The coexistence of unroofed coronary sinus and persistent left superior vena cava is a rare cardiac anomaly that is known as Raghib syndrome in recognition of the person who first described it. Reference Caballero, Izquierdo and Jaurena1 Early diagnosis of this anomaly is important for avoiding brain abscess or brain oedema that can be caused by right-to-left shunt.
In this study, the surgical treatment of intraoperatively detected unroofed coronary sinus, persistent left superior vena cava, and coronary sinus orifice atresia in a patient with tetralogy of Fallot is presented.
Case
A 16-month-old boy with tetralogy of Fallot was admitted. A physical examination detected remarkable cyanosis and systolic ejection murmur in the pulmonary area. In his transthoracic echocardiography, a large ventricular septal defect, valvular and infundibular pulmonary stenosis were observed. The McGoon ratio was 2.0. There was no additional cardiac anomaly. During the initial mediastinal exploration, a rudimentary innominate vein and persistent left superior vena cava were detected. Aorto-bicaval cannulation was performed, and cardiopulmonary bypass was started. Body temperature was decreased to 32°C. Cardiac arrest was performed by antegrade blood cardioplegia and aortic cross-clamp. After right atriotomy, it was observed that the interatrial septum was intact, and there was no coronary sinus ostium. The persistent left superior vena cava was drained into the roof of the left atrium through unroofed coronary sinus. Persistent left superior vena cava was cannulated. Ventricular septal defect was repaired by a polytetrafluoroethylene patch. Muscle bundles in the right ventricle outflow tract were resected. The monocusp pulmonary valve reconstruction was performed with 0.1-mm-thick polytetrafluoroethylene patch, and right ventricle outflow tract reconstruction was completed with autogenic pericardial patch. Right atriotomy was closed and aortic cross clamp was removed. Persistent left superior vena cava was transected from the junction of the left atrium. The atrial side closed primarily. After the ligation and division of the hemiazygos vein, persistent left superior vena cava was passed through the posterior side of ascending aorta and anastomosed to the right superior vena cava by end-to-side anastomosis technique. The patient was weaned from cardiopulmonary bypass without any complication.
After the patient was followed up in ICU for 2 days, he was discharged home on postoperative day 7. During his early post-operative follow-up, there was not any facial oedema or venous dilatation. An echocardiography taken 14 months post-procedure indicated that, there was laminar flow on the persistent left superior vena cava – right superior vena cava anastomosis.
Discussion
This study makes an original contribution to the literature by presenting a surgical procedure for the first patient with persistent left superior vena cava, unroofed coronary sinus, intact atrial septum, coronary sinus orifice atresia, and tetralogy of Fallot. When the literature is reviewed, there are five cases with tetralogy of Fallot, unroofed coronary sinus, and persistent left superior vena cava. Reference Nomura, Kurosawa, Morita, Miyamoto, Tatara and Hanai2,Reference Ueno, Sakata and Iguro3,Reference Jian, Li and Xiao4,Reference Muthialu, Rao and Balakrishnan5,Reference Mallula, Patel, Abdulla and Bokowski6 In these cases, the authors described some surgical techniques for directing of persistent left superior vena cava to the right atrium. These techniques are tunnelling with interatrial patch, reposition of interatrial septum, extracardiac mobilisation, and anastomosis of persistent left superior vena cava.
In particular, the studies designed by Caballero Reference Caballero, Izquierdo and Jaurena1 and Ueno Reference Ueno, Sakata and Iguro3 are similar to our case with detailed explanation of the extracardiac directing of persistent left superior vena cava to the right atrium. In these studies, the authors noted that the extracardiac anastomosis is more effective than intracardiac repair in the presence of complete unroofed coronary sinus. But Ueno and his colleagues preferred the intra-atrial rerouting technique for their patient due to the presence of incomplete unroofed coronary sinus and a history of previous cardiac surgery. Reference Ueno, Sakata and Iguro3
In the preoperative echocardiography of our patient, persistent left superior vena cava, unroofed coronary sinus and coronary sinus orifice atresia could not be detected. This missed diagnosis was due to the absence of coronary sinus dilatation. Reference Hahm, Park, Lee, Choi, Sul and Lee7 In addition, the probable cyanosis of unroofed coronary sinus and persistent left superior vena cava were concealed due to cyanosis caused by tetralogy of Fallot.
In a study of 20 patients, Xie and his colleagues pointed out that transthoracic echocardiography is 65% successful in the diagnosis of unroofed coronary sinus. Reference Xie, Yang and Cheng8 The failure to diagnose unroofed coronary sinus and persistent left superior vena cava can cause postoperative persistent arterial desaturation.
If the innominate vein is wide enough in a patient with persistent left superior vena cava drained into the left atrium, persistent left superior vena cava can be ligated. However, 65% of patients with persistent left superior vena cava do not have an innominate vein or have small ones. Reference Webb, Gamsu, Speckman, Kaiser, Federle and Lipton9 In this instance, persistent left superior vena cava should be directed to the right atrium or any systemic vein. For this purpose, the intra-cardiac rerouting technique can be used. However, this technique may cause impaired pulmonary venous drainage, supramitral gradient or atrial arrhythmia if not performed correctly. Reference Van Son, Hambsch and Mohr10 Because of its close proximity to the roof of the left atrium, the interatrial conduction pathways may be injured during intra-atrial rerouting, causing atrial arrhythmia. For our patients, we preferred the extracardiac anastomosis technique so as not to extend aortic cross-clamp time. Furthermore, with this extracardiac technique, the growth potential of tissues is preserved with the use of native tissue.
In the extracardiac approach, mobilising persistent left superior vena cava is important for surgical success. If necessary, the hemiazygos vein should be ligated and divided from persistent left superior vena cava. The anastomosis side should be chosen carefully. Otherwise, persistent left superior vena cava might be obstructed under ascending aorta or kinked during the procedure.
Conclusion
Our patient is the first patient with association of persistent left superior vena cava, unroofed coronary sinus, coronary sinus orifice atresia, and tetralogy of Fallot reported in the literature review. After the intracardiac repair of tetralogy of Fallot, we directed persistent left superior vena cava to the right atrium through the right superior vena cava successfully. We believe that the extracardiac direct anastomosis technique for persistent left superior vena cava is more effective, practical, and with considerably less risk than the intra-atrial rerouting technique.
Acknowledgements
We thank MD. Numan Ali Aydemir for institutional support. We are also grateful to Anthony Townley for providing language help and writing assistance.
Financial support
This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
Conflicts of interest
None
