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Ann Thorac Surg 2003;76:2091-2092
© 2003 The Society of Thoracic Surgeons

Case report

Atresia of coronary sinus ostium with retrograde drainage via persistent left superior vena cava

Neerod K. Jha, MCha, Apoorva Gogna, MBBSa, Teng H. Tan, MRCPb, Keng Y. Wong, MMedb, Sriram Shankar, FRCSa*

a Departments of Cardiothoracic Surgery, Singapore
b Cardiology, KK Women's and Children's Hospital, Singapore

Accepted for publication February 28, 2003.

* Address reprint requests to Dr Shankar, Department of Cardiothoracic Surgery, Level-2, Children's Tower; KK Women's and Children's Hospital, 100, Bukit Timah Rd, Singapore 219899
e-mail: shankar{at}kkh.com.sg


   Abstract
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 Abstract
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 References
 
Atesia of the coronary sinus ostium (ACSO) with retrograde drainage of cardiac veins via the left superior vena cava (LSVC) is a very rare abnormality. This condition is usually asymptomatic during life and a majority of the cases were reported as incidental postmortem findings. If there is retrograde venous drainage via persistent LSVC, this communication cannot be ligated irrespective of its size or the presence of a communicating vein because of resultant cardiac congestion and edema. We report herein a 9-month-old Chinese female who underwent repair of a perimembranous ventricular septal defect, patent ductus arteriosus and secundum atrial septal defect. During the operation, ligation of LSVC resulted in myocardial congestion and distension of the heart. The release of ligature decompressed the heart immediately.


   Introduction
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 Abstract
 Introduction
Comment
 References
 
Atresia of coronary sinus ostium (ACSO) with a persistent left superior vena cava (LSVC) is a rare cardiac anomaly and only 35 such cases have been reported so far in available English literature [1–6]. In about 50% of such cases, LSVC was the only out flow channel for the coronary sinus [1]. Usually, ACSO with retrograde drainage via LSVC is a benign anomaly without physiologic consequences [2]. However, recognition of this venous anomaly during cardiac surgery has surgical and physiologic implications, especially in patients in whom ligation or division of LSVC is indicated. Interruption of coronary sinus drainage may lead to myocardial edema, ischemia, and necrosis with poor outcome [1].

A 9-month-old Chinese female presented with failure to thrive. Her body weight was 3.9 kg ( < 3rd centile). She was on diuretics. A two-dimensional echocardiogram revealed a large perimembranous ventricular septal defect (VSD) measuring 4.8 mm X 5.2 mm with bi-directional flow. A small secundum atrial septal defect (ASD) with a patent ductus arteriosus (PDA) was also present with left to right shunting.

At operation, a left superior vena cava was found in addition to the right superior vena cava (RSVC). There was a bridging vein connecting the two. After routine cannulation of the aorta, RSVC and inferior vena cava cardiopulmonary bypass was instituted. The PDA was then ligated. The LSVC was snared with a silk tie. After antegrade blood cardioplegia through the right atrium, Dacron patch closure of VSD and direct suture closure of ASD was done. We were unable to locate the orifice of the coronary sinus that appeared to end blindly at its atrial end. We then de-aired the heart and found de-saturated blood coming from the aorta at the cardioplegia site. After the removal of the aortic cross clamp, ligation of LSVC was done, which resulted in significant engorgement of the cardiac veins in all territories and the heart turned bluish in color. Also, there was distension of the heart. The cardiac contractions were poor, although at this point in time we were only 6 to 7 minutes into reperfusion. We, therefore, released the ligature in the LSVC and that resulted in immediate decompression of the heart including cardiac veins. The heart turned pink with good contractions.

The patient recovered uneventfully and remains well on follow-up 6 months after surgery. To further evaluate the venous anatomy, an angiocardiogram done recently has confirmed the diagnosis of LSVC with ACSO and normal drainage of cardiac veins (Fig 1).



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  		Fig 1. Angiocardiogram (left oblique anterior view) showing right atrium (RA), right superior vena cava (RSVC), innominate vein (Inno Vein), left superior vena cava (LSVC), and the coronary sinus (CS) with absent ostium. (LAO = left anterior oblique.)

 

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At the 4th week of development, the sinus venosus has right and left horns, each receiving blood from the 3major systemic veins, namely, the vitelline, umbilical, and common cardinal veins. However, during the 5th week, with the obliteration of the left vitelline vein the left horn begins to regress. By the 10th week, when the left common cardinal vein is also occluded, the left horn has regressed to form the coronary sinus and the oblique vein of ‘Marshall’ [3].

Persistence of the left anterior cardinal vein gives rise to LSVC, which is the most common abnormality of the distribution of the great cardiac veins [3]. This may be accompanied by obliteration of the right common cardinal vein, whereby the RSVC fails to form. Alternatively, both cavae may persist. Atresia of the coronary sinus ostium is usually associated with an unroofed coronary sinus, constituting a small right to left shunt [2, 4]. A classification of coronary sinus abnormalities was suggested by Mantini et al. in 1966, namely, enlarged coronary sinus, absent coronary sinus, atresia of right atrial ostium of the coronary sinus, and hypoplasia of the coronary sinus [4]. Anatomically, ACSO consists of membranous occlusion with or without segmental interruption of coronary sinus [2].

Atresia of the coronary sinus ostium in association with LSVC without the left atrial connection is a very rare entity. In this situation, cardiac venous blood is forced to flow in retrograde direction via the coronary sinus into the LSVC. The LSVC then drains into the innominate vein and finally into the right atrium [1, 2, 5, 6]. Since the first description of this anomaly in 1738 by ‘Le Cat'et al., the majority of the cases so far were identified at autopsy [2]. A retrograde LSVC flow with a normal size or collapsed coronary sinus on angiography or echocardiography may alert the possibility of ACSO. On Doppler ultrasound, retrograde flow of blood away from the coronary sinus may raise the possibility of atresia of its orifice. However, during surgery inspection of the right atrial cavity confirms the diagnosis of ACSO. The LSVC with patent coronary sinus may pose a problem as blood flows via the coronary sinus into the right atrium, thus flooding the operating field. Occasionally, the LSVC may be cannulated separately if it is large enough. Ligation of the LSVC may be required in patients considered for Fontan or bi-directional Glenn procedure to establish an obligatory drainage of systemic veins to the pulmonary arterial circulation [6]. Ligation of LSVC in association with ACSO has a negative effect on the resulting coronary venous hypertension on myocardial perfusion [6]. Postoperative death has been reported after ligation of a LSVC in a patient with coronary sinus atresia [1].

Ligation or temporary occlusion of LSVC can be done safely if there is a bridging vein between the two cavae with a patent coronary sinus ostium [2]. Therefore, in the event that a LSVC with a dilated coronary sinus is detected on a preoperative echocardiogram, special care should be taken during surgical management of cardiac lesions to ensure patency of the coronary sinus before dividing or ligating the LSVC to avoid an adverse outcome.


   References
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 Abstract
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 References
 

  1. Yokota M., Kyoku I., Kitano M., Shimada I., 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]
  2. Santoscoy R., Walters H.L., 3rd, Ross R.D., Lyons J.M., Hakimi M. Coronary sinus osteal atresia with persistent left superior vena cava. Ann Thorac Surg 1996;61:879-882.[Abstract/Free Full Text]
  3. Moore KL, Persaud TVN, editors. The cardiovascular system. In: The Developing Human: Clinically Oriented Embryology, 6th edition. Philadelphia: WB Saunders; 1998:349–55
  4. Mantini E., Grondin C.M., Lillehei C.W., Edwards J.E. Congenital anomalies involving the coronary sinus. Circulation 1966;33:317-327.[Abstract/Free Full Text]
  5. von Ludinghausen M., Lechleuthner A. Atresia of the right atrial osteum of the coronary sinus. Acta Anat 1988;131:81-83.[Medline]
  6. Muster A.J., Naheed Z.J., Backer C.L., Mavroudis C. Is surgical ligation of an accessory left superior vena cava always safe. Pediatr Cardiol 1998;19:352-354.[Medline]



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