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Biomedical subjects

E Villain

Publications and source records attributed to E Villain.

At least 37 records · Page 2Linked to original sources
PubMed2001

Quality of life and perceived health status in surviving adults with univentricular heart.

OBJECTIVE: To evaluate the quality of life in patients with univentricular heart and to determine the impact of sociodemographic and clinical characteristics. DESIGN AND SETTING: Retrospective, cross sectional study conducted in a regional paediatric cardiology centre. PATIENTS: The health records of 89 survivors with univentricular heart (median age 21 years; range 17-49 years) were reviewed. Sixty seven answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The impact of sociodemographic and clinical variables on individual Duke's measures was assessed. RESULTS: The Duke scores of adults with univentricular heart were similar to the normal population. Cyanosis predicted a worse score for physical (p = 0.05) and perceived health measures (p = 0.02). A higher educational level predicted a better score for physical (p = 0.004), mental (p = 0.01), and general health measures (p = 0.02). Orthopaedic problems worsened the social score (p = 0.05). Psychosocial problems worsened the pain score (p = 0.04). In comparison with the other anatomical types, mitral atresia worsened the perceived health score (p = 0.02). Patients younger than 23 years scored better for almost all health and dysfunction measures. CONCLUSIONS: Despite repeated interventions and other disease related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life.

Adolescent↗
PubMed2001

beta(1)-adrenergic antagonists improve sleep and behavioural disturbances in a circadian disorder, Smith-Magenis syndrome.

Smith-Magenis syndrome (SMS) is a clinically recognisable contiguous gene syndrome ascribed to interstitial deletions of chromosome 17p11.2. Patients have a phase shift of their circadian rhythm of melatonin with a paradoxical diurnal secretion of the hormone. Serum melatonin levels and day-night behaviour were studied in nine SMS children (aged 4 to 17 years) given acebutolol, a selective beta(1)-adrenergic antagonist (10 mg/kg early in the morning). Cardiac examination, serum melatonin, motor activity recordings, and sleep diaries were monitored before and after drug administration. The present study shows that a single morning dose of acebutolol suppressed the inappropriate secretion of melatonin in SMS. A significant improvement of inappropriate behaviour with increased concentration, delayed sleep onset, increased hours of sleep, and delayed waking were also noted. These results suggest that beta(1)-adrenergic antagonists help to manage hyperactivity, enhance cognitive performance, and reduce sleep disorders in SMS.

Acebutolol↗
PubMed2001

[Stenosis of the superior caval canal after Mustard and Senning procedures: treatment by dilatation and stent implantation. Two case reports].

The authors report two cases of stenosis of the superior caval canal after Mustard and Senning procedures for transposition of the great arteries in patients paced for atrial arrhythmia. During cardiac catheterisation, it was possible to treat the stenosis by perforation in one case and by balloon dilatation in the second, followed by the implantation of two stents to reestablish vascular patency. In one patient, two pacing catheters were implanted by an endovascular approach without complications three months after stenting; in the other case, epicardial pacing was required because the patient's condition could not wait for endothelialisation of the stent before implanting the pacing catheters. This technique of revascularisation may also be used in children with venous stenosis after implantation of endocavitary pacing catheters, in which the pacing system has to be changed.

Adolescent↗
PubMed2001

[T wave abnormalities on Holter monitoring of congenital long QT syndrome: phenotypic marker of a mutation of LQT2 (HERG)].

The two genes which code for the potassium channels, KCNQ1 and HERG, are responsible for the most common forms of the long QT syndrome, LQT1 and LQT2. Abnormalities of duration and morphology of the ventricular repolarisation are amongst the diagnostic criteria of this syndrome. The morphology of the T waves was studied by 24 hour Holter monitoring in 190 subjects with a long QT syndrome due to KCNQ1 (LQT1) [N = 133] or HERG (N = 57) and in 100 controls, and it was compared with the ECG T wave. The T wave was characterised according to 3 morphological features: grade 0 (G0) = normal, grade 1 (G&) = slight ST depression and grade 2 (G2) = presence of ST elevation of the descending phase of the T wave. The T wave morphology on Holter ECG was normal for most LQT1 and control subjects compared with LQT2 (92%, 96% and 19% respectively, p < 0.01). Grade 1 appearances were observed more often in LQT2 (18 vs 8% for LQT1 and 4% for controls, p < 0.01). Grade 2 appearances were only observed in the cases of LQT2 (63%). The predictive factors of G2 were young age and an anti-sense mutation of the transmembrane domaines of HERG. The authors conclude that Holter monitoring improves detection of T wave changes compared with the ECG. Grade 2 changes seem to be a phenotype marker for a HERG mutation, especially those situated in the transmembrane domaines.

Adolescent↗
PubMed2001

[Quality of life of adults with univentricular hearts].

This study sought to evaluate the quality of life of surviving adults with univentricular heart and to determine the impact of sociodemographic and clinical characteristics on quality of life. The health records of 89 survivors with univentricular heart living in France at the time of the study were reviewed. They were 17 to 49 years old (median: 21). Sixty-seven patients answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The scores of patients who answered the questionnaire were compared with national norms. The impact of sociodemographic and clinical variables on individual Duke's measures was assessed. The Duke scores of adults with univentricular heart were similar to those of the normal population. Cyanosis predicted a worse score for physical (p = .05) and perceived health measures (p = .02). The higher educational level predicted a better score for physical (p .004), mental (p = .01), and general health measures (p = .02). Orthopaedic problems worsened social score (p = .05). Psychosocial problems worsened pain score (p = .04). Mitral atresia, in comparison with the other anatomical types, worsened perceived health score (p = .02). Finally, patients younger than 23 years scored better for almost all of health and dysfunction measures. In conclusion, despite repeated interventions and other disease-related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life. These patients probably developed copying mechanisms, easing the psychological stress, and oriented themselves towards a different set of values in everyday life so that to accept disabilities and to recalibrate personal expectations.

Adolescent↗
PubMed2000

Characteristics and results of epicardial pacing in neonates and infants.

The results of epicardial pacing in infants with isolated congenital complete atrioventricular block (CAVB) are reported. Thirty-four patients, aged 1 day to 20 months (22 patients < 1 month), were paced between 1988 and 1998. Thirty had bradycardia < 50 beats/min with symptoms in 12 patients, and 4 patients were paced because of associated ventricular ectopy or prolonged QT interval. In thirty cases, the electrodes were implanted through a left thoracotomy and connected to an abdominal generator; in four, the subxyphoid approach was preferred. Twenty-two children had dual chamber units. There was no operative death, but three patients died later of cardiomyopathy. Seven infants were reoperated for electrode displacement, infection, exit block, and pacemaker sensitivity. Chronic ventricular thresholds ranged from 0.3 to 2 V except in one case (4 V) and proper atrial sensing was lost in two cases. All children are doing well and the generator has lasted at least 5 years in 16 cases. In conclusion, epicardial pacing in infants with CAVB can be done with satisfactory results. There was no mortality in relation with pacing and thresholds have improved with the use of steroid-eluting electrodes. The deep location of the generator prevents cutaneous erosion and allows implantation of large units with a longer life duration.

Cardiac Pacing, Artificial↗
PubMed2000

[Cardiac pacing in children with breath-holding spells].

Breath-holding spells are common and usually benign. However, the authors chose to implant a pacemaker in children presenting with severe symptoms. Over the last 15 years, 11 children with severe breath-holding spells were paced. All had reflex spells with loss of consciousness, spontaneously or after minor trauma, and 6 had seizures. All had a normal ECG with marked bradycardic responses to ocular pressure. The 24 hour ECG showed pauses (12-25 s) in 4 patients, sudden bradycardia (< 30/min) in 3 patients, and sinus arrhythmia in the remaining 4 patients. Medical treatment has been unsuccessful. Pacemaker implantation was decided because of the severity and/or the frequency of the episodes in 10 children, and because of intolerable familial anxiety in the other one. Age at implantation ranged from 14 months to 5.5 years (mean: 16.5 +/- 20 months). The device was implanted by an epicardial (7) or from an endocardial (4) approach. All had a ventricular demand device, except for one who was paced from the atrium. The results were spectacular, with disappearance of spells and restoration of normal activities. Holter monitoring showed normal function of the pacemakers. Recurrences were observed in 3 patients, either due to loss of capture (2 cases) or to the need for explantation because of cutaneous erosion. Follow-up ranged from 10 months to 14 years (mean: 7.9 +/- 4.2 years); 2 patients were lost to follow-up; 4 patients totally recovered and only 5 are still vagotonic. Two pacemakers have been changed at 13 and 15 years respectively. The authors conclude that although psycho-social factors play a part in breath-holding spells, pacemaker implantation is very effective in suppressing symptoms in severely affected children.

Age of Onset↗
PubMed2000

[Therapy and prognosis of infectious complete atrioventricular block in children].

From 1983 to 1997, we have studied ten children with complete atrioventricular block likely due to myocarditis in order to assess its prognosis and to define a therapeutic strategy. Their age ranged from 6 days to 16 years (median: 4.1 years). All were admitted for sudden complete block, with symptoms in seven: syncope or fainting, seizures, collapse. Three had an asymptomatic bradycardia which was detected on routine auscultation in children with fever or already hospitalized; fever was present in 5. The disease was related to infection on biological data in 4 cases (1 listeriosis and 3 seroconversions for Epstein Barr or cytomegalic or Coxsackie B viruses), on a myocardial biposy in 1 case and on scintigraphic data in 1 case. In the remaining 4, indirect arguments were considered such as infectious context, normal recent ECG, favourable outcome. Five children were given intravenous isoprenalin with ventricular tachycardia in 3. Five were treated with steroids and 3 with specific antiviral agents. Seven patients were paced temporarily. One child died, 6 recovered totally and 3 have a permanent block with a definitive pacemaker implanted in 2. In conclusion, sudden acquired complete atrioventricular blocks are often ill-tolerated in children and have to be treated with transient pacing. Recovery occurs as a rule but some of these blocks may be definitive. Infective myocarditis is likely to be the cause of the disease even if the pathogen agent cannot always be identified.

Adolescent↗
PubMed2000

[Prenatal diagnosis of conotruncal heart diseases. Results in 337 cases].

Prenatal diagnosis of conotroncal disease has developed by examination of the great arteries during morphological foetal ultrasonic scanning in the second term of pregnancy. This study reports the results of a series of 337 of these malformations diagnosed in the foetus over five and a half years. Their incidence with respect to all foetal cardiac malformations is 16.2%. Tetralogy of Fallot and pulmonary atresia with ventricular septal defect make up 56% of these cases, vascular malposition 16%, coarctation with or without interruption of the aortic arch 14%, truncus arteriosus 9% and agenesis of the pulmonary valves 5%. A karyotypic anomaly was found in 28 cases (8.2%) and, in the foetus with a normal standard karyotype, deletion of chromosome 22q11 was identified in 54 out of 237 cases. There was an associated polymalformative syndrome in 29 cases (8.3%). The training of obstetricians has increased the number of antenatal diagnoses of conotroncal abnormalities. Prenatal counselling should be given by a pluridisciplinary team because the association of these malformations with chromosomal abnormalities or with syndromes difficult to diagnose is common.

Cardiovascular Abnormalities↗
PubMed1999

Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children.

BACKGROUND: The clinical manifestations of inherited disorders of fatty acid oxidation vary according to the enzymatic defect. They may present as isolated cardiomyopathy, sudden death, progressive skeletal myopathy, or hepatic failure. Arrhythmia is an unusual presenting symptom of fatty acid oxidation deficiencies. METHODS AND RESULTS: Over a period of 25 years, 107 patients were diagnosed with an inherited fatty acid oxidation disorder. Arrhythmia was the predominant presenting symptom in 24 cases. These 24 cases included 15 ventricular tachycardias, 4 atrial tachycardias, 4 sinus node dysfunctions with episodes of atrial tachycardia, 6 atrioventricular blocks, and 4 left bundle-branch blocks in newborn infants. Conduction disorders and atrial tachycardias were observed in patients with defects of long-chain fatty acid transport across the inner mitochondrial membrane (carnitine palmitoyl transferase type II deficiency and carnitine acylcarnitine translocase deficiency) and in patients with trifunctional protein deficiency. Ventricular tachycardias were observed in patients with any type of fatty acid oxidation deficiency. Arrhythmias were absent in patients with primary carnitine carrier, carnitine palmitoyl transferase I, and medium chain acyl coenzyme A dehydrogenase deficiencies. CONCLUSIONS: The accumulation of arrhythmogenic intermediary metabolites of fatty acids, such as long-chain acylcarnitines, may be responsible for arrhythmias. Inborn errors of fatty acid oxidation should be considered in unexplained sudden death or near-miss in infants and in infants with conduction defects or ventricular tachycardia. Diagnosis can be easily ascertained by an acylcarnitine profile from blood spots on filter paper.

3-Hydroxyacyl CoA Dehydrogenases↗
PubMed1999

Surgical angioplasty of the main coronary arteries in children.

OBJECTIVE: To determine the safety and efficacy of surgical angioplasty of the coronary arteries in children. METHODS: We performed 9 surgical reconstructions of the left main coronary artery and 1 of the right coronary artery ostium in 10 children (mean age 5.7 years; range 2 months-15 years). The basic diseases included the following: congenital atresia of the left coronary artery (n = 2) and atresia of the right coronary artery in a patient with an aortoventricular tunnel (n = 1); stenosis of the left main coronary artery (1) in a patient with Williams syndrome (n = 1), (2) in a patient with familial hypercholesterolemia (n = 1), (3) after the arterial switch operation for transposition of the great arteries (n = 3), (4) after reimplantation of an anomalous left main coronary artery from the pulmonary artery (n = 1), and (5) by compression after a réparation à l'étage ventriculaire procedure (n = 1). Myocardial viability was assessed by single photon emission computed tomography (thallium 201; 7/10). The coronary artery stem was enlarged with a saphenous (n = 5), a pericardial (n = 4), or a polytetrafluoroethylene patch (n = 1). RESULTS: There was 1 hospital death and 9 patients are alive (mean follow-up 46 +/- 30 months; range 12 months to 10.5 years). Eight of 9 survivors had a selective coronary artery angiogram and had normal coronary artery ostia. Two patients had stenosis of the left anterior descending coronary artery, 1 of whom underwent successful internal thoracic artery grafting. CONCLUSIONS: Surgical angioplasty of the coronary stems restores physiologic coronary perfusion and conserves bypass material. It can be performed safely in children and provides encouraging midterm results.

Adolescent↗
PubMed1999

[Myocardial ischemia: an unknown complication of drepanocytosis in children. Apropos of 2 case reports].

BACKGROUND: Myocardial ischemia is a severe and unknown complication in children with sickle cell anemia. CASE REPORTS: Two cases of myocardial ischemia complicated by syncope in one (8-year-old) and cardiomyopathy in the other (3-year-old) are reported. CONCLUSION: Chest pain in children with sickle cell anemia should require ECG and, in case of hypokinetic cardiomyopathy, a myocardial SPECT. More aggressive therapy of sickle cell anemia should be considered in case of documented myocardial ischemia.

Anemia, Sickle Cell↗
PubMed1999

Total cavopulmonary and atriopulmonary connections are associated with reduced heart rate variability.

AIM: To determine whether cavopulmonary connections are associated with abnormalities of heart rate variability. METHODS: Heart rate variability was studied by 24 hour Holter monitoring in 39 patients (mean (SD) age 12.2 (4.1) years) who underwent cavopulmonary connection operations (partial in 12, total in 13, and atriopulmonary in 14). Two control groups were used: 18 healthy children (11.1 (2.5) years) and 16 patients (11.7 (4.3) years) undergoing cardiovascular surgery for biventricular repair of congenital heart disease. All patients were in sinus rhythm and had normal left ventricular function. Four time domain indices were calculated: mean duration of RR intervals (RR), standard deviation of all RR intervals (SD), square root of the mean squared differences of successive RR intervals (r-MSSD), and percentage differences of successive RR intervals of > 50 ms duration (pNN50). Four frequency domain indices were calculated: total power (TP), low frequency (LF), high frequency (HF), and the LF:HF ratio. RESULTS: Heart rate variability indices were identical in the two control groups. Significantly reduced heart rate variability was found in patients with total cavopulmonary connections and atriopulmonary connections compared with the two control groups. In patients with partial cavopulmonary connections, heart rate variability was reduced compared with healthy controls. No differences in heart rate variability could be related to clinical status (New York Heart Association functional class), number of surgical interventions, or presence of right atrial enlargement. CONCLUSIONS: Patients with cavopulmonary connections have significantly reduced heart rate variability and a particularly low vagal drive.

Adolescent↗
PubMed1999

[Compromise of the cardiac autonomic nervous system in patients with Fontan-type circulation: preliminary data].

BACKGROUND: Rhythm disturbances are frequent after Fontan operations. Arrhythmias related to reduced heart rate variability (HRV) have been described in various cardiovascular diseases. METHODS: We attempted to investigate HRV in 12 patients who underwent Fontan operation (age 11.4 +/- 3 years). Results were compared to a control group of 13 children matched for age, sex and heart rate (10.4 +/- 3 years). All patients underwent 24-hour Holter monitoring. The following time domain indexes were calculated: mean duration of RR intervals, standard deviation of all RR intervals (SD), square root of the mean squared differences of successive RR intervals (r-MSSD), percentage of differences between adjacent RR intervals > 50 msec (pNN50). The following frequency domain indexes were calculated: total power (TP), low frequency (LF), high frequency (HF), LF/HF ratio. RESULTS: The following indexes were significantly reduced in Fontan patients: SD (p < 0.0001), r-MSSD (p < 0.0001), pNN50 (p = 0.0002), TP (p < 0.0001), LF (p < 0.0001), HF (p = 0.0001). LF/HF increased significantly (p = 0.04). No differences were detected according to the type of operation (cavopulmonary connection vs atriopulmonary connection) or clinical status. CONCLUSIONS: Patients with Fontan circulation had a significantly reduced HRV and particularly abnormal sympatho-vagal balance. Surgery on the caval veins and the atria alters the intracardiac ganglia that are abundant at the cavo-atrial junction and in the myocardium of the right atrium. Abnormalities of HRV in Fontan patients may act as a co-factor in the initiation of arrhythmia in these patients.

Adolescent↗
PubMed1999

[Congenital long QT syndrome. The value of genetics in prognostic evaluation].

The congenital long QT syndrome (QTL) is a heterogenic clinical and genetic entity characterised by prolongation of the QT interval which may be complicated by syncope and sudden death. Four genes have been identified for the cardiac potassium (KCNQ1, HERG and KCNE1) and sodium (SCN5A). The aim of this study was to assess the prognosis of the disease by the site of mutation identified on the morbid gene. Thirty-two genotyped families participated to this study. Each subject gave a clinical history, an ECG and a search for genetic mutation. Eighteen mutations in the transmembrane domains of KCNQ1 were identified in 25 families and 2 mutations in the C-terminal part were found in 4 families. The phenotype was less severe in C-terminal part mutations: less syncopes and sudden deaths (22 vs 55%, p < 0.001) and a shorter QTc (458 +/- 31 ms vs 479 +/- 31 ms, p = 0.0003). Three mutations were detected in the C-terminal part of HERG in 3 different families. Their phenotype was less severe with syncoped related to hypokalemia. The authors also report the case of a family in which two subjects who were the most severely affected had two mutations, one in HERG and the other in KCNQ1. This study confirms the value of a genetic research in assessing the severity of the congenital long QT syndrome.

Adolescent↗
PubMed1999

[Prenatal diagnosis of transposition of great vessels reduces neonatal morbidity and mortality].

Transposition of the great arteries (TGA) is a common malformation which sometimes has a dramatic presentation at birth but which is completely curable with early and appropriate initial management. Antenatal diagnosis of this condition may change the neonatal prognosis. The authors compared morbidity and mortality in the pre- and postoperative periods of 68 neonates with an antenatal diagnosis of TGA (foetal diagnosis) with that of 250 neonates in whom the diagnosis was made after birth (neonatal diagnosis). The delay before admission to the department was 2 +/- 2.8 hours in the foetal group and 73 +/- 210 hours in the neonatal group (p < 0.01). Severe haemodynamic distress (metabolic acidosis, multi-organ failure) were more common in the neonatal group (p < 0.01). Management on admission was identical in the two groups (p > 0.05). The preoperative mortality was 15/250 in the neonatal group (6%, 95% CI = 3-9%) compared with 0/68 in the foetal group (p < 0.05). The postoperative morbidity was comparable in the two groups (25/235 and 6/68) but the hospital stay was longer in the neonatal group (30 +/- 17 versus 24 +/- 11 days, p < 0.01). Finally, postoperative mortality was significantly higher in the neonatal group (20/235 compared with 0/68, p < 0.01) although the risk factors of death at arterial switch surgery were identical in the two groups. Therefore, antenatal diagnosis of TGA reduces neonatal morbidity and mortality in this condition. Antenatal diagnosis must be developed by the education of obstetricians. The transfer of mothers with a foetus affected by TGA to centres capable of assuming the initial management, sometimes during labour, is essential.

Female↗
PubMed1998

[Recommendations for the treatment of recurrent supraventricular tachycardia in infants].

AIM: The effectiveness and safety of antiarrhythmic agents, mostly digoxin and amiodarone given to prevent recurrences, were compared in 141 infants of less than 1 year (77% < 1 month) with re-entrant supraventricular tachycardia. RESULTS: Digoxin was the drug of first choice in 114 patients at a dose of 10-20 micrograms/kg/d and was effective in 74 cases (65%). Amiodarone was used as first line therapy or after failure of digoxin. It was given at a maintenance dose of 250 mg/m2/d, alone in 22 infants and together with digoxin in another 36; it was effective in 56 cases (96.5%). Early adverse events occurred in six patients receiving digoxin: ventricular fibrillation requiring cardioversion in three, two of whom had Wolff-Parkinson-White syndrome, significant sinus bradycardia in two, accidental overload in one. At further follow-up, one child treated with digoxin but having also gastroesophageal reflux, died suddenly at 3 months of age; autopsy was normal and the digoxin blood level was 3 ng/mL. Among the 58 infants who received amiodarone, there were no proarrhythmia, a slight and transient increase in TSH in six infants and only one required a short-term treatment for hypothyroidism. Prophylactic therapy was maintained for 6 to 12 months and only ten patients had recurrences in the year following withdrawal. CONCLUSION: Amiodarone was found to be safer and more effective than digoxin. No significant side-effect was demonstrated in infants receiving a short-term treatment. Amiodarone may be proposed as first line therapy for prophylaxis of re-entrant supraventricular tachycardia in infancy, especially for those patients with reentry and Wolff-Parkinson-White syndrome.

Amiodarone↗