Persistent Left Superior Vena Cava Draining into the Left Atrium with Absent Right Superior Vena Cava M. Sherafat, M.D., S. Friedman, M.D., and J. A. Waldhausen, M.D. ABSTRACT A 59i-year-old boy with a persistent left superior vena cava entering the left atrium first presented with a cerebral abscess. The right superior vena cava was absent, and no other intracardiac anomalies were present. One year after drainage of the abscess, the cardiovascular anomaly was torrected by placement of a Dacron baffle within the left atrial cavity in a manner which excluded the left superior vena caval blood flow from the left atrium and directed it into the right atrium. Although the initial repair did not hold up, after a second one the patient did well and had no further symptoms. Repair of this cardiovascular anomaly is best carried out by placement of an intraatrial baffle.
n a patient with no intracardiac defects, absence of the right superior vena cava with entry of a persistent left superior vena cava into the left atrium is an extremely rare anomaly. A patient similar to the one reported here, in whom surgical treatment was unsuccessful, has been reported by Tuchman and colleagues .Described herein is an operative technique used successfully in the correction of this vascular anomaly. The patient was born August 11, 1962, the product of an uneventful pregFrom the Departments of Surgery and Pediatrics, University of Pennsylvania School of Medicine, and The Children’s Hospital of Philadelphia, Philadelphia, Pa. Supported by a grant from the Delaware Heart Association. Accepted for publication June 13, 1970. Address reprint requests to Dr. Waldhausen, The Milton S. Hershey Medical Center, The Pennsylvania State University, Hershey, Pa. 17033.
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Left S V C Draining into Lejt Atrium
nancy and delivery. Physical development and exercise tolerance were considered to be normal during the first four years of life, although mild cyanosis and clubbing of the fingers and toes were recognized. Because the child was thought to be asymptomatic, these findings were not further investigated by the family. At 4 years of age the patient was hospitalized at The Children’s Hospital of Philadelphia because of convulsive seizures associated with a fever of unknown origin. T h e diagnosis of brain abscess was made by appropriate studies, and successful surgical treatment was accomplished by aspiration and drainage of the abscess via a burr hole. Recovery was complete, and there was no residual neurological deficit. Physical examination with reference to the heart was entirely negative. NO significant cardiac murmurs were heard; the second heart sound at the base of the heart was normal in intensity and character. The systemic blood pressure was normal; the femoral pulses were readily palpable. An electrocardiogram was within normal limits; the QRS complexes in the precordial leads had a left-sided, adult-type pattern. Roentgenograms of the chest showed a heart of normal size and contour except for an abnormal density in the left upper arch of the cardiac silhouette in the anteroposterior view. This appeared as an oval-shaped, walnutsized vascular shadow that was adjacent to and fused with the cardiac silhouette (Fig. 1). T h e aortic arch descended in the left hemithorax; the pulmonary vascular markings were within normal limits. A definitive cardiac study was performed in October, 1967, when the patient had reached the age of 5 years. This revealed a persistent left superior vena cava which entered the left atrium. A patent right superior vena cava could not be demonstrated. No other intracardiac abnormalities were found. The pressures in the right ventricle and pulmonary artery were normal; no gradient in pressure was found at the pulmonic or aortic valve. The peripheral arterial oxygen saturation was 72%. An injection of contrast material was made into the left superior vena cava and showed a very large, tortuous vessel carrying all of the venous blood from the upper portion of the body directly into the left atrium (Fig. 2). At operation four months later the heart was exposed through a midsternal incision. Absence of the right superior vena cava and entry of the left superior vena cava into the left atrium were confirmed. The inferior vena cava was cannulated and partial cardiopulmonary bypass initiated. When the right atrium was
FIG. 1 . Chest roentgenogram demonstrating the lesion adjacent to the pulmonary artery. VOL. I I , NO. 2, FEBRUARY,
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Venous cineangiogram demonstrating the tortuous left superior Venn cava draining into the left atrium.
opened, the atrial septum was found to be intact. The atrial septum was incised posteriorly and the superior vena cava catheter introduced through the left atrium into the left superior vena cava. Total cardiopulmonary bypass was then instituted. The atrial septum was opened wide, and a baffle consisting of pericardium was placed in such a position that the b!ood from the left superior vena cava
FIG. 3. Operative correction of the partial anomalous systemic venous return to the left atrium. A [email protected]
diverts the vena caval flow into the right atrium. 162
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drained into the right atrium (Fig. 3). The patient tolerated the operative procedure well and postoperatively had normal partial pressure of oxygen in his arterial blood. Five days after operation a return of the cyanosis was observed along with some distention of the superficial veins on both sides of the neck. An injection of contrast material was made into the left superior vena cava. It entered the left atrium immediately upon injection, and the aorta was rapidly visualized. On the sixteenth postoperative day the child was reexplored. When the right atrium was opened, the site of entry of the left superior vena cava into the right atrium was found to be considerably narrowed. There was also a point of detachment of the pericardial baffle from the posterior wall of the left atrium that permitted venous blood from the left superior vena cava to enter the left atrium. The initial repair was undone, and a large Dacron patch was sutured into the left atrium in a manner similar to that used to install the initial pericardial baffle. This patch divided the left atrium so that the left superior vena caval blood entered the right atrium. This reoperation was also well tolerated, and the postoperative period was uneventful. No further cyanosis was observed; a transient cervical venous pulsation was noted, but this subsided progressively during the next few weeks. T h e patient has been followed for sixteen months since his second operation. The cervical venous pulsation is no longer evident, and there are no signs of superior vena caval obstruction. T h e cyanosis has disappeared completely, as has the clubbing of the fingers and toes. The presence of normal circulatory hemodynamics is presumed.
T h e embryology of the venae cavae has been amply described by Campbell and Deuchar [l] as well as by Taybi and his associates [31. Persistence of the left superior vena cava is usually of little surgical significance unless it enters the left atrium, producing a venoarterial shunt. Although such patients may be asymptomatic, the serious potential complications associated with a right-to-left shunt, e.g., brain abscess and cerebrovascular accident, make operative correction mandatory. In the presence of a right superior vena cava, ligation of the left superior vena cava is effective in correcting the abnormal hemodynamics. However, in the rare situation of our patient, as well as in that of the patient reported by Tuchman and coauthors ,the right superior vena cava was absent, making it necessary to transplant the left superior vena cava into the right atrium. Shumacker and associates [21 described 2 patients with multiple intracardiac defects in whom a simple technique was used for anastomosing the left superior vena cava to the right atrium after detaching it from the left atrium. T h e anastomosis remained patent in only 1 of the patients; the other patient developed a right ventricular aneurysm at the site of an outflow tract reconstruction for tetralogy of Fallot. T h e aneurysm occluded the cava-to-atrium anastomosis. In our patient, redirection of blood from the left superior vena cava into the right atrium was accomplished by use of an intracardiac VOL. 11, NO. 2, FEBRUARY,
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baffle. Although this appeared to function well initially, the correction was not maintained because the baffle was too small and several sutures pulled through the tissues. Following a second, successful repair using a Dacron patch as the baffle, the patient became acyanotic and is now apparently cured. T h e major difficulties in the extracardiac venous transplantation repair lie in moving the left superior vena cava to a position anterior to the ascending aorta and in anastomosing it to the right atrium. T h e length of the cava may be inadequate, necessitating some type of plastic procedure as described by Shumacker and his co-workers . Compression of the cava between the aorta and sternum may also be a potential complication. The intracardiac repair requires that a baffle of adequate size be put in place so that caval obstruction does not occur, as happened in our initial attempt. The mild and transient elevation in superior vena caval pressure associated with venous pulsation that followed the second repair was probably related to the relatively small size and underdevelopment of the new right atrial cavity. REFERENCES
1. Campbell, M., and Deuchar, D. C. Left-sided superior vena cava. Brit. Heal-t J . 16:423, 1954. 2. Shumacker, H. B., Jr., King, H., and Waldhausen, J. A. T h e persistent left superior vena cava: Surgical implications, with special reference to caval drainage into the left atrium. Ann. Surg. 165:797, 1967. 3. Taybi, H., Kurlander, G. J., Lurie, P. R., and Campbell, J. A. Anomalous systemic venous connection to the left atrinm or to a pulmonary vein. Amer. J. Roentgen. 94:62, 1965. 4. Tuchman, H., Brown, J. F., Huston, J. H., Weinstein, A. B., Rowe, G., and Crumpton, C. W. Superior vena cava draining into left atrium. Amer. J. Med. 21:481, 1956.
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