooled to 18°C. The aorta was cross-clamped, cardioplegia was given, and the vertical vein was looped. The Shumaker and King repair was performed, wherein an incision was made from the midportion of the right atrium across the atrial septal defect into the posterior wall of the left atrium [2]. The supramitral ring was excised, and the common venous chamber was opened and was then anastomosed to the left atrium. The atrial septal defect was closed using a pericardial patch. The postoperative course was uneventful. One month postoperatively the baby was doing well with no residual defects or pulmonary artery hypertension as demonstrated by echocardiography.
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Comment
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The supramitral ring is a rare cause of congenital mitral stenosis. Only about a 100 cases have been reported since its first description in 1902 [3]. It is commonly associated with ventricular septal defect, atrial septal defect, and patent ductus arteriosus, and it is also a part of the Shones complex. The supramitral ring is very rarely associated with an anomalous pulmonary venous connection and very few reports exist of this association [4]. This condition should be suspected in any infant having total anomalous pulmonary venous connection with an obstruction to the pulmonary venous return. In a patient with both these lesions, it is important not to miss the supramitral ring. It can be detected by preoperative echocardiography, which is the investigation of choice because cineangiography is often unsuccessful in demonstrating the abnormal left atrial membrane [5]. Surgery is done with cardioplegic arrest on total bypass. Deep hypothermia with circulatory arrest can be used for optimum exposure. The Shumaker and King repair, which employs a transatrial approach [2], is most appropriate for such lesions. It enables the surgeon to identify the membrane and excise it easily and completely. Other approaches in which the left atrial common venous anastomosis is done from outside the heart may not be very helpful in identifying or excising the ring or membrane.
The association of the supramitral ring and total anomalous pulmonary venous connection is a very uncommon but correctable lesion. Early diagnosis and complete surgical correction should yield good results.
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References
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- Anabtawi IN, Ellison RG. Congenital stenosing ring of the left atrioventricular canal (supravalvular mitral stenosis) J Thorac Cardiovasc Surg 1965;49:994.[Medline]
- Shumaker Jr HB, King H. A modified procedure for complete repair of total anomalous pulmonary venous drainage Surg Gynecol Obstet 1961;12:763-765.
- Mychaskiw 2nd G, Sachdev V, Branden DA, Heath BJ. Supramitral ring: an unusual cause of congenital mitral stenosis: case series and review J Cardiovasc Surg 2002;43(2):199-202.[Medline]
- Guido M, Roberto MD, Luciano P, et al. Total anomalous pulmonary venous connectionlong-term appraisal with evolving technical solutions. Eur J Cardiothorac Surg 2002;22:184-191.[Abstract/Free Full Text]
- Pascal R, Francoise B, Laurent F, Oliver B, Francine L, Jean-Yves N. Cor triatriatum and total anomalous pulmonary venous connectiona rare, surgically correctable anomaly. J Thorac Cardiovasc Surg 1985;90(3):443-445.[Abstract]
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Abstract
Introduction
