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Ann Thorac Surg 2002;73:653-655
© 2002 The Society of Thoracic Surgeons

Case report

Surgical treatment of coronary sinus orifice atresia with hypoplastic left heart syndrome after total cavo-pulmonary connection

Noritaka Ohta, MD*a, Kisaburo Sakamoto, MDa, Miwako Kado, MDa, Masahiko Nishioka, MDa, Michio Yokota, MDa

a Department of Cardiovascular Surgery, Shizuoka Children’s Hospital, Shizuoka, Japan

Accepted for publication April 26, 2001.

* Address reprint requests to Dr Ohta, Department of Cardiovascular Surgery, Shizuoka Children’s Hospital, Urushiyama 860, Shizuoka 420-8660, Japan
e-mail: ohtan{at}jun.ncvc.go.jp


   Abstract
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 Abstract
Introduction
 Comment
 References
 
Atresia of the coronary sinus orifice is rare. We describe the surgical treatment of coronary orifice atresia in an infant with a persistent left superior vena cava after total cavo-pulmonary connection for hypoplastic left heart syndrome. The diagnosis was made by cardiac catheterization after total cavo-pulmonary connection at 8 months of age. After surgery, cardiac performance deteriorated. At reoperation, the coronary sinus was fenestrated to the left atrium. The patient survived surgical treatment of coronary sinus ostial atresia unroofed to the left atrium, guiding the placement of the fenestration with a probe placed through the open cardiac end of left superior vena cava.


   Introduction
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 Abstract
 Introduction
Comment
 References
 
Atresia of the coronary sinus orifice in a living patient is rare, and most cases have only been identified at autopsy [1]. Forty cases have been reported in the world literature [1–5], but there have been no reports of operations for creating drainage to the atrium. Of the 14 reported cases in living patients, only 1 patient had Fontan circulation, and the diagnosis of coronary sinus orifice atresia (CSA) was made during a Fontan operation.

A 2-day-old boy was transferred to our institution because of suspected cardiac disease. Echocardiography on admission revealed hypoplastic left heart syndrome. The patient weighed 3,700 g with a body size of 52 cm. At 6 days of age, the Norwood procedure was performed. The patient was discharged on the 50th hospital day. Catheterization at the age of 4 months revealed a Qp/Qs ratio of 2.6, a mean pulmonary arterial pressure of 24 mm Hg, a mean right atrial pressure of 5 mm Hg, and a mean left atrial pressure of 15 mm Hg. Pulmonary resistance was 0.99 U. One-stage total cavo-pulmonary connection with a right lateral tunnel was performed at the age of 4 months without an intervening bidirectional Glenn anastomosis. Thereafter, no major coronary perfusion problems or cardiac dysfunction were evident, and the patient was discharged. However, at the age of 10 months, the patient developed massive ascites and pleural effusion. His suckling was poor, and he suffered from persistent vomiting. Hepatomegaly was evident, and his cardiac function had begun to deteriorate. Catheterization revealed CSA with a persistent left superior vena cava (Fig 1). The coronary sinus and left superior vena cava were markedly dilated.



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  		Fig 1. The anteroposterior view demonstrates contrast medium filling a dilated left superior vena cava and coronary sinus. The absence of contrast in either atrium and retrograde flow into the innominate vein confirms atresia of the coronary sinus osteum.

 
The persistent left superior vena cava had not been recognized during the previous operation or catheterization. The superior and inferior vena cava mean pressure was 18 mm Hg. Echocardiography revealed a restrictive atrial septal defect. At reoperation, a dilated left superior vena cava was identified and divided after the institution of cardiopulmonary bypass, and the cephalad end was ligated. Following cardioplegic arrest, the right atrium was opened and absence of the coronary sinus orifice was confirmed. The atrial septal defect was enlarged. The coronary sinus was then unroofed to the left atrium guiding the placement of the fenestration with a probe placed through the open cardiac end of the left superior vena cava. There was a crossing vein between the right superior vena cava and the left superior vena cava. The cardiac end of the left superior vena cava was then ligated. The patient was weaned from the cardiopulmonary bypass without difficulty. Postoperatively, cardiac function was considerably improved, and both the ascites and pleural effusion were resolved.


   Comment
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 Abstract
 Introduction
 Comment
References
 
Atresia of the coronary sinus orifice is rare and is usually diagnosed at autopsy. The first case was reported in 1738 [1], and 40 cases have been reported since then [1–5]. However, there have been no reports of atresia of the coronary sinus orifice with hypoplastic left heart syndrome. The diagnosis of CSA is usually made by cardiac catheterization, when there is retrograde flow through a left superior vena cava (SVC) [6]. Ten cases of atresia of the coronary sinus orifice identified by angiography and 5 cases identified intraoperatively have been reported [1–5]. In 1 patient, who died 2 weeks after surgery, a new connection had been fashioned between the coronary sinus and the left atrium [2].

Usually, the left SVC can be ligated or occluded during cardiopulmonary bypass when there is a crossing vein between the left SVC and the right SVC. However, ligation should be avoided if there is no coronary sinus orifice in the atrium. Our group [7] previously reported the lethal consequences of ligating a left SVC not unroofed to the left atrium in the presence of an atretic coronary sinus orifice.

In the present patient, CSA was diagnosed by angiocardiography after a Fontan operation. The central venous pressure increased with the development of a restrictive atrial communication. This was further compounded by coronary venous hypertension and myocardial dysfunction, leading to a vicious cycle of clinical decompensation. Decompression of the coronary sinus into the left atrium and enlargement of the atrial septal defect were successful. In this case of CSA with hypoplastic left heart syndrome, creation of a new connection between the coronary sinus and left atrium was found to be very useful.


   References
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 Abstract
 Introduction
 Comment
 References
 

  1. Santoscoy R., Walter H., Ross D.R. Coronary sinus ostial atresia with persistent left superior vena cava. Ann Thorac Surg 1996;61:879-882.[Abstract/Free Full Text]
  2. Fulton J.O., Mas C., Brizard C.P., Karl T.R. The surgical importance of coronary sinus orifice atresia. Ann Thorac Surg 1998;66:2112-2114.[Abstract/Free Full Text]
  3. Imai S., Matsumura T., Yamazoe M., Kato T. Atresia of the right atrial orifice of the coronary sinus with persistent left superior vena cava. J Cardiol 1999;34:341-344.[Medline]
  4. Giebel J., Fanghanel J., Hauser S. A case of a persistent left vena cava superior with atresia of the right atrial ostium of the coronary sinus. Anat Anz 2000;182:191-194.[Medline]
  5. Muster A.J., Naheed Z.J., Backer C.L. Is surgical ligation of an accessory left superior vena cava always safe?. Pediatr Cardiol 1998;19:352-354.[Medline]
  6. Yeager S.B., Balian A.A., Gastafson R.A. Angiographic diagnosis of coronary sinus ostium atresia. Am J Cardiol 1985;56:996.[Medline]
  7. Yokota M., Kyoku I., Kitano M. Atresia of 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
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Kisaburo Sakamoto
Michio Yokota
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Right arrow Congenital - cyanotic

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