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Year : 2004  |  Volume : 5  |  Issue : 3  |  Page : 58-61 Table of Contents     

Atretic Coronary Sinus Orifice with Left Superior Vena Cava Draining to Left Atrium in a Child with Ellis-van Creveld syndrome

Department of Cardiovascular Surgery, Istanbul Medical Faculty, Istanbul University, Turkey

Date of Web Publication22-Jun-2010

Correspondence Address:
Ugurlucan Murat
Bozkurt Caddesi, Benli Apt., No: 110-112, Daire:6, 80250 Kurtulus, Istanbul
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Source of Support: None, Conflict of Interest: None

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How to cite this article:
Murat U, Murat B, Eylul K, Ufuk A, Enver D, Ertan O. Atretic Coronary Sinus Orifice with Left Superior Vena Cava Draining to Left Atrium in a Child with Ellis-van Creveld syndrome. Heart Views 2004;5:58-61

How to cite this URL:
Murat U, Murat B, Eylul K, Ufuk A, Enver D, Ertan O. Atretic Coronary Sinus Orifice with Left Superior Vena Cava Draining to Left Atrium in a Child with Ellis-van Creveld syndrome. Heart Views [serial online] 2004 [cited 2023 Mar 30];5:58-61. Available from: https://www.heartviews.org/text.asp?2004/5/3/58/64562

   Introduction Top

Persistent left superior vena cava connecting directly to the left atrium is a very rare congenital malformation.

The embryological development of systemic venous system is a complex process and various developmental abnormalities may occur because of the many transformations during the formation of both vena cavae. One variation is the persistence of a left superior vena cava (LSVC) which usually drains into the right atrium via the coronary sinus. In 8% of the cases however, the anomalous vein directly drains into the left atrium [1],[2] . In such cases, it is believed that the coronary sinus must be absent because of their embryological backgrounds.

We describe here a patient with Ellis-van Creveld Syndrome in whom a persistent left superior vena cava draining to the left atrium was associated with coronary sinus orifice atresia and common atrial chamber.

   Case Presentation Top

A 7-year-old male patient with Ellis-van Creveld Syndrome was admitted to our institution. When referred, he complained of dyspnea and fatigue on exertion. He was evaluated at infancy and the only pathology found was a common atrium.

Physical examination revealed dwarfism (height <3 percentile), short and deformed limbs, widely-spaced teeth and dysmorphic nails by inspection [Figure 1]. On cardiac examination, there was a midsystolic murmur audible over the left sternal edge and the lung fields were clear on auscultation. Transthoracic echocardiography (TTE) revealed only common atrial chamber [Figure 2] and a decision for operative closure of the defect was made.

The operation was performed through a median sternotomy under low flow cardiopulmonary bypass with standard aortic and bicaval cannulations at moderate hypothermia (28°C). A large portion of autologous pericardium was harvested. The LSVC was found to connect directly to the roof of the left atrium.

This unusual anomalous vessel was dissected and controlled before the establishment of extracorporeal circulation. Myocardial protection was achieved with antegrade cold crystalloid cardioplegia and topical ice saline. Following the cardiac arrest, a right atriotomy parallel to the right atrioventricular groove was fashioned. There was no visible coronary sinus orifice in the right atrium. With the administration of cardioplegia, fluid was noted to drain only from the small fenestrations on the floor of left atrium suggesting the diagnosis of coronary sinus orifice atresia. The anomalous LSVC drained directly into the roof of the left atrium. With the unsnaring of the vessel, we noticed that blood drained only through the separate orifice of the LSVC located in the roof of left atrium. Pulmonary venous connections were normal [Figure 3],[Figure 4].

The common atrial chamber was divided into right and left compartments with autologous pericardium and we decided to leave the small fenestrations and LSVC to drain into the left atrial chamber. Sinus rhythm resumed following removal of the aortic cross-clamp. The electrocardiogram findings were normal.

Blood samples were drawn from both of the seperated chambers and oxygen saturations of the right and the left sides were 65% and 98% respectively. Weaning from cardiopulmonary bypass was uneventful. The cardiopulmonary bypass and aortic cross-clamp times were 110 minutes and 88 minutes, respectively. The patient was extubated after eight hours and intensive care unit stay period was one day. The arterial oxygen saturation was 95% in room air. Postoperative TTE confirmed the effective closure of the defect with LSVC draining to the left atrium. Work-up excluded the heterotaxia syndrome with the normal splenic and bronchial structures. The child was discharged after eight days without any clinical symptoms. Another therapeutic intervention was not recommended for persistent LSVC and we asked the patient to return for follow-up periodically. He has been very well for 13 months.

   Discussion Top

Ellis-van Creveld syndrome was first described by Richard W.B. Ellis of Edinburgh and Simon van Creveld of Amsterdam in 19403. It is an autosomal recessive syndrome characterized with disproportionate dwarfism, postaxial polydactyly, ectodermal dysplasia, small chest, common atrium (most common cardiac defect) and other congenital heart defects. The pathology merits a full cardiac evaluation including TTE to detect any possible cardiac anomaly.

Embryological development of the systemic veins involves the umbilical, vitelline and cardinal venous systems. The most common venous abnormality is the persistent LSVC draining to the right atrium via coronary sinus. However, in 8% of cases, the anomalous vein drains into the left atrium [1],[2] . Although it is believed that the coronary sinus must be absent in such patients, there are also a few cases described in the literature in which a persistent left superior vena cava draining to a left atrium is associated with a normal coronary sinus [4] .

The most challenging problem in our case was the unfeasibility of LSVC for any intra- or extra-cardiac repair. The intra-atrial rerouting of vena cava to the right atrium was not considered possible because of the high position of the orifice of the anomalous vessel. On the other hand, when the LSVC was temporarily clamped to determine the feasibility of ligation, the dramatic increase in the venous pressure did not give us the opportunity for ligation. The high risk of thrombosis in venous system precluded also any extracardiac graft interposition procedure. Since it is well known that isolated LSVC, does not cause hemodynamic compromise, we preferred not to repair the persistent LSVC and asked the patient to return for follow-up periodically.

The association of LSVC connected directly to the left atrium with a coronary sinus orifice atresia is rare. Atresia of coronary sinus orifice is usually associated with a variety of congenital cardiac lesions and was first described in 1738 [5]. Although coronary sinus orifice atresia can be diagnosed at cardiac catheterization or TTE by demonstrating retrograde LSVC flow [6],[7] , it is not usually possible, and most defects are diagnosed perioperatively or at autopsy studies. Although these anomalies might be benign in isolated forms, incorrect surgical treatment may have lethal consequences if the LSVC is the only route for venous drainage of the heart. Occlusion of the LSVC in these cases may result in intramyocardial edema and hemorrhage, which is caused by coronary venous hypertension [8]. In our case, there was no continuity between LSVC and coronary sinus. We believe that the abnormal anatomy should be precisely defined during the preoperative evaluation to prevent any fatal consequence that may result from incorrect operation.

The diagnosis of these complex anomalies are usually made during cardiac operation of patients having other associated lesions. Although persistent left superior vena cava is a benign condition, the preoperative evaluation of these patients is critically important in patients with coronary sinus orifice atresia.

   References Top

1.Goor DA, Lillehei CW: Congenital Malformations of the Heart. Embryology, Anatomy, and Operative Considerations. Grune & Statton, New York; 1976: 317-27.  Back to cited text no. 1      
2.Lucas JV, Krabill KA: Abnormal Systemic Venous Connections. In: Emmanouilides GC, Riemenschneider TA, Allen HD, Gutsell HP (eds) Moss and Adam's Heart Disease in Infants, Children, and Adolescents Including the Fetus and Young Adult, 5th edn. Williams & Wilkins, Baltimore; 1995: 874-902.  Back to cited text no. 2      
3.Howard TD, Guttmacher AE, McKinnon W, et al: Autosomal dominant postaxial polydactyly, nail dystrophy, and dental abnormalities map to chromosome 4p16, in the region containing the Ellis-van Creveld syndrome locus. Am J Hum Genet 1997; 61(6): 1405-12.  Back to cited text no. 3      
4.Wiles HB. Two cases of left superior vena cava draining directly to a left atrium with a normal coronary sinus. Br Heart J 1991; 65(3): 158-160.  Back to cited text no. 4      
5.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(4): 948-53.  Back to cited text no. 5      
6.Yeager B, Balian AA, Gustafson RA et al., Angiographic diagnosis of coronary sinus ostium atresia. Am J Cardiol 1985; 56: 996.   Back to cited text no. 6      
7.Sunaga, N. Okubo, K. Hayashi et al., Transesophageal echocardiographic diagnosis of coronary sinus orifice atresia. Am Heart J 1992; 124: 794-796.   Back to cited text no. 7      
8.Yokoto M, Kyoku I, Kitano M, et al. Atresia of the coronary sinus orifice atresia. J Thorac Cardiovasc Surg 1989; 98: 30-32.  Back to cited text no. 8      


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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