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Ann Thorac Surg 2006;81:e16-e17
© 2006 The Society of Thoracic Surgeons

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Case report

Coronary Sinus Orifice Atresia With Left Superior Vena Cava in Patients With Univentricular Heart

^a Department of Pediatric Cardiac Surgery, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
^b Department of Pediatric Cardiology, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland

Accepted for publication January 17, 2006.

^_* Address correspondence to Dr Salminen, Division of Pediatric Surgery and Pediatric Cardiac Surgery, Hospital for Children and Adolescents, University of Helsinki, POB 281, Helsinki, 00029 HUS Finland (Email: jukka.salminen{at}hus.fi).

	Abstract

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Two patients with hypoplastic left heart syndrome had coronary sinus orifice atresia with persistent left superior vena cava. Both patients underwent successfully coronary sinus unroofing. One underwent surgery at the time of the bidirectional Glenn procedure and the other before creation of a total cavopulmonary connection. According to our population-based database, 10.3% of patients with univentricular heart have persistent left superior vena cava, and 2.3% have associated coronary sinus orifice atresia. Our cases highlight the importance of recognizing this anomaly in patients with univentricular heart to avoid high coronary venous pressure, which is potentially lethal.

	Introduction

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The cardiac anomaly coronary sinus orifice atresia (CSOA) with a persistent left superior vena cava (LSVC) is rare. Only four cases with this anomaly have been reported in patients undergoing surgery for univentricular heart (UVH) [1–4], so in these patients the incidence of CSOA and LSVC is unknown. The combination of CSOA and LSVC leads to coronary venous hypertension, and if this is not diagnosed and treated during bidirectional Glenn (BDG) or Fontan-type surgery it is potentially lethal.

	Case Reports

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Patient 1
A 4-year-old girl with previously undiagnosed hypoplastic left heart syndrome (HLHS) was resuscitated at the age of 12 days. At 2 weeks of age she underwent a Norwood I operation. Because myocardial function remained compromised, she received an early BDG at 2 months of age. Initially after BDG, myocardial function deteriorated even further, but it slowly improved with medication for cardiac failure. At 14 months of age, a small LSVC with CSOA was visible in angiography at preoperative catheterization for total cavopulmonary connection. This was repaired by unroofing the coronary sinus to the left atrium and ligating the LSVC. Coronary sinus orifice atresia was found to consist of a thick muscle layer and endocardium. The patient recovered well and her myocardial function normalized postoperatively. At echocardiography and cardiac catheterization 8 months later, connection of the coronary sinus to the left atrium was found to be nonrestrictive. Total cavopulmonary connection was performed thereafter without complications, and she remains well.

Patient 2
A 6-month-old boy with HLHS had successfully undergone Norwood I surgery at 1 week of age. Coronary sinus orifice atresia and small LSVC were diagnosed in angiography prior to BDG. During BDG, the coronary sinus was unroofed and the LSVC was ligated. The recovery was uneventful, and the patient is currently doing well. At echocardiography and cardiac catheterization 3 months later, the connection of the coronary sinus to the left atrium seemed nonrestrictive.

In our country, complicated pediatric cardiology and cardiac surgery is centralized in the Hospital for Children and Adolescents, University of Helsinki, Finland. Every patient with UVH thus comes to our hospital for evaluation. We analyzed the population-based incidence of LSVC and CSOA in patients with UVH born in Finland between January 2000 and December 2004 [5]. Of 87 patients with UVH, 9 had LSVC (10.3%), and 2 of these 9 had CSOA, giving an incidence of 2.3% (2 of 87). Both of these patients with CSOA and LSVC had HLHS. The overall incidence of CSOA and LSVC among our patients with HLHS was 4.7% (2 of 43). Of the patients with HLHS, 28 underwent BDG, including the 2 with CSOA. At echocardiography, retrograde flow in a small LSVC was not diagnosed before the cardiac catheterization and angiography that were performed prior to BDG and total cavopulmonary connection surgery. Angiograms of the innominate vein and LSVC revealed CSOA in both patients. Of the seven cases of LSVC without CSOA, six had a diagnosis of LSVC that was made by echocardiography, with one very small LSVC detected perioperatively.

	Comment

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Left superior vena cava is found in 2% to 4% of all patients with congenital cardiac defects [6]. In patients with CSOA and LSVC, with no coronary venous communication to the left atrium, venous blood from the coronary sinus drains retrograde through the LSVC and innominate vein to the right superior vena cava [1–4, 7]. Other venous connections between the LSVC and the right superior vena cava may also exist [3]. Other cardiac malformations related to CSOA and LSVC coexist in approximately half the cases [1–4, 7].

Surgical ligation of the LSVC in patients with CSOA leads to interruption of the coronary sinus drainage, decreased myocardial perfusion, and impaired myocardial function [7]; fatal outcomes have occurred [8]. Either preoperative echocardiography or angiography is necessary to rule out retrograde flow in a small LSVC associated with CSOA.

For treatment of CSOA and LSVC, we used the technique described by Ohta and colleagues [2]; we created a new connection from the interior of the left atrium by using a guide probe that was advanced through the opened LSVC. Oshima and colleagues [1] created a large, smooth opening between the coronary sinus and the left atrium by using a left superior vena cava flap. In 1 patient with total anomalous pulmonary venous drainage, the coronary sinus drained to the common pulmonary venous confluence. The confluence and the coronary sinus were then anastomosed to the left atrium [7].

In our population-based series, the incidence of CSOA and LSVC in patients with UVH was 2.3%. Even though CSOA and LSVC have been considered extremely rare, the incidence of this anomaly may not be as rare as suspected. Only four cases of CSOA and LSVC have been reported in patients with UVH (ie, three cases of HLHS and one case of tricuspid atresia) [1–4]. Diagnosis of CSOA and LSVC was made in preoperative evaluation in three cases and during surgery in one case. In two cases, nothing was done for CSOA and LSVC during BDG and Fontan operations. The patients recovered, but there is no follow-up data available on them [3, 4]. In one case, during BDG a connection between the atrium and the coronary sinus was created and the LSVC was divided [1]. In another case, total cavopulmonary connection was performed, and nothing was done for the CSOA and LSVC. Postoperatively, cardiac performance deteriorated and the patient suffered pleural effusion and ascites; coronary sinus ostial unroofing was performed 6 months later. After that, cardiac function improved and the ascites and pleural effusion resolved [2]. The postoperative course of one of our patients was very similar. It is obvious that when CSOA and LSVC are left intact after BDG or a Fontan surgery, the resulting coronary venous hypertension leads to a lower coronary arteriovenous gradient and myocardial perfusion, and then to impaired cardiac function.

We conclude that when a patient is considered for BDG or Fontan-type procedures, the possibility of CSOA and LSVC has to be kept in mind. When LSVC is identified in echocardiography in a patient with a univentricular heart, it is crucial to determine the direction of flow in this vein. Selective angiography into the LSVC is advisable to define the anatomy of the coronary sinus [4]. If the diameter of the LSVC is small, it may remain undetected in echocardiography. Angiography of the innominate vein is recommended to determine the occurrence of the LSVC and the direction of flow in it, even if preoperative echocardiography does not reveal the LSVC. An alternative draining site between the coronary sinus and atria should be created, with the LSVC ligated to avoid the harmful effect of chronic coronary venous hypertension on myocardial perfusion and function.

	References

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1. Oshima Y, Doi Y, Misaki T, Ichida F. Surgical repair of coronary sinus orifice atresia Eur J Cardiothorac Surg 2005;27:351-352.[Abstract/Free Full Text]
2. Ohta N, Sakamoto K, Kado M, Nishioka M, Yokota M. Surgical treatment of coronary sinus atresia with hypoplastic left heart syndrome after total cavopulmonary connection Ann Thorac Surg 2002;73:653-655.[Abstract/Free Full Text]
3. Santoscoy R, Walters HL, Ross RD, Lyons JM, Hakimi M. Coronary sinus ostial atresia with persistent left superior vena cava Ann Thorac Surg 1996;61:879-882.[Abstract/Free Full Text]
4. Muster AJ, Naheed ZJ, Backer CL. Is surgical ligation of an accessory left superior vena cava always safe? Pediatr Cardiol 1998;19:352-354.[Medline]
5. Nieminen HP, Jokinen EV, Sairanen HI. Late results of pediatric cardiac surgery in Finlanda population-based study with 96% follow-up. Circulation 2001;104:570-575.[Abstract/Free Full Text]
6. Campbell M, Deuchar DC. Left sided superior vena cava Br Heart J 1954;16:423.
7. Fulton JO, Mas C, Brizard CP, Karl TR. The surgical importance of coronary sinus orifice atresia Ann Thorac Surg 1998;66:2112-2114.[Abstract/Free Full Text]
8. Yokota M, Kyoku I, Kitano M, et al. Atresia of the coronary sinus orifice. Fatal outcome after intraoperative division of the drainage left superior vena cava J Thorac Cardiovasc Surg 1989;98:30-32.[Abstract]

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This Article Abstract Full Text (PDF) 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): Heikki Sairanen Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Salminen, J. T. Articles by Sairanen, H. Search for Related Content PubMed PubMed Citation Articles by Salminen, J. T. Articles by Sairanen, H. Related Collections Congenital - cyanotic