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Biomedical subjects

D Sidi

Publications and source records attributed to D Sidi.

At least 163 records · Page 9Linked to original sources

Heart transplantation in children: mid-term results and quality of life.

From 1987 to 1991, heart transplantation was undertaken in 49 infants and children with either end-stage cardiomyopathies (28 patients) or severe congenital heart disease (21 patients including 16 having already been surgically but unsuccessfully treated). Their age ranged from 13 days to 15 years (mean = 4.5 +/- 4.2 years; median = 2.5 years). There were 12 early and 7 late deaths (overall mortality = 38%), mainly due to graft dysfunction, acute or chronic rejection, and infectious complications, mostly viral. Optimal criteria in selecting both donors and recipients are crucial to reduce early mortality and should never be transgressed despite the critical shortage of organs. The actuarial probability of survival was 64% at 1 year and 57% at 5 years. Our 30 mid-term survivors (62%) were submitted to a close follow up programme which includes endomyocardial biopsies, even in the very young, since non invasive criteria failed to mark every rejection episode. Maintenance therapy was always steroid-free to start with (cyclosporin+azathioprine) but in almost one half of our oldest survivors, it failed to avoid rejection and we had to add low-dose oral steroids for at least several months. Epstein-Barr virus related lymphoproliferations occurred in four patients, two of whom died and two recovered with specific therapy. Renal function was closely monitored: tubular and interstitial lesions were found on renal biopsies and were associated with moderate functional changes. The quality of life of the children who survived heart transplantation was considered as near normal in a little more than one half of the cases but many issues (late coronary disease, drug toxicity, long-term compliance to follow up and therapy) remain significant concerns for the future.

Adolescent↗

Anomalous left coronary artery from the pulmonary artery: results of isolated aortic reimplantation.

Thirty-one consecutive children with anomalous left coronary artery underwent direct aortic reimplantation of the anomalous artery without an associated procedure. There were five deaths (16%; 70% confidence limits, 9% to 26%), three in the hospital and two early (within 3 months). The severity of preoperative left ventricular dysfunction was the only incremental risk factor for mortality: 31% mortality rate among patients with left ventricular shortening fraction of less than 0.20 versus 0% among patients with a left ventricular shortening fraction of 0.20 or more (p = 0.03). There were no late deaths up to 6 years, a survival rate of 84% +/- 7%. Late results were studied in 23 survivors having a follow-up of longer than 12 months. Ninety-six percent were free of symptoms; left ventricular function recovered to normal in all patients; moderate to severe mitral regurgitation decreased to minimal or no regurgitation in most patients (5/7); and the reimplanted anomalous left coronary artery was patent in each patient. Based on this study, we reached five conclusions. (1) Direct aortic reimplantation is technically feasible in most patients with anomalous left coronary artery and yields a high rate of late patency. (2) Left ventricular resection is unnecessary. (3) The mitral valve should not be interfered with at the initial operation, but mitral regurgitation may persist in a few patients and necessitate later operation. (4) In patients with moderate left ventricular dysfunction, the operative risk is low and early operation indicated. (5) In patients with severe left ventricular dysfunction, the operative risk is high; heart transplantation may be suggested, but our current approach favors an immediate corrective procedure.

Actuarial Analysis↗

[Enzymatic activities of the mitochondrial respiratory chain in child cardiomyopathies. 34 cases prospectively studied by endomyocardial biopsy].

The arguments in favour of mitochondrial pathology of certain childhood cardiomyopathies (multi tissue involvement, lactic acidosis, histochemical abnormalities of skeletal muscle) are indirect and may be absent in isolated cardiomyopathy. The authors therefore set up a prospective study of enzyme activity of the mitochondrial respiratory chain directly by endomyocardial biopsy. Fifty children aged 2 months to 16 years were included. Thirty four had cardiomyopathy which was dilated and hypokinetic with thin walls in 21 cases, restrictive in 2 cases, and hypertrophic in 11 cases; the remaining 16 children had either normal hearts (13 catheterised for other reasons) or myocardial hypertrophy due to pulmonary or aortic stenosis (3 cases). Both ventricles were evaluated in 3 cases; macro-surgical biopsies were obtained in 6 cases and skeletal muscle biopsy was obtained in 9 cases. The results indicate the method to be reliable with no difference between the micro and macro biopsies. The absolute values of enzyme activity were too variable to serve as quantitative parameters but some ratios of activity were remarkably stable and allowed a qualitative assessment which was all the more accurate when identical values were obtained in the myocardium, skeletal muscle and liver. The mitochondrial respiration was independent of ventricular pressures and of the type (right or left) of ventricle. Enzyme activity was nearly always normal in dilated cardiomyopathy (20/21) which suggests that it was unaffected by dilatation of the heart and by abnormal myocardial contractility. The results could be normal in myocardial hypertrophy and in valvular stenosis and in over half the cases of hypertrophic cardiomyopathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Intensifying iron chelating therapy with desferrioxamine using implantable venous access catheters (Port-A-Cath)].

Deferoxamine is still today the only preventive and curative treatment of transfusional hemochromatosis. It must be perfused daily, intravenously or subcutaneously, during several hours. Implantable infusion devices (Port-A-Cath) offer intravenous access, allowing to use higher doses, while avoiding local swelling due to subcutaneous injections. This device was inserted in 7 major thalassemic patients who presented with severe complications of iron overload, including 4 of them with signs of cardiac failure. Ferritinemias of all patients were lowered after intensifying iron chelation: cardiac function improved drastically in 2 patients. Devices were responsible for some complications: occlusion in one patient, local infections in two. This way of administration of desferal seems useful in patients with high ferritinemia and/or organic complications related to hemochromatosis.

Acute-Phase Reaction↗

Heart transplantation in children: risk factors of early and late mortality.

In order to identify predictive risk factors of poor outcome following heart transplantation in children, we performed a retrospective analysis of our pediatric recipient population: 31 children, aged 15 days to 15 years (mean = 5.2 +/- 4.9 years). The preoperative diagnosis was cardiomyopathy in 17 (55%), congenital heart disease in 13 (42%) and end-stage valvular disease in 1 (3%). There were 5 operative deaths: hyperacute rejection (2), low cardiac output syndrome (3); 4 in-hospital deaths: infection (2), multiorgan failure (2) and 4 late deaths: acute rejection (1), chronic rejection (1), lymphoma (1), unknown (1). The actuarial probability of survival (+/- SE) was 62% +/- 10% at 1 year and 53% +/- 12% at 2 years. Univariate analysis was used to evaluate the following risk factors: age, diagnosis, hemodynamic decompensation, previous cardiac surgery, ischemic time of the graft, technique of graft preservation, preoperative pulmonary artery pressure, occurrence of postoperative low cardiac output syndrome (LCOS) with pulmonary hypertension (PHT). The occurrence of early LCOS with PHT significantly increased both early and late mortality (78% early mortality, 100% overall mortality). This syndrome occurred in 9 patients (29%) and was attributed to primary graft failure in 2, increased pulmonary vascular resistances in 6 and multiple factors in 1. Although not significant, two factors may increase early survival: young age (less than or equal to 1 year) at operation and improved technique of graft preservation.

Adolescent↗

[Scoliosis and congenital heart diseases in children. Apropos of 44 cases].

In order to evaluate the difficulties of treatment of associated scoliosis and congenital heart disease (C.H.D.), 44 patients who have been treated between 1970 and 1988 were reviewed. The scoliosis was idiopathic in 30 cases, congenital in 11, neurologic in 3. There were 27 females and 17 males. Twenty one patients had a cyanotic C.H.D. (twelve tetralogies of Fallot). No relation was found between the side of the cardiac approach and the side of the thoracic curves. Three cases of right aortic arch with two left thoracic scolioses were noted. Associated anomalies were encountered in 60 p. 100 of cases. The analysis of old chest X-rays, revealed that the scoliosis was already present an average of 5 years before the first orthopaedic consultation. Eight patients had an orthopaedic treatment; in ten cases surgery was decided but not performed. Twenty six patients were operated. (22 posterior and six anterior procedures). The average angulation was 55 degrees pre-operatively, 30 degrees post-operatively and 40 degrees at a 40 months follow-up. In five cases a pseudarthrosis led to re-intervention. One patient died during a posterior procedure, an other post-operatively. Two patients had a severe complication (one heart arrest; one gaz-embolism) but recovered. The surgical prognosis can be evaluated on an original scale including different cardiac parameters, and the importance of the planned surgical procedure. Under a limited mark, no serious complications occurred. In conclusion, a closed collaboration between pediatricians and the orthopaedic team, should lead to more precocious screening of scoliosis in cardiologic children and before the orthopaedic procedure, to a more precise risk factors evaluation.

Child↗

[Right pulmonary artery arising from the proximal ascending aorta. A model of reflex pulmonary hypertension of the left lung?].

The right pulmonary artery arising from the proximal ascending aorta is a rare and severe malformation. This retrospective study of 11 children with this condition was undertaken to determine the conditions of diagnosis, to analyse the results of surgery, and, above all, to clarify the mechanism of the left pulmonary arterial hypertension which was always present. Ten of these patients were 4 to 90 days old. All had severe congestive cardiac failure with iso- or suprasystemic left pulmonary arterial hypertension. The only associated lesions were ventricular septal defect (1 case) and patent ductus arteriosus (7 cases). None of the patients had significant left-to-right shunts and only one had left atrial hypertension: this patient died before surgery could be performed. The other 9 patients underwent surgical correction and the pulmonary pressures immediately fell to normal or almost normal values. The child with the ventricular septal defect died of infection 6 weeks after surgery. The 8 survivors are doing well 1 month to 12 years later and left pulmonary pressures are normal in all, including those (5 cases) with a stenosed (4 cases) or completely occluded right pulmonary arterial circulation (1 case) and in 1 patient with obstructive vascular disease. The eleventh patient was very different: she had no signs or symptoms until 2 years of age, when a right pulmonary obstructive arterial disease but with normal left pulmonary pressures was documented. She was not operated on and remains well nine years later.(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta↗

Anatomic correction of transposition of the great arteries with ventricular septal defect. Experience with 118 cases.

One hundred eighteen patients, 100 with transposition of the great arteries plus ventricular septal defect and 18 with double-outlet right ventricle and subpulmonary ventricular septal defect have undergone arterial switch and patch closure of the ventricular septal defect since February 1983. In transposition of the great arteries the ventricular septal defect was perimembranous in 70 cases, trabecular in 28, and infundibular in 10. Eleven patients had multiple ventricular septal defects. In addition to 18 patients with double-outlet right ventricle, malalignment of the conal septum was present in 19 cases. Coronary type A distribution was recognized in 79 cases, type C in one, type D in 24, and type E in 14. Great arteries were side by side in 19% of cases. Aortic coarctation was present in 31 cases, and subaortic obstruction in 9. Age at operation ranged from 4 days to 4 years (mean, 3.5 +/- 8.3 months), and mean weight was 4.0 +/- 1.6 kg. Thirty-seven infants were younger than age 1 month. Thirty-six patients underwent previous operations: pulmonary artery banding alone (n = 13), pulmonary artery banding and coarctation repair (n = 13), and coarctation repair alone (n = 10). Mean time between the first procedure and the switch was 2.2 months. Six patients with aortic coarctation underwent one-stage repair, through median sternotomy, aortic reconstruction, closure of the ventricular septal defect, and arterial switch. Perioperative mortality was 13.5% (70% confidence limit 10% to 17.6%, n = 16). It was directly related to coronary artery kinking in 50% of deaths and to anatomy and size discrepancy of the great arteries in the remaining deaths. Univariate analysis could not find any significant risk factor of in-hospital mortality. Mean follow-up of 30.3 +/- 23.5 months was achieved in all but 2 survivors. There was one late death. Ten patients underwent 11 reoperations for recoarctation (n = 1), pulmonary stenosis (n = 7), residual ventricular septal defect (n = 2), and stenosis of superior vena cava (n = 1). Two patients needed a permanent pacemaker. Actuarial survival and freedom from reoperation at 5 years were 84.5% +/- 3.6% and 85.7% +/- 4.6%, respectively. We conclude that anatomic correction of complex transposition is a safe method that offers good early and midterm results.

Child, Preschool↗

[Double discordance with ventricular septal defect and pulmonary artery hypertension. A study of 21 cases].

This paper reports a retrospective study of 21 children with atrioventricular and ventriculo-arterial discordance, or double discordance, associated with a large ventricular septal defect responsible for pulmonary hypertension. Other associated congenital defects were: atrioventricular block (5 cases), coarctation of the aorta with neonatal cardiac failure (6 cases), tricuspid valve malformations responsible for significant tricuspid regurgitation (11 cases) and right ventricular hypoplasia (1 case). Two children died before any therapeutic intervention, one from syncope related to atrioventricular block and the other after a decision of therapeutic abstention. Three children underwent total correction with one good result (the only case of situs inversus), one late death and one lost to follow-up. The majority of patients (n = 16) underwent initial palliative surgery consisting in pulmonary artery banding occasionally associated with reconstruction of the aortic arch: there was no early mortality but there were 2 late deaths. Of the 14 survivors, 6 are well after a mean follow-up period of 31 months. Eight underwent open heart surgery with 1 operative death, 6 post-operative complete atrioventricular blocks requiring cardiac pacing and 5 poor results due to aggravation or secondary tricuspid regurgitation leading to 1 cardiac transplantation (death) and 2 reoperations for valvular surgery (1 plasty and 1 tricuspid valve replacement). The overall results of this series are poor: high mortality (33 per cent) and equally high morbidity when direct surgery is undertaken. Two major complications are observed: complete atrioventricular block (55 per cent) and regurgitation of the systemic atrioventricular valve (45 per cent), both of which often necessitate invalidating complementary procedures.(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Preschool↗

[Stenosis of the pulmonary veins in total anomalous pulmonary venous drainage. Associated or iatrogenic abnormality?].

Obstruction of total anomalous pulmonary venous drainage (TAPVD) is sometimes observed in neonates, usually at the site where the collector drains into the caval system. Stenosis of the pulmonary veins themselves before joining the collector is less common. This was observed in 6 cases of intra- and supracardiac TAPVD; the prognosis is usually very bad due to postoperative pulmonary hypertension (PHT) which is difficult to treat. Three of our cases had TAPVD into the coronary sinus with obstruction presenting at birth. Conventional surgery did not reduce the PHT of the first 2 children because the congenital stenosis of the pulmonary veins was not corrected. In the first case, the pulmonary veins resembled fibrous cords and in the second case, the collector was stenosed at its junction with the coronary sinus. These two children died despite surgery. The third child, however, was cured because the obstruction was diagnosed preoperatively and successfully treated when the TAPVD was corrected. In the other three cases, TAPVD to the superior vena caval system was not obstructed but the pulmonary veins retracted progressively after surgery causing PHT and right ventricular failure. After unsuccessful percutaneous dilatation, reoperation revealed obstruction due to exuberant scar tissue with retraction at the site of anastomosis and (or) reconstruction of the pulmonary veins by incision or enlargement with a pericardial patch. One of these children survived after pneumonectomy of the obstructed lung which relieved reflex PHT of the contralateral lung.(ABSTRACT TRUNCATED AT 250 WORDS)

Angiocardiography↗

[Incessant idiopathic ventricular tachycardia in infants].

Reduction of the arrhythmia was obtained in 1 to 5 days, spontaneously (2 cases) of after oral amiodarone (500 to 2000 mg/m2/24 hr) (5 cases) or intravenous flecainide or propafenone in the two most critically ill patients. With the exception of 1 patient who was successfully treated with propranolol for 1 year, amiodarone was prescribed in all cases for 7 to 36 months after reversion to sinus rhythm: 5 children have been taken off treatment and seem to be completely cured 4 to 9 years after withdrawal; the other 3 patients have not had recurrences but have only been treated for 6 to 15 months. One of them has neurological sequellae from a cerebral embolism originating from an atrial thrombus. These results show that incessant ventricular tachycardia in infancy is a serious condition but amenable to medical treatment with high dose amiodarone or, when poorly tolerated, intravenous Class 1c antiarrhythmics for reduction of the arrhythmia, and amiodarone at adequate doses for 2 years to prevent recurrence. With the exception of the rare arrhythmogenic right ventricular dysplasia, the long term prognosis would seem to be excellent and, in general, there would seem to be little need for surgery.

Amiodarone↗

[Severe thrombocytopenia induced by heparin in an infant with acute myocarditis].

A severe heparin-induced thrombocytopenia is reported in a child suffering from acute myocarditis. Thrombocytopenia (80.10(9)/l) occurred on day 5 (D5) of heparin therapy which was thus discontinued during 8 hours but reintroduced on the view of a negative platelet aggregation test using standard heparin. On D10, while thrombocytopenia persisted the presence of two intra-cardiac thrombi led to replace standard heparin by low molecular weight heparin (Fraxiparin), associated with vitamin K antagonist (phenindione). On D12, a 3rd intra-cardiac thrombus required immediate discontinuation of Fraxiparin therapy. Platelet aggregation tests performed on D14 were positive in the presence of standard heparin and of low molecular weight heparin, thus demonstrating the existence of thrombocytopenia induced by standard heparin and secondly by low molecular weight heparin. This observation led the authors to discuss the diagnosis and the therapeutic management of heparin-induced thrombocytopenia.

Acute Disease↗

Percutaneous balloon valvuloplasty in neonates with critical aortic stenosis.

Percutaneous balloon valvuloplasty was attempted in 10 newborn infants with critical aortic valve stenosis and severe congestive heart failure. Three had a very small left ventricle and aortic anulus. In one infant, the aortic valve could not be passed, and in another infant, a technical error resulted in severe valvular damage, aortic insufficiency and death. Among the eight patients who had effective dilation, the stenosis was relieved in seven as assessed by a significant decrease in transvalvular pressure gradient, improvement of left ventricular contraction and eventual inversion of the ductal shunting. The procedure failed in the only patient whose dilation was performed with an undersized balloon. Aortic insufficiency occurred in three infants and was severe (perforated cusp) in one, moderate in one whose valve was dilated with an excessively large balloon and mild and transient in one. None of the three infants with a very small left ventricle recovered (two died and one underwent cardiac transplantation). Among the seven infants with a left ventricle of acceptable size, three underwent subsequent aortic valvotomy; one of these died and two bad good results. The remaining four are doing well 16 +/- 5 months later (mean +/- SD) with mild to moderate residual aortic stenosis and normal left ventricular function. In conclusion, percutaneous balloon valvuloplasty is an acceptable alternative to surgery in neonates with critical aortic valve stenosis. Incidence of complications and good relief of the obstruction depend on a careful technique. Immediate results are similar to those of surgery. Late prognosis depends on the quality of the left heart structures.

Aortic Valve Insufficiency↗

Heart- and heart-lung transplantation in children.

Since January 1987, 16 prepubertal children have undergone heart (13) or heart-lung (3) transplantation. Immunosuppression included cyclosporine and azathioprine and excluded steroids except in case of rejection. The indications for heart transplantation were hypoplastic left heart syndrome (4 infants, mean age = 2 months), congenital heart disease (4 patients, mean age = 5.7 years) and cardiomyopathy (5 patients, mean age = 2.8 years). There were 4 early deaths (acute graft failure in 2, pulmonary hypertension in 1, infection in 1) and 1 late death (heart failure at 3 months). The 8 survivors had a mean follow-up of 12 months (range 1-19 months). Late complications were minimal. There were 4 episodes of rejection in 2 patients. There was no infection, normal somatic growth and no systemic hypertension. Renal function remained within normal limits although mild-to-moderate tubulointerstitial lesions were found in 4 renal biopsies. Three children (9-11 years old) underwent heart-lung transplantation. The early postoperative course was difficult with 6 episodes of rejection and 5 infections. One patient died at 3 months from infectious complications. One child has a complete rehabilitation 8 months posttransplantation. The last patient is clinically well at 7 months but has a residual tracheal stenosis. The long-term fate of these children, and particularly the long-term effects of cyclosporine therapy are unknown. Heart and heart-lung transplantation remain under investigation but may be reasonable approaches for infants and children with end-stage cardiac and/or pulmonary disease.

Acute Kidney Injury↗

[Artificial cardiac stimulation in the newborn infant with complete congenital atrioventricular block. Study of 16 cases].

This is a retrospective study of 16 children with congenital complete atrioventricular block (CAVB) who were fitted with a pacemaker in infancy. All were neonates admitted at the age of 1 to 9 days for bradycardia; 3 had a cardiopathy. In 8 children a permanent pacemaker was implanted in the first two days of life on account of a heart rate slower than 50 beats/min, accompanied with threatening symptoms (heart failure or syncopes) in 4 cases. In 6 children the pacemaker was implanted at the age of 2 to 3 months; in spite of reassuring electrocardiograms, 5 of them were readmitted in an emergency for heart failure or syncope with slow heart rate; the 6th patient had disorders of ventricular excitability. Finally, 2 asymptomatic infants underwent pacing: one at 20 days for bundle branch block, the other at 6 months for slow phase abnormalities. Pacing was epicardial in all patients, the chamber being positioned in the space that separates the kidney from the parietal peritoneum. Ventricular synchronous pacing (VVI) was applied in 14 cases and atrioventricular pacing (DDD) in the two most recent cases. Two children died post-operatively, due to inadequate attachment of the electrode resulting in loss of ventricular capture in one case, and to extensive left atrial thrombosis in the other case. Two children died at a later stage of severe respiratory pathology. The 12 survivors were followed up for a mean period of 3.7 +/- 3.1 years. Three pacemakers were replaced: one at 28 months for infection, the others at 3 and 6 years respectively for running down of the batteries.2 +

Cardiac Pacing, Artificial↗

[Aptitude for sports in children operated on for aortic coarctation. Contribution of the exercise test].

Pronouncing on the fitness of children operated upon for coarctation of the aorta to practise sports, as well as on the nature and level of this activity, depends, to a great extent, upon their blood pressure profile at exercise (BPE) as measured by an exercise test on an ergometric bicycle. We studied thi profile in 43 children aged 11.7 +/- 2.5 years who had undergone surgery for coarctation of the aorta (isolated in 93 p. 100 of the cases) at the age of 3.8 +/- 3.2 years Systolic arterial pressure (SAP) in the upper limbs was normal at rest in 80p. 100 of the children but reached abnormal values at exercise in 81 p. 100 and even exceeded 200 mmHg in 16 p. 100. Diastolic arterial pressure (DAP) in the upper limbs was sometimes slightly elevated at rest, but it clearly decreased at exercise and became normal in all cases. This suggested that systolic hypertension at exercise was due to low peripheral resistance and either to an exercise-induced isthmic gradient or to a poor vascular impedance. As a result of this study, we warn such children against playing sports with a strong static stress (e.g. martial sports) or with a risk of thoracic trauma (e.g. rugby). For the other sports, we make no reservation when the BPE is normal, and we forbid competitions when the BPE is abnormal but the SAP at exercise remains below 200 mmHg. Beyond this value, children must be investigated for residual coarctation of the aorta which may be amenable to a specific treatment.

Adolescent↗

[Severe respiratory syncytial virus infections. Study of 87 infants hospitalized in an epidemic].

During a winter epidemic, 87 infants were admitted to Necker-Enfants-Malades hospital with a severe respiratory syncitial virus (RSV) infection. These infants fell into two groups: 37 infants without any medical history and 50 showing an underlying pathology (immune deficiencies, heart disease, CNS disorders, digestive malformations, allergic manifestations). Of the 37 infants with no medical history, most were below the age of 6 months and the RSV infection was manifested clinically by bronchiolitis or bronchitis. Most of the infants in the other group were more than 6 months of age and presented mostly with pneumonia or bronchiolitis. A respiratory distress syndrome was observed in 17 of the 87 infants, and virtually all of them were younger than 6 months. No significant difference was observed between the two groups with regards to the incidence of respiratory distress. Pulmonary infections complicating the course of the illness, most often due to commensal flora bacteria of the upper respiratory tract, were observed in 19 infants but with no greater frequency in the group at risk. Direct detection of viral antigens in nasopharyngal secretions not only enabled rapid diagnosis in all the infants but also allowed antiviral therapy to be started rapidly. Antiviral treatment by ribavirine, administered over a period of 5 days in 20 mg/ml doses by aerosol was instituted in 10 patients whose course might have become serious. In 8 of these patients, disappearance of the virus from secretions and recovery occurred. Two patients in the group at risk died despite treatment, with one case being considered a true therapeutical failure since the virus was still present in nasopharyngeal secretions.(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Hospitalized↗