[Long QT syndrome in children: analysis of the Lyon series]. / Syndrome du QT long congénital chez l'enfant. Analyse de la série lyonnaise.

Congenital long QT syndrome is a rare and serious disorder in children. In addition to the clinical and electrocardiographical diagnostic criteria, molecular biochemistry has identified six genes which are implicated in this pathology. Our study involved a retrospective analysis of 23 patients aged less than 21 with congenital long QT syndrome, followed up for an average of two years. Genotypes were obtained for all of the patients. There were unfortunately two deaths, one of which had a mutation in the SCN5A gene. The other patient had a double mutation of the SCN5A and KCNE2 genes. Symptomatic patients had QT and QTc intervals noticeably longer than the asymptomatic patients, although this difference was not shown to be significant. LQT3 patients as well as those with a double mutation were affected more severely because two of the three LQT3 patients and one of the two patients with a double mutation suffered a cardiac arrest. Three patients in our study showed no mutation. Nevertheless, two of them suffered a severe cardiac event. This confirms the limits of genetic diagnosis, which could be envisaged in all cases. All of the clinical and ECG data should be combined with the genetic analysis in order to confirm the diagnosis.

[Propionibacterium acnes infective endocarditis. Study of 11 cases and review of literature]. / Endocardites infectieuses a Propionibacterium acnes. Etude de 11 cas et revue de la littérature.

BACKGROUND: Propionibacterium acnes, a gram positive, anaerobic, skin commensal bacillus, is too often considered a biologic fluid contaminant, of blood cultures in particular. Its implication has been shown in various infections, including brain abscess, ocular infections, osteitis, and acne. It is also the cause of infective endocarditis (IE). METHODS: Retrospective, observational study of 11 patients with P. acnes IE, hospitalised between 1993 and 2001 at the Louis Pradel Hospital, Lyon-Bron, and review of 20 published cases. RESULTS: P. acnes IE is rare, though its prevalence is probably underestimated. It is most likely to affect men (71%), and affects all ages (children 4/31 cases). An entry point, probably cutaneous, is rarely confirmed. P. acnes IE often develops on valve prosthesis (42%), and embolisms are common (61%). The infective site is usually aortic (55%). The often-subtle symptoms and slow growth of the organism in vitro complicate the diagnosis, which is often made at a late stage, when valvular and peri-valvular destruction has become major. Despite the high sensitivity of P. acnes to most antimicrobials, a surgical intervention is very often needed (81%). The mortality is relatively high (15% to 27%). Examination of pathologic specimens by polymerase chain reaction increases the sensitivity and speed of its detection. The identification of P. acnes in a biologic specimen, valvular tissue in particular, requires a thorough knowledge of the clinical context before concluding to contamination, and mandates close surveillance of the patient. P. acnes can be the cause of IE long before it has been detected.

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.

Echocardiographic findings in Friedreich's ataxia.

Echocardiographic examination of 21 patients with Friedreich's ataxia (age 7 to 28 years) showed cardiac abnormalities in 90% of the cases. They were characterized by varying degrees of septal hypertrophy in 81%, left ventricular free wall hypertrophy in 61%, and a slight reduction of left ventricular internal dimension in 57% of the cases. Asymmetric septal hypertrophy (ASH) with a septal/left ventricular free wall ratio of over 1.3 was found in 29% of the cases, and systolic anterior motion (SAM) of the mitral valve in three patients. Two other patients showed evidence of a different type of cardiomyopathy with marked symmetric left ventricular hypertrophy and marked left ventricular enlargement.

Anomalous origin of left coronary artery from the pulmonary artery: evolution of left ventricular function and perfusion after surgery in a 44-year-old man.

Anomalous origin of left coronary vessel from the pulmonary artery is an almost universally fatal form of congenital heart disease unless appropriate corrective surgery is performed at an early age. A case was diagnosed in a symptomatic 44-year-old man who presented an impairment of systolic left ventricular function. The abnormality was successfully treated with ligation of the left coronary artery combined with left internal mammary artery anastomosis. Left ventricular function improved dramatically six months after surgery, both at rest and during exercise. Such an evolution suggests that surgery may be indicated not only in infants but also in adults with this congenital heart abnormality.

[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.

[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.

[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.

[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.

[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.

[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.

[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.

[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)

[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.

[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.

[Long-term prognosis of congenital atrioventricular block]. / Pronostic à long terme des blocs auriculo-ventriculaires congénitaux.

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)

[Hydatid cyst of the heart diagnosed with bidimensional echography. Apropos of a case]. / Kyste hydatique du coeur diagnostiqué par échographie bidimensionnelle. A propos d'un cas.

A hydatid cyst of the left ventricular posterior wall was diagnosed by 2D echocardiography in a 36 year old North African patient presenting with exertional dyspnoea and atypical chest pain. Clinical examination was negative but the surface ECG showed inverted T-waves in the infero-lateral leads. Postero-anterior and lateral chest X rays were initially interpreted as normal but 2D echocardiography immediately revealed a cystic cardiac tumour which very probably was hydatid, given the patient's origin. Thorough investigation showed no other localisations and hydatid serology was also negative. Left ventricular and selective coronary angiography were performed to determine the vascular relationships of the cyst: ablation was carried out under cardiopulmonary bypass and pathological examination confirmed the diagnosis of hydatid disease.

[Remote results of corrective surgery for aortic coarctation in newborn infants and infants]. / Résultats tardifs de la chirurgie correctrice de la coarctation aortique chez le nouveau-né et le nourrisson.

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.

[Coronary anomalies associated with the Williams-Beuren syndrome. Apropos of 2 cases]. / Anomalies coronaires associées au syndrome de Williams-Beuren. A propos de deux observations.

Two coronary artery anomalies associated with the Williams-Beuren syndrome are reported. The first case was a 14 year old child with severe supra-aortic stenosis associated with severe hypoplasia of the ascending aorta; 2 D echocardiography and angiography showed a voluminous aneurysm of the left main coronary artery. Aortoplasty with a patch gave a good early result. The second case was a two and a half months old baby operated as an emergency after cardiac arrest and who died at the end of surgery. The baby had severe supra-aortic stenosis, occlusion of the left coronary ostium by the left anterior cusp which was abnormally adherent to the aortic wall with subendocardial infarction and reaction fibroelastosis. The frequency of coronary anomalies associated with the Williams-Beuren syndrome is probably underestimated. Because of their severity they must be looked for routinely, by echocardiography and angiography. Their tendency to progress is an indication for early surgical correction of severe supra-aortic stenosis.