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Unlabelled: There is only a few data on sudden unexpected death (SUD) in the pediatric population, as well as the role of sport., Materials and Methods: Between 1980 and 2003, 2220 autopsies were performed at the Lyon's forensic institute for SUD (all ages) as defined by world health organization., Results: Fifty-seven cases of SUD of cardiac origin concerned 6 to 18-year-old children. Hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy remained the main causes of SUD. Mitral valve prolapse were frequently observed and for the most part of the cases associated with other diseases. Twenty-four were observed during physical activity., Conclusion: Our study confirms the low incidence of SUD and its male predominance in the pediatric population (47 males, 10 females). It seems that physical activity is not the decisive factor for SUD. The authors emphasized the necessity to get a rapid access to cardiac resuscitation devices, as well as the formation of collaborators. Some preventive measures could be done.

Objective: Evaluation of physical capacity in kidney transplanted children for sport practice adequacy., Population and Methods: A retrospective study was carried out in 32 children transplanted at least one year before evaluation. We studied auxology, respiratory function testing, cardiovascular parameters at rest and after exercise, and aerobic capacity performed during a triangular effort test., Results: Kidney transplanted children were smaller and had a higher body mass index (IMC). Breathing parameters and cardiac ultrasound examinations were normal in all but one child. After exercise, four children had high systolic blood pressure requiring a treatment adaptation. Lower values of maximal aerobic power (Pmax) and oxygen maximal uptake (VO2max) were noticed, mainly in girls., Conclusion: Kidney transplanted children have a good physical exercise tolerance. Therefore they can be allowed to practise sports at school or in a club after a normal effort test (taking into account the risk of transplant trauma).

Between 1990 and 1997, 122 neonates aged 8.7 +/- 7.5 days, 75 boys (61.4%), were referred for coarctation of the aorta which was isolated (54 cases) or associated with one (52 cases) or more (20 cases) ventricular septal defects. Hypoplasia of the aortic arch, diagnosed in 52 cases, was more common in children with ventricular septal defects (p < 0.05). The diagnosis was later in isolated coarctation (10.6 +/- 6.8 days) than in cases with shunts (7.8 +/- 7.7 days) and/or hypoplasia of the aortic arch (5.1 +/- 4.3 days). One hundred and nineteen patients were operated, including 112 of left thoracotomy (24 had pulmonary artery banding in addition) at the age of 1.1 +/- 2.7 months, and 7 by sternotomy of first intention for aortic repair and closure of ventricular septal defect. After thoracotomy, closure of the ventricular septal defect was undertaken at 11.3 +/- 10.8 months in children who had undergone previous pulmonary banding and at 3.5 +/- 2.4 months in the absence of banding. Early mortality after aortic repair was 2.5% and late mortality 9.5%, higher in cases of large ventricular septal defects and hypoplasia of the aortic arch (p < 0.001). Follow-up varied from 55 days to 7.8 years (3.99 +/- 2.24 years). Global survival was 97.5% at 1 month and 98.2% at 8 years. In coarctation with ventricular septal defect survival was 95.6% at 1 month and 74.7% at 8 years with a worse prognosis in cases with large single interventricular shunts. Restenosis was observed in 28.5% of cases, 2.25 +/- 3.8 months after aortic surgery (88.5% of cases before the 6th month) and was generally treated by percutaneous aortic angioplasty (10 cases performed 13.5 +/- 12 months after surgery). In all, two factors seemed to increase the risk of death (hypoplasia of the aortic arch and large ventricular septal defects) and restenosis was observed in 1 out of 4 cases, usually before the 6th postoperative month.

This retrospective study analysed the outcome of children with hypertrophic obstructive cardiomyopathy. Between 1969 and 1992, 40 patients all under 20 years of age (mean = 10.9 +/- 6.2 years) were followed up for this condition. There was a positive family history of hypertrophic cardiomyopathy and/or sudden death in 21 cases (53%); 21 (53%) were symptomatic: > NYHA Stage II dyspnoea (n = 13); chest pain (n = 8); syncope (n = 7) or palpitations (n = 4). Thirty-two patients were treated by betablockers, 1 by verapamil, 2 by amiodarone (associated with propranolol in 1 case); 7 patients underwent surgery and 6 others, asymptomatic, had no specific treatment. The mean follow-up period was 10.2 +/- 6.2 years with no drop-out: 13 patients died (1 non-cardiac death, 2 of unknown causes and 10 of cardiac causes, including 8 sudden deaths) giving an annual global mortality of 3.2%; the actuarial 5 and 10 year survival rates were 90 and 85% respectively. The 27 survivors were compared with the 13 patients who died: of the 11 clinical and paraclinical criteria examined, only a previous history of syncope correlated with global mortality (p = 0.004) and sudden death (p = 0.0008).

The use of aortic angioplasty rather than surgery for restenosis of coarctation of the aorta after initial surgery remains controversial. The efficacy and complications of these two techniques have never been compared in prospective and retrospective studies. Between 1976 and 1992, 56 patients were treated for secondary restenosis of coarctation of the aorta: 29 by angioplasty and 27 by surgery. The case reports of these patients were reviewed retrospectively. The average follow-up was 1.5 +/- 0.3 years of the angioplasty group and 5.4 +/- 0.8 years for the surgical group. The reduction of systolic blood pressure in the right arm was significant and identical in the two groups (p < 0.05). The residual pressure gradient was less immediately and at term in the surgical group (p < 0.05). No fatalities were observed in either group. There were no aneurysmal complications. A neurological complication (posterior column syndrome) was observed after surgery (3.7%). The global rate of complications was higher after surgery (6.7% vs 33%). The rate of further restenosis was higher after angioplasty (18.5% vs 3.7%). The low rate of complications, the shorter hospital stay without repeat thoracotomy were in favour of the angioplasty procedure but the greater immediate and long-term efficacy of surgery on the pressure gradient was in favour of the latter solution. A prospective long-term study is necessary.

The aim of this study was to assess the long term prognosis of congenital atrioventricular block (AVB). From 1965 to 1990, 42 cases of congenital AVB (22 antenatal or natal diagnoses and 20 presumed congenital AVB according to Yater's criteria). The AVB was isolated in 28 cases and associated with cardiac disease in 14 cases (8 of which were corrected transposition of the great arteries). The average age of the patients was 14 years (range 32 years to 18 months) at the time of the study. There was a clear female predominance (64%). Maternal connective tissue disease was present in 18% of cases (in the group of children born after 1977 when maternal connective tissue diseases was systematically looked for). Cardiac failure was present in 10 cases (8 with associated AVB); syncope and sudden death were observed in 11 cases. The indication for pacemaker therapy was the presence of poor prognostic factors: syncope, poorly controlled cardiac failure, low heart rate, increased QRS duration, prolonged QTc, infrahisian AVB, long pauses or arrhythmias on Holter monitoring. The only significant prognostic factors in this series were a previous history of syncope, increased QRS duration and a QTc of over 0.45 seconds. Fourteen patients were paced (endocavitary pacing only from 1981), usually in the DDD mode: 8 for syncope, 2 for cardiac failure, 4 for a poor prognostic factor.(ABSTRACT TRUNCATED AT 250 WORDS)

A 16-month-old boy who had a heart-lung transplantation is doing well 16 months postoperatively. The HLT can be a successful treatment for very young patients. Most of the postoperative management can be assessed with noninvasive techniques. Normally, the allograph grows with the recipient.

The poor results of palliative surgery in the hypoplastic left heart syndrome has led Bailey to propose neonatal cardiac transplantation for this condition. The authors report the case of a neonate who underwent transplantation 2 days after birth for hypoplastic left heart with mitro-aortic atresia, diagnosed in utero. The child was prescribed triple immunosuppressive therapy (steroids, azathioprine, ciclosporine) and is now 24 months old. An episode of acute rejection during the first month was treated with a short intensive course of intravenous steroids. At the fourth month, the child underwent aortic angioplasty for an isthmic stenosis. Growth is retarded (- 2SD) and he has mild renal failure but psychomotor development has been normal. The number of neonatal cardiac transplantations remains limited by parental refusal and the lack of donor organs. Despite encouraging medium term results, questions remain as to the long-term viability of the graft and the patient's renal function.

In recent years, thanks to a better understanding of the pathophysiology of congestive heart failure and progress made in the pharmacology of cardiovascular drugs, new therapeutics have been advocated in the treatment of congestive heart failure. Among them, converting enzyme inhibitors are the most useful. However, the classical association of digoxin-furosemide and general measures remains a very effective first-choice treatment in most cases. Only in particular situations, such as cardiomyopathy and decompensated atrio-ventricular insufficiency, should priority be given to converting enzyme inhibitors. Phosphodiesterase inhibitors are essentially used within the context of post-cardiac surgery intensive care. Beta-blockers which have been recently proposed for treatment of adult patients must not be used, as there is still no data available on their effectiveness and tolerance in pediatric patients.

This review is a retrospective study of the cases of 103 patients under 20 years of age with idiopathic dilated cardiomyopathy and represents the whole experience of the Department of Paediatric Cardiology of the Hospital of Cardiovascular Disease, Lyon, between January 1970 and December 1988. The aetiological investigations were constantly negative: hypertrophic, restrictive and secondary cardiomyopathies were excluded. The clinical, radiological, electrocardiographic and echocardiographic data of each patient was noted at the time of admission and at the end of the clinical course. There were many more infants in this series. The onset of the disease was usually sudden. All patients were given medical treatment. The study period ranged over 19 years. Forty one patients (39.8%) died mainly of terminal cardiac failure. Sixty two patients are still alive, of whom 39 are completely cured, 2 have undergone cardiac transplantation, and 21 have a variable degree of chronic invalidity. The six months survival was 77.5%; the 1 year survival was 70% and the 5 year survival was 60%. Over one third of deaths (36.6%) occurred during the first month, 56% during the first 3 months and 70% during the first year. A statistical analysis comparing the initial criteria to death by the chi 2 test revealed the following prognostic factors: age at presentation (32.9% of deaths in patients under 2 years of age compared to 56.6% in the group over 2 years of age, p less than 0.025) and a family history of myocardial disease. The other clinical, radiological and echocardiographic parameters had no predictive value in this series and it was not possible to identify potential candidates for cardiac transplantation.

The authors discuss the percutaneous balloon dilatation procedure and the results obtained in 10 children (age range: 2.5-13 years) over a 2 year period (April 1986-April 1988). Six cases of coarctation restenosis were observed and good clinical and hemodynamic results were noted in 2 cases, while in 4 cases the procedure was not totally successful. For the 4 cases of stenosis of bioprosthetic valves in right ventricular-to-pulmonary arterial conduits, good results were obtained with a significant reduction of the trans-stenotic gradient from 50 to 23 mmHg. While long-term effectiveness of the procedure is still uncertain, those encouraging preliminary results suggest that this procedure could be considered as an alternative treatment for coarctation restenosis and stenosis of bioprosthetic valves in right ventricular to pulmonary artery conduits.

The purpose of this study was to evaluate the efficacy technique and follow-up results of balloon valvuloplasty for congenital valvular pulmonary stenosis. Percutaneous dilatation was performed in 48 patients aged 0.5 to 21 years (m = 7.5 yrs) from two pediatric cardiology centers (Lyon and Montreal). The right ventricular peak systolic pressure ranged from 42 to 180 mmHg (m = 93) before dilatation and fell from 24 to 105 mmHg (m = 48) immediately after dilatation. The pulmonary valvular peak systolic pressure gradient ranged from 22 to 156 mmHg (m = 73) and fell to 4 to 70 mmHg (m = 27) after the procedure (P less than 0.001). Follow-up continuous doppler data was available from 33 patients at 1 to 37 months (m = 9.2 months) after dilatation. The maximal instantaneous gradient from right ventricle pulmonary artery ranged from 0 to 74 mmHg (m = 27).

One hundred eighteen patients who survived Mustard immunités (n = 103) or Senning operations (n = 15) performed between 1972 and 1984 for isolated transposition of the main arteries were followed-up for over a mean period of 3.67 yr (up to 12 yr). The authors have examined the potential complications which can alter the long-term prognosis in this type of atrial correction: heart rhythm disorders (regression of sinus rhythm of 3.82% yr), mechanical complications, haemodynamic dysfunction. After atrial correction the survival curve underwent an actuarial regression of 1.3% yr. All these potential complications should be detected by regular survey and may indicate anatomical correction, the results of which have yet to be fully evaluated.

This study takes stock of the current results of physiological correction (Mustard's or Senning's operation) in simple transposition of the great vessels, at a time when anatomical corrections, or detranspositions, are developing. Between January 1, 1974 and December 31, 1984, 168 neonates with simple transposition of the great vessels were operated upon and followed up for a mean period of 3.67 years (up to 11 years and 10 months). Thirty of them died before correction (12.7% mortality rate with palliative surgery) and 15 immediately after corrective surgery (11.2%). Among the 111 children who survived corrective surgery, the results were satisfactory in 62.7%, fair in 17.8% and poor in 10.2%; the late mortality rate was 9.3%. Post-correction morbidity mainly consisted of mechanical complications suspected in one-third of the patients (with 3 consecutive deaths) and heart rhythm disorders (50% of patients in this series had abnormal Holter recordings), with predominance of atrial rhythm disorders (regression of sinus rhythm was 3.82% per annum). This, after correction the survival curve underwent an actuarial regression of 1.31% per annum. This primary and secondary morbidity and mortality justifies a switch to anatomical corrections the results of which remain to be fully evaluated.

Prostaglandin E1 (PGE1) was administered to increase ductus patency in 26 neonates who present a cyanotic heart disease with right ventricular obstruction (1st group, 13 cases) or congestive heart failure with left ventricular outflow obstruction (2nd group, 13 cases). Clinical improvement occurred in all but the oldest infant in the first group. The efficacy of PGE1 is inconstant in the second group, but medical status was very poor before treatment. We could usually reduce the dose of PGE1 but in no case we could stop the drug before surgery. Surgery could be delayed several hours or days to get an hemodynamic and biologic satisfactory condition and even a few weeks until the infants and their pulmonary arteries had grown (1st group). Side effects are common but relatively minor.

Between September 1st 1974 and June 1st 1976, 12 infants under the age of 6 months have been operated on by a dacron patch aortoplasty for coarctation of the aorta. The associated lesions were a patent ductus arteriosus in each case, a VSD in 8 cases, and a trans-position of the great arteries in 5 cases. A pulmonary artery banding was performed with the aortoplasty in 8 cases, and a Blalock-Hanlon operation in one instance. There were two operative deaths (17 p. 100), amongst them one in a 1400 g premature infant, and a late death (3rd month). Two infants have a clinical aspect of recoarctation. In four infants, the post-operative hemodynamic and angiographic study carried out before the treatment of associated intra-cardiac lesions, shows a good result of the coarctation repair. This particular technique, although not performed as a routine in the infant with coarctation of the aorta, seems to be of interest in the most severe forms of the disease, with diffuse isthmus narrowing and intra-cardiac defects.

Two cases of right pulmonary artery to left atrium in the newborn are described. Both patients presented with refractory hypoxemia cardiomegaly, hypovascular lung fields. Moreover one patient presented with continuous murmur and localised X-ray shadow. Echocardiographic left atrium enlargement suggested diagnosis and angiography confirmed it. Spontaneous improvement in one child is due to a decrease in pulmonary vascular resistance and has allowed the patient to attain an age and a weight in which surgical ligation has been performed more safely.

The authors report four cases of metabolic cardiomyopathy with lipid infiltration diagnosed by skeletal muscle and myocardial biopsy in children with no clinical signs of muscular dystrophy. Normal or increased serum and urinary carnitine levels excluded a primary carnitine deficiency. A deficiency in muscular-palmityl-carnitine-transferase was demonstrated. This pathogenic mechanism may be an indication for treatment with carnitine, but the results are less spectacular than in primary carnitine deficiency states.

Forty five patients, 2 months to 42 years of age, had valved tubes implanted for ventriculo-pulmonary discontinuity. The underlying malformations were: certain forms of Fallot's tetralogy (16 cases), transposition of the great arteries with ventricular septal defect and pulmonary stenosis (8 cases), truncus arteriosus (9 cases), double outlet right ventricle (6 cases), other malformations (4 cases). Hospital mortality (at 30 days) was 31% (14 cases). This was mainly due to technical difficulties related to the large number of palliative procedures and to irreversible pulmonary hypertension, present in 43% of cases with fatal outcomes. Thirty one patients were followed up for 6 months to 7 years (mean 3 +/- 1,6 years). There were 3 late deaths, one during reoperation for a residual shunt. All survivors underwent clinical assessment with hemodynamic control in 17 cases (15 routine control catheterisations). To date it has only been necessary to change one valved tube: a good result was obtained.

Approximately 50% of patients with Trisomy 21 have congenital heart lesions and they account for 2.7% of the admissions to the Pediatric Cardiac Unit of the Cardiac Hospital of Lyons. In a series of 91 cases observed over a 10 year period (1070-1980) undergoing hemodynamic and angiographic investigation, 9 out of 10 cases - as previously reported - had the following abnormalities: endocardial cushion defect, 42.7% (39% of which were complete atrioventricular canals), ventricular septal defect, 33%, and Fallot's tetralogy, 12%. These lesions were characterised by the high incidence and precocity of pulmonary hypertension (69 out of 72 cases with a left-to-right shunt . A comparison of the hemodynamic data of children with Trisomy 21 with ventricular septal defects, and children without Trisomy 21 of the same age with ventricular septal defect showed the pulmonary arterial resistances to be significantly higher in the group with Trisomy 21, suggesting that Trisomy 21 plays a role in the pathogenesis of severe early pulmonary hypertension. The operative risk is much higher in these patients, especially with openheart surgery (4 operative deaths out of 12 cases of closure of VSD). The surgical indications are difficult to define because of the context of malformation and mental retardation, the high incidence of pulmonary hypertension and the high opertive risk requiring a large and detailed dialogue with the family.

Over the past 3 years, a Mustard operation has been performed in 38 infants aged 30 days to 24 months, among them 29 less than one year of age. There were 27 TGV with intact ventricular septum, 8 TGV and VSD, (isolated in 3, and associated with PS in 2 and with coarctation of the aorta in 3), 2 TGV with isolated PS, and 1 TGV with aorta-pulmonary window. The operation was carried out under deep hypothermia, circulatory arrest and limited by-pass, using a pericardial patch in all cases but one. The operative mortality is 8% (3 cases) for the whole group, 7% in infants less than one year of age, and 3% for all TGV with intact ventricular septum. The long term results are reviewed, with a follow-up of 2 to 36 months (mean 17 months).

A case of severe Fallot's tetralogy is presented; there was angiographic evidence of progression towards pulmonary atresia. The relationship between Fallot's tetralogy and pulmonary atresia with open septum is discussed, as is the variability of the pulmonary circulation found in cases of pulmonary atresia with septal defect, and the treatment is also discussed.

Of a consecutive series of 91 babies aged less than 2 years, operated on for closure of a ventricular septal defect during the last four years, the 61 cases with a minimum post operative follow up period of 18 months were retained for review. Closure of the ventricular septal defect was carried out directly 41 times, and after pulmonary artery banding in the other 20 cases, using deep hypothermia with a short period of circulatory arrest and cardiopulmonary bypass.

A review of 122 cases of symptomatic coarctation of the aorta in neonates and infants confirmed the need for early corrective surgery in the majority of cases, in relation to the usual coexistence of associated cardiovascular malformations. The operative risk in the newborn up to one month of age was very high (42 %) but fell very sharply thereafter (5%). Restenosis was the principal late complication (36 %), persistent hypertension being rare in this age group. The risk of restenosis (not a significant vital risk during secondary surgery) should not weigh in the balance against life saving surgery where clinical deterioration is observed despite medical therapy or due to associated malformations.