[Intravenous thrombolysis in pediatric arterial ischemic stroke: a case report and a review of the literature]. / L'utilisation de la thrombolyse intraveineuse dans l'infarctus cérébral artériel aigu de l'enfant, en dehors de la période néonatale : à propos d'un cas et revue de la littérature.

Pediatric arterial ischemic stroke (AIS) is little known by pediatricians, as it is rare and difficult to diagnose. Current therapeutic approaches include platelet aggregation inhibitors, anticoagulation using heparin or thrombolysis with rt-PA, and therapeutic abstention in the absence of consensus, in contrast with stroke in adults. We report the case of a 3-year-old child who benefited from intravenous thrombolysis with rt-PA 2h after a major AIS of the anterior and middle left arteries due to embolism from the heart. Neurological progression was marked by a persistent, severe functional deficiency, in spite of the early use of thrombolysis. No hemorrhagic complication occurred. This observation raises the problem of therapeutic indications in childhood AIS and more particularly the question of the use of intravenous thrombolysis. Currently, few studies exist on this subject and only a few case report of children and teenagers having received effective intravenous or intra-arterial thrombolysis further to an AIS are available in the literature. A single study describes 46 cases of children with AIS having received thrombolysis but reports numerous complications. However, the absence of symptomatic hemorrhagic complications is noteworthy. There is currently insufficient evidence to recommend thrombolysis in pediatric AIS; nevertheless it can be proposed in certain rare cases, similar to the observation reported herein.

Long-term clinical and hemodynamic evaluation of porcine valved conduits implanted from the right ventricle to the pulmonary artery.

OBJECTIVE: This retrospective study was initiated to evaluate the long-term results of valved prosthetic conduits implanted in the right ventricular outflow tract in patients with complex ventricular-pulmonary discontinuity. METHODS: A cohort of 103 patients out of 127 (24 early deaths, 19%) operated on between 1973 and 1996 with porcine valved conduits was available for evaluation, with a follow-up ranging from 1 to 21.6 years (mean follow-up 8.4 +/- 6 years). A total of 74 hemodynamic studies were performed after the operation, 50 patients having undergone at least 1 cardiac catheterization during the follow-up period. RESULTS: There were 16 late deaths, and the actuarial survivals, including early mortality, were 72.9% +/- 4% at 5 years, 63.1% +/- 5% at 10 years, and 58.2% +/- 5% at 15 years, at which time 20 patients were still available for review and exposed to the risk of dying. The mean peak systolic gradient across the right ventricular outflow tract was plotted as a function of time, showing a gradual increase and a significant step-up after the eighth year, from 43 +/- 36 to 69 +/- 19 mm Hg (P < .005). Reoperation was required for progressive conduit obstruction between 1.1 and 17.7 years after implantation (mean 7.4 +/- 4.8 years) in 25 patients (24%, 70% CL 15%-33%), with generally very few symptoms, or for residual ventricular septal defect in 3 patients. Freedom from reoperation was 79.5% +/- 5% at 10 years and 65.8% +/- 7% at 15 years. CONCLUSIONS: Porcine conduits may represent a valuable alternative to biologic substitutes with similar long-term results. Given the few symptoms, progressive conduit stenosis after the eighth postoperative year imposes a yearly noninvasive patient evaluation during the follow-up.

Mechanical valve in aortic position is a valid option in children and adolescents.

OBJECTIVE: The choice of a valve substitute remains a challenge in young patients, with numerous reports of early degeneration and calcification of biological valves in this age group. Therefore an assessment of the long-term results after mechanical aortic valve replacement in children was initiated. METHODS: A retrospective study was conducted in 54 consecutive patients aged 1.1 to 17 years (mean 12.8 +/- 4 years) operated on between 1975 and 1993. Aetiology was congenital in 34 patients, rheumatic in 13, infectious in 5, and dystrophic in 2. Concomitant surgery included mitral valve replacement (10), aortic annulus enlargement (9), correction of truncus arteriosus (7), Bentall operation (2), coarctation repair (2), tricuspid valvuloplasty (2), correction of double outlet right ventricle (1), and replacement of a right ventricle to pulmonary artery conduit (1). A Bjork-Shiley valve was implanted in 14 patients, and a St Jude Medical valve in 40. All patients were given Warfarin with a monthly INR control. Follow-up was completed through questionnaires mailed to referring physicians and direct clinical examination. RESULTS: Overall early mortality was 13% (7 cases), and 6% (2 cases) in the 32 patients operated on after 1984. Follow-up was complete in 45 survivors (2 lost to follow-up), with a total follow-up of 261 patient-years. There were 6 late deaths, 4 being cardiac and due to persistent LV dysfunction, and 2 valve-related, due respectively to major gastro-intestinal bleeding and massive thromboembolism. Linearized rates of valve thrombosis and anticoagulant-related hemorrhage were both 0.3% per patient-year. Actuarial survival rate was respectively 84.5% at 5 years and 70.2% at 10 years. Reoperation was necessary in 3 patients for recurrent LV outflow tract obstruction. One patient with severe LV dysfunction is awaiting a heart transplant. CONCLUSION: We conclude that the longterm outcome after mechanical aortic valve replacement in children and adolescents is satisfactory and comparable to currently available reports on biological substitutes. The mandatory anticoagulant therapy is well tolerated in this age group.

[Long-term results after surgery of coarctation of the aorta in neonates and children]. / Résultats à long terme après chirurgie de coarctation aortique chez le nouveau-né et l'enfant.

Surgery is the treatment of choice for coarctation of the aorta in childhood. Coarctation presenting in the neonatal period carries a poorer functional and vital prognosis and it may be opposed to the paucisymptomatic forms observed in infants and children. Coarctation in the neonatal period presents with severe cardiac failure and is often associated with hypoplasia of the transverse aorta and/or other complex congenital malformation. Improved neonatal intensive care and the introduction of prostaglandin E1 have considerably reduced the immediate mortality by enabling surgery to be undertaken under the best possible haemodynamic conditions. However, early and late mortality in this group remain significantly higher due to associated cardiac lesions; in this context, the management varies with some groups carrying out surgery in one stage and others in two stages. Despite progress in neonatal surgery and operative techniques to increase the diameter of the transverse aorta, hypoplasia may persist and be a cause of restenosis or secondary hypertension. In this group of coarctations, the main problem is the timing of surgery in order to reduce the risks of restenosis and hypertension to a minimum. Restenosis is diagnosed by clinical examination. Doppler ultrasonography and eventually confirmed by magnetic resonance imaging (MRI). The risk factors for restenosis are young age at surgery, the type of procedure performed and the presence of extensive aortic hypoplasia. Recurrent, localised forms are accessible to percutaneous angioplasty when performed 6 months to 1 year after surgery; extensive restenosis and restenosis in older children should be referred for reoperation. Some subjects become hypertensive in the absence of residual obstruction and, in these cases, MRI should be requested to detect hypoplasia of the aortic arch. However, hypertension may be observed alone or only occur during exercise: late surgery and the length of follow-up seem to be associated with its occurrence. Aortic aneurysms occur after aortoplasty with a patch, a technique which has now be abandoned for this reason. Nevertheless, this risk is also associated with percutaneous angioplasty of restenosis, justifying systematic diagnostic MRI. In summary, coarctation of the aorta in children has a good overall prognosis at medium-term, the neonatal forms having considerably benefited from progress in the management of this condition in the intensive care unit and from advances in surgical technique. However, long-term cardiological follow-up remains necessary to detect the two potential complications: restenosis and hypertension.

[Cardiopathies in trisomy 21. Therapeutic indications]. / Les cardiopathies de la trisomie 21. Indications thérapeutiques.

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.

[Pediatric cardiac transplantation. Long-term results and problems]. / Transplantation cardiaque pédiatrique. Résultats à long terme et problèmes actuels.

The aim of this study was to establish an assessment for cardiac transplantation by retrospective analysis of data from two French centres. Screening for acute rejection was ensured by systematic myocardial biopsies for patients aged over 10 years, and by clinical non-invasive follow up, ECG and Doppler echo in the younger ones. Coronaropathy was screened for by coronarography or stress echography. Eighty three cardiac transplantations were performed in 82 patients aged 9.2 +/- 6 years. The follow up was 5.8 +/- 4.8 years. Survival was 86% at 1 month, 76% at 1 year, 73% at 5 years and 60% at 10 years and did not differ for age at transplantation (p = 0.88) or the initial pathology (p = 0.25). Twenty-nine patients died in the period between 3 days to 11 years, of which 13 were during the first month and 9 after one year (of which 4 were acute rejections and 2 coronaropathies). The incidence of acute rejection was 1.8 episodes per patient; 42% were late rejections, through non-compliance for half of them. Systematic biopsy was useful in the first 3 months post transplant. Eight patients (9.6%) presented with coronaropathy of the graft, progressive for 5 of them. Renal function was altered in 12% of cases, especially in the patients transplanted before the age of 2 years. The results of paediatric cardiac transplantation are satisfactory. Acute rejection and therapeutic compliance for adolescents, graft coronaropathy, and renal function of infants remain the principal elements for the long term prognosis.

[Medium-term results of non-invasive follow-up after cardiac transplantation in childhood and in adolescence]. / Expérience à moyen terme du suivi non invasif après transplantation cardiaque chez l'enfant et l'adolescent.

Between December 1984 and September 1996, 43 cardiac transplantations were carried out in 40 patients aged 2 days to 21 years (one third under 10 years of age) for cardiomyopathy (21 cases), congenital heart disease (19 cases) or retransplantation (3 cases). The average waiting time for transplantation was 80 days: this delay increased by a factor of five in 2 years (from 1 month, before 1994, to 5 months at present). Twelve patients dies, including 6 before the 8th day. The 28 survivors were prescribed triple immunosuppressive therapy: the average follow-up was 4.4 years (range 3 months to 11 years). Monitoring rejection was carried out by non-invasive methods based on clinical, electrocardiographic and Doppler echocardiographic observations. Any suspicion of acute rejection led to endomyocardial biopsy for confirmation and therapeutic guidance. There was a total of 47 episodes of acute rejection (0.3 per patient), mainly in the first 3 months: acute rejection was less common in the younger children. Graft function was normal in 71% of cases. Five children have a pacemaker implanted during the first month. Despite continuous steroid therapy, 82% of patients had normal staturo-ponderal growth. The myocardial mass of the graft increased in parallel with the body surface area. Nephrotoxicity of ciclosparine was responsible for significant renal failure in 19% of patient and seemed more common in the young children. Psychological disturbances were commonest in adolescence and could result in poor treatment compliance (4 cases, with 1 death and 2 retransplantations). Despite satisfactory medium-term results, nephrotoxicity of ciclosporine, long-term graft function and psychological difficulties of adolescents remain unresolved so that transplantation is reserved for terminal cardiac disease resistant to all other forms of treatment.

[Mid-term results of treatment of aortic coarctation in neonates]. / Résultats à moyen terme du traitement chirurgical de la coarctation aortique du nouveau-né.

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.

[Variation in cholesterol, lipoproteins and triglycerides after heart transplantation in children]. / Variations du cholestérol, des lipoprotéines et des triglycérides après transplantation cardiaque chez l'enfant.

Total cholesterol, HDL and LDL-cholesterol and triglyceride levels may contribute to the development or progression of coronary artery disease of the transplanted heart. The aim of this retrospective study was to determine the short and long-term lipid profiles of transplanted children and to identify factors influencing these dyslipidemias. Twenty-three patients aged 9.5 +/- 5.9 years at cardiac transplantation were followed up for 5.8 +/- 3.1 years. All were on triple therapy with normal diets. The total cholesterol increased by 17% during the first year (4.47 +/- 1.01 mMol/l to 5.25 +/- 1.22 mMol/l at 1 year: p < 0.05) with a peak at 3 months of 5.31 +/- 1.28 mMol/l correlating with the dosage of prescribed corticosteroids. LDL-cholesterol levels increased by 20% during the first year (2.26 +/- 0.67 mMol/l to 3.29 +/- 0.99 mMol/l at 1 year: p = 0.018). HDL-cholesterol levels increased from 1.02 +/- 0.27 mMol/l to a maximum of 1.55 +/- 0.4 mMol/l at 1 year, p < 0.05. Lipoprotein A1, a protecting sub-fraction of HDL, did not change significantly. Changes in triglyceride levels were not significant despite a tendency to hypertriglyceridaemia in the early phases. After one year, serum cholesterol and lipoprotein levels remained higher than the initial values. These results show that cardiac transplant children are exposed to the risk of atherogenic hyperlipidaemia and require systematic lipid profile monitoring, dietary advice and lipid lowering drugs.

[Bacterial endocarditis in children]. / L'endocardite infectieuse chez l'enfant.

The authors undertook a retrospective study of 69 cases of infective endocarditis (IE) in 68 children treated from 1971 to 1992. The comparison between two groups (Group I comprising 34 patients treated between 1971 and 1981; Group II comprising 34 patients treated between 1982 and 1992) based on a review of the literature showed that the natural history of paediatric IE has changed during these two decades: a slight increase in the incidence in young children. The sequellae of rheumatic heart disease play no role in determining IE in France. Congenital heart disease plays a major role (72% of cases) with increasing numbers having undergone surgical treatment for more complex lesions. Mitral valve prolapse has become a more common cause with multiple portals of entry, predominantly buccal and oto-rhino-laryngeal. Blood cultures are positive in 75% of cases, the commonest organisms being Streptococci and Staphylococci, but the frequency of uncommon pathogens is increasing. Echocardiography plays a major role in the diagnosis and inventory of IE (vegetations demonstrated in 64% of cases in Group II). Although mortality is progressively decreasing (3% in Group II) because of more frequent surgical indications (32% in Group II) and more severe sequellae: only 27% of children in Group II were cured without sequellae or aggravation of their previous cardiac lesion.

[Echocardiographic evaluation of the growth of cardiac graft in children with heart transplantation]. / Evaluation échographique de la croissance du greffon cardiaque chez l'enfant transplanté.

The evaluation of the growth of the cardiac transplant in children was studied in four young children and three infants by echocardiography after orthotopic transplantation. These children were all under 13 years of age at the time of transplantation and have been followed up for more than two years. The age of the recipients ranged from 2 days to 12.8 years (average 10.7 years) and that of the donors from 7 days to 27 years (average 6.7 years). All patients received triple immunosuppressor therapy. The follow-up was 29 to 48 months (average 39.7 months) in the infants and 28 to 71 months (average 50.25 months) in the children. In the 7 patients the global follow-up period ranged from 28 to 71 months (average 45.8 months). No episodes of acute rejection or hypertension were observed during the study period. The echocardiographic parameters studied were the left ventricular end diastolic dimension, left ventricular end systolic dimension, left ventricular mass, left ventricular mass index. The date was gathered prospectively during the study at monthly intervals after the 3rd postoperative month. The donor/recipient weight ratio varied from 0.83 to 5 (average 1.89). The growth of the recipients was normal in 5 out of 7 cases and moderately retarded in 2 cases. The left ventricular end diastolic dimension, left ventricular end systolic dimension and left ventricular mass increased linearly with the body surface area of all patients. The growth was faster in the infant patients.(ABSTRACT TRUNCATED AT 250 WORDS)

[Role of echocardiography in the diagnosis of congenital abnormalities of the thoracic aorta]. / Place de l'échocardiographie dans le diagnostic des anomalies congénitales de l'aorte thoracique.

Echocardiography has revolutionized the diagnosis and follow-up of congenital heart disease over the last 20 years. Permanent technological innovation in the field of ultrasonic investigation and in the limitations inherent to this technique are illustrated in the assessment of congenital disease of the aorta, the subject of this review. The role of echocardiography associated with Doppler techniques in the investigation of congenital disease of the aorta varies with age: there is no rival technique in investigation of the foetus; in neonates, infants and young children, the role of ultrasound is preponderant because of the excellent echogenicity and the high incidence of congenital aortic disease occurring in a clinical context of cardiorespiratory distress. The limitations and insufficiencies of the techniques are greater in adolescents and adults in whom other non-invasive techniques are possible in acceptable practical conditions. The reality of progress in diagnosis is demonstrated by the possibility of therapeutic indications based only on the association of clinical and echocardiographic data without need for diagnostic catheterization and angiography. The limitations of ultrasonic techniques should however be recognized to avoid inappropriate usage.

[Current results of the treatment of transposition of the great vessels. Apropos of a series of 168 cases including 138 physiologic corrections]. / Résultats actuels du traitement de la transposition des gros vaisseaux. A propos d'une série de 168 cas dont 138 corrections "physiologiques".

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.

[Genetics and congenital heart diseases]. / Génétique et cardiopathies congénitales.

We describe in this article the recent data on the genetics of congenital heart defects (CHD) organised by type of CHD although each predisposing genetic factor is associated with a whole variety of CHD types. The recent progress resulting from animal models, molecular cytogenetics and CHD familial cases studies allow a better understanding of the determinism of CHD. This lead in term to improved counselling of parents of affected children and of CHD adults who would like to become parents. Nevertheless, more progress is needed to reach a better accuracy in prediction.

[Double outlet left ventricle: a rare and unusual cardiopathy. Apropos of 7 new cases]. / Le ventricule gauche à double issue:une cardiopathie rare et étonnante de diversité.

Double outlet left ventricle is a very rare condition due to an abnormality of conotruncal morphogenesis. The authors report 7 new cases to the 119 already published, one with an anatomical variation not previously described. Three of the cases reported were of the most usual type similating tetralogy of Fallot. Two of these cases underwent complete correction with excellent results 13 months and 2 years after surgery. The third patient aged 6 months is well after initial palliative neonatal surgery. A case with an L-malposition pedicle with subpulmonary ventricular septal defect and pulmonary outflow tract obstruction died after early palliative surgery (Blalock-Taussig). A case with subaortic ventricular septal defect, pulmonary stenosis, and tricuspid atresia, underwent physiopathological correction (Fontan procedure) after a Waterston shunt and is well at 19 years of age. The other two cases presented more unusual anatomical forms aortic outflow obstruction: one had hypoplasia of the aortic arch with an isthmic coarctation requiring a Crafoord procedure in the neonatal period associating with banding followed by complete correction at 19 months of age. After 3 years, the patient is asymptomatic. The last case with atresia of the aortic valve and severe hypoplasia of the ascending aorta died after corrective surgery of first intent. Other cases have been described in the literature with different clinical presentations: absence of pulmonary or aortic obstruction; intact interventricular septum. The anatomical variability is due to the complex embryogenesis of the conotruncal region and explains the clinical diversity of this congenital cardiac malformation.

[Total anomalous pulmonary venous connection. Results of repair in 50 infants]. / Retour veineux pulmonaire anormal total. Résultats de la correction chez 50 nourrissons.

Fifty infants with isolated total anomalous pulmonary venous connection (TAPVC) were operated between 1/01/73 and 31/12/87. The average weight at surgery, which was performed under hypothermia with circulatory arrest in 92 per cent of cases, was 4.5 Kg. The preoperative pulmonary to systemic pressure ratios (PAP/PS) enabled identification of two groups of patients: Group I: TAPVC without severe pulmonary hypertension (PAP/PS less than 0.85) (n = 35), and Group II: TAPVC with severe pulmonary hypertension (PAP/PS greater than 0.55) (n = 15). The hospital mortality was 22 per cent (8 cases) in Group I compared with 73 per cent (11 cases) in Group II (p less than 0.05). Patients in Group II were younger (64 days compared with 137 days, p less than 0.02), lighter (p less than 0.05) and had preoperative mean pulmonary artery systolic pressures of 83 mmHg (p greater than 0.001). Three patients in Group I required early reoperation for stenosis of the pulmonary veins at the site of repair resulting in pulmonary hypertension, and all died. The global survival was 28 patients with an average follow-up of 7 years (range 1 to 15 years). Six of these patients were reoperated (2 phrenoplications, 4 atrial shunts). All survivors are asymptomatic and have no conduction defects. Control echocardiography in 15 of the 28 survivors was judged to be normal. These results show that obstructive forms of TAPVC (Group II) carry a very poor prognosis: immediate results in this group could only be improved by earlier surgery. The clinical long-term results in those who survive surgery are very satisfactory.

[Primary tumors of the heart. Diagnostic, anatomic and therapeutic aspects]. / Les tumeurs primitives du coeur. Aspects diagnostiques, anatomiques et thérapeutiques.

Primary cardiac tumours are rare. They are usually benign, the most common ones being left atrial myxomas. The authors report their experience of 16 cardiac tumours operated between 1978 and 1985. The patients were 12 adults and 4 children. The tumours were benign in 14 cases and malignant in 2 cases. Complete (14 cases) or incomplete ablation of the tumour was carried out under cardiopulmonary bypass with an early mortality of 6.25 per cent (1 case) and late mortality of 12.5 per cent (the two malignant tumours). The tumours were identified as myxomas in 11 cases, rhabdomyomas in 2 cases, fibroma in 1 case and malignant sarcomas in 2 cases. The average follow-up period of the 13 survivors is 30 months (range 4 months to 5 years) with excellent clinical and anatomical results. Clinical and paraclinical diagnosis of these tumours does not pose any major problems nowadays because of the reliability of the methods of investigation. However, the nature of the tumour is not always predictable and the operative findings are fundamental, not so much for the treatment which is relatively stereotyped but for the prognosis. Age is not a significant prognostic factor as excellent results may be obtained in the very young and the very old despite a sometimes precarious preoperative clinical condition. The benign or malignant nature of the tumour is the only real factor which affects the prognosis.

[Left ventricular assistance by postoperative mechanical devices in neonates]. / Réentraînement ventriculaire gauche par assistance mécanique postopératoire chez le nouveau-né.

Circulatory assistance in children and neonates has not been extensively reported. It poses specific problems related mainly to miniaturisation of systems. The authors report three cases of left ventricular assistance with the Biomedicus centrifugal pump followed by functional recovery in neonates operated for d-transposition of the great arteries. The assistance was installed by cannulation of the left atrial appendage and the ascending aorta, the canules being connected to the Biomedicus pump. The duration of assistance was 53, 116 and 120 hours respectively. The beginning of left ventricular recovery was observed after 24, 48 and 70 hours of assistance and the patients were weaned of assistance under stable haemodynamic conditions. One of the major problems during the period of assistance is decoagulation, 2 out of 3 patients requiring removal of thrombi during the first 12 hours of assistance. On the other hand, no cases of systemic embolism or thrombosis in the pump itself were observed. Decoagulation was maintained by continuous intravenous heparin with an initial dosage of 5 U/kg/h adapted to the activated cephalin time and anti Xa levels measured every 6 hours. No infectious complication was observed and the sternal wound healed satisfactorily despite systematic delayed closure of the thorax. The quality of results requires cooperation of a surgical infrastructure used to techniques of circulatory assistance, a well equipped neonatal intensive care unit and a permanently accessible laboratory for monitoring coagulation status.

[The Ross procedure in the acute phase of infectious endocarditis in childhood]. / Intervention de Ross à la phase aiguë de l'endocardite infectieuse de l'enfant.

The Ross procedure of aortic valve replacement with a pulmonary autograft has several advantages in childhood over mechanical prostheses or homografts, especially in infectious endocarditis requiring early surgery. Between January 1997 and July 1998, 3 children with no known previous cardiac disease, aged 14 months, 10 and 11 years, had aortic valve infectious endocarditis. The causal organism was not identified in 1 case and the other two were due to staphylococcus aureus and corynebacterium diphteriae. All children had severe, rapidly progressive aortic regurgitation complicated by pulmonary oedema in the baby and systemic emboli in the two older children. Surgery was performed within 9 days, 1.5 month and 2 months after the onset of the disease. The postoperative course was uncomplicated in the 3 cases. Postoperative Doppler echocardiography showed absence of autograft dysfunction or stenosis, with the presence of pulmonary regurgitation in 1 case. Pulmonary autograft has the advantages of not requiring anticoagulation, of allowing growth of the aortic ring, of not being limited by the age of the patient and of having a low risk of degeneration and infectious endocarditis. Therefore, it seems particularly indicated for cases of complicated infectious endocarditis requiring early aortic valve replacement. The early (4.8%) and late (4.3%) mortality rates were comparable to those of other techniques and are lower than those associated with valve replacement with mechanical prostheses in cases of endocarditis (8.5% versus 40%). The secondary morbidity is 18.8% with dysfunction of the autograft and/or stenosis of the pulmonary homograft. Despite a limited follow-up, aortic valve replacement by a pulmonary homograft seems better than aortic valve replacement with a homograft or mechanical prosthesis, especially in cases of complicated infectious endocarditis requiring surgery in the acute phase. Further studies are required to confirm these encouraging results.

[Dobutamine echocardiography in children after heart transplantation]. / Echocardiographie sous perfusion de dobutamine chez l'enfant après transplantation cardiaque.

Coronary disease of the transplanted heart is the principal cause limiting long-term survival of patients and grafts. In view of the invasive nature of coronary angiography, dobutamine echocardiography has been proposed as a non-invasive diagnostic method for this disease. The aim of this study was to determine the feasibility and reliability of this investigation in transplanted children. Twenty-one echoes were performed with dobutamine infusions in 17 patients transplanted at 10 months to 16.9 years of age (average 8.4 years), and followed up 1.1 to 10.1 years (average 4.4 years): 4 were on antihypertensive drugs but none were treated by betablockers. Dobutamine echocardiography was performed according to the standard protocol used in adults. The maximal level was attained in all cases. No major side effects were observed. The maximal heart rate attained 57 to 89% of the theoretical maximal rate, an increase of 44 to 184% compared with the basal heart rate. The maximal systolic blood pressure rose to 120 to 194 mmHg, an increase of 8 to 109% compared with resting values. The contractility scores and segmental contractile index were normal in 18 cases, abnormal at the maximal level in 2 cases (hypokinesia of segments 8 and 9 and akinesia of segments 10 and 16 with an index of 1.2), abnormal at the lowest levels (hypokinesia of segment 7 with an index of 1.1) and maximal level (hypokinesia of segments 1 and 7 with an index of 1.2) in one case. These results were concordant with coronary angiography performed within 2 to 8 days of echocardiography, and considered as the diagnostic investigation of reference (sensitivity 75%, specificity 100%, positive predictive value 100% and negative predictive value 93%). The authors conclude that dobutamine echocardiography is a non-invasive method easily performed with low risk in transplanted children but its diagnostic performance in coronary disease of the transplanted heart should be confirmed in larger studies.