Successful Operative Repair of Acyanotic Tetralogy of Fallot

Transcription

1 Successful Operative Repair of Acyanotic Tetralogy of Fallot in a 61-Year-Old Man Ronald J. Sisel, M.D., Clarence S. Weldon, M.D., and G. Charles Oliver, M.D. ABSTRACT A case of acyanotic tetralogy of Fallot-classic tetralogy anatomically with a predominant left-to-right shunt-that was repaired operatively in a 61-year-old man is reported. This represents, to our knowledge, the oldest reported patient with a tetralogy malformation to receive successful corrective surgery. The patient s relatively mild pulmonic obstruction was probably the most significant factor in his long survival. Paradoxical embolization and progressive severe congestive heart failure were the main indications for operation. A 6 I-year-old man underwent successful surgical repair of a tetralogy of Fallot malformation. Surgical correction of tetralogy in adults of the ages of 53 [41 and 54 [9], in whom good results were achieved, have been previously reported. As far as we have been able to determine, our patient represents the oldest patient to receive successful corrective surgery for this condition. A 61-year-old man was admitted to Barnes Hospital on February 11, He had been studied eight months previously by cardiac catheterization, and a diagnosis of tetralogy of Fallot, acyanotic type, had been made. During the eight-month interval following catheterization, he complained of progressively increasing shortness of breath on exertion, orthopnea, and swelling of his lower extremities. A right homonymous hemianopia suddenly appeared in December, 1968, and was interpreted as due to paradoxical embolization. The patient was transferred to Barnes Hospital from a local hospital after he had had two cyanotic episodes associated with respiratory arrest and runs of ventricular extrasystoles. Review of the past history revealed the presence of a cardiac murmur at birth. At 5 years he was noted to have blue lips when climbing stairs. Cyanosis was not noted again until December, 1968, when it appeared transiently. During his life he had had two sudden episodes of neurological deficit including the aforementioned right homonymous hemianopia. The first episode occurred in 1951 and was described as a transient paresis of his right side, with a subsequent weakness of his right hand that persisted for three months. Throughout his life he had complained of dyspnea with exertion, and there had been marked progression of this symptom in later years. From the Departments of Medicine and Cardiovascular Surgery, Barnes Hospital, Washington University, St. Louis, Mo. Supported in part by U.S. Public Health Service Grant HE from the National Heart Institute, Washington, D.C. Accepted for publication July 13, Address reprint requests to Dr. Oliver, Cardiovascular Division, Department of Medicine, Washington University School of Medicine, 4550 Scott Ave., St. Louis, Mo THE ANNALS OF THORACIC SURGERY

2 CASE REPORT: Repair of Tetralogy at 61 Years I II Ill avr avl avf v2 v3 va v6 FIG. 1. Electrocardiogram demonstrating right-axis deviation, right ventricular enlargement, and questionable old anteroseptal myocardial infarction. On admission the patient was breathing comfortably in the supine position and was receiving a slow intravenous drip of lidocaine in solution. The vital signs were within normal limits, and the lungs were clear. The jugular venous pulse showed large, flicking A waves. A systolic thrill was palpable over the base of the heart. A grade 5/6 systolic ejection murmur was heard loudest at the base but radiated throughout the precordium. The murmur was not changed by respiration and was preceded by a questionable ejection click in the pulmonk area. The second heart sound at the base was single. The right ventricular impulse was slightly overactive, and the left ventricular impulse was not palpable. The liver was felt 3 cm. inferior to the right costal margin. There was neither cyanosis nor clubbing. On neurological examination a right homonymous hemianopia was noted. The hemoglobin was 16.4 gm. per 100 ml. The electrocardiogram (Fig. 1) showed right-axis deviation, right ventricular enlargement, questionable old anteroseptal myocardial infarction, and occasional multifocal premature ventricular contractions. The chest roentgenogram showed right ventricular enlargement and a large, dilated left main pulmonary artery. The cardiac catheterization findings are summarized in the Table. The intracardiac anatomy as determined angiographically was essentially that of a tetralogy of Fallot with a large anterior ventricular septa1 defect (VSD), infundibular pulmonic stenosis, and probable valvular pulmonic stenosis (Fig. 2). The aorta was slightly dextraposed. Oximetry revealed a bidirectional shunt across the VSD with the predominant shunting from left to right (a pulmonary-to-systemic flow ratio of 2.6:l). The systolic pressure in the right ventricle equaled the systemic systolic pressure. The pulmonary artery pressure was elevated to 50 mm. RESULTS OF CARDIAC CATHETERIZATION (JULY, 1968) O2 Saturation Pressure Site (%) (mm. Hg) Superior vena cava Inferior vena cava Right atrium 52 A:15, 3 Right ventricle Pulmonary artery 78 Aorta , % fi Left ventricle VOI.. 11, NO. 6, JUNE,

3 SISEL, iz ELDOh, AND OI.IVEK FIG. 2. Right ventricular angiogram demonstrating large VSD and filling of the aorta and pulmonary artery. (A0 = aorta; PA = pulmonary artery; LV = left ventricle; RV = right ventricle.) Hg. There was a systolic gradient of 100 mm. Hg across the right ventricular outflow tract. The patient s clinical condition remained stable after admission. Two weeks later an operation was performed. After cardiopulmonary bypass was established, a right ventriculotomy was made. A large mass of infundibular muscle including the septa1 and parietal bands of the crista supraventricularis was excised. The pulmonic valve was moderately stenotic. There were three wellformed pulmonary sinuses with a partial fusion of all three commissures. A commissurotomy to the annulus was performed. A large, anteriorly placed VSD measuring approximately 2.5 cm. in diameter was closed with a patch of Teflon cloth. An infraannular gusset of pericardium was sutured to the right ventriculotomy to allow an adequate diameter for the outflow tract. At the completion of the operation, the pressure in the right ventricle was 40 mm. Hg. The postoperative course was complicated by a transient episode of complete heart block that was treated by demand pacemaker. The patient was discharged on March 12, 1969, with a normal sinus rhythm. Since operation he has had a marked increase in exercise tolerance and has been doing well. COMMENT The mean survival age in tetralogy patients without pulmonary atresia is 12 years [l]. On occasion, survival to adulthood does occur, and a small number of patients have survived to the fifth decade [3]. Most 600 THE ANNALS OF THORACIC SURGERY

4 CASE REPORT: Repair of Tetralogy at 61 Years of the reported long-term survivors have been patients with moderately severe cyanosis, indicating large right-to-left shunts. Those with socalled acyanotic tetralogy with lesser right-to-left shunts have the most favorable outlook, but they also are limited in physical capacity and very infrequently survive beyond 40 years of age [S]. As far as we have been able to determine, the oldest patients reported prior to this who have received a successful corrective operation for tetralogy malformation were a 53-year-old woman reported by Coles and his associates [4] and a 54-year-old man reported by Friesinger and Bahnson [9]. Since long survival with untreated tetralogy is rare, it is of interest to review briefly the factors associated with an unusual longevity. In infancy the presence of a patent ductus arteriosus [17] contributes to the hypoperfused pulmonary circulation. Bopp and co-workers [Z] reported a 34-year-old tetralogy patient with pulmonary atresia (pseudotruncus arteriosus) and a patent ductus arteriosus. However, postmortem examination reports of nearly all tetralogy patients with prolonged longevity show a normally closed ductus. Clearly, other physiological compensatory mechanisms assume importance as the patient matures; these include the development of a bronchial collateral circulation and a polycythemic response to the hypoxemia. Despite the above, the most important factor influencing survival seems to be the degree of hemodynamic abnormality resulting from the anomalous cardiac condition. The anatomical factor most important in this regard is the degree of pulmonic stenosis. Most of the patients with a long survival were reported in the precatheterization era, before a precise measurement of the degree of functional pulmonic stenosis was possible. Both patients [4, 91 successfully corrected in their sixth decade did have a more severe degree of pulmonic obstruction, and consequently greater right-to-left shunting, than our patient as determined by systemic arterial desaturation studies. Savard and colleagues [16] have shown that when a VSD is greater than 1 sq. cm. per square meter of body surface area, the systolic pressure becomes equal in both ventricles and they behave physiologically as a single ventricle. The VSD in a tetralogy malformation assumes these proportions. Therefore, the degree of left-to-right or right-to-left shunting is determined by the relative resistance within the pulmonic and systemic circuits. Since the pulmonary vascular resistance is invariably small in comparison with the systemic vascular resistance, the major resistance to flow in the pulmonic circuit is determined by the degree of pulmonic obstruction, which may be valvular, subvalvular, or supravalvular or a combination of all three. Depending on the magnitude of this resistance, the shunt may be predominantly right-to-left (severe pulmonic obstruction) or left-to-right (mild pulmonic obstruction) or there may be equal flow in both circuits (balanced shunt with VOL. 11, NO. 6, JUNE,

5 SISEL, \VELDON, AND OLI\ ER moderate pulmonic obstruction). In our patient the pulmonic obstruction was relatively mild; this was probably the most significant factor in his long survival. Controversy exists concerning whether or not patients such as these represent examples of true tetralogy of Fallot or whether they should be classified as having a VSD and pulmonic stenosis. Stated somewhat differently, should one adopt a purely anatomical definition, as described by Fallot [7], or should one depend more on a hemodynamic or functional definition and require, as do Harley [lo] and Nadas [14], the presence of a large VSD and pulmonary stenosis of sufficient severity to produce a net right-to-left shunt? If one adopts the latter definition, our patient would not qualify as having tetralogy of Fallot. Other authors do not accept this rigid definition. Cooley and Hallman [5] state that since these patients possess the same anatomical features as patients with the cyanotic variety of the disease and since they differ mainly in the degree of pulmonic stenosis, they should be classified as having tetralogy but should be designated as having an acyanotic or atypical variety [ll, 151. McCord, Van Elk, and Blount [12] regard tetralogy of Fallot as a broad clinical and hemodynamic spectrum with multiple and varied combinations of pulmonic stenosis and VSD resulting in right-to-left, left-to-right, or bidirectional shunting. Since the patient reported here had the classic anatomical features of tetralogy [6] both angiographically and at operation but had a predominant left-to-right shunt, we believe his pathology fits the category of acyanotic or atypical tetralogy. Of interest is the finding of progressive severe congestive heart failure in our patient, since congestive failure is generally not seen as a clinical manifestation in patients with tetralogy [13]. Arteriosclerotic heart disease probably contributed to the failure; however, the operative result suggests that the abnormal hemodynamics were important etiologically. The VSD with its sizeable left-to-right shunt significantly increased the left ventricular work load [ 181, thereby promoting heart failure. Operative closure of the VSD, therefore, significantly reduced the left ventricular work load. Resection of the pulmonary stenosis and the pulmonary valvuloplasty further reduced right ventricular work load. The significance of the altered hemodynamics as a cause of the patient s congestive heart failure is borne out by the gratifying operative result. Due to the rarity of tetralogy in adults, the indications for operation are not well formulated. An important indication would be to prevent the possibility of paradoxical embolization; cerebral complications are the most frequent causes of death in long-term survivors [ZI. In our judgment the prime indication for operation is a deteriorating clinical course. It is our opinion that when the anatomical condi- 602 THE ANNALS OF THORACIC SURGERY

6 CASE REPORT: Hepail. of Tetralogy at 61 Years tion is favorable and the symptoms are severe and have failed to respond to medical management, operation should be performed. REFERENCES Abbot, M. E. Atlas of Congenital Cardiac Disease. New York: American Heart Association, Bopp, P., Rast, J., and Duchosal, P. Unusual longevity in Fallot s tetralogy and pseudotruncus arteriosus. Brit. Heart J. 25:735, Bowie, E. A. Longevity in tetralogy and trilogy of Fallot: Discussion of cases in patients surviving 40 years and presentation of two further cases. Amer. Heart J. 62:125, Coles, J. C., Gergely, N. F., and Buttigliero, J. B. Congenital heart disease in the adult. Arch. Surg. (Chicago) 89:130, Cooley, D. A., and Hallman, G. L. Surgical Treatment of Congenital Heart Disease. Philadelphia: Lea & Febiger, P Edwards, J. E., Carey, L. S., Neufeld, H. N., and Lester, R. G. Congenital Heart Disease. Philadelphia: Saunders, Vol. 11, p Fallot, A. Contribution B l anatomie pathologique de la maladie bleue (cyanose cardique). Marseiile Med. 25:77, Friedburg, C. K. Diseases of the Heart. Philadelphia: Saunders, P Friesinger, G. C., and Bahnson, H. T. Tetralogy of Fallot: Report of case with total correction at 54 years of age. Amer. Heart J. 71:107, Harley, H. R. S. What is Fallot s tetralogy? Amer. Heart J. 62:729, Lendrum, B. L., Agustsson, M. H., Arcilla, R. A., Gasul, B. M., and Mercado, H. G. Natural history of patients with acyanotic tetralogy of Fallot. Circulation 24:979, McCord, M. C., Van Elk, J., and Blount, S. G. Tetralogy of Fallot: Clinical and hemodynamic spectrum of combined pulmonary stenosis and ventricular septal defect. Circulation 16:736, Nadas, A. Pediatric Cardiology (2d ed.). Philadelphia: Saunders, Pp. 629, 640. Ibid., p Rowe, R. D., Vlad, P., and Keith, J. 0. Atypical tetralogy of Fallot: A noncyanotic form with increased lung vascularity. Circulation 12:230, Savard, M., Swan, H. J., Kirklin, J. W., and Wood, E. H. Hemodynamic alterations associated with ventricular septal defects. In A. D. Bass and G. K. Moe (Eds.), Symposium on Congenital Heart Disease. Washington, D.C.: American Association for the Advancement of Science, P Taussig, H. B. Tetralogy of Fallot: Especially the care of the cyanotic infant and child. Pediatrics 1:307, Tikoff, G., Schmidt, A. M., Thorne, J. L., and Kuida, H. Clinical and physiologic sequelae of large ventricular septal defects. Amer. J. Med. 42:497, 1 OK7 VOL. 11, NO. 6, JUNE, g