[Kawasaki disease in a young infant: difficulties for diagnosis and early complications]. / Syndrome de Kawasaki chez 1 nourrisson: difficultés diagnostiques et complications liées à l'âge.

UNLABELLED: We report 1 case of Kawasaki disease in young infant, featuring atypical presentation and severe outcome. CASE REPORT: A 3-month-old patient was admitted with initial cervical lymphadenitis, and lasting fever. He subsequently developed the classical manifestations of Kawasaki disease. Treatment with intravenous gamma globulins was successful only after a second perfusion. The early echocardiography revealed a coronary vasculitis, which progressed rapidly and was followed by complications as right coronary thrombosis, several aneurysms, and some others as exsudative enteropathy, aseptic meningitis and hepatitis. CONCLUSION: Patients less than 6 months with Kawasaki disease are at increased risk of atypical presentation, severe prognosis and are often predicted to be non-responsive to the first administration of IVIG.

Transcatheter closure of patent foramen ovale in patients with platypnea-orthodeoxia: results of a multicentric French registry.

BACKGROUND: Dyspnea and the decrease in arterial saturation in the upright position in elderly subjects is described as platypnea-orthodeoxia syndrome (POS). POS is secondary to the occurrence of an atrial right-to-left shunt through a patent foramen ovale (PFO). METHODS: This French multicentric study reports on 78 patients (mean age 67 +/- 11.3 years) with POS who had transcatheter closure of the PFO; frequently associated diseases were pneumonectomy (n = 36) and an ascending aortic aneurysm (n = 11). In all patients, the diagnosis was confirmed by transthoracic or/and transesophageal echocardiography. Five different closure devices were used: Amplatz (n = 45), Cardioseal (n = 13), Sideris (n = 11), Das Angel Wings (n = 8) and Starflex (n = 1). Closure was successful in 76 patients (97%). RESULTS: Oxygen saturation increased immediately after occlusion from 84.6 +/- 10.7% to 95.1 +/- 6.4% (p < 0.001) and dyspnea improved from grade 2.7 +/- 0.7 to grade 1 +/- 1 (p < 0.001). A small residual shunt was immediately observed in 5 patients (3 with the Cardioseal device, 1 with the Sideris and 1 with the Amplatz) leading to the implantation of a second device in one case (Cardioseal). Two early deaths occurred unrelated to the procedure (one due to sepsis probably related to pneumonectomy, another due to respiratory insufficiency). Other complications were: a small shunt between the aorta and the left atrium, two atrial fibrillations and a left-sided thrombus which disappeared with anticoagulant therapy. At a mean follow-up of 15 +/- 12 months, there were 7 late deaths related to the underlying disease. CONCLUSION: Percutaneous occlusion of the foramen ovale is safe and gives excellent results thanks to continuing improvement in available devices. This technique enables some patients in an unstable condition to avoid a surgical closure.

[Restrictive cardiomyopathy due to myofibrillar myopathy]. / Une cardiomyopathie restrictive révélatrice d'une myopathie myofibrillaire.

UNLABELLED: Early and severe cardiomyopathy may be related to myofibrillar myopathy. CASE REPORT: We report a one-year-old child with early and severe restrictive cardiomyopathy. The diagnosis of myofibrillar myopathy was obtained on skeletal muscle and endomyocardial biopsies. The patient died despite inotropic support and mechanical ventilation. CONCLUSION: Myofibrillar myopathy must be considered when exploring the etiology of a restrictive cardiomyopathy in children. The diagnosis relies on examination of endomyocardial or skeletal muscle biopsy samples.

Serial echocardiographic measurements of the pulmonary autograft in the aortic valve position after the Ross operation in a pediatric population using normal pulmonary artery dimensions as the reference standard.

Serial echocardiographic measurements of the annulus and sinus were obtained in children before the Ross operation, and early and late postoperatively. Values were compared with normal standards for the aorta and pulmonary artery (PA). There was no significant difference between PA annulus measurements before surgery and the corresponding autograft immediately afterward (1.73 +/- 0.60 cm preoperatively; 1. 63 +/- 0.58 cm postoperatively, p = NS). Late after surgery the mean annulus diameter was enlarged compared with the normal aorta (DeltaZ 1.9 +/- 2.4), but remained relatively unchanged compared with the normal PA (DeltaZ 0.7 +/- 1.1, p <0.01). In contrast, the autograft sinus was dilated early after surgery (1.83 +/- 0.58 cm preoperatively; 2.18 +/- 0.73 cm postoperatively, p <0.01). Mean sinus Z score further increased compared with both the aorta (DeltaZ 1.3 +/- 1.7) and PA (DeltaZ 1.3 +/- 1.6). Use of standard PA measurements may be important in the assessment of autograft enlargement. Minimal change in autograft Z scores over time suggests that annulus enlargement is mainly due to somatic growth. In contrast, the autograft sinus showed an immediate and continued disproportionate increase in size over time, suggesting that sinus enlargement is largely due to passive dilation.

[Percutaneous closure of an right-left interatrial shunt acquired after pneumonectomy]. / La fermeture percutanée du shunt droit-gauche interauriculaire acquis après pneumonectomie.

Right to left shunt through a patent foramen ovale after pneumonectomy is a rare complication. Its clinical presentation is a severe dyspnea with posture dependency. The diagnosis is easily confirmed by contrast echocardiography. The purpose of this article is to expose the interest of interventional catheterization and transcatheter closure. This method appears to be a viable alternative to surgery for those patients with precarious respiratory function.

[Prognosis and outcome of idiopathic dilatation of the right atrium in children. A cooperative study of 15 cases]. / Pronostic et évolution de la dilatation idiopathique de l'oreillette droite chez l'enfant.

Idiopathic dilatation is a rare abnormality corresponding to isolated aneurysmal dilatation of the right atrium, the outcome of which is not well known. Therefore a multicentric retrospective study was set up by the paediatric working group of the French Society of Cardiology recensing 7 boys and 8 girls who were diagnosed with this condition between 1971 and 1993. Ten of the children were asymptomatic and the diagnosis was suggested by the chest X-ray: one neonate had cardiac failure secondary to atrial tachycardia. The diagnosis has been facilitated by echocardiography since 1980. In this series, since 1993, four diagnoses were made antenatally. The outcome was variable : eight children are alive and well with follow-up periods ranging from 2 to 15 years (average 6 years) : four children have had cardiac arrhythmias : benign atrial extrasystoles (1 case), junctional reentrant tachycardia (1 case). The other two had more severe arrhythmias with flutter in a 7 year-old and one neonatal atrial tachycardia. The outcome was favourable with medical treatment. Three children underwent surgical atrial resection : the outcome has been good in these 3 cases with follow-up periods of 4, 13 and 18 years. This series shows that idiopathic dilatation of the right atrium is usually a well tolerated abnormality but unexpected complications may arise which can be severe such as arrhythmias, or which may be potentially threatening such as interatrial thrombosis. Management consists of either follow-up to diagnose complications which require appropriate treatment of systematic surgical correction as some authors suggest.

[French experience in the closure of atrial septal defects of the ostium secundum type with the Sideris button occluder]. / Expérience française de la fermeture des communications interauriculaires de type ostium secundum par la prothèse boutonnée de Sideris.

From February 1992 to November 1995, four French teams used the Sideris button occluder to close 122 ostium secundum, foramen ovale or surgical fenestration atrial septal defects in 121 patients aged 2 to 79 years with body weights of 10 to 96 kg. a left-to-right shunt in 110 cases (average QP/QS = 2.09) or right-to-left shunt in 12 cases. The usual type of prosthesis was used in 115 cases, 8 centered on a guide wire, reverse type in 5 cases and the "centering-device" type in 2 patients. Nineteen implantation attempts were abandoned before releasing the prosthesis. The immediate results were: closure of the atrial septal defect in 116 patients: 59 were completely occluded, 43 had minimal residual shunts. Five patients were operated for non-buttoning or malposition of the prosthesis. In one other case, the device was removed by catheterisation. During follow-up ranging from 1 month to 3 years, 20 patients were operated for varying complications, the commonest of which was malposition of the prosthesis (17 cases) with a shunt of variable volume. In one other case, a second device was inserted. Seventy-seven patients were reviewed at 1 year, 28 a 2 years and 6 at 3 years. The residual shunts decreased with time but only completely disappeared in half the cases. Secondary fractures not requiring surgery were observed in 5 patients. Failures and complications were the result of various causes which are discussed. Successive technological improvements and the experience of the medical teams should reduce this incidence, but caution is required especially in the treatment of young children.

[Percutaneous valvotomy of aortic valve stenoses in children]. / Valvulotomie percutanée des sténoses valvulaires aortiques de l'enfant.

A retrospective cooperative study of percutaneous aortic valvuloplasty was undertaken in 12 French centres from 1985 and included 90 children over 3 months of age (average 8.5 +/- 5.2 years) treated for isolated aortic valve stenosis with peak transvalvular pressure gradients of 80.7 +/- 23 mmHg. over 50 mmHg in 92% of cases. The majority of cases were performed by a retrograde femoral arterial approach with inflation of a balloon with a diameter approximating that of the aortic annulus. There was one serious complication (lethal collapse occurring before dilatation) and 12% of local complications due to arterial trauma: the latter were temporary or accessible to simple therapeutic measures. Overall, the pressure gradient decreased by an average of 39.2 +/- 25.4 mmHg. There were 15 immediate failures (17%) and 21 partial results (23%) requiring a further procedure at varying intervals after the valvuloplasty. There were 54 primary successes (60%) with annulation of the pressure gradient and this result was sustained in 45 of the 51 cases followed up for an average of 34 +/- 21 months. Aortic regurgitation was observed or aggravated in 29 children; at the end of the study, this remained a serious problem in 15 cases (17%). The authors conclude that interventional catheterisation is an elegant. simple and relatively economical alternative to conventional surgery. It is as safe, but less immediately effective overall; aortic continence may be compromised in the long-term. In the absence of technical innovations, a parallel development of the two therapeutic procedures is to be expected.

Idiopathic right pulmonary artery aneurysm with pulmonary valve insufficiency.

A case of idiopathic right pulmonary artery aneurysm with pulmonary valve insufficiency simulating a mediastinal teratoma occurred in an asymptomatic 13-year-old boy. The key to correct diagnosis was pulmonary angiography. The patient was successfully treated with surgery.

International experience with secundum atrial septal defect occlusion by the buttoned device.

Several devices are available for transcatheter occlusion of atrial septal defect. This report describes the international experience with the buttoned device. During a 4.5-year period ending in February 1993, 180 transcatheter atrial septal defect occlusions were performed with the buttoned device. Patient age varied between 0.6 and 76 years and stretched atrial defect diameter between 5 and 25 mm. The defects were closed with 25 to 50 mm devices delivered through 8F (148 patients) or 9F (32 patients) sheaths. Twelve patients were adults whose defects were closed to prevent recurrence of cerebrovascular accidents caused by presumed paradoxic embolism. In the remaining patients the atrial defect was closed to treat the left-to-right shunt. The atrial septal defects were effectively occluded as demonstrated by (1) decrease in pulmonary-to-systemic flow ratio from 2.1 +/- 0.6 (mean +/- SD) to 1.05 +/- 0.1 (p < 0.01) by oximetry; (2) normalized S2 and disappearance of the diastolic murmur by auscultation; and (3) improvement in right ventricular volume overloading by echocardiogram. However, trivial to small shunts could be detected by color Doppler studies in 76 (45%) of 168 patients in whom such data are available. Complications included unbuttoning in 13 and whole-device embolization in 1. All patients remained stable, and retrieval of the device and surgical closure of the atrial septal defect were accomplished in 10 patients. Transcatheter retrieval was used in the remaining 4 patients. The incidence of unbuttoning, a major complication of the procedure, appeared to decrease with the increasing experience of the investigators and with device modification (third-generation). The follow-up duration varied between 1 month and 4 years. Six patients required surgery during the follow-up period. In the remaining patients (n = 160), clinical examination did not reveal signs of atrial shunts. Color Doppler studies revealed either complete disappearance of the previously demonstrated shunts or further diminution of their size. The results indicate that transcatheter occlusion of the atrial septal defects with buttoned devices is feasible, relatively safe, and effective, and it appears to be a viable alternative to surgery for some patients with secundum atrial septal defect. Complications are infrequent and should improve with experience.

[Reconstruction of the left ventricle by circular endoventriculoplasty with septal exclusion]. / La reconstruction du ventricule gauche par plastie circulaire endoventriculaire avec exclusion septale.

Since 1984 the authors have developed a technical modification of left ventricular surgery after myocardial infarction. The principle is to reorganise the contractile muscle in a circumferential manner by excluding the fibrous akinetic parts of the interventricular septum. The operation consists of implanting sutures distally then resecting the exteriorized fibrous zones and finally mobilising the scarred endocardium in the zones inaccessible to resection (septum and the base of the anterior and posterior papillary muscles) up to the limits of the viable myocardium. A patch of septal endocardium or dacron lined with pericardium is sutured in the contractile muscular zone. One hundred and fifty patients have been operated for cardiac failure (37%), angina (40%) or arrhythmias (10%). One third of patients required intra-aortic balloon pumping in the preoperative period. Myocardial revascularisation was associated in 75% of cases. Surgery was performed as an emergency in 33 cases (25% mortality); in the remaining 117 cases the mortality was 5%. Postoperative control assessment (115 immediate postoperative and 60 one year controls) showed the left ventricular geometry to be almost normal and the global ejection fraction to have increased by an average of 17%. This technique of left ventricular remodelling with septal exclusion enables the surgeon to perform a more physiological repair in patients without cardiac failure and to extend the surgical indications in patients with cardiac failure.

[Endoluminal closure of ductus arteriosus. A cooperative study]. / Fermeture du canal artériel par voie endoluminale. Etude coopérative.

Thirty-two patients from 3 Pediatric Cardiological Centres underwent attempted endoluminal transcatheter closure of a patent ductus arteriosus. The patients' age ranged from 8 months to 67 years, but there were only 2 adults (18 and 67 years) in this series. All patients had a murmur which was continuous in 28 cases and systolic in 4 cases. Nine patients had left ventricular hypertrophy on the electrocardiogram and radiological cardiomegaly. Closure of the patent ductus was attempted by Rashkind's technique using a double umbrella of 12 mm diameter for ductus less than 4 mm diameter (26 cases), and an umbrella of 17 mm diameter when the ductus was wider (6 cases). The immediate results judged by retrograde aortography showed total occlusion in 12 patients and incomplete closure in 17 others with persistence of a minimal left-to-right shunt. In 3 cases, embolisation of the umbrella into the left pulmonary artery (2 cases) or right pulmonary artery (1 case), required thoracotomy to extract the umbrella and at the same time section-suture of the ductus arteriosus. Color Doppler echocardiographic follow-up showed the disappearance of the residual shunt in seven children.

[Fetal macrosomia in a diabetic mother]. / Macrosomie du foetus de mère diabétique.

Foetal macrosomia is arbitrarily defined as an excessive weight for the gestational age, was found to be present in 36 p. 100 of 43 diabetic pregnancies. This finding may indicate incomplete normalization of maternal metabolic control despite the use of new forms of insulin delivery systems, such as insulin pumps. However, in our series the macrosomia was usually moderate and without consequences on the foetal outcome. The levels of insulin, but not those of the insulin-like growth factor (IgF1), were increased in the umbilical cord blood of macrosomic newborns of diabetic mothers. Cardiac septum hypertrophy correlated with maternal glycosylated haemoglobin levels better than with macrosomia. We therefore suggest that in all diabetic pregnancies the cardiac septum thickness should be measured in utero by sonocardiography.

Performance of recipient hearts after heterotopic transplantation.

Twenty-two heterotopic heart transplantations were performed, in 19 of which the evolution of the recipient heart was estimated. Within the first postoperative hours the recipient heart was often more effective than the donor heart. The discrepant rhythm between the 2 hearts did not result in any pathological findings. The increased pressures in the recipient's right heart always decreased, sometimes within several weeks. The left atrial volume was found to be reduced while left ventricular contraction was sometimes unchanged, sometimes improved and in a few cases showed definite improvement with a mean decrease of the ultra-sound diastolic diameter of 20 mm on echocardiography. The technique of heterotopic systems seems to be a useful indication for end-stage cardiomyopathies with pulmonary hypertension (class IV) which is generally considered a contraindication for orthotopic heart transplantation.

[Anatomical and hemodynamic evolution at midpoint follow-up with the assisted heart in heterotopic heart transplantation. Apropos of 21 cases]. / Evolution anatomique et hémodynamique à moyen terme du coeur assisté dans la transplantation cardiaque hétérotopique. A propos of 21 observations.

Between November 1978 and March 1985, 27 cardiac transplant operations were performed at the Arnault Tzanck Institute; Barnard's heterotopic method was used in 21 cases. In 16 cases, follow-up was prolonged to assess the effects on the assisted receiving heart. In the first postoperative hours the receiving heart is often more effective than the graft. There were no pathological consequences due to the two different rhythms. The increased pressures in the right cavities of the receiving heart decreased but sometimes this look several weeks. The volume of the left atrium decreased. Left ventricular contraction was unchanged in some cases but in others it improved significantly. This was accompanied by an average decrease of 20 mm in echocardiographic left ventricular end diastolic internal dimension. This technique of heterotopic assistance seems particularly suitable for advanced stages of cardiomyopathy with stage IV pulmonary hypertension.

[Monitoring and rehabilitation of heterotopic cardiac transplantation patients during the period of convalescence]. / Surveillance et réadaptation des transplantés cardiaques hétérotopiques à la période de convalescence.

Heterotopic cardiac transplantation is a procedure performed in patients with severe pulmonary hypertension. Surgery is usually preceded by a long period of inactivity. This paper reports the results of a programme of cardiac readaptation, the complications observed, the improvement obtained and the problem replated to the absence of graft innervation. Eight patients who underwent heterotopic cardiac transplantation underwent the readaptation programme. This began with an exercise ECG on which the physical training programme was based. Sessions of respiratory and muscular physiotherapy were also arranged during the same period; a second exercise ECG was performed at the end of the programme to assess progress. Clinical, biological, electrocardiographic and echocardiographic parameters were carefully monitored. The results of the final exercise ECG showed the average maximal work capacity to be 90 watts for 3 minutes. The average increase in heart rate on effort was 25.92%. This was significantly higher than the resting heart rate (p less than 0.01). Two complications were observed during the programme: ventricular fibrillation of the cardiac graft and graft rejection on the 35th postoperative day, diagnosed by echocardiography. Progression of exercise capacity was observed in all patients. The two patients who made the least progress were those who underwent the fewest sessions of physical readaptation. This improvement was mainly related to peripheral muscular fitness. The training programme was well tolerated by all patients; the principal factor limiting an increase in cardiac output on effort was the absence of graft innervation. The heart rate can only be increased by two mechanisms: an increased venous return and raised concentrations of circulating catecholamines.(ABSTRACT TRUNCATED AT 250 WORDS)

[Supraventricular tachycardia and fetal edema. Apropos of 2 cases]. / Tachycardie supraventriculaire et anasarque foetales. A propos de deux observations.

Two cases of supraventricular tachycardia responsible for hydrops fetalis emphasize the interest of an antenatal echocardiography. The systematic use of this technique will increase the frequency of diagnosis of this etiology in other cases where the prognosis is less favorable. The in utero treatment of this rhythmic disorder consists in administering Digoxin to the mother and the newborn child must receive excellent care from the moment of the birth.

[Evaluation of the severity of congenital aortic valve stenosis by M-mode echocardiography. Comparison with hemodynamics and data from the literature]. / Estimation de la sévérité d'une sténose valvulaire aortique congénitale par échocardiographie TM. Comparaison avec l'hémodynamique et les données de la littérature.

Previously published M mode echocardiographic formulae for the prediction of transvalvular aortic pressure gradients and/or systolic left ventricular pressure, were tested in a series of 26 children aged 2 to 17 years with compensated valvular aortic stenosis. The formulae using the end systolic parameters Ws/Ds expressing the ratio of the thickness of the left ventricular posterior wall to the diameter of the ventricular cavity were useful, irrespective of the value of the constant of proportionality "C" (225 or 245). Formulae using the end diastolic measurements of the left ventricular posterior wall; of the interventricular septum and diameter of the left ventricle also gave a good estimation of the transvalvular aortic gradients or left ventricular pressure.