of Simple D-Transposition of the Great Arteries

Transcription

1 Growth after Surgical Repair of Simple D-Transposition of the Great Arteries Robert J. Levy, M.D., Amnon Rosenthal, and Alexander S. Nadas, M.D. M.D., Aldo R. Castaneda, M.D., ABSTRACT The effect of the Mustard procedure on growth was assessed in 45 patients with simple D-transposition of the great arteries (DTGA) surviving for at least one year after operation. Growth failure (below the third percentile for height, weight, or both) was found in 25 of the 45 patients preoperatively and in 8 patients postoperatively. The principal factors associated with poor growth before repair were advancing age, increased pulmonary and systemic flow, and subpulmonic stenosis. In those patients without postoperative growth failure, growth had returned to the normal range within two years. All patients with retarded growth after the Mustard procedure had had preoperative growth failure as well. In addition, all 8 patients with postoperative growth failure had one or more major residual hernodynamic abnormalities, whereas residual lesions were present in only 10 of the 37 patients with normal postoperative growth. Simple transposition or D-transposition of the great arteries with an intact ventricular septum (DTGA) is a severe cardiac malformation that results in poor somatic growth [7, 14, 151. The uniformly severe hypoxemia and increased pulmonary blood flow in DTGA 3,191 are probably the major factors responsible for the growth disturbance. Apparently the subnormal height and weight status in these patients is not influenced by intrauterine growth retardation since the birth From the Departments of Cardiology and Cardiovascular Surgery, The Children s Hospital Medical Center, and the Departments of Pediatrics and Surgery, Harvard Medical School, Boston, MA. Supported in part by Training Grant HL05855 and Program Project Grant HL10436 from the National Institute of Health, Bethesda, MD. We would like to express our appreciation to Dr. Olli Miettinen for his advice on statistics. In addition, we thank Ms. Pam Stover for the illustrations and Ms. Margaret Wall for typing the manuscript. Accepted for publication Sept 6, Address reprint requests to Dr. Levy, Department of Cardiology, The Children s Hospital Medical Center, 300 Longwood Ave, Boston, MA weight distribution in this group is normal [12]. Similarly, accompanying congenital malformations likely to impair growth are infrequently associated with DTGA [8]. Successful surgical repair of the cardiac lesion with the Mustard procedure results in normal hemodynamics [3, 171, and preliminary reports suggest that growth may also improve after repair 17, 141. The purpose of the present study was to determine the growth pattern in patients with DTGA before and after the Mustard procedure. Specifically, we addressed ourselves to the following questions: (1) How does the postnatal growth status of patients with DTGA prior to repair differ from the normal pattern? (2) Is there improvement in growth status after the Mustard operation, and over what period of time does it occur? (3) What are the relevant factors that influence growth before and after surgical repair? Methods The records of all patients followed at The Children s Hospital Medical Center, Boston, with the diagnosis of simple DTGA were reviewed. Children who had survived the Mustard operation to at least one-year follow-up were selected and their growth with respect to height and weight was analyzed. Head and chest measurements were not available. Relevant clinical, laboratory, and physiological data were reviewed. All patients had undergone preoperative cardiac catheterization and 33 of the 45 had postoperative catheterization. The 12 patients without postoperative cardiac catheterization were clinically thought to have excellent repairs. All patients had had balloon atrial septostomy or surgical septectomy prior to the Mustard operation. Eight of them had associated subpulmonic stenosis (systolic gradient 2 50 mm Hg). The growth status of the patients with DTGA was assessed by utilizing the Stuart and Reed [20] growth charts from the Children s Hospital Medical Center, Boston. For the purposes of the by The Society of Thoracic Surgeons

2 226 The Annals of Thoracic Surgery Vol 25 No 3 March 1978 present study, growth failure was defined to be height, weight, or both below the third percentile for chronological age. The data were analyzed according to the chisquare statistic with the Yates correction when indicated. Group t testing was used to compare categories whenever appropriate. HEIGHT 0 Pro-op Post-op N.45 Results Forty-five patients with DTGA were studied (Table 1). The mean birth weight of the study population was 3.25 kg (range, 2.3 to 3.9 kg). None of the patients had extracardiac anomalies. PREOPERATIVE GROWTH STATUS. The study population had markedly impaired growth for both height and weight (Fig 1, Table 1). Growth failure was present in 25 of the 45 patients (55%) prior to the Mustard operation ( p < 0.001). Ten of these 25 patients were below the third percentile for both height and weight, 10 were below that percentile for weight only, and 5 were below it for height alone. POSTOPERATIVE GROWTH STATUS. Height and weight for the patients with DTGA had improved significantly as of the latest postoperative follow-up (Table 1, Fig 2). All 37 patients without delayed growth at the latest follow-up had returned to a normal distribution about the median within two years after repair (Fig 2). Among these 37 patients the catch-up in growth was even more striking in the 17 patients with preoperative growth failure evaluated at one year. Of these 17,16 (43% of the37) had achieved a normal growth pattern (Fig 2). PERCENTILE Fig 1, Height and weight according to percentile range for 45 patients with simple transposition before and after Mustard repair. After operation, height status improved significantly (x2 = 9.5; p < 0.01) as did weight status (xz = 29.2; p <O.OOZ). Eight patients had persistent postoperative growth failure; 4 of these were below the third percentile for height and weight and 4 were below the third percentile for weight alone. FACTORS AFFECTING GROWTH. The chief fac- Table 1. Growth Btfore and After Mustard Operation in Patients with Simple Transposition of the Great Arteries Age at Repair Follow-up Median Height Median Weight (yr) Period (yr) Percenhle Percentile Growth Failure" No. of Catrgory Patients Mean Range Mean Range I'reop Postop Preop Postop Preop Postop All patients ' (55%) Rh (17%) Sex Male h (52%) 5' (1R%) Female SO llh (61%) 3 (17%) Palliation SeptUStOmy IS " (47%) 1 (7%) Srptectomy '' (60%) 7' (23%) Repair Hypothermia IR x "' (38%) 2 (11%) Normothermia "' (66%) 6" (22.2%) "Numbrr of patients below third percentile for height, weight, or both. DCwnparison to predicted in New England population p < 0 W1. ''Comparison between preoprative growth failure in patients with hypothermic circulatory arrest versus normuthermia: 18 = 0.05

3 227 Levy et al: Growth after Repair of Simple D-Transposition i HEIGHT WEIGHT 0 Pre-op Post-qJ 1 year 0 4:'es"ps Postoperative growth failure was strongly associated with preoperative growth failure, residual lesions, and older age at repair. All 8 patients with postoperative growth failure had had subnormal growth preoperatively, and in addition, as a group they underwent the Mustard operation at a significantly older mean age (3.2 versus 1.7 years; p = 0.01). The 8 patients with persistent growth failure after the Mustard operation all had major residual lesions (Table 3). The incidence of specific residual lesions was significantly higher in patients with than in those without postoperative growth failure (x2 = 10.8; p < 0.001). < 3rd PERCENTILE Fig 2. Growth status of37patients withoutpostoperative growth failure before and after Mustard repair. Catch-up growth, for both height and weight, was achieved within two years after repair. tors associated with preoperative growth retardation were age, subpulmonic stenosis, and increased pulmonary and systemic flow. The effect of advancing age on preoperative growth failure in patients with DTGA is shown in Figure 3. There was marked deterioration with time. Weight initially was more severely affected than height, but by 3 years of age the discrepancy was no longer evident. Preoperative growth failure was significantly related to increased pulmonary and systemic blood flow (Table 2). In addition, 7 of the 8 patients with subpulmonic stenosis had preoperative growth failure; this represented a significantly greater incidence than occurred in the rest of the patients (x* = 9.1; p < 0.001). The weight status of patients managed by atrial septectomy or Mustard operation using normothermic cardiopulmonary bypass was relatively poorer preoperatively, reflecting significantly older age at repair (p < 0.001). There were no important physiological differences related to type of palliation or choice of repair. Comment The study indicates that patients with DTGA and normal antenatal growth develop subnormal growth in the postnatal period that deteriorates with advancing age. Delayed growth is associated with increased pulmonary and systemic flow, usually manifested clinically by congestive heart failure. It is unrelated to the severity of arterial desaturation. Surgical repair with the Mustard operation results in return to normal grqwth within one to two years unless major residual lesions are present. The few previous reports ongrowth of patients with DTGA [7,14,15] are inconclusive because of the small size of the populations studied [q, grouping together of simple and complex lesions [7,14,15], or lack of information on postoperative results [15] or postoperative hemodynamic assessment [7]. Mehrizi and Drash [15] studied the height and weight of 83 patients with transposition, including complex cases as well as patients with intact ventricular septum. They found 62 (75%) to be below the sixteenth percentile for height and 65 (79%) below that percentile for weight. The operative status of these patients and factors responsible for the delayed growth were not assessed. Feldt and associates [7] reported on 9 postoperative patients with transposition who improved in mean weight from -2.1 to -1.5 standard deviations (SD) and in mean height from -2.3 to -1.3 SD. Mair and colleagues [14] reported accelerated growth after operation in patients with transposition. Postoperatively, 45 of 49 patients (92%) were in "excellent or good' physical condition. Detailed growth status and factors affecting growth were

4 228 The Annals of Thoracic Surgery Vol 25 No 3 March 1978 Height 0 Weight Fig 3. Influence of advancing age on growth in children retardation 16, 231 and extracardiac anomalies [2, with simple transposition prior to repair with Mustard pro- 5, 61. The present study analyzed both preoperacedure. By age3 years, 80% of the children were below the third percentile for height and were below the third tive and postoperative growth in patients with percentile for ziwight. well-defined lesions, with normal birth weight distribution, without extracardiac anomalies, not evaluated. The present study confirms the and with relatively uniform hemodynamics. Our suggestions made by previous investigators and findings clearly show that, all other factors being establishes the beneficial effect on growth of favorable, correcting the abnormal cardiovassuccessful repair with the Mustard procedure. cular hemodynamics can improve abnormal Cardiac operations for various congenital heart growth in patients with DTGA. diseases have generally been held to be of uncer- The principal factors resulting in preoperative tain benefit to postoperative growth. While some growth failure in the present study-age, inauthors have found that operation helps growth creased pulmonary-systemic flow, and subpul- [7, 10, 151, several other studies have shown no monic stenosis-have been studied to some exsignificant improvement in growth after defini- tent in patients with other lesions. While a gentive cardiovascular operations [l, 2,5, 6,221. The eral decline in growth with advancing age is discrepancy in postoperative results may be noted in patients with many types of congenital partly attributed to the fact that these studies heart disease [15, 16,231, the specific time course were based on a small number of patients with a has not been previously documented. Congeswide spectrum of hemodynamic abnormalities tive heart failure is known to be associated with and included patients with intrauterine growth poor growth in patients with ventricular septa1 Table 2. Preoperative Physiological Data and Growth (mean f standard error) Mean Mean No. of Age Sao, PAP SFI PFI Growth Status Patients (yr) ( (mm Hg) (L/min/m2) (L/min/m2) Growth failure t 2 13 t t 0.6" 11.7 t 1.3b Normal * 2 15 t t 0.3" 7.3 t l.lb Total t ? * 1.1 Significance of comparison between groups: "p = (t = 2.40); "p = 0.05 (t = 2.15). Sao, = systemic oxygen saturation; PAP = pulmonary artery pressure; SFI = systemic flow index; PFI = pulmonary flow index.

5 229 Levy et al: Growth after Repair of Simple D-Transposition Table 3. Sequelae of Mustard Operation for Simple Transposition in 45 Patients Factor Growth Failure Normal Growth Total no. of patients 8 37 Age in years (mean 3.2 (k 0.41) 1.9 (k 0.24) standard error) No. with residual 8 10 abnormality Type of residual abnormality Subpulmonic stenosis 4 4 Complete heart block 2a 0 Sick sinus syndrome la la Complete SVC 2 3 obstruction Atrial left-to-right lb 0 shunt Coarctation of aorta 0 1 Sudden death 0 1 C Tacemaker. bpulmonary-systemic flow ratio, 4: 1. cdied seven years after repair. SVC = superior vena cava. defect [16] and patent ductus arteriosus [lo, 241, but attempts to establish the physiological basis for these observations have been inconclusive [9, 11, 221. The response to repair in the present study suggests that successful physiological correction of the cardiovascular hemodynamics improves retarded growth and therefore implies that marked growth impairment is caused by increased pulmonary flow and failure. Interestingly, preoperative growth disturbance in the patients with DTGA was not directly related to the severity of cyanosis. The mean systemic oxygen saturations measured in patients who had preoperative growth failure and those who did not were virtually identical. Similarly, Linde and co-workers [13] found poor growth to be associated with the presence rather than the degree of cyanosis. In adolescent cyanotic patients, systemic oxygen saturation has been shown to correlate with delayed bone age [25]. The absence of a relationship between stature and systemic oxygen saturation in our patients is not surprising because none were adolescents. Subpulmonic stenosis was strongly associated with preoperative growth failure and was the most common residual lesion in the group with continuing growth failure after the Mustard operation. The mechanisms by which subpulmonic stenosis and certain other residual lesions contribute to postoperative growth failure in the study patients have not been well established. Valvular pulmonic stenosis has been observed in association with delayed growth [15], and so has complete heart block [211, found in several patients after the Mustard repair. Similarly, no clear mechanism is known for an adverse growth effect of the sick sinus syndrome or vena caval obstruction, but anorexia probably plays a part in causing growth failure in these conditions [l]. Sex, repair by hypothennic circulatory arrest, and type of surgical palliation had no measurable influence on growth in our study. Investigations of patients with other congenital cardiac lesions have shown growth to be more severely affected in males than in females [2, 13, 15, 231. Clearly this is not the case in patients with DTGA. Furthermore, the normal growth observed after repair utilizing deep hypothermia in our patients is similar to the growth data obtained by Clarkson [4] in patients with large left-to-right shunts repaired by a similar technique. The results of this study lead to the following conclusions concerning management of DTGA: (1) Early Mustard repair is advisable since growth status in this lesion deteriorates with age, and growth failure before the operation predisposes to postoperative growth failure. Furthermore, deep hypothermia was not associated with any deleterious growth effects in the present study. (2) Abnormal postoperative growth by one to two years after the repair is an indication to perform cardiac catheterization since all patients with postoperative growth failure in the present study had significant residual lesions. References 1. Adams FH, Land GW, Disenhoun RB: Observation on the physique and growth of children with congenital heart disease. J Pediatr 44:674, Bayer LM, Robinson SJ: Growth history of children with congenital heart defects. Am J Dis Child 117:564, Chonpsaur GL, Sokol DM, Trusler GA, et al: Repair of transposition of the great arteries in 123 pediatric patients. Circulation 47:1032, Clarkson PM: Growth following corrective cardiac

6 230 The Annals of Thoracic Surgery Vol 25 No 3 March 1978 operation in early infancy, in Heart Disease in Infancy. Edited by B Barratt-Boyes. London, Livingstone, 1973, p Eid EE: A follow-up study of physical growth following failure to thrive with special reference to a critical period in the first period of life. Acta Paediatr Scand 60:39, Engle MA, Hoswalde GR, Goldberg HP, et al: Persistent problems pertaining to patency of the ductus arteriosus: I. Persistence of growth retardation after successful surgery. Pediatrics 1:70, Feldt RH, Strickler GS, Weidman WL: Growth of children with congenital heart disease. Am J Dis Child 117:573, Greenwood RD, Rosenthal A, Parisi L, et a1 Extracardiac abnormalities in infants with congenital heart disease. Pediatrics 55:485, Huse DM, Feldt RH, Nelson RA, et al: Infants with congenital heart disease: food intake, body weight, and energy metabolism. Am J Dis Child 129:65, Krovetz LJ: Weight gain in children with patent ductus arteriosus. Dis Chest 44:274, Lees MH, Bristow VD, Griswold HE, et al: Relative hyperventilation in infants with congenital heart disease and undernutrition. Pediatrics 36:183, Levy RJ, Rosenthal A, Fyler DC, et al: Birthweight of infants with congenital heart disease. Am J Dis Child (in press) 13. Linde LM, Dunn OV, Schillson R, et a1 Growth in children with congenital heart disease. J Pediatr 61:418, Mair DD, Danielson GK, Wallace R, et al: Long term follow-up of Mustard operation survivors. Circulation 5O:Suppl 2:46, Mehrizi A, Drash A: Growth disturbance in congenital heart disease. J Pediatr 70:413, Menahem S: The clinical growth of infants and children with ventricular septal defects. Aust Paediatr J 8:1, Mustard WT: Successful two stage correction of transposition of the great vessels. Surgery 55:469, Noonan JA, Nadas AS, Rudolph AM, et al: Transposition of the great arteries: a correlation of physiologic and autopsy data. N Engl J Med 263:493, 637, 689, 739, Plauth WH, Nadas AS, Bernhard WF, et a1 Changing hernodynamics in patients with transposition of the great arteries. Circulation 42131, Reed RB, Stuart HC: Patterns in growth in height and weight from birth to eighteen years of age. Pediatrics 24:904, Rosenthal A, Castaneda AR: Growth and development after cardiovascular surgery in infants and children. Progr Cardiovasc Dis 18:27, Stocker FP: Oxygen consumption in infants with heart disease. J Pediatr 80:43, Suoninen P: Physicalgrowth of children with congenital heart disease. Acta Paediatr Scand [Suppl] 225:1, Umansky R, Hauck AJ: Factors in the growth of children with ventricular septal defects. Pediatrics 30:540, White RI, Jordan CE, Fischer KC, et a1 Delayed skeletal growth and maturation in adolescent congenital heart disease. Invest Radio1 6:326, 1971