Complete Repair of Taussig-Bing Abnormality

Transcription

1 Complete Repair of Taussig-Bing Abnormality Norman B. Thomson, Jr., M.D.* T he congenital cardiac abnormality described by Taussig and Bing in 1949 is a variant of transposition of the great vessels [61. In this anomaly, the aorta arises from the right ventricle; the pulmonary artery arises anteriorly and straddles a large ventricular septal defect. Thus, the pulmonary artery arises from both ventricles but is anterior to the aorta. Lev [3] lists four types of Taussig-Bing abnormalities; he notes that the pulmonary trunk may arise completely or mostly from the right ventricle, equally from both ventricles, or mostly from the left ventricle. In the abnormality described by Taussig and Bing, a muscle ridge extends from the anterior aspect of the large ventricular defect to the anterior ventricular wall between the aortic and pulmonary valve annuli. I wish to describe a complete repair of such an abnormality in a 16-year-old child. This child was first seen at the Buffalo Children s Hospital at 3% years of age for evaluation of cyanosis. Cyanosis was present at birth and had persisted and increased in its intensity until the patient s first admission. A systolic murmur had been noted over the left sternal border by the family s physician when the child was 4 months old; a chest x-ray was obtained at that time (Fig. 1). The child s weight and growth had been normal for the first year of life; after that, however, growth and development were below normal. On admission the patient weighed 11.5 kg. (25 Ibs.) and was 89.5 cm. (35 in.) tall. Respiratory rate was 24, pulse rate 132 per minute, and blood pressure mm. Hg. The patient had cyanosis of the lips with cyanosis and clubbing of the fingers and toes. There was a systolic thrill over the precordium and a grade 4 of 6, high-pitched systolic ejection murmur loudest at the left second and third intercostal spaces. The murmur radiated to the left of the sternum toward the apex of the heart and the left axilla. The murmur was not transmitted into the neck. There was no diastolic murmur present. Liver and spleen were not palpable and there was no peripheral edema. An ECG was interpreted as showing combined ventricular hypertrophy (Fig. 2). Cardiac catheterization carried out in 1952 revealed a right ventricular and pulmonary artery pressure equal to the aortic pressure. A right-to-left shunt was present, but its site was undetermined. The diagnosis was ventricular septal defect with Eisenmenger s complex. From the Department of Cardiovascular Surgery, Children s Hospital, Buffalo, N.Y. and the Department of Surgery, State University of New York at Buffalo, School of Medicine, Buffalo, N.Y. Accepted for publication May 15, *Present address: Cardiovascular Center, 600 Gresham Drive, Norfolk, Va THE ANNALS OF THORACIC SURGERY

2 Repair of Tazissig-Bing A bnorrnality FIG. I. X-ray of patient taken at 4 rnonths of age when nzupmiir fil-st identified. She was admitted to the hospital at the age of 6 with a purulent meningitis, from which she recovered without sequelae after treatment with appropriate antibiotics. One year later she was admitted again for cardiac catheterization and further evaluation. Cyanosis was still present, and the patient now complained of severe exertional dyspnea. A grade 4 of 6 systolic ejection murmur was heard over the left sternal border. This murmur radiated throughout the precordium. The pulmonic second sound was markedly exaggerated and was followed by a grade 2 diastolic murmur. Cardiac catheterization was performed; the results again were not entirely satisfactory. The final diagnosis was a large ventricular defect with elevated pulmonary vascular resistance, an Eisennienger s type of defect. Slie was then followed in the Chrtliac Clinic at Children s Hospital with the diagnosis of an unusual type of transposition. In 1959, when tlie patient was 11 years old, the systolic murmur changed its character. Tlie duration antl intensity decreased from a grade 4 to a grade 3 of 6 systolic murmur. The piilmonic diastolic murmur which had appeared two years earlier had increased in intensity, antl the patient noted further increase in her exertional dyspnea. Slie was readmitted for evaluation in January, At this time there was a palpable systolic thrill along the left sternal border, and a grade 3 systolic murmur was heard in the pulmonic area which radiated throughout the precordium; the pulmonic second sound was markedly exaggerated and was followed by a grade 2 to 3 mitldiastolic murmur along the left sternal border. Tlie data obtained at this cardiac catheterization and tlie angiocardiographic studies yielded tlie diagnosis of a Taussig-Bing abnormality (Table 1). It was felt that although pulmonary vascular resistance had considerably increased, the pulmonary flow was significantly greater than systemic flow. Consequently, the patient was advised to have an operation. Slie was admitted to the hospital in May, 1965, for definitive surgery (Figs. 2, 3). The anticipated plan of surgery was that the Taussig-Bing abnormality could be converted to a classic complete transposition by appropriate sutures within the right ventricle. A Mustard procedure could then be carried out at the atrial VOL. 4, NO. 5, NOV.,

3 FIG. 2. Patient s ECG s. 3 years of age: Combined right hypertrophy. Normal axis (paper speed 25 mm. per src.}. 16 years: Partial right bundle branch block with right ventricle hypertrophy and strain; less left vmtricle forces (paper speed 50 mm. per sec.). 17 years: Postoperative. A complete right bundle brunch block prevents evaluation of right veiltrick hypertrophy (paper speed 50 mm. per sec.).

4 Repaar of Taussig-Bing A bnormality FIG. 3. Chest x-ray before complete repair shows the relatively small heart with the tremendously enlarged main pulmonary artery and the decreased vascularity of the peripheral lung fields accompanying the severe pulmonary vascular disease. level [l, However, after opening the right ventricle it was apparent that a direct correction from within the right ventricle would be possible. OPERATIVE FINDINGS AND COURSE At operation, pressures were measured in all chambers of the heart (Table 1). The pulmonary artery was four times the diameter of the aorta and arose anterior to the aorta from the right ventricle at about the normal position. The aorta, however, arose further to the right than is normal and clearly from the right ventricle. There was a large single right coronary artery descending in the right atrial-ventricular groove. The first branch of this right coronary artery was large and crossed the pulmonary artery just below the pulmonary annulus. This vessel then became the anterior descending coronary artery; the left coronary artery did not supply the anterior descending branch. The left coronary arose from the aorta in a normal position and passed beneath the pulmonary artery to the left ventricle as a circumflex branch. There were no abnormalities of systemic or pulmonary venous return, and the ductus was obliterated. After establishing extracorporeal perfusion, the right ventricle was opened obliquely between the two major branches of the right coronary artery. A huge ventricular septa1 defect was present, measuring 6.5 cm. in diameter. The tricuspid valve appeared to be normal and was supported by papillary muscles with normal distribution and source of origin. All the tricuspid valve substance was inferior and posterior to the ventricular defect. The aorta arose from the right ventricle. The aortic valve annulus was 10 cm. to the right mitral valve. The pulmonary artery arose partially from the right ventricle, but mostly from the left ventricle. There was marked hypertrophy of the right ventricle, most extensive in the outflow tract. Arising from the anterior portion of the ventricular

5 ~~~ THOMSON TABLE 1. DATA OBTAINED AT CARDIAC CATHETERIZATION AND AT TIME OF OPERATION *4,* Cardiac Catheterization Corr&tive Operation Oxygen Pressures Pressures Pressures Saturation Before After (mm. Hg) (%) Perfusion Correction Sample Site (mm. Hg) (mm. Hg) - - Superior vena cava Right atrium Right ventricle apex Right ventricle outflow Pulmonary 88/ /40 58/39 artery Pulmonary vein Left atrium Left ventricle Aorta 81/ FZG. 4. Artist s drawing of the anatomy as drawn from the specimen. R.I.D., right inferior portion of the defect; L.S.D., left superior portion of the defect which is divided by the muscle pillar arising from the remnant of the ventricular septum and inserting into the tissues between the aorta and pulmonary valves. 424 THE ANNALS OF THORACIC SURGERY

6 Repair of Taussig-Bing Abnormality FIG. 5. (1) Site of transverse ventriculotomy incision. (2) Position of the insertion of the patch to isolate the aortic outflow area from the rest of the right ventricle. (3) Position of the superior patch beneath the pulmonary valve annulus. septum was a ridge of muscle, 1.5 cm. in diameter, which was inserted between the aortic and pulmonary valves just anterior to their junction. The valves of these two vessels appeared to lie in the same transverse plane (Fig. 4). The intracardiac repair was achieved utilizing two patches of Teflon felt material (Fig. 5). One patch was sutured (with interrupted sutures supported by pledgets of Teflon felt material) to the undersurface of the muscle ridge and down into the left ventricular chamber 3 cni. below the pulmonary valve and cephalad to the anterior leaflet of the mitral valve (which was easily identified through the large ventricular defect). The patch isolated the left ventricular outflow from the pulmonary valve area. A second patch was then sutured to the undersurface of the muscle ridge and around the periphery of the ventricular defect and over the annulus of the aortic valve. Blood from the left ventricle then exited into the aorta inferior to the first patch and posterior to the second patch (Fig. 6). Abundant hypertrophy of the outflow area of the right ventricle was then resected. Because the outflow tract of the right ventricle might be somewhat obstructed by virtue of the tunnel that had been created, a short longitudinal incision was then made into the right ventricle below the anomalous right coronary artery; a third Teflon patch was placed in the outflow area of the right ventricle. The right ventricular blood now exited over the two patches placed within the right ventricle and beneath the outflow tract patch in the right ventricular wall. A 2 by 3 cm. ostium secundum defect was identified and closed

7 THOMSON FIG. 6. Composite drawing: (A) Inferior patch. (B) Superior patch. (C) Area Of markedly hypertrophied right ventricular outflow tract that was resected. (D) Position of the muscle bundle. through a separate incision in the right atrium. The right ventricular transverse incision was then closed. The total period of extracorporeal perfusion was 3 hours and 27 minutes. Thirteen 10-minute periods of aortic cross-clamping were utilized with periods of coronary perfusion between each cross-clamping. After discontinuing the perfusion and allowing the patient to obtain normal temperature and what appeared to be a normal blood volume, the pressures were measured; they are given in Table 1. We were disappointed at the failure of a significant fall in the pulmonary artery pressure, but encouraged by the lack of evidence of obstruction to the outflow of either ventricle. A lung biopsy was taken from the right middle lobe; photomicrographs of the biopsy are shown in Figure 7. The patient was returned to the Intensive Care Unit with the endotracheal tube in place and was ventilated with an Engstrom respirator. At the end of 4 hours the patient had good respiratory exchange; arterial blood gas studies indicated normal ph, pc02, and poz when the patient was off the respirator and breathing 40% oxygen on her own. The endotracheal tube was removed. She recovered gradually from the operative procedure; however, a slight peripheral cyanosis remained. On the 14th postoperative day she developed evidence of increasing cardiac failure with accumulation of right pleural fluid. She responded rapidly to an increase in Digoxin, the utilization of diuretics, and a thoracentesis of 800 cc. of amber fluid from her right chest. Her arterial oxygen saturation was 87% when she was breathing room air; it rose to 94% when she was breathing 40% oxygen. She was discharged from the hospital on the 32nd postoperative day; at this time her lungs were clear and she had a grade 2 systolic murmur at the pulmonic area followed by a soft pulmonic diastolic murmur of grade 1 to 2 intensity. The pulmonic second sound, however, remained markedly increased in intensity. She was followed at monthly intervals. Her last follow-up was in November, 1965, 7 months postoperatively. At that time it was noted that she had marked increase in her exercise tolerance. She could climb three flights of stairs without dyspnea but with some slight increase in cyanosis. She was attending high school full-time and had not had any absences from school. She had gained ten pounds 426 THE ANNALS OF THORACIC SURGERY

8 Repair of Taussig-Bing Abnormality FIG. 7. Lung biopsy. (~160.) Severe pulmonary arteriolar hypertrophy. Tissue removed at autopsy showed the same degree of far advanced arteriolar disease. since the operation, and it seemed that her cyanosis and clubbing had subsided since operation. She had a grade 1 ejection systolic murmur at the left sternal border and a grade 2 diastolic murmur lower along the left sternal border; the pulmonic sound was still increased in intensity. It was believed, however, that the pulmonic second sound was not as loud as it had been at discharge from the hospital. Her liver and spleen were not palpable, and she had no peripheral edema. The blood pressure was mm. Hg, and her pulse was 97. Chest x-ray was similar to that observed immediately before operation. The ECG showed a complete right bundle branch block (Fig. 2). She was exercised with forty steps which increased her heart rate from 90 to 120. The heart rate promptly fell to 100 within one minute. It was felt she had shown definite improvement and that a cardiac catheterization was warranted in 6 months to determine the hemodynamics. On January 27, 1966, she and her family were exposed to carbon monoxide gas as a result of a defective flue in their home. All other members of the family recovered. The patient was admitted to another hospital at 7:30 a.m. She was not noted to be in any distress. Examination at that time suggested that there was no essential change in the findings compared to the examination performed at Buffalo Children s Hospital in November. The patient did have an injected throat and conjunctivitis; she was placed on tetracycline and was continued on her maintenance dose of Digoxin. At 11:30 that night she complained of feeling warm and was soon found to be without respirations and heart action. Adrenalin was given intracardially. In spite of all efforts, she died. Autopsy was performed at Children s Hospital 11 hours after death; no obvious cause for death was identified. Specifically, there were no pulmonary emboli, no evidence of heart failure, no evidence of myocardial infarction, and the repair, as noted in the drawings of Figures 4, 5, and 6, was intact. The right ventricular hypertrophy which had been present at the time of operation had

9 THOMSON decreased to the extent that the thickness of the right ventricular wall was 70% of that of the left ventricular wall. DISCUSSION The abnormality in this patient is identical to that first described by Taussig and Bing. The diagnosis may be suspected from the ECG, chest x-ray, and clinical picture. Cardiac catheterization will further support the diagnosis by the identification of the oxygen saturations at the appropriate levels. The diagnosis, however, can only be established with certainty by angiocardiographic studies. The physiological findings determined at cardiac catheterization will not distinguish the Taussig-Bing abnormality from transposition of the great vessels [Z]. The marked hypertrophy of the outflow tract of the right ventricle in our patient allowed a resection of this area; by combining this with a small right ventricular outflow patch, an adequate outflow area was achieved. Pressures measured in the operating room at the conclusion of the operative procedure disclosed no gradient between the left ventricle and aorta or the right ventricle and distal pulmonary artery. The persistence of the pulmonary hypertension, however, was interpreted as an ominous finding; indeed, her postoperative course was marked by respiratory distress and cyanosis which disappeared with the breathing of oxygen. Whether the cyanosis was due to difficulties in diffusion or the opening of pulmonary-arterial venous shunts, is not answered by any of the studies performed. The intent of the operation originally was to convert the defect into a complete transposition of the great vessels with an intact septum by closing the ventricular defect; a Mustard procedure at the atrial level was then to be done. However, upon opening the right ventricle, the possibility of doing a complete direct repair within the right ventricle was presented; this seemed technically easier than converting the patient to a complete transposition. What the long-term catheterization studies might have demonstrated in this patient are unknown, unfortunately. However, lung microscopic sections taken at the time of autopsy showed no essential change in the severity of the degree of arterial disease demonstrated by the biopsy taken at operation. It would seem possible that should a Taussig-Bing abnormality be present without the muscular ridge arising from the anterior area of the ventricular defect and extending to the tissues between aortic and pulmonary artery, a repair could be achieved within the ventricle by fashioning a U-shaped tunnel somewhat similar to the technique utilized in correcting a double-outlet right ventricle. The difference would be that in the Taussig-Bing abnormality the lateral and posterior portions of the superior edge of this patch must be sutured into the left 428 THE ANNALS OF THORACIC SURGERY

10 Repair of Taussig-Bing Abnormality ventricular musculature, thus isolating the left ventricular outflow tract from the pulmonary artery and directing the blood across the right ventricle and into the aorta. For such a tunnel to function effectively, this operation should perhaps be limited to the older patients; or, conceivably, the tunnel could be created out of pericardium which might grow if the repair were carried out in a smaller patient. If the direct repair is not warranted due to the anatomical variations encountered, the ventricular septa1 defect can be closed to convert the heart to a classic complete transposition of the great vessels with an intact septum; a Mustard procedure could then be carried out at the atrial level. In reconstructing the sequence of events leading to the death of this patient, several facts are missing. No blood carbon monoxide levels were obtained at the time of admission; no other laboratory data is available; and there were no ECG s or chest x-rays. Thus questions of carbon monoxide exposure, the evidence of heart failure, or the development of arrhythmias with the presence of a significant degree of infection, are not settled. The blood carbon monoxide level at time of autopsy, 11 hours after death, was 4%. One wonders whether the degree of peripheral oxygen desaturation plus carbon monoxide intoxication, might possibly have led to the development of an arrhythmia so common in patients with pulmonary hypertension. We believe that the patient reported here is the first patient in whom a complete repair of a Taussig-Bing abnormality has been attempted. Undoubtedly additional patients will be operated upon for this abnormality now that a complete correction of transposition of the great vessels is possible. Some patients will be encountered in whom the diagnosis before operation will be transposition of the great vessels, and the true anatomy revealed only at operation. If the surgeon is aware of the possibilities of repair of the Taussig-Bing abnormality, then the original operative plan will not be abandoned, only altered. The findings at autopsy, as depicted in the artist s drawing of the actual specimen, would indicate that the repair is technically possible and certainly should be carried out before severe vascular disease develops. SUMMARY The clinical history, cardiac catheterization data, and ECG s of a patient with a classic type of Taussig-Bing abnormality is presented. The technique of direct repair carried out within the ventricle is described and variations in this technique are discussed. The operative repair of this abnormality should be carried out before severe vascular changes have developed in the pulmonary arterioles. VOI.. 4, NO. 5, NOV.,

11 THOMSON REFERENCES 1. Aberdeen, E., and Waterston, D. J. Successful correction of transposed great arteries by Mustard s operation. Lancet 1: 1233, Beuren, A. Differential diagnosis of the Taussig-Bing heart from complete transposition of the great vessels with a posteriorly overriding pulmonary artery. CircuZation 21: 1170, Lev, M., Rimoldi, H. J. A., Eckner, F. A. O., Milhushi, B. P., Meng, L., and Paul, M. H. The Taussig-Bing heart. Arch. Path. (Chicago) 81:24, Mustard, W. T. Successful two-stage correction of transposition of the great vessels. Surgery 55:469, Mustard, W. T., Keith, J. D., Trussler, G. A., Fowler, R., and Kidd, L. The surgical management of transposition of the great vessels. J. Thorac. Cardiov. Surg. 48:953, Taussig, H. B., and Bing, R. J. Complete transposition of the aorta and levoposition of the pulmonary artery. Amer. Heart J. 37:551, THE ANNALS OF THORACIC SIJKGERY