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Biomedical subjects

D Sidi

Publications and source records attributed to D Sidi.

At least 37 records · Page 2Linked to original sources
PubMed2002

Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia.

BACKGROUND: Friedreich's ataxia encodes a protein of unknown function, frataxin. The loss of frataxin is caused by a large GAA trinucleotide expansion in the first intron of the gene, resulting in deficiency of a Krebs cycle enzyme, aconitase, and of three mitochondrial respiratory chain complexes (I-III). This causes oxidative stress. Idebenone, a short chain quinone acting as an antioxidant, has been shown to protect heart muscle against oxidative stress in some patients. OBJECTIVE: To assess the efficiency of idebenone on cardiac hypertrophy in Friedreich's ataxia. DESIGN: Prospective, open trial. SETTING: Tertiary care centre. METHODS: Idebenone (5 mg/kg/day) was given orally to 38 patients with Friedreich's ataxia aged 4-22 years (20 males, 18 females). Cardiac ultrasound indices were recorded before and after idebenone treatment. RESULTS: After six months, cardiac ultrasound indicated a reduction in left ventricular mass of more than 20% in about half the patients (p < 0.001). The shortening fraction was initially reduced in six of the 38 patients (by between 11-26%) and it improved in five of these. In one patient, the shortening fraction only responded to 10 mg/kg/day of idebenone. No correlation was found between responsiveness to idebenone and age, sex, initial ultrasound indices, or the number of GAA repeats in the frataxin gene. CONCLUSIONS: Idebenone is effective at controlling cardiac hypertrophy in Friedreich's ataxia. As the drug has no serious side effects, there is a good case for giving it continuously in a dose of 5-10 mg/kg/day in patients with Friedreich's ataxia at the onset of hypertrophic cardiomyopathy.

Adolescent↗
PubMed2002

[3D-transthoracic echocardiography: a selection method prior to percutaneous closure of atrial septal defects].

Prior to putting in place a percutaneous device, the assessment of the atrial septal defect anatomy is mandatory. The 3D transthoracic echocardiography is a non-invasive method bringing an imaging of the septal defect surface and its borders. Fifty-two patients ageing from 3 to 16 years old had a rotational 3D transthoracic echocardiography. Thirty-six (69%) were selected for a percutaneous closure (borders > 7 mm and a septal surface/atrial septal defect ratio > 2). Thirty-two of these selected patients (89%) benefited from the Amplatz prosthesis implantation with success. The maximal 3D diameter of the septal defect was 20 + 4 mm (14-30) compared to the mean size of prosthesis at 22 mm (18-30). Four of the 36 patients (aneurismal septum or a borderline septal surface/atrial septal defect ratio) were secondarily oriented to surgeons. Sixteen patients (31%) were selected directly to a surgical closure. The lack of borders or septal surface were confirmed by the surgical view. The 3D transthoracic echocardiography allows to define new criteria for the selection of patients prior to a percutaneous closure of atrial septal defects. This prospective study demonstrates that 9 out of 10 patients who had the 3D criteria had a percutaneous closure of the septal defect.

Adolescent↗
PubMed2002

[Marfan syndrome in the newborn and infants less than 4 months: a series of 9 patients].

We report a series of 9 children with neonatal Marfan syndrome. The diagnosis was made on a striking facial dysmorphia associated with arachnodactyly type skeletal anomalies. They all had cardiovascular anomalies: aortic dilatation 9 times, and mitral and/or tricuspid valve prolapse 8 times. Three children underwent ascending aorta replacement with the Yacoub technique. There was one operative death in a young infant and the two survivors required further surgery for gross mitral insufficiency and an aneurysm of the horizontal aorta. Three children died before the age of 1 year from cardiac insufficiency linked to massive leaks of all the valves. The three youngest children in the series did not undergo any procedure and were followed up for a slowly growing dilatation of the initial aorta. The prognosis of neonatal Marfan syndrome is weighted with a heavy mortality. The timing of surgical intervention in case of excessive dilatation of the aortic root is difficult to determine. It depends not only on the aortic diameter but also on the aortic valve function and the existence of other mitral or tricuspid lesions. Surgical treatment for lesions of the initial aorta and the atrioventricular valves improves the short-term prognosis but the risk of re-intervention is higher.

Aortic Diseases↗
PubMed2002

[Percutaneous closure of a paravalvular mitral regurgitation with Amplatzer and coil prostheses].

The authors report a case of percutaneous closure of paravalvular mitral regurgitation in a 9 year old child. This patient had complete atrioventricular canal with levo-isomerism completely repaired with replacement of the left atrioventricular valve by a mechanical prosthesis. One year after an episode of infectious endocarditis, the patient was readmitted because of jaundice due to haemolysis attributed to paravalvular mitral regurgitation. Transoesophageal echocardiography demonstrated a crescent-shaped dehiscence of the prosthesis opposite the left atrial appendage associated with a small paraseptal regurgitation. First of all, a 6 mm Amplatzer Septal Occluder and a 5 x 5 coil were introduced by retrograde catheterisation to treat the regurgitation due to the valve dehiscence. This procedure was completed 3 months later by the implantation of a second 8 mm Amplatzer prosthesis for persistent haemolysis. Closure of the paraseptal regurgitation resulted in a reduction of the patient's transfusional requirements.

Child↗
PubMed2002

[Percutaneous closure of an interatrial communication via the internal jugular route using an Amplatzer prosthesis].

Closure of interatrial communications by interventional catheterisation is recommended and effective. The usual approach is via the femoral vein. However, difficulty in positioning the probe and the guide in a stable fashion across the septal defect occurred during closure of the interatrial communication by the percutaneous route in a 71 year old female with severe scoliosis. This deformity most certainly contributed to the failure of the femoral approach. The decision for closure by the internal jugular route was taken and allowed closure of the interatrial communication to be achieved. The internal jugular route can be an alternative for closure of interatrial communications with difficult access.

Aged↗
PubMed2002

[Followup of adults with a Fontan-type cavopulmonary derivation].

The Fontan procedure is a technique used in single ventricle syndromes and initiates a circulation which bypasses the right ventricle. It was described by Fontan in 1971. Many variations of this operation have been described and classified under the term of total cavopulmonary derivation. These operations result on long-term functional benefit. However, the 15 year survival rate is only 55 to 80%. This procedure (often carried out in childhood) is considered to be palliative rather than curative. The mortality is due to long-term complications, the frequency of which increases with the length of follow-up of the patient. The causes are: the Fontan circulation itself (arrhythmias, thrombosis, inferior caval stasis (hepatic, exsudative enteropathy), recurrence of cyanosis): the underlying cardiac disease (ventricular failure, atrioventricular valvular regurgitation); other complications (endocarditis and extracardiac, in particular orthopedic problems). Any of these complications may be an indication for cardiac transplantation, a high risk procedure because of previous multiple surgical interventions.

Adult↗
PubMed2002

[Geographic origin of endomyocardial fibrosis treated at the central hospital of Maputo (Mozambique) between 1987 and 1999].

Endomyocardial Fibrosis (EMF) is a restrictive myocardiopathy of unknown aetiology, which is specific to tropical areas. In order to document the geographical distribution of this disease in Southern Mozambique, we reviewed the medical records of patients treated at Maputo Central Hospital between 1987 and 1999. Analysis was restricted to cases originating from the 3 provinces of Southern Mozambique. Cumulative incidences were computed for the period of 1987-1999, by age group, sex and residential district at disease onset. The geographical distribution of the disease in the 34 districts (administrative subdivisions) of the study area was mapped. Between 1987 and 1999, 118 cases of EMF originating from Maputo, Gaza and Inhambane provinces were diagnosed at Maputo Central Hospital. Seventy seven patients (65%) were living in Inhambane Province at the time of disease onset. Cumulative incidence for this province was 6.9/100,000 over the study period of 1987-1999. Comparatively, cumulative incidences were respectively 1.7/100,000 for Maputo and 1.0/100,000 for Gaza Province (chi 2 = 64.3, p < 10(-5)). All the patients were native from 22 out of the 34 districts of these 3 provinces. Affected districts were all located in the coastal area and higher cumulative incidence was computed for Inharrime district (18.3/100,000). This study confirmed that endomyocardial fibrosis is endemic in some areas of South Mozambique, but did not allow to measure the true magnitude of the disease which is probably grossly underestimated. The geographical distribution of EMF in South Mozambique reinforce the environmental hypothesis of aetiologic disease.

Adolescent↗
PubMed2001

Presence of increased stiffness of the common carotid artery and endothelial dysfunction in severely obese children: a prospective study.

BACKGROUND: Epidemiological studies suggest that obesity-induced atherosclerosis may start in childhood, but this process has never been demonstrated. We looked for arterial changes and investigated their relation to cardiovascular risk factors in obese children. METHODS: Non-invasive ultrasonographic measurements were made in 48 severely obese children and 27 controls to investigate arterial mechanics and endothelial function. Plasma lipid concentrations, indices of insulin resistance, and body composition were assessed in the obese children. FINDINGS: The obese children had significantly lower arterial compliance than the healthy controls (median 0.132 [0.022-0.273] vs 0.143 [0.112-0.237] mm(2).mm Hg; p=0.02) and lower distensibility (0.60 [0.10-1.00] vs 0.70 [0.50-1.10] mm Hg(-1).10(-2); p=0.0001). Conversely, the obese children had higher values than the controls for wall stress (3.36 [2.00-5.01] vs 2.65 [2.13-3.54] mm Hg.10(2); p=0.0001) and incremental elastic modulus (1.68 [0.72-10.8] vs 0.96 [0.64-1.47]; p=0.0001). Endothelium-dependent and independent function were also lower in the obese than in the control children. An android fat distribution was positively correlated with indices of insulin resistance and plasma triglyceride concentrations and was negatively correlated with plasma HDL-cholesterol concentration and arterial compliance. Endothelial dysfunction was correlated with low plasma apolipoprotein A-I and with insulin resistance indices. INTERPRETATION: Severe obesity in children is associated with arterial wall stiffness and endothelial dysfunction. Low plasma apolipoprotein A-I, insulin resistance, and android fat distribution may be the main risk factors for these arterial changes, which are of considerable concern as possible early events in the genesis of atheroma.

Adolescent↗
PubMed2001

Results of the arterial switch operation in neonates with transposed great arteries.

BACKGROUND: The arterial switch operation is judged the best palliative operation for neonates with transposed great arteries. We aimed to assess the value of analysing a large series of unselected cases by this technically demanding operation and formulate a realistic prognosis. METHODS: We reviewed all 432 neonates (mean age at operation 7 days, mean weight 3.25 kg) who underwent an arterial switch operation between 1987 and 1999. Follow-up (mean time: 4.9 years) was complete in 412 patients. FINDINGS: Survival probability and freedom from reoperation was 94% and 78% at 10 years, respectively. 26 patients died, 16 because of myocardial ischaemia. Risk factors for death included early experience, low weight, associated cardiovascular malformations (especially hypoplasia of the right ventricle or aortic arch), and difficult patterns of coronary arteries. The risk of the coronary artery pattern was greatly reduced in those who had recent operations. At last follow-up, 90% of patients had normal life without treatment, and 94% a normal heart function on echocardiography. INTERPRETATION: The arterial switch operation in neonates achieves excellent results mid-term. Obstruction of the translocated coronary arteries is responsible for most deaths and a substantial number of reoperations. Although confirmation is needed, these results allow anticipation of a favourable long-term prognosis.

Disease-Free Survival↗
PubMed2001

Selective coronary angiography in patients younger than 1 year of age.

Precise assessment of coronary artery anatomy is needed in congenital coronary malformations and acquired coronary obstruction in children. The aim of the study was to describe our experience of selective coronary angiography (SCA) in newborns and infants. One hundred and three patients younger than 1 year of age underwent SCA. Indications were preoperative screening for coronary artery lesions or anomalous pattern (41 patients), signs of myocardial ischemia (43 patients), surgical difficulties during the reimplantation of the coronary arteries without signs of ischemia (15 patients), and other indications (4 patients). Success rate of selective injection was 100% with no significant complication. In the presence of clinical suspicion of myocardial ischemia, abnormalities of coronary artery pattern was found in 17 of 43 patients. Precious information for the surgical management was obtained in 13 of 41 patients. SCA can be performed safely even in newborns and small infants. Skills in catheterization and knowledge about the coronary artery anatomy are needed to perform SCA.

Coronary Angiography↗
PubMed2001

Absorbable pulmonary artery banding in tricuspid atresia.

A child with tricuspid atresia, concordant ventriculoarterial connections, large ventricular septal defect, and elevated pulmonary artery pressure underwent pulmonary artery banding with a polydioxanone ribbon. This procedure was successful in this patient as the ventricular septal defect became restrictive while the banding was fully absorbed after 5 months. This technique could be included in the panel of surgical strategies for patients with single ventricle physiology and potential but insufficient subpulmonary stenosis in early infancy.

Absorbable Implants↗
PubMed2001

A new percutaneously adjustable, thoracoscopically implantable, pulmonary artery banding: an experimental study.

BACKGROUND: In patients who undergo left ventricular retraining, multiple reoperations are often necessary to adjust the pulmonary artery banding. The availability of a percutaneously adjustable band would be very useful. METHODS: Ten lambs (10 to 25 kg) underwent pulmonary artery banding using a new device, 7 by thoracotomy and 3 by thoracoscopy. The possibility of percutaneously adjusting the band was evaluated immediately after operation in 10 animals and at 3 months in 8 animals. RESULTS: One death occurred on the day of the procedure from displacement of the device and another death was from infection. Immediate hemodynamic studies proved the feasibility of increasing right ventricular afterload in a precise and reversible way. After 3 months the band could still be precisely loosened or tightened in all but 1 animal. Autopsy revealed that all the devices were in the correct position and no fibrosis or adhesions were present around the devices, and there was no residual stenosis noted on the pulmonary artery. CONCLUSIONS: This new device may be a valuable alternative to the repeated pulmonary artery banding needed for ventricular preparation.

Animals↗
PubMed2001

Results of the Lecompte procedure in malposition of the great arteries and pulmonary obstruction.

OBJECTIVE: Assessment of the Lecompte procedure, our repair method of choice in malposition of the great arteries with pulmonary stenosis. METHODS: A retrospective analysis of 42 patients (median age at operation, 1.4 years) operated on between 1986 and 1999 for various forms of great artery malposition, ventricular septal defect, and pulmonary stenosis. Relevant associated findings included the insertion of a tricuspid papillary muscle on the conal septum (nine patients), absence of conal septum (six patients), hypoplasia of a side pulmonary artery (four patients), and hypoplasia of the right ventricle (one patient). A preliminary systemico-pulmonary shunt was created in 28 patients and a cavo-pulmonary anastomosis in one patient. At operation, the conal septum (whenever present) was resected (36 patients), the pulmonary bifurcation was usually translocated over the ascending aorta (37 patients), and the main pulmonary artery was enlarged with a patch of pericardium. A monocusp valve was fashioned within the patch in 40 patients. The follow-up information was complete in 32 patients and ranged from 0.4 to 14 years (mean, 5.4+/-3.2 years). RESULTS: The survival rate at 5 years was 92+/-5%. Three patients died post-operatively (mortality, 7%) and none during follow-up. The freedom from reoperation was 86+/-8 and 51+/-22% at 5 and 10 years, respectively. Six patients were reoperated, all for a pulmonary stenosis. Calcification of the monocusp patch was present in all. Pulmonary stenosis developed in three further patients: one underwent percutaneous dilatation and two are awaiting surgery. No procedural factors thought to have a potential correlation with the development of right ventricular outflow tract stenosis could be individualized on univariate analysis. CONCLUSIONS: The Lecompte procedure, which allows early repair of these defects, provides overall good results. The need to reoperate on the right or left ventricular outflow tract seems reduced in comparison with the Rastelli operation, the other alternative.

Analysis of Variance↗
PubMed2001

Prevalence of 22q11 deletion in fetuses with conotruncal cardiac defects: a 6-year prospective study.

OBJECTIVES: Conotruncal malformations (CTMs) are a major feature of 22q11 microdeletion (22qdel). The prevalence of 22qdel in fetuses harboring these defects is unknown. We assessed the prevalence of 22qdel in a population of fetuses with conotruncal cardiac defects. STUDY DESIGN: Consecutive fetuses (n = 261) with a CTM and a normal karyotype were included in the study. All fetuses were screened for 22qdel by means of fluorescent in situ hybridization. RESULTS: A 22qdel was found in 54 fetuses (20.7%). The proportion of 22qdel for each CTM was: tetralogy of Fallot (14/100), pulmonary atresia with ventricular septal defect (11/61), tetralogy of Fallot with absent pulmonary valves (6/16), interrupted aortic arch (10/22), truncus arteriosus (9/29), and complex transpositions of the great arteries (4/33). Additional vascular anomalies were present in 75%. Typical abnormal facial appearance at birth or at autopsy was observed in 80%, and thymus hypoplasia, in 76%. The pregnancy was terminated in 41 of 54 cases, including an intrauterine death in one case. The 22qdel was inherited in 7.7%. CONCLUSION: Prevalence of the 22qdel is high in fetuses with CTMs. The risk of mental retardation associated with the respective risk of cardiac surgery for each type of CTM may strongly influence prenatal counseling.

Abortion, Therapeutic↗