Abstract
We treated two patients with a rare developmental complex. The persistent left superior vena cava draining into the left atrium (PLSVC into LA) was associated with an absent coronary sinus and an atrial septal defect. Ligation of PLSVC and patch-repair of the atrial septal defect were successfully performed in one stage. The atrial septal defect was located in the upper and posterior aspect of the interatrial septum and appeared to be an unique type of atrial septal defect. In the other patient, additional multiple cardiac defects were associated with this syndrome, including ventricular septal defect, pulmonary stenosis, tricuspid insufficiency, and complete transposition of the great arteries. Palliative Blalock procedure was used for this patient. The PLSVC into LA was discovered accidentally in both cases during heart catheterization and it was clearly demonstrated by venography. For a preoperative recognition of PLSVC, computed tomograms of the heart are of great assistance. Surgical correction of the persistent superior vena cava was emphasized for treatment of this syndrome.
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Raghib, G., Ruttenberg, H.D., Anderson, R.C., Amplatz, K., Adams, P., and Edwards, J.E.: Termination of left superior vena cava in left atrium, atrial septal defect, and absence of coronary sinus. Circulation 31: 906–918, 1965.
Lee, M.E. and Sade, R.M.: Coronary sinus septal defect. Surgical consideration. J. Thorac. Cardiovasc. Surg. 78: 563–569, 1979.
Winter, F.S.: Persistent left superior vena cava. Angiology 5: 90–132, 1954.
Taybi, H., Kurlander, G.J., Lurie, P.R., and Campbell, J.A.: Anomalous systemic venous connection to the left atrium or to a pulmonary vein. Am. J. Roentgenol. 94: 62–77, 1965.
Mantini, E., Grondin, C.M., Lillehei, C.W., Edwards, J.E.: Congenital anomalies involving the coronary sinus. Circulation 33: 317–327, 1966.
Frank, C.G. and Maloney, J.V. Jr.: Surgical significance of the coronary sinus. J. Cardiovasc. Surg. 9: 420–427, 1968.
Goor, D.A. and Lillehei C.W.: Congenital malformation of the heart. Embryology, anatomy, and operative considerations. pp. 402–404, Grune & Stratton, New York, 1975.
Rastelli, G.C., Ongly, P.A., and Kirklin, J.W.: Surgical correction of common atrium with anomously connected persistent left superior vena cava: Report of case. Mayo Clin. Proc. 40: 528–532, 1965.
Meadows, W.R. and Sharp, J.R.: Persistent left superior vena cava draining into the left atrium without arterial oxygen unsaturation. Am. J. Cardiol. 16: 273–279, 1965.
Shumacker, H.B. Jr., King, H., and Waldhausen, J.A.: The persistent left superior vena cava surgical implications, with special reference to caval drainage into the left atrium. Ann. surg. 165: 797–805, 1967.
Helseth, H.K. and Peterson, C.R.: Atrial septal defect with termination of left superior vena cava in the left atrium and absence of the cronary sinus. Recognition and correction. Am. Thorac. surg. 17: 186–192.
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Okumori, M., Hyuga, M., Ogata, S. et al. Raghib’s syndrome: A report of two cases. The Japanese Journal of Surgery 12, 356–361 (1982). https://doi.org/10.1007/BF02469635
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DOI: https://doi.org/10.1007/BF02469635