Ann Thorac Surg 1999;68:1414-1416
© 1999 The Society of Thoracic Surgeons
Case Reports
Repair of cor triatriatum associated with partially unroofed coronary sinus
Jacques A.M. van Son, MD, PhDa,
Rüdiger Autschbach, MD, PhDa,
Friedrich W. Mohr, MD, PhDa
a Herzzentrum, University of Leipzig, Leipzig, Germany
Address reprint requests to Dr van Son, Herzzentrum, University of Leipzig, Russenstrasse 19, D-04289 Leipzig, Germany
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Abstract
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A young patient with cor triatriatum, secundum atrial septal defect, persistent left superior vena cava, partially unroofed coronary sinus, and moderate tricuspid regurgitation was documented to have both a left-to-right shunt from the common pulmonary venous chamber to the right atrium and a right-to-left shunt from the partially unroofed coronary sinus to the left atrium. Resection of the membrane dividing the left atrium, closure of the atrial septal defect and the partially unroofed coronary sinus with pericardial patches, and a tricuspid annuloplasty resulted in an excellent hemodynamic result.
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Introduction
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Cor triatriatum is a rare congenital cardiac anomaly in which the pulmonary veins enter a chamber that is separated from the remainder of the left atrium by a diaphragm in which usually there are one or more restrictive orifices. The prevalence of coexistence of cor triatriatum and a persistent left superior vena cava (SVC) in excess of an association with other associated anomalies has been suggested as indicating a relationship between these two anomalies [1–5]. Recently, we successfully performed operative correction in a young patient in whom cor triatriatum was associated with a left SVC and a partially unroofed coronary sinus. The physiology in this patient was unique in the sense that both a left-to-right shunt and a right-to-left shunt at the atrial level were documented.
An 8-year-old boy was referred with the diagnosis of cor triatriatum, secundum atrial septal defect, left SVC, and moderate tricuspid regurgitation. The patient presented with complaints of exertional dyspnea and hepatic congestion. Radiologically there were increased pulmonary vascular markings and cardiomegaly. On physical examination, the liver was found to be enlarged. Echocardiography and cardiac catheterization confirmed the diagnoses of cor triatriatum (Fig 1 ), secundum atrial septal defect, left SVC, and tricuspid regurgitation. The innominate vein was diminutive. Besides a left-to-right shunt between the common pulmonary venous chamber and the right atrium, an additional right-to-left shunt at the atrial level was documented, probably secondary to a partially unroofed coronary sinus (Fig 2). The oxygen saturations in the common pulmonary venous chamber and the left atrium were 100% and 95%, respectively. The pressures in the common pulmonary venous chamber, the right atrium, the left atrium, and the pulmonary artery were 10, 9, 5, and 37/16 mm Hg, respectively.
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Fig 1. Transthoracic echocardiogram (apical four-chamber view) of cor triatriatum with atrial septal defect between common pulmonary venous chamber and right atrium (RA) demonstrates the diaphragm (arrows) extending from the left pulmonary vein (LPV) to the atrial septum. (LA = left atrium; LV = left ventricle; RV = right ventricle; VS = ventricular septum.)
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Fig 2. Diagram demonstrating cor triatriatum associated with secundum atrial septal defect, persistent left superior vena cava, and partially unroofed coronary sinus. There is both a left-to-right shunt from the common pulmonary venous chamber (CPVC) to the right atrium (RA) (curved closed arrow) and a right-to-left shunt from the partially unroofed coronary sinus to the left atrium (LA) (angled closed arrow). Open arrow indicates communication between the common pulmonary venous chamber and the left atrium.
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At operation, through a median sternotomy, a large pericardial patch was harvested and stored in saline solution. After aortic and bicaval venous cannulation, using cardiopulmonary bypass and moderate hypothermia, the diaphragm separating the common pulmonary venous chamber and the remainder of the left atrium was exposed through a right atrial transseptal approach. There was a 1.5-cm large orifice in the center of the diaphragm. The common pulmonary venous chamber communicated with the right atrium through a large secundum atrial septal defect. The coronary sinus was confirmed to be partially unroofed.
The membrane separating the common pulmonary venous chamber from the true left atrium was excised flush with the atrial septum and the left atrial free wall. The unroofed segment of the coronary sinus and the secundum atrial septal defect were closed with pericardial patches. The tricuspid valve was rendered competent by performance of a de Vega type of annuloplasty.
Postoperative echocardiography demonstrated normal anatomy, absence of residual shunts, and a competent tricuspid valve. The systemic arterial oxygen saturation was 100%.
The patient was discharged from the hospital on the eighth postoperative day. At 12-month follow-up, the patient is in excellent clinical condition.
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Comment
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Cor triatriatum produces symptoms by causing pulmonary venous congestion and pressure loading of the right side of the heart. As in our patient, the clinical symptoms may be attenuated by a relatively large opening in the diaphragm or the presence of an atrial septal defect between the common pulmonary venous chamber and the right atrium, or both. Other mechanisms that may circumvent pulmonary venous obstruction are the presence of a condition associated with decreased pulmonary blood flow (for instance pulmonary stenosis or tetralogy of Fallot) or the presence of partial anomalous pulmonary venous connection [1, 2, 5, 6]. Our patient had unique physiology in the sense that both a left-to-right shunt and a right-to-left shunt at the atrial level existed. On the basis of reduced pulmonary venous chamber hypertension, the diagnosis was made relatively late in life.
For exposure of the dividing diaphragm in cor triatriatum, either a left or right atrial approach, or a combination, may be used [1, 2, 5]. A left atrial approach is useful when the left atrium is enlarged, which may be the case in adults. In infants and children, however, the left atrium is usually small. In this circumstance, the confluence of the atrial septum and the diaphragm may be difficult to expose because the septum is retracted anteriorly, and the mitral valve may become injured. A right atrial transseptal approach generally provides superior exposure of the diaphragm and may be preferred, especially in the presence of associated anomalies, as in the reported patient.
In the presented case the partially unroofed coronary sinus (that was associated with a persistent left SVC) was closed with a pericardial patch. In the presence of a left SVC with completely unroofed coronary sinus (and absent innominate vein), the usual repair technique consists of intraatrial rerouting of the left SVC to the right atrium with a pericardial patch. In this circumstance, however, we prefer an extracardiac repair technique in which the left SVC is divided close to its entrance into the roof of the left atrium and anastomosed to the right SVC [7]. In the case of a small or absent right SVC, the left SVC may be anastomosed to the right atrial appendage or left pulmonary artery. The same technique, in addition to resection of the diaphragm separating the left atrium, may be applied in the unique circumstance of a left SVC with unroofed coronary sinus in association with cor triatriatum. Alternatively, in such cases the diaphragm may be partially detached toward the mitral valve and used to reroute the pulmonary venous blood to the mitral valve orifice [8].
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References
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Van Son J.A.M., Walther T., Mohr F.W. Repair of divided left atrium associated with unroofed coronary sinus. Cardiol Young 1998;8:253-255.
Accepted for publication March 27, 1999.
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Creation of coronary sinus using left atrial diaphragm in the patient with cor triatriatum and unroofed coronary sinus.
Ann. Thorac. Surg.,
May 1, 2006;
81(5):
1893 - 1895.
[Abstract]
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Abstract
Introduction
