[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.

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.

[A rare pathological association: Fallot's tetralogy and asymmetric septal hypertrophy]. / Une association pathologique rare: tétralogie de Fallot et hypertrophie septale asymétrique.

The association of asymmetric septal hypertrophy and Fallot's tetralogy is very rare. The authors describe a case in an infant documented by angiography and 2D echocardiography. The prevalence of complex forms of Fallot's tetralogy and the diagnostic and therapeutic problems which they pose, are discussed.

[Unusual recording of a giant pulmonary A wave on echocardiography in pulmonary atresia with an intact septum]. / Enregistrement inhabituel d'une onde "A" géante pulmonaire en échographie (TM) lors d'une atrésie pulmonaire è septum intact.

An infant with cyanotic congenital heart disease underwent M mode echocardiography, cardiac catheterisation and angiography. A diagnosis of pulmonary atresia with intact ventricular septum was made. Echocardiography showed an average sized right ventricle with tricuspid valve thickening, as previously described in this condition. In the pulmonary incidence, a giant A wave, occuring after the P wave of the surface ECG, without a systolic valvular opening, was recorded. This probably corresponded to the motion of the diaphragm separating the pulmonary infundibulum from the true pulmonary artery. The possibility of recording the giant A wave of the fibrous diaphragm should be recalled in patients with pulmonary atresia with intact ventricular septum.

[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.

[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.

[Monodimensional echographic study of echogenic masses in the left ventricular outflow tract in aortic valve insufficiencies. Apropos 5 cases]. / Etude échographique monodimensionnelle des masses échogènes de la chambre de chasse du ventricule gauche lors des insuffisances aortiques. A propos de 5 observations.

The presence of a mass in the left ventricular outflow tract is often a sign of severe pathology. The authors report 4 cases illustrating this echocardiographic diagnosis. In bacterial or mycotic endocarditis these masses suggest either aortic valve vegetations or prolapse of an aortic cusp. Two causes may be observed in patients with aortic bioprostheses: paravalvular leak with rocking of the sewing ring and destruction of the bioprosthetic cusps. In the 4 cases presented hemodynamic and angiographic investigations were contra indicated because of the risk of embolism of bacterial vegetations. Echocardiography gave precise diagnosis of the causal disease process and led to early surgical cure. Correlations between the anatomical and echocardiographic appearances are described and discussed.

[Preliminary study on the resumption of work after aorto-coronary bypass]. / Etude préliminaire sur la reprise du travail après pontage aorto-coronarien.

A questionnaire was sent 8 months to 3 years after aorto-coronary bypass to 98 patients who, before surgery, had had to abandon their professional activities because of ischaemic heart disease to assess the numbers who had returned to work: thirty six patients (37%) had not returned to work for medical or personal reasons; only one patient had been refused by his employee; sixty two patients (63%) had returned to work, 81% full-time and 19% with a different job. Only 19% had to stop working secondarily for medical reasons. The criteria thought initially to influence the chances of resumption of professional activity were analysed: factors not influencing the return to work were: type of work before operation (judged by the physical activity involved and the statute of salaried or independent worker), the severity of surgery (number of grafts and associated resection of aneurysm), a subjective assessment of physical condition after surgery (91% or patients not returning to work admitted to feeling well); uncontrollable factors influencing the return to work were: age of patient (average 51,7 years for those returning to work, compared to 55,1 years for the others), previous history of myocardial infarction (2,5 times more common in those not returning to work); finally, controllable factors influencing return to work were: the duration of unemployment before surgery (3,3 months for those returning full-time, compared to 16,4 months for those not returning to work); the period between surgery and resumption of activity which averaged 5 months and should not exceed 6 months. Three factors seemed to be particularly important: apart from the shortest possible period of unemployment before surgery, early physical reeducation after surgery based on chest physiotherapy and readaptation to physical activity and a psychological preparation for the return to work which should be started even before surgery.

[Aneurysm of the sinus of Valsalva rupturing into the pulmonary infundibulum. Echographic study]. / Anévrysme du sinus de Valsalva rompu dans l'infundibulum pulmonaire. Etude échographique.

Rupture of a Sinus of Valsalva aneurysm into the pulmonary infundibulum with ventricular septal defect was diagnosed by M mode echocardiography The differential diagnosis was prolapse of an aortic valve cusp associated with ventricular septal defect. The relevant features were the presence of an echo anterior to the anterior aortic wall. This echo was related to the aortic valve as a small posterior movement was recorded at aortic valve closure. Systolic fluttering of the pulmonary valve suggested a degree of subvalvular obstruction. Diastolic fluttering was related to the aortic regurgitation immediately below the pulmonary valve. This case confirms the hypothesis that the anterior echo produced by the aortic valve is in fact the left anterior coronary cusp.

[Osler aortic aneurysm in isthmic coarctation. Clinical and angiographic aspects and postoperative results]. / Anévrysme aortique oslérien sur coarctation isthmique. Aspects cliniques, angiographiques et résultats postopératoires.

A case of probable infective aneurysm complicating isthmic coarctation of the aorta, is reported. On radiology, the aneurysmal mass seemed to have developed in less than two years. However, it was not possible to culture the causative organism. The pre- and postoperative angiographical appearance after one-stage radical surgery of the lesion and its complication are described.

[Aortic insufficiency and syphilitic coronary ostial disease. Pre- and postoperative angiographic aspects]. / Insuffisance aortique et coronarite ostiale syphilitique. Aspects angiographiques pré et postopératoires.

The authors report the case of a 50 year old patient with severe aortic incompetence (stage IV on angiography) and syphilitic left coronary ostial disease confirmed by complementary investigations. Management comprised aortic valve replacement and saphenous vein aorto-coronary bypass. The postoperative angiographic appearances showed normal valve function and a permeable aorto-left anterior descending bypass graft. The outcome was also satisfactory from the clinical point of view.

[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)

[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.

[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.

[Action of digoxin on systolic and diastolic time intervals. An echocardiographic study in infants]. / Actions de la digoxine sur les intervalles de temps systoliques et diastoliques. Etude échocardiographique chez l'enfant.

The effects of digoxin on systolic and diastolic time intervals were studied in 25 children and infants the majority of whom had congenital heart disease by M mode echocardiography. The recordings were performed before and after the administration of digoxin. Serum digoxin levels were measured to confirm therapeutic dosage. After digoxin, the right and left ventricular pre-ejection periods, the duration of the corrected electromechanical systole and the Weissler indices decreased, and the isovolumic relaxation periods increased. The ventricular ejection times were unchanged except for the corrected right ventricular ejection time which was only slightly decreased. Our results concerning left ventricular systolic time intervals are in agreement with other studies in children. As no other studies of the effects of digoxin on the right ventricular systolic time intervals, or of the right and left isovolumic relaxation time are available, confirmatory studies are required. The decrease in the right and left pre-ejection periods, electromechanical systole and the Weissler indices, is interpreted as being related to the positive inotropic effect of digoxin whilst the increase in isovolumic relaxation reflects only a decrease in preload. This study allows a better understanding of the effects of digoxin on the different phases of the cardiac cycle and a better appreciation of its action potential.

[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.

[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.

[2-dimensional echocardiographic diagnosis of an intracardiac thrombus in children]. / Diagnostic par échocardiographie bidimensionnelle d'un thrombus intracardiaque chez l'enfant.

Eight cases of intracardiac thrombi in infants and children were compiled in a cooperative study involving five paediatric cardiological centres. Two babies were hospitalised for cardiac failure due to a severe supraventricular arrhythmia. Two-dimensional echocardiography (2D echo) showed a left atrial thrombus which disappeared after anticoagulant therapy. The third case was unusual: 2D echo performed 4 months after a Senning operation for complete transposition of the great arteries showed stenosis of the pulmonary venous canal and a thrombus above the stenosis: the mass was echogenic, rounded, of variable density and in contact with the pulmonary veins. These findings were confirmed at autopsy. The fourth case was a 34 month old child with Fallot's triad in whom 2D echo showed a right ventricular thrombus, confirmed at surgery. The four remaining cases were thrombi detected in patients with congestive cardiomyopathy. The thrombus was adherent to the left ventricular lateral wall or apex. Two of these thrombi disappeared after anticoagulant therapy, one of which after hemiplegia. 2D echo is a useful tool for the diagnosis and surveillance of intraatrial or intraventricular thrombi. Intraatrial thrombi may be due to supraventricular arrhythmias in children; intraventricular thrombi are usually seen in association with poor left ventricular contractility. The diagnosis of thrombosis should lead to institution of anticoagulant or even fibrinolytic therapy in order to avoid systemic embolism.

[Angiography of the left ventricle: study of the kinetics of the wall. A new theoretical approach]. / Angiographie du ventricule gauche: étude de la cinétique des parois. Nouvelle approche théorique.

Authors present a new study of left ventricular local wall motion. A critical study about three methods is analysed. This original analysis method is based on the calculation of local ejection fractions from a mathematical model: a theoretical ideal systolic contour is evaluated from an ejection fraction value and for a left homogeneous ventricular contraction. The left regional wall motion is appreciated from the comparison between real systolic contour and theoretical systolic contour. At first the use problems of classical regional wall motion study are described. This presentation is to develop the mechanical and mathematical particularities of our original method. On the other one the study of computer-derived shape is to allow systematical and quick application to quantify the left ventricle contractility.