Therapy and Prevention Congenital Heart Disease

From April 1984 to January 1986, anatomic surgical correction was performed in 50 newborn (2to 23-day-old, mean 8 + 5 [SD]) infants with simple transposition of the great arteries. Before surgery, balloon atrial septostomy was performed in all patients, prostaglandin E1 was infused in 42, and left ventricular shape on a two-dimensional echocardiogram was considered "satisfactory" in 48. Surgery was performed in patients on cardiopulmonary bypass without cardiac arrest; the pulmonary artery was reconstructed by end-to-end anastomosis according to Lecompte's maneuver with a pericardial patch. In all but one patient coronary artery transfer was possible regardless of the distribution of these vessels. There were eight early deaths (16%), but only four (10%) of the last 41 patients treated died. There was one late death (2%) due to a secondary myocardial infarction caused by compression of the left coronary artery. Reoperation was successfully performed in two patients for supravalvar pulmonary artery stenosis. The only late medical complication was a transient episode of myocardial ischemia 6 months after surgery. The 41 late survivors were in excellent condition, were in sinus rhythm, and had a normal left ventricle 1 to 22 months after surgery (mean 7.2 ± 5.4 [SD]). Aortic growth was normal; pulmonary artery supravalvar stenosis occurred in six patients (mild in four). We conclude that anatomic correction can be applied successfully in the first few days of life in newborns with simple transposition of the great arteries, regardless of coronary distribution. Circulation 75, No. 2, 429-435, 1987. ANATOMIC CORRECTION of transposition of the great arteries (TGA) is theoretically a better therapy than atrial repair, since it does not introduce any additional intracardiac anomaly and it restores the left ventricle to its natural systemic function. But anatomic correction is a delicate operation requiring the transfer of the coronary arteries and reconstruction of the pulmonary artery. The long-term fate of these vascular structures remains unknown. Since the first successes were reported in 1975,1 2 anatomic correction appeared for the majority of pediatric cardiologic centers as the method of choice in managing TGA with ventricular septal defect (VSD).3 4 The use of anatomic correction for simple TGA is more questionable for two main reasons: (1) atrial repair5 of simple TGA provides excellent immediate and satisfactory midterm From Service de Cardiologie Pddiatrique, Departement de Pediatrie, Hopital des Enfants-Malades, Paris, and Clinique de Chirurgie Cardiovasculaire, Hopital Marie-Lannelongue, Le Plessis Robinson, France. Address for correspondence: Daniel Sidi, Service de Cardiologie Pediatrique, Departement de Pediatrie, Hopital des Enfant-Malades, 149, Rue de Sevres, 75743 Paris Cedex 15, France. Received July 17, 1986; revision accepted Oct. 2, 1986. Vol. 75, No. 2, February 1987 results, and (2) anatomic repair carries an important risk of failure because of the natural underdevelopment of the left ventricle, which soon after birth becomes a low-pressure ventricle. Therefore, anatomic correction has to be performed either after preliminary preparation of the left ventricle, or in the newborn. A two-stage procedure for treatment of TGA was introduced and successfully developed by Yacoub et al. ,6 but in our hands this method failed, mainly because of a high mortality and morbidity associated with the first stage of pulmonary arterial banding.8'9 Furthermore, this preliminary stage may alter left ventricular function,'0 and may dilate the future proximal aorta,' 12 inducing aortic insufficiency.3 1` Encouraged by the successful report by Hougen et al.`3 in Boston, in April 1984 we decided to attempt to treat simple TGA by anatomic correction in the neonatal period. Well-informed parental consent was obtained in each case and the protocol was applied only to a selected group of patients who were less than 2 weeks old and had a suitable left ventricle. We report here our results with this approach. 429 by gest on A ril 7, 2017 http://ciajournals.org/ D ow nladed from