CASE REPORTS. Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

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1 CASE REPORTS Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Definitive Surgical Treatment by Saphenous Vein Interposition in a 17-Month-Old Child P. Venugopal, M.D., and S. Subramanian, F.R.C.S. ABSTRACT A 17-month-old child underwent successful operative correction of anomalous origin of the left coronary artery from the pulmonary artery by saphenous vein interposition. The clinical, hemodynamic, and angiographic features of the anomaly are presented. Three months after the operation, cardiac catheterization and angiography revealed a patent graft with good antegrade flow and improvement in left ventricular function. ince origin of the left coronary artery from the pulmonary artery was described by Abrikossoff [l] in 191 1, much interest has centered about the diagnosis and management of this malformation. According to Keith, Rowe, and Vlad [5], the majority of infants with origin of the left coronary artery from the pulmonary artery die before 6 months of age. In their experience, when symptoms occurred during early infancy, no patient survived beyond 2 years of age. Wesselhoeft and colleagues ill], in a collected series of 140 patients reported in 1968, found the so-called infant syndrome in 105 patients. Only 8 of the group survived to childhood, and half of these were incapacitated with mitral regurgitation. While the clinical and hemodynamic features of this lesion are well documented, many different therapeutic procedures have been suggested. There is little disagreement that the ideal definitive therapy for this anomaly is to separate the coronary artery from the pulmonary artery and anastomose it to the aorta. Cooley and associates [3] in 1966 reported successful definitive repairs in 4- From the Division of Cardiovascular Surgery, Children s Hospital, Buffalo, N.Y. Accepted for publication June 11, Address reprint requests to Dr. Subramanian, Division of Cardiovascular Surgery, Children s Hospital, 219 Bryant St., Buffalo, N.Y VOL. 19, NO. 4, APRIL,

2 VENUGOPAL AND SUBRAMANIAN and 5-year-old children; in 2 a composite Dacron and vein graft was used for interposition. They advocated that definitive therapy be delayed until that age for technical reasons. It is increasingly recognized, however, that the longer the child awaits correction, the more serious are the effects of myocardial ischemia, resulting myocardial fibrosis, secondary endocardia1 fibroelastosis, mitral regurgitation and arrhythmia, heart failure, and danger of sudden death. Operative mortality has been high because of ventricular fibrillation soon after ligation or at the time of anastomosis. Availability of circulatory support increases the safety of the operation and possibly avoids fatalities. We report a 17-month-old child with such an anomaly who has successfully undergone saphenous vein graft interposition between the aorta and left coronary artery under deep hypothermia, circulatory arrest, and limited cardiopulmonary bypass. A 17-month-old black girl was admitted to Children s Hospital, Buffalo, for the first time on January 7, 1973, with complaints of tachypnea and cough of two days duration. The child, a product of a full-term, normal gestation, had been noted to be breathing fast and eating poorly. She was found to be rather quiet and easily fatigued. General examination revealed a normally developed child with a respiration rate of 60 per minute and a regular heart rate of 150 per minute. The liver was palpable 2 cm below the right costal margin. There was no murmur. A prominent third heart sound was heard at the apex. Auscultation revealed bilateral basal crepitations. A roentgenogram of the chest showed cardiomegaly with increased pulmonary vascular markings. The electrocardiogram (Fig. 1) showed a QRS axis of +30 degrees, evidence of left atrial and left ventricular hypertrophy, FIG. I. Preoperative ECG shows left atrial and lgt ventricular hypertrophy with strain pattern. 452 THE ANNALS OF THORACIC SURGERY

3 ~I CASE u pow: Correction of Anomalous LCA aiid ;I strain pattern with flat T waves in VS and VS. Initially she was thought to be in cardiac failure due to endocardial fibroelastosis and was treated with digitalis. She did not improve clinically, and cardiac catheterization and angiocardiography were done on January 11. The data revealed no shunt (Table 1) and normal right ventricular and pulmonary artery pressures. The left ventricular end-diastolic pressure was slightly elevated. Angiocardiography revealed a large right coronary artery and late visualization of collaterals over the apex of the left ventricle with retrograde filling of the left coronary artery and then the main pulmonary artery. There was slight mitral regurgitation. The left ventricle was large and contracted poorly. Ventricular volume measurements revealed a very poor ejection fraction (26%) and a markedly increased left ventricular mass (Table 2). On January 19 she was operated upon, using the technique of surfaceinduced deep hypothermia, circulatory arrest, and limited cardiopulmonary bypass. Anomalous origin of the left coronary artery from the pulmonary artery was confirmed and was seen to be on the posterolateral aspect of the pulmonary artery. The left ventricle was found to be fibrotic and felt gritty, especially at the apex, indicating secondary fibrotic changes. The left coronary artery was 1 mm in size. An oval segment was taken from the wall of the pulmonary artery, including the ostium of the left coronary artery, to obtain a wide anastomosis. A reversed segment of freshly obtained saphenous vein from the patient s brother, whose histocompatibility with the patient is outlined in Table 3, was used as a vascular conduit from the ascending aorta to the coronary artery. The anastomosis was done with 6-0 Prolene suture for the coronary arterial end and 5-0 for the aorta. The total period of circulatory arrest was 26 minutes. The rest of the procedure was uneventful. During the postoperative period she continued to have a gallop and sinus tachycardia and was maintained on digoxin. She also had a grade 216 ejection systolic murmur, possibly due to the disparity in size of the vein and left coronary artery. She was discharged on the seventeenth postoperative day. Since that time she has been well, although the heart size is still large with an intermittent fourth heart TABLE 1. CARDIAC CATHETERIZATION DATA Preop. Postop. Oxygen Oxygen Pressure Sat. Pressure Sat. Site (mm Hg) (%) (mm Hg) (%) svc (3) IVC (5) RA (4) 59 (4) RV /5 73 PA 25/15 (18) 61 27/11 (16) 71 PV (8-10) 100 (7) 100 LV 85/ Aorta 88/60 (72) (70) 99 SVC = superior vena cava; IVC = inferior vena cava; RA = right atrium; RV = right ventricle; PA = pulmonary artery; PV = pulmonary vein; LV = left ventricle..ti VOL. 19, NO. 4, APRIL,

4 VENUGOPAL AND SUBRAMANIAN TABLE 2. LEFT VENTRICULAR MEASUREMENTS AT ANGIOGRAPHY Measurement Preoperative Value Postoperative Value End-diastolic volume (ml) 75.5 (index, 169.0) 56 (index, 114) End-systolic volume (ml) 56.0 (index, 125.0) 29 (index, 58.5) Stroke volume (ml) 20.0 (index, 45.0) 27 (index, 55.5) Ejection fraction (%) 26 (heart rate, 118) 48 (heart rate, 140) Cardiac output (L/min) 2.35 (index, 5.26) 3.8 (index, 7.7) Left ventricular wall thickness (mm) 6.7 Left ventricular mass (gm) 78 (index, 175) sound. There is no evidence of heart failure. The ECG shows persistence of left ventricular hypertrophy with T-wave changes. She was restudied three months postoperatively. The left ventricular enddiastolic pressure was still slightly elevated. The graft was patent (Fig. 2) and demonstrated antegrade flow in the coronary artery. Left ventricular function had improved, the ejection fraction being 48% (see Table 2). Comment Since the prognosis for origin of the left coronary artery from the pulmonary artery is generally poor once symptoms have appeared, much interest has been shown in an operative approach designed to alleviate or correct this condition. While there is a consensus that medical treatment is indicated for congestive heart failure [ 101, there is still disagreement concerning the optimal time for operation and the type of procedure that should be performed. Operative treatment has been attempted with a variety of techniques. Among these are: (1) using a systemic-to-pulmonary artery anastomosis in an effort to increase both oxygenation and pressure in the pulmonary artery; (2) pulmonary artery constriction; and (3) creation of an increased blood supply to the left ventricle from the pericardium and other structures by means of irritants. Each of these procedures has had disappointing results. Ligation of the anomalous left coronary artery to prevent coronary steal and to raise the perfusion pressure of the left ventricular myocardium has been done extensively, especially in children below the age of 2 years. Results of ligation have generally been poor in infants. Likar, Criley, and Lewis [6] reported a collected series of 27 patients who underwent ligation of the anomalous vessel; 16 survived, 13 of whom were improved. All 11 deaths occurred in infants 2 to 16 months of age. The survivors included 9 infants 1 to 22 months of age, 6 children 2 to 12 years of age, and 1 patient 16 years of age. One could TABLE 3. RESULTS OF LYMPHOCYTOTOXICITY TESTS FOR HISTOCOMPATIBILITY BETWEEN PATIENT AND HER FAMILY MEMBERS Member First Locus Second Locus Mother 2, 10 7, 17 Father 9,? 5, 12 Donor (brother) 9, 10 5, 17 Patient?, 10 12, THE ANNALS OF THORACIC SURGERY

5 CASE REPORT: Correction of Anomalous LCA FIG. 2. Selective injection into vein shows patent graft with antegradeflow into the left coronary artery (LCA) system. (A0 = aorta; PA = pulmonary artery.) assume that most of the survivors in the infant group would have lived without the operation. Transplantation of the left coronary artery provides the most direct approach, since a two-coronary-artery system is produced and normal circulation in the coronary arteries results. Mustard [8] described an operation in which the anomalous left coronary artery is detached from the pulmonary artery in such a fashion that a portion of the pulmonary artery is removed at the coronary ostium to enhance performance of the anastomosis with the left common carotid artery. Although the anastomosis was accomplished, both infants died immediately of ventricular fibrillation. Apley and associates [2] planned a similar operation in a 12-month-old girl using the left subclavian artery, but anastomosis could not be done for technical reasons. The coronary artery was ligated, and the infant died soon afterward. Meyer and co-workers "71 in 1968 reported the successful utilization of the above technique in a 3-month-old infant, and Pinsky and co-workers [9] in 1973 reported 2 such patients. Cooley and associates [3] in 1966 reported successful definitive repair using Dacron graft and autogenous saphenous vein graft in 2 patients aged 4 and 5 years; they advocated that definitive repair should be delayed until this age with medical management up to that time. The long-term fate of these grafts is still uncertain although the aortocoronary saphenous vein bypass operation is technically successful in children and has beneficial effects; however, obliterative changes and dilatation may occur in the saphenous vein grafts in patients who have no systemic or distal coronary artery disease. In a recent report by El-Said and colleagues [4] in which they followed up 10 patients operated on since 1965, a repeat study of 6 patients found an occluded graft in 1, stenosis at the site of anastomosis in 2, and saccular dilatation of the distal graft in 1 patient. The authors stated that these patients represent their early experience with the saphenous vein graft technique, however, and they anticipate better coronary flow and longer graft patency in their present patients with current techniques of graft preparation and anastomosis. VOL. 19, NO. 4, APRIL,

6 VENUGOPAL AND SUBRAMANIAN There is no disagreement on the fact that early correction of this anomaly largely prevents all the secondary effects of myocardial ischemia which are inevitable with delayed definitive treatment. Many of the earlier attempts at reconstruction of the coronary artery have been unsuccessful partly because circulatory support, which not only would have protected against fatal arrhythmias but also would have made the operations technically simpler, was not used. The technique of surface-induced deep hypothermia and circulatory arrest with limited cardiopulmonary bypass allowed us to perform this procedure with a satisfactory margin of safety. This patient showed improvement in left ventricular function after the corrective operation. References 1. Abrikossoff, A. Aneurysma des linken Herzventrikels mit abnormer Abgangsstelle der linken Koronararterie von der Pulmonalis bei einem fuenfmonatlichen Kinde. Vjrchows Arch Pathol Anat 203:413, Apley, J., Horton, R. E., and Wilson, M. G. The possible role of surgery in the treatment of anomalous left coronary artery. Thorax 12: 18, Cooley, D. A., Hallman, G. L., and Bloodwell, R. D. Definitive surgical treatment of anomalous origin of the left coronary artery from the pulmonary artery: Indications and results. J Thorac Cardiovmc Surg 52:798, El-Said, G. M., Ruzyllo, W., Williams, R. L., Mullins, C. E., Hallman, G. L., Cooley, D. A., and McNamara, D. G. Early and late results of saphenous vein grafts for anomalous origin of left coronary artery from pulmonary artery. Circulation 47, 48 (Suppl 111):2, Keith, J. D., Rowe, R. D., and Vlad, P. Heart Disease in Infancy and Childhood. New York: Macmillan, P Likar, I., Criley, J. M., and Lewis, K. B. Anomalous left coronary artery arising from the pulmonary artery in adults: A review of therapeutic problems. Circulation 33:727, Meyer, B. W., Stefanik, G., Stiles, Q. R., Lindesmith, G. G., and Jones, J. C. A method of definitive surgical treatment of anomalous origin of the left coronary artery. J Thorac Cardiovmc Surg 56: 104, Mustard, W. T. Anomalies of the Coronary Artery in Pediatric Surgery. Chicago: Year Book, Vol 1, p Pinsky, W. W., Fagan, L. R., Kraeger, R. R., Mudd, J. F. C., and Willman, V. L. Anomalous left coronary artery: A report of two cases. J Thorac Cardiovmc Surg 65:810, Wagner, H. R., Nadas, A. S., and Hugenholtz, P. G. Anomalous left coronary artery originating from the pulmonary artery: Detailed follow-up report of successful ligation of the aberrant vessel in two infants. Pediatrics 40:320, Wesselhoeft, H., Fawcett, J. S., and Johnson, A. L. Anomalous origin of the left coronary artery from the pulmonary trunk: Its clinical spectrum, pathology, and pathophysiology -based on a review of 140 cases with seven further cases. Circulation 38:403, THE ANNALS OF THORACIC SURGERY