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Biomedical subjects

J Kachaner

Publications and source records attributed to J Kachaner.

At least 163 records · Page 9Linked to original sources
PubMed1987

[Apparently idiopathic primary myocardiopathies in children. The role of metabolic etiology].

Thirty-three children presenting with "primitive" cardiomyopathy observed from January 1984 to December 1985 underwent a protocol of investigations consisting of histo-enzymatic study of the deltoid muscle, metabolic studies (glucose, free fatty acids, lactate, pyruvate, 3-hydroxybutyrate, aceto-acetate, carnitine, amino-acids blood levels after a 15 hour-fast; urinary organic acids chromatography) and a study of the fatty acids oxidation in cultured fibroblasts. In all children cardiac involvement was predominant and had been the cause for hospitalization. Cardiomyopathies of the hypertrophic type have an early onset, most often are part of a complex picture of extra-cardiac involvement and frequently have a lethal evolution. On the contrary, hypokinetic dilated cardiomyopathies are most often isolated, have a later onset and a less severe course. In 2 cases, an early hypokinetic dilated cardiomyopathy evolved toward hypertrophy. Peripheral muscular involvement is very frequent (lipidosis, mitochondrial aggregates or specific aspects) (60% of cases) in dilated as well as hypertrophic types. A precise etiological diagnosis or a strong presumption was possible in 12 of 33 cases: 2 with hereditary deficiency of the fatty acids beta-oxidation, 1 carnitine systemic deficiency, 1 Friedreich ataxia, 1 central core disease, 1 coxsackie B1 myocarditis, 6 strong suspicions of respiratory chain deficiency.

Cardiomyopathy, Dilated↗
PubMed1987

[Trial of prevention of coronary aneurysm in Kawasaki's disease using plasma exchange or infusion of immunoglobulins].

Coronary aneurysms are present in 15-25% of cases of Kawasaki disease and are responsible for the morbidity and rapid mortality in this disease. We treated 20 children aged 20 +/- 16 months, less than 15 days (8 +/- 3.2) after onset of the disease, either by plasma exchange (7 exchange transfusions and one plasmapheresis) or by high dose intravenous gammaglobulins. No coronary anomalies or cardiac dysfunction prior to or after therapy (mean follow-up 8.3 +/- 4 months) was found. Tolerance to therapy was good and the course of the disease was arrested by exchange transfusions or shortened by gammaglobulin infusion. We conclude as have the Japanese, that this therapy is efficacious and safe, and should be initiated early.

Child, Preschool↗
PubMed1987

[Treatment by prostaglandin E1 of isolated coarctations in newborn infants with acute cardiac failure].

In neonates with isolated coarctation of the aorta presenting with acute neonatal heart failure refractory to classical treatment, emergency surgery is associated with a 30% death-rate. We rather suggest to add to treatment a perfusion of prostaglandin E1 in order to dilate the ductus arteriosus and thereby the aortic isthmus. This was performed successfully in 9 of 12 neonates aged 5 to 16 days: coarctation was relieved and heart failure disappeared. As soon as the left ventricle became hyperkinetic again (2 to 6 days later), prostaglandin was discontinued. Coarctation reappeared with either chronic heart failure (3 cases) or severe arterial hypertension (5 cases). Eventually one child recovered without surgery and the other 11 were operated on at an average age of 24 days, with only one death.

Alprostadil↗
PubMed1987

[Scimitar syndrome in infants. Physiopathology and therapeutic implications in 12 cases].

Twelve children, aged 6 days to 6 months (mean: 42 +/- 23 days) were diagnosed as presenting with the scimitar syndrome. In this syndrome, anomalous pulmonary venous drainage, hypoplasia of the right pulmonary artery and parenchyma, variety of "sequestrum" of the right pulmonary artery and parenchyma, variety of "sequestrum" of the right pulmonary base usually fed by subdiaphragmatic branches of the aorta are associated. Respiratory disorders were constant, severe in 8 cases. X-rays showed "dextro-cardia" and "hypoplasia of the right lung". Endocardiac investigation assessed the diagnosis and showed pulmonary arterial hypertension in 11 cases, with (5) or without (6) associated heart defect. One case with good tolerance remains under simple supervision; 4 were operated on from the associated lesions only and died; 7 took benefit of a simple surgical removal of the branches feeding the sequestrum, with one death and 6 successes. The poor prognostic significance of the sequestrum is thus indirectly demonstrated and the value of its devascularization is emphasized.

Cardiac Catheterization↗
PubMed1986

[Arrhythmia after atrial correction of transposition of the great vessels. Holter recording study of 123 surgically treated patients].

Sinus node dysfunction after intra-atrial repair of transposition of the great arteries by a Mustard or Senning procedure is well known. We undertook this study to evaluate the frequency, the nature, the severity and evolution of these dysrhythmias by performing Holter monitoring in 123 children followed up over 5 years; 302 Holter recordings were reviewed. The patients were divided into 3 groups of increasing severity: I = no sinus node dysfunction, II = moderate sinus node dysfunction, III = severe sinus node dysfunction with bradycardia of less than 30/min and/or pauses of over 2000 ms. The association of atrial hyperexcitability was classified in 3 subgroups: A = no extrasystoles, B = at least 4 extrasystoles per 24 hours, C = atrial tachycardia (focal tachycardia or flutter) after the first six postoperative months. There were only 15% of normal recordings (IA) and the majority of children (58%) were classified in the intermediary groups (IB, IIA and IIB). Sinus node dysfunction tended to become more severe with time in nearly 30% of the 69 cases followed up sequentially. The bradycardia tended to become more severe and associated with episodes of atrial tachycardia: the frequency of type B and C increased to 30% in Group I, to 68% in Group II and to 91% in Group III. The attacks were severe, especially in patients with a mediocre postoperative haemodynamic result. This explains the global mortality of 3%, the morbidity of 15% and the pacemaker implantation rate of 12%.(ABSTRACT TRUNCATED AT 250 WORDS)

Arrhythmias, Cardiac↗
PubMed1986

[Results of anatomical correction of simple transposition of the great vessels in 46 newborn infants].

Between the 4th April 1984 and 6th December 1984, a strategy of anatomical correction of transposition of the great arteries was undertaken in the newborn if three criteria were respected: parental consent, patient's age of less than 15 days and satisfactory left ventricular geometry. We report our results in 47 consecutive cases operated by the same surgeon at an average age of 8.2 +/- 5.3 days. Atrioseptostomy was performed in all cases before surgery, with a ratio of left to right systolic ventricular pressures greater than 0.66 (m = 0.8) and an infusion of prostaglandin E1 was maintained until surgery in 40 cases. The geometry of the left ventricle assessed by echocardiography was considered to be normal in 23 cases, satisfactory in 22 cases and unfavourable in 2 cases. Except for one case with the great vessels situated in the frontal plane in which an atrial correction was decided upon during surgery, all the patients underwent detransposition under cardiopulmonary bypass without cardiac arrest, irrespective of the coronary anatomy. The pulmonary tract was reconstructed by direct anastomosis with a piece of tanned (first 25 cases) or autologous (following 21 cases) pericardium. There were 18 immediate deaths (17%) but only 4 out of 37 after September 1984 (11%); the causes of failure were: 5 technical errors, 1 irreducible postoperative pulmonary hypertension and 2 probable left ventricular failures. The 38 survivors were followed up for an average of 6.8 +/- 5.4 months (range 1 to 21 months; 28 cases were followed up for over 3 months).(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta↗
PubMed1986

[Evaluation of the systolic pressure of the right ventricle by echocardiographic study of left ventricular geometry].

Right ventricular systolic pressure is an important parameter in paediatric cardiology. A critical haemodynamic situation is attained if it exceeds the systemic pressure: strain proximal to severe pulmonary stenosis or the result of pulmonary hypertension. In these circumstances, the patient must be closely followed up at the least, and appropriate treatment, often surgical, has to be instituted. Ambulatory methods of assessing this parameter are either unreliable, like the surface electrocardiogram, or relatively complex, like certain ultrasonic methods. We therefore looked for a method relating the systolic pressures of the two ventricles and the end systolic geometry of the left ventricle as assessed simply from a short axis view of the left ventricle at the level of the junction between the chordae and papillary muscles. Two orthogonal diameters are measured and the ratio of the two dimensions calculated: this ratio which we call the "septal curvature" reflects left ventricular compression by the pressure that the right ventricle exerts through the interventricular septum. Seventy-two children aged 6 hours to 18 years (average 5.2 years) hospitalised for pre or post-operative investigation of congenital heart disease were evaluated by this method. The results were compared by statistical analysis with the ratio of ventricular pressures measured directly almost simultaneously during cardiac catheterisation. In general, a linear relation was observed between septal curvature (x) and ratio of pressures (y); y = 0.88, x - 0.63 and R = 0.92.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1984

[Surgical treatment of complete atrioventricular canal in early infancy].

Ninety children with complete atrioventricular canal without any significant associated abnormality underwent surgery between 1973 and 1982. The first group of 26 infants, including 7 with trisomy 21 (average age 5.3 +/- 3.8 months) had severe pulmonary hypertension and underwent pulmonary artery banding: 10 died in the immediate or early postoperative period (38 p. 100). The mortality was 50 p. 100 in children under 6 months of age. In two cases, the banding was a failure because it was ineffective or "vicious" and both patients had to undergo reoperation 2 to 6 months later for total correction which resulted in 1 death and 1 poor result; the surviving 14 patients were improved: 3 are waiting for total correction under good conditions and 11 underwent open heart surgery 36 +/- 16 months after banding with 7 good results and 4 average results because of residual significant mitral regurgitation associated in one case with atrioventricular block requiring pacing. The overall results of this group were 42 p. 100 mortality, 19 p. 100 average results and 27 p. 100 good results; 12 p. 100 are waiting for surgery. The second group included 64 children, with 34 with trisomy 21 (average age 17.4 +/- 16.7 months) who underwent complete correction straight away (before 2 years in 50 cases, before 1 year in 30 cases). The operative mortality was 28 p. 100, but only 7 p. 100 in the 15 cases in which pulmonary atrial pressure did not exceed 2/3 of the systemic pressure and 35 p. 100 in the other 49 cases with severe pulmonary hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗
PubMed1984

[Severe tracheo-bronchial compression caused by a circumflex aorta. Apropos of 3 cases treated by aortic uncrossing].

The cases of three children with an encircling right aortic arch and left sided descending aorta illustrate the fact that this rare abnormality may cause severe compression of the tracheo-bronchial axis. A ventricular septal defect was associated to the abnormal vascular arch in all three cases. The compressive structure was the aorta and these children could only be extubated after surgical uncrossing . Before diagnosing tracheomalacia in a child operated for an abnormal vascular arch, residual compression must be excluded by tracheo-bronchial fibroscopy.

Aorta, Thoracic↗