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Coarctation or isthmic stenosis of the aorta is defined as an abnormal obstruction situated at the junction of the aortic arch and the descending aorta near the site of ligamentus arteriosus. It is a common malformation representing 5 to 7.5% of all congenital heart diseases. Coarctation of the aorta is 2 to 3 times commoner in boys than in girls. Two clinical forms may be distinguished: asymptomatic isolated coarctation of childhood, the surgical treatment of which was first performed by Crafoord in 1944, and coarctation of the neonate and infant associated in over 2/3 of cases with other cardiovascular malformations, especially tubular hypoplasia of the aortic arch. The surgical correction of isolated coarctation is best performed between 6 months and 1 year of age in other to limit the incidence of residual hypertension. A modified Crafoord technique decreases the risk of restenosis and enables treatment of associated hypoplasia of the aortic arch in the same surgical procedure.

Left heart obstructive lesions, in particular interrupted aortic arch or severe forms of coarctation with hypoplasia of the aortic arch, are the main cause of cardiac failure in the neonate and are often at the root of multiple organ failure which worsens the prognosis. Based on a retrospective study of 35 neonates admitted between July 1984 and June 1994, the authors attempted to identify the prognostic factors for admission to the intensive care unit and the optimal timing for operation of these patients. All neonates with a ductus-dependent aortic obstructive lesion and severe multiple (at least four) organ failure, were included in the study. There was a high mortality (54%) including firstly 7 patients who died in the three days following admission to the intensive care unit (20%); this was so-called "medical" mortality for which there was no identifiable poor prognostic factor. On the other hand, the surgical mortality (12 out of 28 cases, 43%) was significantly different in neonates operated before recovery from multiple organ failure (72%) and those operated after recovery from multiple organ failure (17%). Based on these results, the authors propose a therapeutic strategy based on prolonged preoperative intensive care until the initial multiple organ failure is reversed rather than early surgery.

A retrospective study of 150 children under 3 months of age who underwent repair of coarctation of the aorta in the same center (between 1972 and 1987) was undertaken to assess the problems posed by surgery without cardiac catheterisation or angiography. The patients were divided into two groups: Group A, comprising 104 children operated after invasive investigations, and Group B, comprising 46 children operated on Doppler echocardiographic data alone. The two populations were comparable and "hypoplastic aortic arch" type coarctation was present in over 60% of cases. However, there were more ventricular septal defects in Group B (67.5%) than in Group A (49%). There were no significant diagnostic errors in Group B (one case of interrupted aortic arch diagnosed at surgery). The 1 month survival was the same in the two groups (82%). The indications of pulmonary artery banding were less frequent in Group B although there were more ventricular septal defects in these patients. These results confirm the value of Doppler echocardiography in the context of urgent surgery of congenital heart disease.

Twenty infants underwent coarctectomy between 1972 and 1979, 12 were less than one month old at surgery. One infant died after the procedure (5% mortality). Seven underwent open heart surgery later in the first year of life for associated intracardiac defects; there were 3 early and one late death. Of the 15 survivors, 12 have been followed up for 6 months to 6 years (mean 3 years). All are actually asymptomatic. Arterial blood pressure is slightly higher in the leg than in the arm in 8 patients (as in normal children), equal in leg and arm in 2 and lower in the leg than in the arm in 2 (recoarctation). Postoperative catheterization was done in six patients. A pressure gradient across the anastomotic site was confirmed in two (32 and 28 mm Hg). In the other four, no pressure gradient was found, and the site of the anastomosis was hardly or not at all detectable on the angiogram. Coarctectomy in newborns and infants now carries a low operative mortality. With proper operative technique, normal growth of the anastomotic site is usually obtained.

Between 1972 and 1984, 141 infants of less than 12 months of age were operated upon for coarctation of the aorta. The abnormality was isolated in 41 cases (29 p. 100) and associated with ventricular septal defect (VSD) in 58 cases (41 p. 100), with transposition of the great vessels with or without VSD in 16 cases (11.3 p. 100), with cardiac valve disease with or without VSD in 11 cases (7.8 p. 100) or with miscellaneous intracardiac lesions in 15 cases (10.6 p. 100). Resection-anastomosis (Crafoord) was performed in 89 cases (63 p. 100), subclavian flap aortoplasty (Waldhausen) in 36 cases (26 p. 100) and dacron aortoplasty in 16 cases (11 p. 100). Cerclage of the pulmonary artery was combined with one or another of these operations in 65 cases (46 p. 100). Twenty-five patients (17.5 p. 100) died within 30 days of the operation, and 28 patients (24.7 p. 100 of those who survived surgery) died at a later stage. Three infants were lost sight of. Follow-ups ranged from 1 to 13 years (mean: 4.01 years). Fifteen infants (13.3 p. 100 of those who survived surgery) were reoperated upon for recurrence of the coarctation. Four infants (3.5 p. 100) now present with clinical signs of recoarctation and are awaiting treatment.

244 cases of coarctation of the aorta were treated surgically from 1953 to 1988. The age of the patients ranged from 3 days to 59 years with an average of 11.2 years. The average age of the patients decreased progressively over the years. 22% of the patients (54 patients) were less than one year old at the time of the operation. The percentage of infants presenting with decompensation and associated cardiac malformation increased progressively over the years. A total of 40 out of 54 patients belong to this group. The surgical procedure--resection and shunt--was a standard one in 89% of all cases. A prosthesis was required in 4.5% of all cases, and a widening patch in another 5.3%. An unusual technique had to be used in 1.7% of cases. The surgical mortality was of 12 patients (4.9%), all belonging to the infant group (3 days to 13 months old). No mortality was observed among older patients, nor among the elective surgical cases. 9 patients were treated for recurrence of coarctation. No case of paraplegia was noted in this series.

An 11 to 15 year follow up (mean 12.7 years) of 90 patients undergoing surgery for coarctation of the isthmus of the aorta is reported. There were 7 late deaths. Of the survivors 87% were normotensive (B.P. less than or equal to 160/100 mmHg) and 90% were asymptomatic and leading a normal life. Five cases-all with an aortic prosthesis-had to be reoperated for recurrent coarctation. These figures are comparable with those found in the literature. The best long-term results are obtained in patients who have the operation while young (less than 20 years), who have no associated abnormality, and whose coarctation can be treated by resection with direct anastamosis.

142 infants under 6 months of age were operated on for coarctation of the aorta from 1972 to 1978. Coarctation was isolated in 46 (32.4%) and associated with another heart defect in 96. Surgery was decided upon because of congestive heart failure sometimes associated with arterial hypertension refractory to medical treatment: 9 patients died during surgery. 89 underwent Crafoord's operation and in 44 ligature of the subclavian artery or aortoplasty using the subclavia were carried out. Early postoperative death rate was 30.9% and 13 patients (13.2%) died later. Recoarctations occurred in 25 patients (27%); more than half of these patients were less than 1 month-old at the time of surgery. Recoarctation was more frequent (46.4%) in cases with than in cases without (18.7%) aortic hypoplasia and it was more severe (5 out of 7 deaths) when there was an associated cerclage resulting in a double obstacle to right and left ventricles ejection.

Between 1972 and 1983, 29 newborns and infants were treated surgically for coarctation of the aorta. The mean age at operation was 2 months 7 days. 76% of the patients (22/29) had an associated intracardiac malformation, 8 requiring correction. The early mortality rate (due to coarctation repair) was 7% (2/29). The total mortality rate was 31% (7/29). Four of the 7 late deaths occurred during surgical correction of an intracardiac malformation. Twenty children were followed up for 1 to 10 years (mean 4 1/2 years) after surgery. Four patients (20%) had a recoarctation. All 4 recurrences occurred within 2 years after surgery, and in patients operated on during the first month of life. Blood pressure at the latest outpatient visit was above the 97th percentile in 6 children, 4 of whom had recoarctation. Among the patients with no evidence of recurrence, 86% had a blood pressure between the 50th and the 97th percentile or just above the 97th. Five patients underwent a maximal exercise test: none developed excessive elevation of systolic blood pressure, and no pressure difference appeared between arm and leg during the exercise test in those who had none at rest. Thus, early surgical correction improves the prognosis of infantile coarctation syndrome, but recoarctation may occur; long term follow-up is advisable.