[Cardiac failure in children]. / Insuffisance cardiaque de l'enfant. Nouvelles approches physiopathologiques.

The understanding of cardiac failure pathophysiology in children has advanced greatly in recent years. Whilst cardiac failure secondary to left ventricular dysfunction with viral or metabolic cardiomyopathy is comparable in pathophysiological terms with adults, other aetiological mechanisms are also implicated, linked both to anatomical anomalies in congenital cardiopathy and their surgical repair. The role of the neuroendocrine system with adrenergic activation of the renin-angiotensin system, secretion of natriuretic peptides and the inflammatory system, before and during surgical repair procedures, have been proven both experimentally and clinically. Understanding these pathophysiological mechanisms opens new therapeutic perspectives for improving the management of children with congenital cardiopathy in cardiac failure and reducing postoperative mortality and morbidity.

[Congenital cardiopathy: indications for anticoagulant treatment]. / Cardiopathies congénitales: indication d'un traitement anticoagulant.

Congenital cardiopathy is common in children with thromboembolic disease. There are no objective clinical data for medium and long term treatment. Vitamin K antagonists could be envisaged for this. Children with a mechanical valve should be anticoagulated. This has been validated in adults, there has been no study in children which contradicts this, and the serious nature of thromboembolic complications in this group is significant. In atrial fibrillation, the most frequent indication for oral anticoagulants is after a Fontan procedure, because of the thromboembolic risk factors. Anticoagulation following Fontan or similar procedures relies on the thrombotic factors and their temporal distribution, being more pronounced in the first postoperative year; anticoagulants should be given over this period. In Eisenmenger's syndrome haemorrhage is common: these patients are not anticoagulated unless there is an indication such as recent pulmonary embolus. During interventional catheterisation thromboembolic accidents are rare, occurring early, and more commonly with certain prostheses. In adults with an intra-atrial prosthesis oral anticoagulants are used for three months, and then replaced by antiplatelet drugs. In congenital cardiopathy the stent is often pulmonary or aortic, and the incidence of thromboembolic accident is therefore very low. For a venous channel or in the context of a Fontan procedure, oral anticoagulants are justified for between three and six months. It is to be hoped that prospective studies, of which some are in progress, will provide further guidance for these indications.

Coronary events after arterial switch operation for transposition of the great arteries.

BACKGROUND: Transfer of the coronary arteries is a crucial step during the arterial switch operation (ASO) for transposition of the great arteries. This retrospective study aims to assess the incidence and risk factors of coronary events after ASO and sensitivity of noninvasive tests in the diagnosis of the coronary obstruction. METHODS AND RESULTS: Between 1982 and 2001, 1304 newborn and infants had an ASO and the 1 198 hospital survivors had a 59-month mean follow-up. Coronary events occurred in 94 patients (7.2%; 95% CI, 6 to 9). Survival without coronary events were 92.7, 91, and 88.2% at 1, 10, and 15 years, respectively. The incidence was bimodal: high early and slow later. Multivariate analysis showed correlation with type B or C coronary pattern and major operative events (P<0.0001 and P=0.0024). In a subset of 324 patients who underwent a coronary artery angiography, lesions were observed in 22 patients (6.8%; 95% CI, 5 to 10). Multivariate analysis showed correlation with only type B or C coronary pattern (OR=20.8, P=0.0002). All of these patients had electrocardiogram and echocardiogram, 174 patients also had a treadmill test, and 115 patients had a myocardial scintigraphy. The association of these tests had the highest diagnosis sensitivity, 75%. CONCLUSIONS: After ASO, coronary events are not rare, occurring most often early and are an important cause of death. Coronary repair can be needed lately. Noninvasive tests are not sensitive enough to detect significant delayed coronary artery stenosis and coronary artery angiography should be performed.

[Prevalence and diagnosis of coronary lesions after arterial switch]. / Prévalence et dépistage des lésions coronaires après switch artériel.

In anatomical repair procedure of transposition of the great arteries (arterial switch), translocation of the coronary arteries is crucial and coronary complications remain the principal cause of death. The aim of this retrospective study was to assess the prevalence of coronary lesions and to evaluate the diagnostic methods to prevent their consequences. From 1982 to 2001, 1,304 patients were operated for transposition of the great arteries by the switch procedure at the Marie Lannelongue Surgical Centre. The average follow-up of the survivors was 59 months (3 days to 17 years) during which 324 patients underwent coronary angiography. All had an ECG and an echocardiogram (N = 324); 174 underwent exercise stress testing and 115 had myocardial scintigraphy. Of the 324 patients who underwent coronary angiography, 22 had coronary lesions (6.8%; 95% CI 5-10). In multivariate analysis a type II coronary network by the Marie Lannelongue Classification was related to the risk of coronary lesions (OR = 0.28; p < 0.0002). Each non-invasive method studied separately had a low sensitivity (< 50%) for the detection of these lesions. The association of ECG, echocardiography and myocardial scintigraphy had the best sensitivity at 75%. Therefore, after the arterial switch procedure non-invasive investigations are not sensitive enough to diagnose coronary lesions and systematic coronary angiography and aortography should be performed in all patients.

Long-term results after valvotomy for congenital aortic valvar stenosis in children.

As interest increase in the Ross procedure performed as a therapeutic option for children with congenital aortic valvar stenosis, it becomes increasinly important to know the late results of aortic valvotomy in this population. We have therefore examined retrospectively the medical records of 121 consecutives survivors undergoing aortic valvotomy before 10 years of age between 1974 and 1992. The mean age at the first valvotomy was 29 months, with a range from 3 days to 10 years. The mean duration of follow up was 9.4 years, with a range from 1.6 to 22 years. Fifteen patients (12.3%; 70% CL: 10-16) died: 9 following reoperation, and 6 late after surgery. Death was related to the hearts in 86% of cases. The actuarial survival rate was 79% (70% CL: 72/84) at 10 years. Young age at the first valvotomy, and the number of procedures, emerged as risk factors of secondary mortality. Reoperations on the aortic valve, 73 in all, were required in 56 patients. The second procedure was done after a mean interval of 6 years, with a range from 1 day to 18 years. This was for restenosis in three-quarters of the cases. The aortic valve was replaced in 30 patients, at a mean of 9 years, and with a range from 9 months to 18 years, after the first procedure. The survival without replacement at 20 years was 29% (70% CL: 15-49). No factor was identified with a relationship either to reoperation or valvar replacement. Long term results after aortic valvotomy, therefore, show a high late mortality, frequent reinterventions, and an almost inescapable eventual need for valvar replacement. The ongoing use of the Ross operation is justified, even if longterm studies in children are still needed to validate its use.

[Comparison of long-term results of arterial switch and Senning procedure in transposition of great vessels with intact ventricular septum]. / Comparison des résultats à long terme du switch artériel et de l'opération de Senning dans la transposition des gros vaisseaux à septum intact.

One hundred and five survivors after the 30th day of complete cure of transposition of the great arteries with intact ventricular septum between 1980 and 1985 were followed up. Fifty-four had an arterial switch (AS) in a single stage at an average age of 10 +/- 9 days and 51 had Senning's procedure (S) at an average age of 4 +/- 2.5 months. The average follow-up (97% of patients) was 5.8 +/- 1.1 years for the AS group and 9.3 +/- 2.3 years for the S group. The actuarial survival at 5 years was 100% in the AS group and 85.8% in the S group (p < 0.01) (8 late deaths). In the AS group, 3 patients were reoperated for stenosis of the pulmonary artery and, in the S group, 4 patients underwent 6 reoperations. All but 3 patients in the S group and all but 1 patient in the AS group are in functional Class I of the NYHA classification. Doppler echocardiographic studies have shown mild to severe dysfunction of the systemic ventricle in 2% of the AS group and 26% of the S group (p < 0.001). Holter monitoring, performed in 70% of patients in the S group showed sinus node dysfunction in 60% and sinus rhythm in 40% of cases. In conclusion, good functional results were observed at over 5 years in both groups. However, the absence of late mortality and the minimal incidence of systemic ventricular dysfunction in the AS group confirm the authors' choice of indication of arterial switch for the treatment of transposition of the great arteries with intact ventricular septum.

[Neonatal heart transplantation. First results]. / Transplantation cardiaque néonatale. Premiers résultats.

Two 5- and 17-days old neonates with hypoplastic left heart syndrome respectively underwent orthotopic heart transplantation. The donor-recipient weight ratio was +58 percent and +88 percent; ischemic time was 144 and 167 min. The immunosuppressive protocol included thymoglobulin during the induction period and a classical 3-drug therapy, with a rapid tapering off of prednisone over 3 weeks. No infectious complication was observed; each infant experimented one episode of acute rejection, successfully treated with prednisolone. Forty-three months and 10 months later, the 2 children are doing well, with normal renal function and normal growth. No late rejection episode was observed. Heart transplantation in neonates is feasible, the short-term and mid-term results are good. Despite important ethical problems, heart transplantation represents a great hope for neonates with inoperable congenital heart defects.

[Immediate and mid-term results of surgery of aortic valve stenosis in the newborn infant]. / Résultats immédiats et à moyen terme de la chirurgie chez le nouveau-né avec sténose aortique.

Between January 1980 and June 1990, 47 consecutive neonates with severe aortic stenosis underwent surgical aortic commissurotomy at Marie-Lannelongue Hospital. The average age at operation was 5 days. Other cardiac abnormalities were present in 27 children and left ventricular fibroelastosis in 11 children. Closed heart aortic valvotomy via the apex of the left ventricle was performed in 26 patients and open heart commissurotomy in 21 patients. Immediate per- or postoperative death was observed in 14 cases (29.8%). Six patients died after open heart valvulotomy (29%) and 8 after closed heart valvulotomy (31%). The mortality was higher in children with a critical preoperative status: 60% versus 22% (p less than 0.05); in cases with an associated cardiac malformation: 45% versus 6% (p less than 0.01) or fibroelastosis: 91% versus 11% (p less than 0.001). None of the patients was lost to follow-up which lasted an average of 47 +/- 41 months. Ten secondary deaths were observed, of which 4 were sudden, 4 after reoperation and 2 due to mitral stenosis with pulmonary hypertension. The overall one year survival was 57% (IC 70%: 40-64); the 5 year survival was 46% (IC 70%; 30-60). Reoperation was necessary in 12 children. Survival without reoperation at 1 and 5 years was 49% (IC 70%: 41-56) and 43% (IC 70%: 29-59) respectively. At the last follow-up examination, 97% of the 23 survivors had good left ventricular function; the systolic pressure gradient was less than 50 mmHg in two thirds of patients and aortic regurgitation was minimal or absent in three quarters of patients. In critical aortic stenosis in neonates, surgery is associated with a high immediate and secondary mortality. The results at medium term in the survivors are good with respect to symptoms, left ventricular function and obstruction to left ventricular ejection.

Anatomic repair of Taussig-Bing hearts.

From 1978 through 1990, 27 consecutive patients underwent anatomic repair of Taussig-Bing heart (TBH) malformation. Group 1 (seven patients) underwent intraventricular repair by rerouting of the left ventricular blood stream through a tunnel patch from the ventricular septal defect (VSD) to the aorta. Group 2 (20 patients) underwent an arterial switch with VSD closure. Indications for each type of repair were based on the following criteria: when the great vessel relation is more or less anteroposterior, repair is performed at the arterial level; when side by side, the minimal tricuspid pulmonary valve distance is evaluated; if less than aortic valve diameter or if there are abnormal tricuspid chordae, repair is performed at the arterial level; if greater, pulmonary artery banding is performed in order to wait until suitable weight for intraventricular repair. The mean age at operation was 13.2 months. Coarctation of the aorta was present in 12 patients, abnormal tricuspid chords in eight, and subaortic stenosis in seven. The great vessels were in D-transposition in 12 patients, and they were side by side in 15. Seventeen patients underwent previous palliative surgery. Early (two patients, 7.4%) and late (two patients, 8%) mortality rates were related in group 1 to subaortic stenosis and in group 2 to myocardial infarction. Mean follow-up of 40 +/- 24 months was achieved in 22 survivors. They were all in New York Heart Association (NYHA) class I, without medication. Actuarial survival and freedom from reoperation rates were, respectively, at 5 years 73 +/- 14.6% and 58 +/- 13% (70% confidence limits [CL]).(ABSTRACT TRUNCATED AT 250 WORDS)

[Use of PTFE (Gore-Tex) in pulmonary systemic anastomoses. Apropos of 50 cases]. / L'utilisation du PTFE (Gore-Tex) dans les anastomoses systémiques pulmonaires. A propos de 50 cas.

Between 1977 and 1981 palliative anastomosis procedures between the subclavian and pulmonary arteries using a PTFE (Gore-Tex) prosthesis for anatomical reasons (short subclavian artery or trajectory incompatible with a Blalock-Taussig anastomosis) were carried out in 50 patients from 1 day to 24 years of age with cyanotic congenital heart disease. Two groups of patients were identified: ten patients in which the PTFE prosthesis was used as an "extension" to the subclavian artery, and 40 patients in which the PTFE was interposed between the respected subclavian and pulmonary arteries. There were three deaths (6 p. 100) at 1,7 and 15 days after surgery (mean mortality 5,7 and 12,3 p. 100). There were no major complications in the survivors. Two prostheses became occluded, one of which was reanastomosed to the ascending aorta. The follow-up ranges from 6 months to 5,5 years. The angiographic investigations performed before complete repair demonstrated the patency and good function of these anastomoses.