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1 A New Procedure for the Transposition of the Great An Experimental Study Palliation of Vessels Francis Robicsek, M.D., Harry K. Daugherty, M.D., Wilfred Tam, M.D., Paul W. Saqger, M.D., and Emanuel Bagby T ransposition of the great vessels is a congenital anomaly in which the aorta originates anteriorly from the right ventricle and the pulmonary artery posteriorly from the left ventricle. This condition has an unfavorable clinical prognosis, and the patient s survival depends largely on associated cardiac defects that allow communication between the otherwise isolated systemic and pulmonary circuits. As a method of palliation, Blalock and Hanlon [6] presented in 1948 an experimental procedure that allowed closed creation of atrial septal defects. Clinical application of this method was reported two years later by the same authors [7]. Rashkind and Miller [28] recently demonstrated that sizable intraatrial communications could be created by rupturing the foramen ovale with a balloon catheter. Helmsworth et al. [19], Cooley et al. [ll], and others [Z, 17, 431 recommended that in cases where transposition is complicated with ventricular septal defect and pulmonary hypertension, creation of an atrial defect should be supplemented by a pulmonary banding [25] procedure. Now, 20 years after its first application, the Blalock-Hanllon operation, with its modification [18, 401, is still the most commonly used method of palliation in transposition of the great vessels. Implantation of the right pulmonary veins into the right atrium as an alternative to the creation of an atrial septal defect was recommended by Lillehei and Varco [221 in The same hemodynamic principle, but with a different technical approach, was used by Edwards and associates [14] in The Baffes procedure [3] combines transplantation of the right pulmonary veins into the right atrium with anastomosis of the inferior vena cava to the left atrium. From the Department of Cardiovascular and Thoracic Surgery, Charlotte Memorial Hospital, Charlotte, N.C This work was supported by grants from The John A. Hartford Foundation, Inc., of New York, N.Y. Accepted for publication Aug. 28, VOL. 7, NO. I, JAN.,

2 ROBICSEK ET AL. During the past 15 years, anatomical correction of this disease has been attempted [4, 5, 20, 21,26,411, but never successfully. An effective method of physiological correction, described in 1954 by Albert [l], consists of intraatrial rearrangement of the caval and pulmonary veins in such a way as to provide the pulmonary circuit with venous blood and the systemic circuit with oxygenated blood. This method was later modified by others [12, 23, 38, 421 who used various autogenous and synthetic materials to achieve this redirection of the atrial blood flow. The intraatrial retransposition, however, is the ideal method available [Z]; unfortunately, it carries a very high operative risk, especially in the very young and the very ill. This is why various palliative measures that do not require the usage of cardiopulmonary bypass still enjoy wide popularity. The purpose of this paper is to present a different operation for possible application in patients with transposition of the great vessels and pulmonary stenosis. This procedure combines cava-pulmonary anastomosis with an additional anastomosis of the right pulmonary veins to the central end of the divided superior vena cava. METHOD AND MATERIALS Twenty-four mongrel dogs of both sexes were anesthetized intravenously with pentobarbital, intubated, and ventilated with a Jefferson respirator. The chest was entered through the right fourth interspace and the pericardium opened posterolateralry to the phrenic nerve. The superior vena cava was divided proximal to the orifice of the azygos vein and its distal end was anastomosed end to end to the peripheral end of the divided right pulmonary artery. At the completion of the cava-pulmonary anastomosis, the lung was ventilated and the pulmonary circulation was reestablished for four to five minutes. The superior vena cava was then cross-clamped again and 1,000 U. heparin sulfate was injected into the right pulmonary artery. The right pulmonary veins were FIG. 1. Schematic presentation of the procedure. 22 THE ANNALS OF THORACIC SURGERY

3 Transposition of Great Vessels detached from the left atrium and anastomosed end to end to the central end of the superior vena cava (Fig. 1). Following the anastomosis, the clamps were removed, and the venous blood from the distal segment of the superior vena cava was allowed to enter the circulation of the right lung and from there to flow through the central segment of the superior vena cava into the right heart. RESULTS The operation was generally well tolerated by the animals, who received the usual postthoracotomy care after surgery. Postoperative complications were common, however. Atelectasis and pleural effusion were encountered frequently. Appropriate treatment consisted of administration of antibiotics and repeated thoracocentesis. Nine of the 24 animals died within one week following the operation, all from pulmonary complications. Thrombosis of one of the two anastomoses was found in 4 of them. There were 3 late deaths, 2 due to infection of the right pleural cavity and 1 caused by distemper. Angiographic studies were carried out one week to three months after the operation; 15 cc. of 50% Hypaque was injected through a catheter introduced into the superior vena cava. The cava-pulmonary anastomosis was patent in all animals. Rapid clearance of dye from the lung into the right atrium was demonstrated in 9 dogs. Hold-up of dye in the pulmonary veins was present in 3 animals. These 3 animals were proved to have severe Stricture or occlusion of the pulmonary vein-vena cava anastomosis at autopsy. Hemodynamic studies were done in 5 dogs with open anastomoses. The pressures in the inferior caval veins, right heart, and pulmonary artery were found normal. The pressure in the superior vena cava, however, was moderately elevated (9 to 16 mm. Hg) in all animals. Indicating the patency of the pulmonary vein-superior vena cava anastomosis, a left-to-right shunt ranging from 25 to 62% of the systemic flow was demonstrated in all 5 dogs. The surviving animals were sacrificed one week to three months after the operation. A cava-pulmonary anastomosis of satisfactory caliber was found in all of them. The pulmonary vein-vena cava anastomosis, however, was completely occluded in 3 dogs. Two additional animals had stricture of this anastomosis, which was judged to be significant. The lungs appeared normal and not congested except for some patchy atelectasis and fibrin daposits usually observed following thoracotomies. The hearts were somewhat enlarged, and 3 dogs showed frank left-ventricular dilatation. DISCUSSION Cava-pulmonary anastomosis is a procedure designed to deliver venous blood into the pulmonary circuit in certain forms of congenital heart disease. The physiological basis of this method has been described independently by several groups of investigators, including Carlon and associates in Italy [8-101, Darbinian and Mohalkin [13, 241 in Russia, and Glenn and associates [15, 161 and the authors of this paper [ in the United States. In 1958 we successfully applied this principle in an 1 l-year-old boy with transposition of the great vessels and pulmonary stenosis [24, 321. The patient is still doing well 10 years later. Experiences similar to ours have been reported by Glenn [15] and Carlon [8]. The beneficial effect of the cava-pulmonary anastomosis upon the patient who has transposition but no pulmonary hypertension un- VOL. 7, NO. 1, JAN.,

4 ROBICSEK El' AL. doubtedly stems from the fact that this operation delivers pure venous blood into the right lung, thus improving the degree of oxygenation. Unfortunately, due to the nature of the anomaly, this blood will be drained through the right pulmonary veins into the left heart and from there will recirculate into the left lung. The addition of the pulmonary vein-superior vena cava anastomosis eliminates this vicious circle and delivers the blood into the right heart, from which it is forwarded into the arterial circulation. Our experiments have demonstrated that this procedure is anatomically and physiologically feasible. It must be considered that this procedure was performed on normal dogs, so that the operation places the burden of a large left-to-right shunt on the heart, thus worsening the hemodynamics. In a patient with transposition, one may hopefully expect the opposite effect. The fact that a significant number of animals developed thrombosis and late occlusion of the anastomosis indicates that while the procedure may have potential merit, further experimental experience must be gained before clinical application can be considered. SUMMARY The authors present a new method of palliation that drains the circulation of the right lung into the systemic atrium in transposition. The superior vena cava is anastomosed end-to-end to the peripheral end of the transected right pulmonary artery. The right pulmonary veins are detached from the left atrium and implanted into the central stump of the superior vena cava. Possible application of this procedure in the palliative treatment of transposition of the great vessels without pulmonary hypertension is discussed. REFERENCES 1. Albert, H. M. Surgical correction of transposition of great vessels. Surg. Forum 5:74, Ankeney, J. L., and O'Grady, T. J. The treatment of transposition of the great vessels. Ann. Thorac. Surg. 5:262, Baffes, T. G. A new method for surgical correction of transposition of the aorta and pulmonary artery. Surg. Gynec. Obstet. 102:227, Bailey, C. P., Cookson, B. D., Downing, D. F., and Neptune, W. B. Cardiac surgery under hypothermia. J. Thorac. Surg. 27:72, Bjork, V. O., and Bouckaert, L. Complete transposition of the aorta and pulmonary artery: An experimental study of surgical possibilities for its treatment. J. Thorac. Surg. 28:632, Blalock, A., and Hanlon, C. R. Interatrial se tal defect: Its experimental production under direct vision. Surg. Gynec. 0 f stet. 87:183, Blalock, A., and Hanlon, C. R. The surgical treatment of complete transposition of the aorta and pulmonary artery. Surg. Gynec. Obstet. 90:1, Carlon, C. A. Personal communication. 24 THE ANNALS OF THORACIC SURGERY

5 Transposition of Great Vessels 9. Carlon, C. A., Mondini, P. G., and De Marchi, R. Su una anastomosi vasculari per la terapia chirurgia di aluni vizi cardiovasculari. G. Ztal. Chir. 6:760, Carlon, C. A., Mondini, P. G., and De Marchi, R. Surgical treatment of some cardiovascular diseases (a new vascular anastomosis). J. Znt. CoU. Surg. 16:1, Cooley, D. A., Hallman, G. L., Bloodwell, R. D., and Leachman, R. D. Two-stage surgical treatment of complete transposition of the great vessels. Arch. Surg. (Chicago) 93:704, Creech, O., Mahaffey, D. E., Sayegh, S. F,, Sailors, S. F., and Sailors, E. L. Complete transposition of the great vessels: A technique for intra-cardiac correction. Surgery 43:349, Darbinian, T. M. Functional study of the anastomosis of the superior vena cava to the right pulmonary artery. Sovet. Med. 21:28, Edwards, W. S., Bargeron, L. M., and Lyons, C. Reposition of right pulmonary vein in transposition of the great vessels. J.A.M.A. 188:522, Glenn, W. W. L. Circulatory bypass of the right side of the heart: IV. Shunt between superior vena cava and distal right pulmonary arteryreport of clinical application. New Eng. J, Med. 259:117, Glenn, W. W. L., and Patino, J. F. Circulatory bypass of the right heart. Preliminary observations on the delivery 9f vena caval blood into the pulmonary arterial circulation. Azygos vein-pulmonary artery shunt. Yale J. Biol. Med. 27:147, Haller, J. A., Jr. In discussion of Willman, V. L., Cooper, T., Mudd, J. G., and Hanlon, C. R. Treatment of ventricular septa1 defect by constriction of pulmonary artery. Arch. Surg. (Chicago) 85:745, Hallman, G. L., and Cooley, D. A. Complete transposition of great vessels: Results of surgical treatment. Arch. Surg. (Chicago) 89:891, Helmsworth, J. A., Kaplan, S., Keirle, A. M., and Jones, D. V. Results of palliative and curative operations for transposition of aorta and pulmonary artery. Circulation 29(Suppl. I): 114, Idriss, F. S., Goldstein, I. R., Grana, L., French, D., and Potts, W. S. A new technique for complete correction of transposition of the great vessels: An experimental study with preliminary report. Circulation 24:5, Kay, E. G., and Cross, F. S. Surgical treatment of transposition of the great vessels. Surgery 38:712, Lillehei, C. W., and Varco, R. L. Certain physiologic, pathologic and surgical features of complete transposition of the great vessels. Surgery 34:376, Merendino, K. A., Jesseph, J. E., Herron, P. W., Thomas, G. I., and Vitto, R. R. Interatrial venous transposition: A one-stage intra-cardiac operation for the conversion of complete transposition of the aorta and pulmonary artery to corrected transposition. Theory and clinical experience. Surgery 42: 898, Mohalkin, E. N. Anastomosis of the upper vena cava to the pulmonary artery in patients with congenital heart disease with blood flow insufficiency in the lesser circulation. Eksp. Khir. Anest. 1:3, Muller, W. H., Jr., and Dammann, J. F., Jr. The treatment of certain congenital malformations of the heart by creation of pulmonary stenosis to reduce pulmonary hypertension and excessive pulmonary blood flow. Surg. Gynec. Obstet. 95:213, Mustard, W. T., Chute, A. L., Keith, J. D., Sirek, A., Rowe, R. D., and Vlad, P. A surgical approach to transposition of the great vessels with extracorporeal circuit, Surgery 36:39, Mustard, W. T., Keith, J. D., Trusler, G. A., Fowler, R., and Kidd, L. The surgical management of transposition of the great vessels. J. Thorac. Cardiovasc. Surg. 48:953, VOL. 7, NO. I, JAN.,

6 ROBICSEK ET AL. 28. Rashkind, W. J., and Miller, W. W. Creation of an atrial septal defect without thoracotomy: A palliative approach to complete transposition of the great arteries. J.A.M.A. 196:991, Robicsek, F., Temesvary, A., and Kadar, R. L. A new method for the treatment of congenital heart disease associated with impaired pulmonary circulation. Acta Med. Scand. 154: 151, Robicsek, F., Sanger, P. W., and Taylor, F. H. Three-year follow-up of a patient with transposition of the great vessels, atrial septal defect and pulmonary stenosis treated by vena cava-pulmonary artery anastomosis. J. Thorac. Cardiovasc. Surg. 44:817, Robicsek, F., Sanger, P. W., Taylor, F. H., Najib, A., and Tavana, M. Complete bypass of the right heart. Amer. Heart J. 66:792, Robicsek, F., Sanger, P. W., Taylor, F. H., and Najib, A. The azygos steal syndrome in cava-pulmonary anastomosis. Ann. Surg. 158: 1007, Robicsek, F., Sanger, P. W., Taylor, F. H., and Daugherty, H. K. Peripheral stricture of the pulmonary artery treated by cava-pulmonary anastomosis. Ann. Surg. 160:1066, Robicsek, F., Sanger, P. W., Gallucci, V., and Bagby, E. Long-term complete circulatory exclusion of the right side of the heart. Hemodynamic observations. Amer. J. Cardiol. 18:867, Robicsek, F., Sanger, P. W., Gallucci, V., and Daugherty, H. K. Long-term circulatory exclusion of the right heart. Surgery 59:431, Sanger, P. W., Robicsek, F., and Taylor, F. H. Vena cava-pulmonary artery anastomosis: 111. Successful operation in case of complete transposition of great vessels with interatrial septal defect and pulmonary stenosis. J. Thorac. Cardiovasc. Surg. 38: 166, Sanger, P. W., Robicsek, F., Taylor, F. H., Najib, A., Tavana, M., and Gallucci, V. Is the right heart an absolutely necessary organ? Bull. SOC. Int. Chir. 5:645, Sanger, P. W., Robicsek, F., Robicsek, L. K., and Gallucci, V. The clinical application of cava-pulmonary anastomosis. Dis. Chest 48: 145, Sanger, P. W., Robicsek, F., Taylor, F. H., and Gallucci, V. Observations on partial and complete circulatory exclusion of the right heart. J. Cardiovasc. Surg. 6:30, Schuster, S. R., Kiernan, E., Rosenkranz, J., and Bozier, A. A new technique for the creation of an interatrial septal defect with clinical application. J. Thorac. Cardiovasc. Surg. 46:510, Senning, A. Surgical correction of transposition of the great vessels. Surgery 45:966, Shumacker, H. B., Jr. A new operation for transposition of great vessels. Surgery 50:773, Willman, V. L., Cooper, T., Mudd, J. G., and Hanlon, C. R. Treatment of ventricular septal defect by constriction of pulmonary artery. Arch. Surg. (Chicago) 85:745, ?HE ANNALS OF THORACIC SURGERY

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