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E Villain

Publications and source records attributed to E Villain.

At least 19 recordsLinked to original sources
PubMed2006

[Obstetrical management of patients at risk of neonatal lupus syndrome: review of the literature].

Fetuses and infants of women with anti-SSA/Ro and anti-SSB/La antibodies are at risk of neonatal lupus syndrome, featuring skin lesions, hematological and hepatic disorders, and congenital heart block (CHB) in the absence of severe cardiac malformation. The prevalence of CHB in newborns of anti-SSA/Ro positive women with known connective tissue disease is 1 to 2% and the risk of recurrence ranges from 10 to 17%. CHB is definitive and is associated with significant morbidity (pacemaker must be implanted in 2/3 of cases) and mortality (16 to 19%). Myocardial involvement may either be associated or appear subsequently. Other manifestations are discussed. For anti-SSA/Ro positive pregnant women, echocardiograms should be performed every 2 weeks from 16 to 24 weeks of gestation, and every week in case of past history of CHB. Electrocardiogram should be performed in the first days of life for all children to detect incomplete CHB. Therapy for CHB detected in utero is based on fluorinated steroids, especially betamethasone. Its efficiency is variable.

Antibodies, Antinuclear↗
PubMed2006

[Transcatheter exclusion of a giant aneurysm of the left pulmonary artery using covered stents].

An 11 year-old boy with an Alagille syndrome was referred for an urgent echocardiography because of new onset of febrile hemoptysis. Follow-up in our institution to that point had shown severe hypoplastic pulmonary arteries. The diagnosis of tricuspid endocarditis complicated by pulmonary embolisms and aneurysm of the left pulmonary artery was done. Despite effective antiobiotics, the evolution was marked by rapid progression in size of the left pulmonary aneurysm. Since the surgery was not an option because of high risk of death, this aneurysm was treated by a trans-catheter technique using covered stents (CP stents, Numed Inc). Its exclusion allowed to stop its progression in size and to avoid its rupture.

Aneurysm, Infected↗
PubMed2005

Permanent junctional reciprocating tachycardia in children: a multicentre study on clinical profile and outcome.

OBJECTIVES: To investigate the clinical profile, natural history, and optimal management of persistent or permanent junctional reciprocating tachycardia (PJRT) in children. METHODS AND RESULTS: 85 patients meeting the ECG criteria for PJRT were enrolled in a retrospective multicentre study. Age at diagnosis varied from birth to 20 years (median 3 months). Follow up ranged from 0.1 to 26.0 (median 8.2) years. At the time of referral, 24 of 85 patients (28%) had congestive heart failure that was resolved with medical treatment in all patients. Eighty three patients received drug treatment initially. Amiodarone and verapamil were the most effective with a success rate of 84-94% alone or in association with digoxin. Radiofrequency ablation of the accessory pathway was performed in 18 patients. There was a trend for a relation between age at ablation and the result of the procedure, failures being more common in younger patients (three of six procedures in younger and 15 of 18 in older children were successful; p = 0.14). Two patients with persistent left ventricular dysfunction on echocardiography but with no symptoms of congestive heart failure died suddenly one month and three years after diagnosis. PJRT resolved spontaneously in 19 patients (22%). Age at diagnosis of PJRT was not a predictor of spontaneous resolution. CONCLUSIONS: PJRT is a potentially lethal arrhythmia in children with tachycardia induced cardiomyopathy. Spontaneous resolution of tachycardia is not uncommon. Antiarrhythmic treatment is often effective. Radiofrequency ablation should be performed in older children or when rate is not controlled, especially in patients with persistent left ventricular dysfunction.

Adolescent↗
PubMed2005

[Pediatric cardiac pacing: indications, implant techniques, pacing mode].

Over the last years, pacing leads design and pacemaker (PM) generator size, reliability, and longevity have markedly improved, so that reliable paediatric implant can now be performed at any age with a low complication rate. Main indications include congenital and postoperative atrioventricular block (AVB) and postoperative sick sinus syndrome. Implantation of a PM is mandatory for children who are symptomatic from syncope or congestive heart failure and for those who have advanced block persisting more than 10 days after cardiac surgery. Criteria for pacing have been established in relation with the bradycardia and prophylactic pacing is recommended in children with congenital AVB and a mean heart rate <50 beats/minute. The majority of paediatric cardiologists recommend epicardial pacing in children less than 10 kg and when venous access to the heart is limited by congenital anomalies or prior operation; for older children, transvenous implantation has become the technique of choice. As heart rate is the main determinant of cardiac output at exercise in children with normal heart structures, the VVI-R mode is an alternative to dual chamber transvenous pacing in young patients. Patients with isolated sinus failure are paced in the atrium. Although the majority of patients are doing well, late complications within the paediatric population include venous thrombosis and difficulties in lead extraction. Myocardial dysfunction in children with congenital AVB is increasingly reported, but it is not determined whether it is due to the underlying disease or to right ventricular apical pacing and adverse remodelling.

Bradycardia↗
PubMed2005

[Sudden death].

Explore the source record for details and available documents.

Arrhythmias, Cardiac↗
PubMed2004

Cardiac abnormalities 15 years and more after adriamycin therapy in 229 childhood survivors of a solid tumour at the Institut Gustave Roussy.

The purpose of this paper was to determine the cardiac status in children 15 years or more after adriamycin therapy for a solid tumour. Of the 447 pts, 229 pts were fully studied and 218 were not. The following cardiac evaluations were proposed to all the 447 consecutive patients (pts): (1) cardiac Doppler US by one of two expert cardiologists; (2) cardiac rhythm and conduction abnormalities including 24-hour holter ECG; (3) (131)l-mlBG myocardial scintigraphy; (4) serum brain natriuretic peptide levels at rest; (5) an exercise test with VO(2) max measurement. The radiation doses delivered to 6 points in the heart were estimated for all patients who had received radiotherapy. Congestive heart failure was diagnosed in 24 of 229 (10%) evaluated pts, with a median interval of 15 years (0.3-24 years) from the first symptom after adriamycin treatment. Among the 205 remaining pts, 13 asymptomatic pts (6%) had severe (n=4) (FS<20%) or marked (n=9) (20< or =FS<25%) systolic dysfunction. In the 192 others, the median meridional end-systolic wall stress was 91 (53-135) and it exceeded 100 g cm(-2) in 52 pts. Using a Cox model, only the cumulative dose of adriamycin and the average radiation dose to the heart, were identified as risk factors for a pathological cardiac status. In conclusion, the risk of cardiac failure or severe abnormalities increases with adriamycin treatment, radiotherapy and time since treatment, even after a follow-up of 15 years or more. In our series, after an average follow-up of 18 years, 39% of the children had a severe cardiac dysfunction or major ventricular overload conditions. The risk increases with the dose of adriamycin and radiation received to the heart, without evidence for threshold.

Adolescent↗
PubMed2004

[Cardiac syncope in children].

Syncope is a frequent problem in childhood; generally, it is an isolated event and the common causes are benign. However, in some circumstances, syncope can herald a potentially lethal problem, especially when occurring during exercise. Routine evaluation includes history, physical examination and a 12-lead standard ECG should be performed in all cases. Worrying features which should be an indication for further investigation include syncope during exercise, collapse in a swimming pool, history of familial sudden death, and abnormalities on clinical exam or ECG. Structural cardiac abnormalities that may cause syncope and sudden death include aortic stenosis, hypertrophic cardiomyopathy and coronary malformations. All children with unrepaired or repaired congenital heart disease who experienced a syncope should be referred to a specialist. Primary arrhythmias that are easily diagnosed on ECG are the long QT syndrome, complete atrio-ventricular block and Wolff-Parkinson-White syndrome; ST elevation in V1-V3 may reveal a Brugada syndrome. Another arrhythmia which is known to be potentially fatal if undiagnosed is the catecholaminergic ventricular tachycardia; the baseline ECG is normal but the arrhythmia is easily reproduced during exercise testing. Finally, vasovagal syncope is the most likely cause of syncope in the young and it usually easily recognized.

Arrhythmias, Cardiac↗
PubMed2004

Low incidence of cardiac events with beta-blocking therapy in children with long QT syndrome.

AIMS: To evaluate the effect of beta-blockers in children with long QT syndrome (LQTS) we reviewed the outcome of 122 patients (pts). METHODS: LQTS was diagnosed in 24 neonates and in 98 pts aged 0.5-15 years. Diagnosis was made because of syncope in 51 pts, bradycardia in 10 neonates and family history in 61 pts. The longest QTc ranged from 400 to 700 ms. Thirteen pts had 2:1 atrioventricular block and/or ventricular arrhythmias. Screening for mutations was performed in 118 pts. All children were treated with beta-blockers, annually checked by exercise testing and/or 24 h ECG monitoring. RESULTS: Four pts died. Survivors were followed-up for 1-18 years (7.5 +/- 5.3 years). Five neonates and 3 older pts received a prophylactic pacemaker (1 death) so that only 111/122 pts survived and were followed-up with beta-blockers alone. None of them died and five experienced a non-fatal cardiac event. There was no cardiac event among pts who were diagnosed because of familial history and among symptomatic KCNQ1 pts who were effectively treated with beta-blockers. CONCLUSION: The outcome of children with LQTS under effective beta-blockers is favourable. Persisting arrhythmia or symptoms despite beta-blockers should aim at identifying other genotypes than KCNQ1.

Adolescent↗
PubMed2004

[Neonatal forms of congenital long QT syndrome].

UNLABELLED: The neonatal congenital long QT syndrome (LQTS) is rare and of bad prognosis due to the presence of severe ventricular arrhythmia and conduction abnormalities. METHODS: we included 24 propositus newborns from our population with LQTS. Genetic study was possible in 19 cases. RESULTS: the diagnosis of LQTS was made according to a QT prolongation associated with a sinusal neonatal bradycardia (n=9) or a 2/1 AV block (n=15). The onset presentation consisted of syncope (n=2), torsades de pointes (n=7), cardiovascular collapse (n=5), cardiac arrest (n=1). The mean QTc was at 550+60 ms. During the neonatal period the treatment consisted of beta-blocking agents in all cases, associated with a definitive pacemaker implantation in 10 cases with 2/1 AV block. Three newborns with a 2/1 AV block died during the first month of life (one case due to a septecemia after implantation of a pacemaker, and two who were waiting for that implantation). All survivors remained asymptomatic during a follow-up period of 7 years. In all cases with a 2/1 AV block we identified mutations in HERG (n=8). Newborns with isolated sinusal bradycardia presented all a mutation in KCNQ1 (n=9). CONCLUSION: the LTQS with 2/1 AV block is preferably associated with mutation in HERO with a bad initial prognosis.

Female↗
PubMed2004

[Congenital complete atrio-ventricular block].

Congenital complete atrio-ventricular block is a rare condition, the result of an anatomical anomaly of the conduction pathways, or even the trans-placental passage of maternal antibodies causing fetal myocarditis with fibrosis of the conduction tissue. It is not clear whether AV block discovered later in childhood is really "congenital". Whatever the age of the child, the only treatment is pacemaker implantation. Cardiac failure and syncope are absolute indications for implantation. Children whose heart rate is below 50 beats per minute (bpm) represent a group at high risk of syncope or even sudden death and must be paced, even if asymptomatic. Moreover, certain patients with immunological complete AV block have cardiopathy and must also be paced. In our department, the route for pacing is epicardial in younger children, in practice weighing less than 10-15 kg, and endocavitary in older children. The choice is either double chamber pacing, which restores AV conduction initiated by the child's sinus, or ventricular pacing with activity-controlled heart rate. Although worrying complications have been described, such as venous thrombosis, infections related to repeated interventions, and delayed cardiomyopathy, the results of paediatric pacing are generally satisfactory and the great majority of children with congenital complete AV block lead a normal life.

Age Factors↗
PubMed2003

[Neonatal lupus syndrome: review of the literature].

PURPOSE: Neonatal lupus syndrome include skin lesions, hematological and hepatic disorders, and congenital heart block (CHB) in the absence of severe cardiac malformation. This rare disorder is closely linked to transplacental transport of anti-SSA/Ro and anti-SSB/La maternal antibodies. CURRENT KNOWLEDGE AND KEY POINTS: The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is 2% and the risk of recurrence ranges from 10 to 17%. Skin and systemic lesions are transient, whereas CHB is definitive and is associated with significant morbidity and mortality (estimated at 16-19%). A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or may appeared secondarily. Mothers of children with CHB are usually asymptomatic or have Gougerot-Sjögren, or undifferentiated connective tissue disease. Mothers of children with cutaneous manifestations may present with more severe disease and systemic lupus erythematosus. In anti-Ro/SSA positive pregnant women, echocardiograms should be performed at least every 2 weeks from 16 to 24 weeks gestation. Electrocardiogram should be performed for all children. FUTURE PROSPECTS AND PROJECTS: The efficiency of prophylactic treatment of CHB is not established. Therapy for CHB detected in utero is not standardized and involves fluorinated steroids (especially betamethasone).

Causality↗
PubMed2003

[Wolff-Parkinson-White syndrome in the child. A case report with associated atrial fibrillation].

UNLABELLED: In children, Wolff-Parkinson-White (WPW) syndrome is often revealed by reentrant tachycardia episodes. Rarely, this syndrome can be the cause of syncope or of sudden death, resulting from rapid conduction of atrial fibrillation to the ventricles through the accessory pathway. CASE REPORT: The authors report the case of a child, who had a WPW syndrome diagnosed after birth because of a supraventricular tachycardia and who was later asymptomatic. At ten years of age, he was admitted because of palpitations due to atrial fibrillation and rapid conduction through the accessory pathway. CONCLUSION: In a child with WPW syndrome, tachycardia with wide and irregular QRS complexes should evoke the involvement of atrial fibrillation, with rapid conduction to the ventricle. Drugs blocking conduction through the atrio-ventricular node are contra-indicated and the condition is an indication for radiofrequency ablation.

Atrial Fibrillation↗
PubMed2003

[Predictive factors for late complete atrio-ventricular block after surgical treatment for congenital cardiopathy].

The late occurrence of complete atrio-ventricular block (CAVB) after cardiac surgery is rare but potentially responsible for cases of late sudden death. We searched for factors allowing prediction of this complication, retrospectively reviewing the case notes of 11 patients in hospital with complete AVB, 2 months to 10 years after correction of a cardiac malformation. All had a normal pre-operative ECG. The diagnosis had been made based on symptoms in 8 patients: syncope or collapse (4 cases) symptoms on effort (3 cases) or fatigue (1 case). In the others the diagnosis had been made on ECG. The block was infra-His in 5 patients who had electrophysiology. ECG analysis showed that all the patients had CAVB immediately post-operatively lasting 3-14 days. After restoration of conduction the ECGs showed the following anomalies compared to the pre-operative ECGs: long PR (1 case), long PR + right bundle branch block (2 cases), long PR + left axis deviation (1 case), RBBB + left deviation or rotation of the QRS axis (3 cases), long PR + RBBB + left axis deviation (4 cases). All of these patients had been fitted with a cardiac stimulator. In conclusion, the children who had CAVB immediately post-operatively lasting more than 48 hours and who then had an ECG showing different QRS compared to the pre-operative QRS and/or long PR had a risk of late complete AVB. These patients should have electrophysiology and a stimulator must be implanted in those who have an infra-His block.

Bundle of His↗
PubMed2003

[Results of prenatal management of fetuses with supraventricular tachycardia. A series of 66 cases].

OBJECTIVE: To describe the prenatal management and outcome of a series of 66 fetuses with supraventricular tachycardia (SVT). MATERIAL AND METHODS: The perinatal data of 66 fetuses with SVT were retrospectively studied from January 1990 to December 2000. Junctional tachycardia was found in 50 fetuses and atrial flutter was found in 16 fetuses. Two groups were studied depending on the absence (n=40) or the presence of hydrops (n=26) at the time of the diagnosis. All fetuses but one were treated prenatally via the mother. Anti-arrhythmic drugs used were: digoxin, sotalol, flecainide or amiodarone. RESULTS: Group of fetuses with no hydrops: digoxin was used in 32 cases and allowed 26 fetuses to be converted to sinus rhythm (80%). One intra uterine death (IUD) occurred in this group. Hydropic fetuses group: nine fetuses were converted to sinus rhythm using either flecainide (n=7) or amiodarone (n=2) as first line therapy, whilst digoxin alone or in association with sotalol failed to restore sinus rhythm in all cases. After first line therapy, SVT persisted in 10 fetuses. Nine fetuses received amiodarone alone or in association with digoxin as second line therapy, five of whom were converted to sinus rhythm. Among the 18 alive neonates treated by amiodarone in utero, three presented elevated thyroid stimulating hormone at day 3-4 and required thyroid hormonal substitution therapy for 2-6 months with normal outcome.

Amiodarone↗
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

[Homozygotous mutation of the SCN5A gene responsible for congenital long QT syndrome with 2/1 atrioventricular block].

Long QT syndrome is characterized by a prolongation of the QT interval on the surface ECG. This clinically and genetically heterogeneous cardiac disease is potentially lethal due to ventricular polymorphic tachyarrhythmias leading to syncope or sudden death. It is transmitted according to different mendelian modes due to mutations in several genes coding for cardiac ion channels. Heterozygous mutations in KCNQ1, HERG, SCN5A, KCNE1 and KCNE2 genes are responsible for the dominant form without deafness whereas homozygous mutations in KCNQ1 and KCNE1 are responsible for the recessive form (Jervell and Lange-Nielsen syndrome) associated with congenital deafness. We report the case of a 5 year-old boy referred for syncope with a prolongation of the QTc interval (526 ms) and a 2/1 Atrio-Ventricular (AVB) block on the surface ECG. Under beta-blocking therapy, the sinus rate decreased and the 2/1 AVB disappeared. Electrophysiological study evidenced an infra-hisian block and a unipolar ventricular endocardial pacemaker was implanted. A V1777M missense mutation was identified in the C-terminal part of SCN5A, cardiac sodium channel gene, at the homozygous state in the proband and at the heterozygous state in both parents and 2 sibblings. Only the proband had a severe phenotype with syncope and AV conduction anomalies. All other genetically affected subjects were asymptomatic. This study provides evidence for the involvement of homozygous LQT3 forms in "functional" AVB.

Amino Acid Sequence↗
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↗