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Study Objectives: To examine the results of right heart derivations and clinical outcomes according to preoperative characteristics and operative strategy implemented., Methods: Fontan operations were performed in 65 patients (mean age = 10.3 years, 41 males). The majority of cardiopathies were single ventricles (SV) with (49% of patients) or without (26%) tricuspid atresia. A palliative bidirectional cavo-pulmonary (BCP) anastomosis was performed prior to Fontan in 15 patients. Intra-atrial Fontan tunnelling was performed in 43 patients, Kreutzer-type operations in 10, and extracardiac tubes were used in 8 patients. The mean duration of follow-up was 6.1 +/- 0.3 years., Result: The 30-day mortality was 13.8%. Early mortality was higher among patients with SV with than without tricuspid atresia (P < 0.01), and among patients < 4 years old. Early reoperations were required in 5 patients, including dismounting in 1, BCP anastomosis after Kreutzer procedure in 1, and tube thrombosis in 1 patient. A single death occurred past 30 days, and late adverse events included protein-losing enteropathy in 1 patient, complete atrioventricular block in 1, and tube thrombosis treated with heparin in 2 patients. At the end of follow-up, 75% were in New York Heart Association functional class I., Conclusion: Our intermediate-term results of Fontan-type operations were satisfactory, and steadily improving. The prognosis was better in patients operated at age 4 or older. A prior BCP anastomosis improved the results. A higher morbidity was observed with intra- than with extra-atrial Fontan procedures. The merit of fenestration procedures with respect to morbidity remains the be evaluated.

The aim of this retrospective study was to analyse cases of infectious endocarditis (IE) of native or repaired ventricular septal defects (VSD) to determine its incidence, the circumstances of its occurrences, the outcome and prognosis of this complication. From 1966 to 2002, 36 IE occurred in 19 boys and 17 girls: the age at diagnosis was 13.4 +/- 11.8 years; 26 had an isolated VSD and 10 had VSD associated with a minor lesion. Eleven of the 36 cases (30.5%) had been previously operated: repair of an isolated VSD with a patch in 5 cases, associated with a Crafoord procedure for coarctation of the aorta in 2 cases, three times with conservative treatment of associated aortic regurgitation (AR) and with ligature of patent ductus arteriosus (PDA) in 1 case. Twenty-five of the 36 cases (69.5%) had not been operated before: 21 isolated type 1 VSD; 2 VSD + AR, 1 VSD with PDA (undiagnosed), 1 VSD with valvular pulmonary stenosis (PS). The portal of entry was post-surgical in 7 out of 36 cases (19.4%): 4 VSD patches, 2 VSD patches + Crafoord and 1 VSD patch with ligature of PDA. The source of infection was dental in 14 out of the 36 cases (38.9%): one isolated VSD repair with residual shunt, 11 native VSDs, and 2 cases of unoperated VSD + AR. The other infectious causes (15 = 41.7%) were ENT (2 cases), skin (2 cases), gastrointestinal (2 cases), pulmonary (1 case) or unknown (8 cases), on operated lesions (3 VSD patches + AR) or native lesions (12 cases: 10 isolated VSDs, 1 VSD with PSD and 1 VSD with PS). Twelve episodes occurred (33.3%) despite antibiotic prophylaxis, 7 out of 7 post-surgical and 5 out of 14 dental cases. The commonest localisation was the tricuspid valve (10 cases, always in isolated VSD). Embolism was observed in 60% of right heart endocarditis (always multiple) and in 55% of IE of the left heart (single embolism). Early surgery was required in 6 patients (16.7%). The risk of early surgery was higher in patients with VSDs associated with other lesions (4 out of 10 = 40%) than in isolated VSD (2 out of 26, 7.7%, p = 0.027). Thirteen patients underwent secondary surgery after an average interval of 2.96 years, median 0.86 years (from 4 months to 22.8 years) for VSD repair (10 cases), aortic valve replacement (2 cases) and aorto-aortic conduit (1 case). The global follow-up period was 7.4 +/- 8.3 years, from 28 days to 27.9 years (median 3.3 years). Five deaths were observed on average 3.7 +/- 6.2 years after the episode of IE (median 6 months): 2 were early, occurring less than 6 months after IE and directly related to the infective episode. The survival was 97.1% at 1 month, 94.3% at 6 months, 91.4% at 1 year and 86.6% at 5 and 10 years after IE. VSD is a benign cardiac lesion, the prognosis of which can be severely compromised by infectious endocarditis: surgical repair reduces the risk but does not totally exclude it because of minor associated abnormalities. Prophylactic antibiotic therapy and the diagnosis of latent infectious problems, particularly dental, remains essential before and after cardiac surgery.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Acute infectious myocarditis in childhood has a very poor initial outcome, but the long-term outlook is relatively good for the survivors. This retrospective study was based on cases of acute myocarditis admitted to two hospital departments with different modes of recruitment. Firstly, a polyvalent paediatric intensive care unit where 12 children (mean age 12 months) were admitted during the acute phase of myocarditis. The initial symptoms were non-specific and misleading, the diagnosis being established at autopsy in 9 cases. Only 4 children presented with typical cardiac failure. The clinical signs were hepatomegaly, sinus tachycardia, cardiomegaly, ECG ST-T wave changes and biological signs of multiple organ failure. Left ventricular function was very poor with a fractional shortening of only 17%. The causal agent was usually viral. The clinical course was marked by a high early mortality (11/26, 42%) within 23 hours of hospital admission. Secondly, a paediatric cardiology unit where 81 children (mean age 15 months) were followed up after acute infectious myocarditis. Thirteen cases were taken from our first series and were included for long-term follow-up; 76.5% had premonitory signs of infection and 71% were in cardiac failure, Classes III or IV, during the hospital admission. The causal agent was identified in 30 cases (37%) and was usually a virus (22 cases). Treatment was classical (association of digitalis, diuretics, angiotensin converting enzyme inhibitors, anticoagulants and beta-sympathomimetics when necessary).(ABSTRACT TRUNCATED AT 250 WORDS)

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).

In emergency cases of babies with a single ventricle and an obstruction to aortic outflow, low pulmonary flow or, on the contrary, high pulmonary flow, only palliative surgical procedures can be proposed. The authors set out to determine the prognosis of a population with this type of lesion having undergone one of the three following procedures: systemic pulmonary shunt, pulmonary artery banding, repair of the aortic arch (usually associated with pulmonary banding). One hundred and nineteen (63%) of the 185 patients hospitalised between 1/01/1970 and 31/12/1991 in the paediatric cardiology unit of the Cardiac Hospital of Lyon with a diagnosis of single ventricle, underwent one of these three procedures as a treatment of first intention. The survival of the 22 patients who underwent pulmonary artery banding (90 +/- 6%, 85 +/- 8%, 85 +/- 8% at 1.5 and 10 years respectively) was significantly better than that of the patients undergoing systemico-pulmonary shunt (63 +/- 6%, 53 +/- 6% and 49 +/- 6% at 1.5 and 10 years respectively). On the other hand, repair of an obstacle of the aortic arch was a precarious procedure as the survival was only 23 +/- 11%, 16 +/- 11% and 16 +/- 11% and 1.5 and 10 years respectively). These results suggest, with the reserve inherent to the methodology of retrospective studies of small populations, that it is not illogical to continue to propose pulmonary artery banding for babies with single ventricle associated with high pulmonary flow. This procedure should only be envisaged after strict selection of candidates and providing there are facilities for intensive postoperative care.(ABSTRACT TRUNCATED AT 250 WORDS)

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 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)

The authors analysed the anatomical and clinical prognostic factors of single or common ventricle. This retrospective study was based on a series of 185 patients hospitalised between 1/2/70 and 31/12/91 in the paediatric cardiological unit of the Cardiological Hospital of Lyon with this condition. A number of anatomical and clinical parameters were identified in this population. For each parameter, a given patient could only relate to single modality and a survival graph determined by Kaplan-Meier analysis was established for each modality. For each variable, the survival curve of the most frequently encountered modality served as a reference and the other modalities were compared with it using a logrank test. The different modalities potentially related to patient survival were then entered into a multivariable model using logistic regression. The results of this study of multivariable analysis using the Odds-Ratio (OR) independently suggest that four variables may influence negatively survival of patients with a single ventricle: pH < or = 7.3 on admission (OR = 3.55), a non-left ventricular morphology of the main ventricular chamber (OR = 3.11), the presence of an obstacle on the aortic outflow (OR = 5.58) and a total anomalous pulmonary venous drainage (OR = 26.88).

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.

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.