[Congenital heart disease and nuchal translucency with normal karyotype. Report of 3 cases]. / Cardiopathie congénitale et clarté nucale avec caryotype normal. A propos de 3 cas.

We report three pregnancies where enlarged nuchal translucency was discovered at the first trimester transvaginal ultrasound examination; congenital heart disease developed later. Two cases of hypoplastic left heart were diagnosed prenatally at the mid-trimester sonographic examination. The pregnancies were terminated. In the third case, a supravalvular pulmonary stenosis was discovered on the second day of life. Further investigations demonstrated a mutation on the elastin locus, thus confirming the diagnosis of Williams-Beuren syndrome. The role of nuchal translucency as a risk marker for congenital heart disease is discussed.

[Additional contribution of high amplification electrocardiography in the follow-up of children with muscular dystrophy]. / Apport supplémentaire de l'électrocardiographie à haute amplification dans le suivi cardiologique des enfants myopathes.

Fifteen children and adolescents with muscular dystrophy underwent investigation by Doppler echocardiography and high amplification electrocardiography to evaluate the quality of left ventricular function. High amplification ECG showed minor intra-atrial conduction defects in 3 cases and abnormal late micro potentials without arrhythmogenic late potentials in 3 other cases. Significant abnormalities were observed in 40% of children examined. Doppler echocardiography showed relative conservation of systolic left ventricular function but a decrease in indices of contractility was observed in 46% of cases, especially in patients with Duchenne muscular dystrophy. Abnormalities of left ventricular distensibility were observed earlier and were more common (53% of cases). A significant decrease in cardiac output was found in 50% of patients, especially among the older and more severely affected children irrespective of the type of muscular dystrophy. These two investigations provide complementary information for the evaluation of left ventricular function of children with muscular dystrophy and they are proposed routinely for the follow-up of these patients.

[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.

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)

[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.

[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.

[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.

[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.

Anomalous origin of left coronary artery with aortopulmonary window - a case report with surgical correction and delayed control.

A 9-month-old male baby was investigated for a massive left-to-right shunt at the arterial level. On right heart catheterization a patent ductus could not be demonstrated but retrograde arterial angiography showed an aorto-pulmonary window. During corrective surgery on cardiac bypass, the left coronary artery was found to arise from the aorto-pulmonary window, this was corrected by dividing the communication distal to the origin of the left coronary artery. On follow-up investigation 6 months after operation the baby was without symptoms and showed a fully functional left coronary artery. To our knowledge this is the first case of such a congenital anomaly to be described.