Asian Cardiovasc Thorac Ann 2003;11:261-262
© 2003 Asia Publishing EXchange Ltd
Atresia of Coronary Sinus Ostium With Coronary Venous Flow to Left Atrium
Nilgün Bozbu
a, MD,
Mehmet Balkanay, MD,
Serpil Ta
, MD,
Vedat Erentug, MD,
Cevat Yakut, MD
Department of Cardiovascular Surgery, Kouyolu Heart and Research Hospital, Istanbul, Turkey
For reprint information contact: Nilgün Bozbua, MD Tel: 90 216 326 6969 Fax: 90 216 339 0441 email: nbozbuga{at}kosuyolu.gov.tr Kouyolu Heart and Research Hospital, 81020 Kadiköy, Istanbul, Turkey.
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ABSTRACT
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The rare cardiac anomaly of atresia of the coronary sinus ostium with a large communication between the coronary sinus and the left atrium was discovered during a mitral valve replacement operation in a 44-year-old woman.
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INTRODUCTION
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Atresia of the coronary sinus ostium with a large communication between the coronary sinus and the left atrium, whereby coronary venous flow enters the left atrium, is a rare cardiac anomaly. It is even more rare in the absence of an interatrial communication.1–2 A case of atresia of the right atrial orifice of the coronary sinus was diagnosed after performing a cardiotomy for mitral valve replacement.
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CASE REPORT
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A 44-year-old woman with a 10-year history of rheumatic mixed mitral valve disease underwent mitral valve replacement. She was in New York Heart Association functional class II with normal sinus rhythm, severe mitral regurgitation, left ventricular enlargement (end-diastolic diameter 56 mm), left atrial (LA) enlargement (53 mm), and pulmonary hypertension (60 mm Hg). The mean transmitral gradient was measured as 9 mm Hg, and LA pressure was 25 mm Hg. During the operation, retrograde coronary sinus cardioplegic delivery could not be performed via a closed right atrial approach. The heart was arrested with antegrade blood cardioplegia through the aortic root. After a left atriotomy, an enlarged coronary sinus ostium, 2.5 cm in diameter, was found in an inferoposterior location in the left atrium (Figure 1
). Myocardial protection was maintained using retrograde coronary sinus cardioplegia via the LA orifice. The normal anatomic opening of the coronary sinus ostium was not detected in the right atrium (Figure 2). Neither persistent left superior vena cava (SVC) with atresia of the coronary sinus orifice nor an interatrial communication was found intraoperatively. The patient underwent mitral valve replacement with a 25-mm bileaflet prosthesis (Sulzer Carbomedics, Inc., Austin, TX, USA). Her postoperative course was uneventful.
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Figure 1. (A) Diagram and (B) operative photograph of anomalous opening of the coronary sinus into the left atrium.
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Figure 2. (A) Diagram and (B) operative photograph of atresia of the orifice of the coronary sinus into the right atrium.
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DISCUSSION
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Atresia of the coronary sinus orifice is a rare and intrinsically benign cardiac malformation that is usually diagnosed at autopsy.2 Only a few cases have been diagnosed during life. This is the only case of coronary sinus malformation we have encountered in 7,275 operations using retrograde coronary sinus cardioplegia since 1996. Atresia of the coronary sinus consists of membranous occlusion of the coronary ostium, thereby interrupting the usual direct drainage of the coronary sinus to the right atrium. Between the blind end of the coronary sinus and the right atrium, there can be either short- or long-segment interruption. There may have been a double coronary sinus during embryonic development, with drainage by way of a left SVC or a coronary sinus-LA window.3,4 While an anomalous left SCV provides the major route for venous drainage from the coronary sinus, there can be small communicating veins between the coronary sinus and the right atrium or an orifice in the left atrium. The anomaly may be isolated or combined with an atrial septal defect (inferoposterior) or complex cardiac malformations.4 In most cases, a considerable left-to-right shunt exists at the atrial level, which can be misinterpreted as an atrial septal defect. There is either complete unroofing of the coronary sinus or atresia of its connection to the right atrium. Atrial appendage anomalies exemplified by both right and left isomerism and ventricular hypoplasia have been seen.5 Diagnosis before the operation is often difficult. In nearly half of cases, a persistent left SVC has afforded the only outflow channel for the coronary sinus, and occasionally this may have surgical implications.1
No specific disability resulting from atresia of the coronary orifice has been documented.5 Coronary angiograms and attention to the venous phase might be informative. Atresia of the coronary orifice is an intrinsically benign cardiac anomaly with important surgical implications. It is difficult to diagnose this abnormality by cardiac catheterization and even during surgery. However, the recognition of this lesion during repair of associated cardiac lesions is of vital importance to the cardiac surgeon. Furthermore, retrograde coronary sinus cardioplegia delivery cannot be achieved by closed transatrial coronary sinus cannulation. Procedures that interrupt coronary venous drainage, such as ligation or redirection of a left SVC or closure of a dorsal atrial septal defect, can potentially lead to myocardial congestion, ischemia, and necrosis.
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REFERENCES
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- Franz C, Mennicken U, Dalichau H, Hirsch H. Abnormal communication between the left atrium and the coronary sinus. Presentation of 2 cases and review of the literature. Thorac Cardiovasc Surg 1985;33:113–7.[Medline]
- Watson GH. Atresia of the coronary sinus orifice. Pediatr Cardiol 1985;6:99–101.[Medline]
- Sahinoglu K, Cassell MD, Miyauchi R, Bergman RA. Human persistent left superior vena cava with doubled coronary sinus. Anat Anz 1994;176:451–4.[Medline]
- Adatia I, Gittenberger-de Groot AC. Unroofed coronary sinus and coronary sinus orifice atresia. Implications for management of complex congenital heart disease. J Am Coll Cardiol 1995;25:948–53.[Abstract]
- Roberts WH, Engen P, Fujimoto E. Anomalous opening of coronary sinus into the left atrium. Anat Anz 1980;147:471–2.[Medline]
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ABSTRACT
Introduction
