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Ann Thorac Surg 1996;61:879-882
© 1996 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Coronary Sinus Ostial Atresia With Persistent Left Superior Vena Cava

Departments of Cardiovascular Surgery and Pediatric Cardiology, Children's Hospital of Michigan, Wayne State University School of Medicine, Detroit, Michigan

Accepted for publication November 16, 1995.

	Abstract

Top Footnotes Abstract Introduction Material and Methods Comment References

 
Background. Atresia of the coronary sinus orifice with a persistent left superior vena cava is an intrinsically benign cardiac anomaly with important surgical implications.

Methods. The medical records of 5 patients with atresia of the coronary sinus orifice with a persistent left superior vena cava were reviewed retrospectively, and a computer search of the world literature describing this cardiac malformation was undertaken.

Results. The 5 patients ranged in age from 9 months to 5 years. In 2, the diagnosis was made preoperatively by angiocardiography, and in 3, the abnormality was found incidentally at the time of cardiotomy for repair of associated congenital heart disease. Four of the 5 patients underwent repair of associated cardiac lesions. During operation, care was taken to avoid disruption of left superior vena cava flow to prevent coronary venous obstruction. All patients survived and are doing well at follow-up.

Conclusions. Atresia of the coronary sinus orifice with persistent left superior vena cava is, in itself, a benign anomaly without physiologic consequence. However, the recognition of this lesion during repair of associated cardiac lesions is of vital importance to the cardiac surgeon. Interruption of this sole route of coronary venous drainage can potentially lead to myocardial ischemia and necrosis.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Comment References

 
Atresia of the coronary sinus orifice (CSOA) with a persistent left superior vena cava (LSVC) is a rare cardiac anomaly with 30 cases reported in the world literature [1–18]. The vast majority (24) of the reported cases were identified at postmortem examination. Of the 6 cases reported in living patients, one diagnosis was made intraoperatively [17], and five diagnoses were made during angiography [10, 13, 14]. We report 5 cases; in 2, the diagnosis was made at angiography and in 3 intraoperatively. We also review the world literature regarding this rare and potentially hazardous condition.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Comment References

 
The case reports of our 5 patients are presented.

Patient 1
A 9-month-old infant had a diagnosis of double-outlet right ventricle (DORV) with pulmonary stenosis. He had undergone a left-sided modified Blalock-Taussig shunt when he was 1 month of age. Eight months later, preoperative cardiac catheterization confirmed the diagnosis of DORV and did not reveal any other cardiac abnormalities. Intraoperatively, a small LSVC was occluded with a silk tie used as a tourniquet. At the time of cardioplegia administration, the coronary sinus became distended, and close inspection within the right atrium confirmed CSOA. The tourniquet was removed from the LSVC, and immediate decompression of the coronary sinus resulted. The patient underwent successful correction of DORV and pulmonary stenosis. The postoperative course was uneventful, and he remains well.

Patient 2
A 2-year-old boy with DORV and unrestricted pulmonary blood flow had undergone placement of a pulmonary artery band at 4 months of age. Twenty months later, preoperative cardiac catheterization demonstrated DORV with a large perimembranous ventricular septal defect. Innominate vein angiography demonstrated an LSVC with retrograde flow. Direct injection of contrast media into the coronary sinus through the LSVC documented CSOA and retrograde flow (Fig 1). The patient underwent uneventful repair of DORV with special care to avoid interruption of the LSVC flow. The postoperative course was unremarkable, and the patient is currently doing well.

View larger version (108K): [in this window] [in a new window]   Fig 1. . (Patient 2.) (A) Anteroposterior (AP) and (B) lateral angiocardiograms. The catheter course is from the inferior vena cava through the right atrium into the right superior vena cava, the innominate vein, and the distal left superior vena cava. The AP view demonstrates contrast medium filling the atretic coronary sinus (black arrow) with retrograde flow into the cardiac veins (open arrowheads). The pulmonary artery band is evident.

View larger version (80K): [in this window] [in a new window]   Fig 2. . (Patient 3.) (A) Anteroposterior and (B) lateral angiocardiograms. The catheter course is identical to that in Figure 1. The atretic coronary sinus is indicated by the black arrows. The hemiazygos connection (open arrowheads) from the left superior vena cava joins the azygos vein (black arrowheads), which drains normally into the right superior vena cava.

Patient 5
A 3-year-old boy underwent closure of a large perimembranous ventricular septal defect and resection of a discrete subaortic stenosis. At the time of operation, an LSVC was discovered, and exploration in the right atrium demonstrated CSOA. Care was taken to avoid disruption of LSVC flow, and the operation proceeded without difficulty. The patient is currently doing well.

	Comment

Top Footnotes Abstract Introduction Material and Methods Comment References

 
Coronary sinus ostial atresia with an LSVC is usually associated with an anomalous connection between the coronary sinus and the left atrium [19, 20]. Atresia of the coronary sinus ostium without this anomalous communication is extremely rare. Since the first description in 1738 [1], 30 cases of CSOA with an anomalous LSVC have been reported in the world literature (Table 1). In 24 instances, the condition was discovered at postmortem examination, and in five, the diagnosis was made at preoperative angiography [1–16]. A single case of intraoperative diagnosis of CSOA in a transplant donor heart has been reported [17]. We describe 5 additional cases; in 2 patients, the diagnosis was made at angiocardiography and in 3, CSOA was found incidentally at the time of cardiotomy for repair of congenital heart disease (Table 2).

Although obstruction to coronary venous drainage caused by a small LSVC has been suggested, no specific disability from CSOA has been documented. The lesion can pose a grave hazard for the cardiac surgeon if it is not recognized before or at the time of cardiac surgical intervention. Occlusion of the small LSVC, which is uncommonly performed when a crossing vein between the LSVC and the RSVC is present, can lead to acute venous obstruction of the coronary system causing myocardial congestion and ischemia.

The relationship between CSOA plus LSVC and other cardiac anomalies is unclear. Other cardiac malformations coexist in approximately 56% of reported cases (see Table 2); however, there is no lesion specifically related to CSOA. Coronary artery disease and ischemic cardiomyopathies have been reported to occur with this condition, but there is no proven cause-effect relationship [10, 12].

The diagnosis of CSOA is usually made at postmortem examination; there are few reports of preoperative or intraoperative identification. Five previous cases of angiographic identification have been reported [10, 13, 14]. The angiograms in our patients clearly demonstrate the atretic opening of the coronary sinus with retrograde drainage into an anomalous LSVC (see Fig 1). Findings at the time of angiography that should alert the investigator include retrograde LSVC flow and a coronary sinus that is not enlarged in the presence of an LSVC. These findings warrant advancement of the angiographic catheter into the coronary sinus to determine the anatomy and to rule out possible stenosis or atresia of the coronary sinus ostium. Preoperative echocardiography can also be helpful by demonstrating a nondilated coronary sinus in the presence of an LSVC. Also, Doppler studies can identify retrograde LSVC flow.

The persistence of an LSVC is not an uncommon finding for the cardiac surgeon and is usually seen during operation to correct other congenital heart defects. When a crossing vein between the LSVC and the RSVC exists, the LSVC can usually be ligated or occluded during the proper conduct of cardiopulmonary bypass. Ligation, temporary occlusion, or vigorous manipulation of the LSVC in the presence of CSOA, however, should be avoided. For example, Yokota and colleagues [16], described a disastrous consequence of ligating an LSVC in the presence of an atretic coronary sinus ostium. Autopsy findings in their patient revealed massive myocardial congestion and hemorrhage as well as an infarct of the right ventricular free wall. Interrupting the only egress of coronary sinus flow can cause coronary venous congestion leading to myocardial ischemia and eventual necrosis. It is, therefore, paramount to recognize or rule out CSOA prior to interrupting flow in an LSVC.

Coronary sinus ostial atresia is a rare malformation. It is, in itself, a benign anomaly without physiologic consequence and is, therefore, usually documented incidentally at postmortem examination. The recognition of this lesion, however, is of vital importance to the cardiac surgeon. Interruption of the sole route of coronary venous drainage can lead to myocardial ischemia and necrosis.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Comment References

 
Address reprint requests to Dr Walters, Department of Cardiovascular Surgery, Children's Hospital of Michigan, 3901 Beaubien Blvd, Detroit, MI 48201.

	References

Top Footnotes Abstract Introduction Material and Methods Comment References

 

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Robert Santoscoy Henry L. Walters, III Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Santoscoy, R. Articles by Hakimi, M. Search for Related Content PubMed PubMed Citation Articles by Santoscoy, R. Articles by Hakimi, M.