[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.