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Ann Thorac Surg 1998;66:2112-2114
© 1998 The Society of Thoracic Surgeons

Case Reports

The surgical importance of coronary sinus orifice atresia

James O. Fulton, FCS(SA)a, Carlos Mas, MDa, Christian P.R. Brizard, MDa, Tom R. Karl, MDa

a Cardiac Surgical Unit and the Department of Cardiology, Royal Children’s Hospital, Melbourne, Australia

Accepted for publication June 5, 1998.

Address reprint requests to Dr Karl, Cardiac Surgical Unit, Royal Children’s Hospital, Flemington Rd, Parkville, 3052 Victoria, Australia
e-mail: (cardiac{at}cryptic.rch.unimelb.edu.au)


   Abstract
Top
 Abstract
Introduction
 Case reports
 Comment
 References
 
Coronary sinus orifice atresia is rare. We describe two cases, one with an atrioventricular septal defect and another with supracardiac totally anomalous pulmonary venous drainage. The association with the latter has not been described previously. The importance of diagnosing the defect is emphasized and surgical treatment is discussed.


   Introduction
Top
 Abstract
 Introduction
Case reports
 Comment
 References
 
Coronary sinus orifice atresia is rarely diagnosed before autopsy. There have been sporadic reports of coronary sinus orifice atresia associated with a variety of congenital cardiac lesions, as well as in patients without cardiac disease [1–5]. If this defect is not diagnosed or suspected in patients having cardiac operation for associated lesions, incorrect surgical treatment may have lethal consequences [5].


   Case reports
Top
 Abstract
 Introduction
 Case reports
Comment
 References
 
Case 1
A 6-month-old male infant was referred to us with the diagnosis of left isomerism, a complete atrioventricular septal defect (AVSD), and a patent ductus arteriosus. On examination this 5.12-kg child was in no distress, although he was tachypneic and cyanosed with crying. There was a pansystolic murmur audible over the left sternal edge, and the lung fields were clear on auscultation. Chest x-ray showed cardiomegaly and pulmonary congestion, whereas the electrocardiogram showed sinus rhythm with biventricular hypertrophy and left axis deviation. On echocardiography, the diagnosis of sinus solitus, interrupted inferior vena cava, patent ductus arteriosus, and complete AVSD with a single atrioventricular valve was made. Cardiac catheterization confirmed the diagnosis, with a left superior vena cava (SVC) draining to the coronary sinus. Incomplete opacification of the proximal coronary sinus did not demonstrate the coronary sinus orifice, but drainage appeared to be retrograde into the left SVC. There was systemic pulmonary artery pressure and mild atrioventricular valve regurgitation.

A two-patch repair of the AVSD was undertaken on cardiopulmonary bypass with moderate hypothermia (28°C) and cold cardioplegic cardiac arrest. At operation the anatomy was as suspected from the preoperative investigations. With the initiation of cardiopulmonary bypass, the patent ductus arteriosus and the left SVC were ligated. There was no visible coronary sinus orifice in the right atrium, and an unroofed coronary sinus to the left SVC was suspected initially. After completion of AVSD repair and deairing of the heart, the aortic crossclamp was removed. Cardiac activity resumed in complete heart block and atrioventricular sequential pacing was begun. The child was weaned from cardiopulmonary bypass with poor hemodynamic findings and pulmonary hypertension, although well supported with inotropic agents. Nitric oxide was administered and intraoperative transesophageal echocardiography showed thickened poorly contracting ventricles. Subepicardial hemorrhage of the left ventricle was noted. In view of these findings and the possibility of coronary artery injury, cardiopulmonary bypass was recommenced and cardioplegia given so the heart could be inspected. Although there was no evidence of circumflex coronary artery injury, a previously placed left atrioventricular valve commissuroplasty suture was removed. The ligature on the left SVC was removed and atrioventricular sequential pacing initiated. After a period of support, the patient was successfully weaned from bypass and given dopamine (5 µg/kg per minute, noradrenaline 0.3 µg/kg per minute, glyceryl trinitrate 1 µg/kg per minute, and nitric oxide at 50 ppm). The chest was closed using a polytetrafluoroethylene membrane sutured to the skin edges because of the myocardial hemorrhage and poor hemodynamics. After 48 hours the child’s chest was closed formally. Follow-up echocardiography showed marked improvement of the left ventricular wall thickening. Pulmonary hypertension persisted in the presence of nitric oxide administration, but the patient was weaned from nitric oxide over 72 hours. Cardiac catheterization was repeated 3 weeks postoperatively, and there was moderate right atrioventricular valve regurgitation with a tiny residual ventricular septal defect and severe pulmonary hypertension. Full opacification of the left SVC was achieved and is shown in Figure 1.



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  		Fig 1. Angiogram of the coronary venous system illustrating the absence of the coronary ostium with opacification of the length of the coronary sinus and connection with the left superior vena cava (SVC).

 
Case 2
A 2.5-kg male infant with dysmorphic facial features had nonobstructed, supracardiac totally anomalous pulmonary venous drainage (TAPVD) diagnosed by transthoracic echocardiography. In addition, there was septooptic dysplasia with absent septum pellucidum and associated hydrocephalus. In view of the unobstructed nature of the TAPVD and the uncertain neurologic status, it was decided to defer cardiac operation until the neurologic issues were clarified. At age 2 months, he developed bronchiolitis and remained oxygen dependent with hyperinflated lung. Subsequently his condition deteriorated, requiring endotracheal intubation and ventilation. The contribution that his cardiac condition made to this deterioration was not known, but correction of the TAPVD was planned. Through a median sternotomy using cardiopulmonary bypass and deep hypothermia and circulatory arrest (16°C) with cold cardioplegic cardiac arrest, the vertical vein was dissected and the pulmonary venous confluence was identified. Also evident was a connecting vein running from the coronary sinus to the inferior aspect of the pulmonary venous confluence (Fig 2). The confluence was anastomosed to the left atrium and the vertical vein was ligated after the inferior connecting vein had been transected. The right atrium was opened, and no coronary sinus orifice could be identified nor was there a Thebesian valve seen (Fig 3). With the administration of cardioplegia, fluid was noted to drain from the transected inferior connecting vein suggesting the diagnosis of coronary sinus orifice atresia. A connection was fashioned between the coronary sinus and the left atrial appendage to allow drainage of the coronary sinus blood to the left atrium. There was a large atrial septal defect that was patched with autologous pericardium to complete the repair. After the aortic cross-clamp was removed, sinus rhythm resumed. The electrocardiogram findings were normal, and pulmonary artery pressures and left atrial pressures were low. The patient was weaned from cardiopulmonary bypass without difficulty and hemostasis was secured. The patient’s lungs were hyperinflated, and he required peak inspiratory pressure of at least 28 cm of water, with a positive end-expiratory pressure of 8 cm and a fraction of inspired oxygen of 100% to maintain oxygen saturation of 85%. The patient’s chest was closed with a polytetrafluroethylene membrane because of the hyperinflated lungs. Sternal closure was achieved after 48 hours without cardiac compromise but ventilator pressures remained high. The child remained dependent on a ventilator, and bronchomalacia and left main bronchus hypoplasia were diagnosed on bronchography. After 2 weeks of full support, treatment was withdrawn because of the combination of severe lung disease and a neurologic condition with a poor prognosis. At autopsy, the diagnosis of coronary sinus orifice atresia was confirmed and the repair of the TAPVD was unobstructed.



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  		Fig 2. Anatomy in case 2 showing the connection of the coronary sinus to the inferior aspect of the common pulmonary venous confluence, which drains into the vertical vein.

 


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  		Fig 3. Autopsy photograph of the interior of the right atrium showing absence of the coronary sinus orifice with the Eustachian valve, the tricuspid valve (white asterisk) and the tendon of Todaro (black arrow) visible.

 

   Comment
Top
 Abstract
 Introduction
 Case reports
 Comment
References
 
The first recorded case of coronary sinus orifice atresia was reported in 1738 [6]. It appears that coronary sinus orifice atresia can be divided into cases with drainage to a persistent left SVC and those with drainage directly to the cardiac chambers (through fenestrations in the floor of the left atrium or by communications from the coronary sinus and its veins to their related atria and ventricles) [1]. This latter group includes patients with an absent coronary sinus, in whom associated cardiac lesions appear to be more frequent [6]. The diagnosis in patients without any associated cardiac disease is incidental, although there has been a case report of thrombotic occlusion of the distal left anterior descending coronary artery in the presence of unobstructed coronary arteries in a 43-year-old woman [3].

Coronary sinus orifice atresia in AVSD with left isomerism similar to that in our patient was reported in an autopsy study by Adatia and Gittenberger-de Groot [6]. The developmental relationship between isomerism and coronary sinus abnormalities is not known. There did not appear to be any obstructing venous valves as an alternate explanation for the coronary sinus obstruction. The cardinal vein persisted as the ascending connecting vein joining the left brachiocephalic vein to the common pulmonary venous confluence. Other congenital cardiac lesions associated with coronary sinus orifice atresia with left SVC include atrial septal defect, ventricular septal defect, partial AVSD, transposition of the great arteries, tricuspid atresia, and mitral atresia.

The diagnosis of coronary sinus orifice atresia is usually made at cardiac catheterization when there is retrograde flow through a left SVC. Transthoracic echocardiography might be able to demonstrate retrograde left SVC flow using Doppler interrogation [7]. Recently, transesophageal echocardiography has been used to diagnose coronary sinus orifice atresia by detecting flow reversal in the coronary sinus [8].

In case 1 there was insufficient opacification of the left SVC and coronary sinus at cardiac catheterization for this to be certain preoperatively. As a result of the absent coronary sinus, communication to the right atrium was not fully appreciated and the left SVC was ligated. Although there were no ST-segment changes on the electrocardiogram after ligation, the patient’s complete heart block and need for cardiac pacing precluded accurate interpretation of the electrocardiogram. Watson [4] reported rapid onset of ST-segment changes and bradycardia with occlusion of the left SVC in one case with coronary sinus orifice atresia. The poor myocardial contractility was no doubt caused by the intramyocardial edema and hemorrhage triggered by coronary venous hypertension [5]. After thorough inspection of the heart, it was evident that there was no coronary sinus orifice and this was confirmed on angiography, when the coronary sinus was fully opacified after repair of the AVSD.

In case 2, the patient’s size and the presence of severe lung disease would have made a preoperative transthoracic echocardiographic diagnosis of the coronary sinus abnormality difficult. Fortunately, the diagnosis was suspected after inspection of the cardiac chambers for a coronary sinus orifice. By giving a flush of cardioplegia we confirmed the diagnosis, and the transected vein was then anastomosed to the left atrial appendage, as simple unroofing of the coronary was thought to be too hazardous because of a diminutive left atrium and absence of the usual intracardiac landmarks of the atrioventricular node. The patient’s severe associated neuroloigc and pulmonary abnormalities resulted in early death, but at no stage did the patient have any evidence of myocardial dysfunction after repair of the TAPVD and coronary sinus.

In conclusion, in any patient who has a left SVC, thorough inspection of the atrial chambers of the heart is necessary to rule out coronary sinus orifice atresia. Although this condition might be associated with no other cardiac abnormality and is a benign diagnosis, inadvertent ligation has lethal consequences. The unique association of TAPVD with coronary sinus orifice atresia was demonstrated.


   References
Top
 Abstract
 Introduction
 Case reports
 Comment
 References
 

  1. Falcone M.W., Roberts W.C. Atresia of the right atrial ostium of the coronary sinus unassociated with persistence of the left superior vena cava: a clinicopathological study of 4 adult patients. Am Heart J 1972;83:604-611.[Medline]
  2. Shinpo H., Van Praagh S., Parness I., Sanders S., Molthan M., Casteneda A. Mitral atresia with a large left ventricle and an underdeveloped or absent right ventricular sinus: clinical profile, anatomic data and surgical considerations. J Am Coll Cardiol 1992;19:1561-1576.[Abstract]
  3. Gerlis L.M., Gibbs J.L., Williams G.J., Thomas G.D.H. Coronary sinus orifice atresia and persistent left superior vena cava. Br Heart J 1984;52:648-653.[Abstract/Free Full Text]
  4. Watson G.H. Atresia of the coronary sinus orifice. Pediatr Cardiol 1985;6:99-101.[Medline]
  5. Yokota M., Kyoku I., Kitano M., et al. Atresia of the coronary sinus orifice. J Thorac Cardiovasc Surg 1989;98:30-32.[Abstract]
  6. Adatia I., Gittenberger-de Groot A.C. Unroofed coronary sinus and coronary sinus orifice atresia: implications for management of complex congenital heart disease. J Am Coll Cardiol 1995;25:948-953.[Abstract]
  7. Yeager S.B., Balian A.A., Gustafson R.A., Neal W.A. Angiographic diagnosis of coronary sinus ostium atresia. Am J Cardiol 1985;56:996.[Medline]
  8. Sunaga Y., Okubo N., Hayashi K., et al. Transesophageal echocardiographic diagnosis of coronary sinus orifice atresia. Am Heart J 1992;124:794-796.[Medline]



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This Article
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Tom R. Karl
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