Heart Views

CASE REPORT
Year
: 2004  |  Volume : 5  |  Issue : 3  |  Page : 58--61

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


Ugurlucan Murat, Basaran Murat, Kafali Eylul, Alpagut Ufuk, Dayioglu Enver, Onursal Ertan 
 Department of Cardiovascular Surgery, Istanbul Medical Faculty, Istanbul University, Turkey

Correspondence Address:
Ugurlucan Murat
Bozkurt Caddesi, Benli Apt., No: 110-112, Daire:6, 80250 Kurtulus, Istanbul
Turkey




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 2020 Aug 8 ];5:58-61
Available from: http://www.heartviews.org/text.asp?2004/5/3/58/64562


Full Text

 Introduction





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





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 [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

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2Lucas 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.
3Howard 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.
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