Mitral valve repair in patient with absent right superior vena cava in visceroatrial situs solitus
We report on a 74-year-old woman with an absence of right superior vena cava in visceroatrial situs solitus who underwent mitral valve plasty for severe mitral regurgitation. Preoperative three-dimensional computed tomography revealed an absent right and persistent left superior vena cava that drained into the right atrium by way of the coronary sinus. Perioperaively, placement of pulmonary artery catheter, site of venous cannulation, and management of associated rhythm abnormalities were great concern. Obtaining the information about this central venous malformation preoperatively, we performed mitral valve plasty without any difficulties related to this anomaly.
KeywordsAbsent right superior vena cava Persistent left superior vena cava Visceroatrial situs solitus Mitral valve plasty
Persistent left superior vena cava (PLSVC) with absent right superior vena cava (RSVC) in visceroatrial situs solitus is an extremely rare congenital anomaly [1, 2]. This anomaly can exist alone, and is difficult to diagnose because the hemodynamics of patients with this condition are normal and there may be lack of clinical symptoms. It is often discovered accidentally while undergoing through examination for heart disease, during pacemaker implantation, or central venous catheter insertion. Before the surgical intervention, this anomaly should be diagnosed correctly to avoid any complications. We report one patient with this anomaly who underwent mitral valve plasty for severe mitral regurgitation.
Our perioperative management plans were as follows; (1) placement of a pulmonary artery catheter through a right femoral vein under fluoroscopy; (2) insertion of a venous drainage cannula through the PLSVC itself and inferior vena cava; and (3) placement of temporary pacing wires to the right ventricle to manage the associated rhythm abnormalities.
Persistent left superior vena cava with absent RSVC in visceroatrial situs solitus is extremely rare, occurring in 0.07 to 0.15% in patient with transvenous pacemaker or defibrillator implantations, 0.09 to 0.13% in postmortem cases of congenital heart disease [1, 2, 3]. Although absence of RSVC in patients with visceroatrial situs solitus is by itself of no hemodynamic significance, its diagnosis before surgery or other invasive procedure is important to avoid various management difficulties, which include the following. (1) Implantation of a transvenous pacemaker or defibrillator. (2) Placement of pulmonary artery catheter for intraoperative or intensive care unit monitoring. (3) Systemic venous cannulation for cardiopulmonary bypass or extracorporeal circulation, (4) Cavopulmonary anastomosis, and (5) Orthtopic heart transplantation [1, 2]. The diagnosis can be confirmed by TTE, TEE, venous angiography, computed tomography or magnetic resonance imaging. Preoperative MDCT is useful for detection and directly visualizing for whole image of these venous anomalies [4, 5]. In this particular case, our perioperative strategy regarding the placement of pulmonary artery catheter and the venous cannulation site in preparation for cardiopulmonary bypass seemed to be reasonable. A venous cannula can be inserted into PLSVC in either way: a cannula can be inserted from the coronary sinus, or an L-shaped cannula can be directly inserted into the PLSVC. Direct insertion of cannula into coronary sinus would be dangerous from the standpoint of coronary sinus injury . In this patient, PLSVC was well exposed so taping and cannulation can be carried out without any difficulty, it is no need for cannulation to PLSVC through coronary sinus.
A higher incidence of arrhythmia and conduction system abnormalities has been described in patients with PLSVC. A dilated coronary sinus stretches the atrioventricular nodal tissue, or the early conduction tissue has close proximity to the cardinal venous tissue and this leads to sinus node dysfunction [7, 8]. This case did not demonstrate abnormalities of cardiac conduction system preoperatively, although all these rhythm abnormalities are not necessarily related to those venous anomalies itself, careful management in certain types of arrhythmias is need perioperatively.
We report our case of treating a patient with the absent RSVC in visceroatrial situs solitus who underwent mitral valve plasty. Preoperative MDCT is useful non-invasive imaging modality for detection of central venous anomaly. Perioperative strategy including placement of pulmonary artery catheter, selection of venous cannulation site in preparation for cardiopulmonary bypass and control of associated supraventricular rhythm abnormalities leads successful management and good clinical result.
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