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Biomedical subjects

E Villain

Publications and source records attributed to E Villain.

At least 73 records · Page 4Linked to original sources

[Catecholamine induced ventricular tachycardia: a cause of severe syncope during adolescence].

A 15-year-old female teenager had recurrent syncopes and had been unsuccessfully treated for 6 years for seizures and hysteria. Syncopes were always triggered by emotions, or efforts, but electrocardiograms were normal between the episodes. A 24-hour-monitoring during a syncope, and provocative tests (exercise testing and isoprenaline administration) showed severe ventricular arrhythmias with premature polymorphous beats, followed by ventricular tachycardia and then ventricular fibrillation. These "catecholamine-induced" ventricular tachycardias are very rare but are always fatal without treatment. Syncopes are stress-induced and the diagnosis is almost always delayed, because the patients have a normal electrocardiogram, with normal QT interval. The arrhythmia appears beyond a "threshold" sinus rate and is easily reproducible by exercise testing. Betablockers are the only efficient treatment to prevent sudden death.

Adolescent↗

Prolonged QT interval in neonates: benign, transient, or prolonged risk of sudden death.

To determine the factors relating to prognosis, the records of 15 neonates with persistent prolongation of the QT interval on the electrocardiogram after the fourth day of life were reviewed. Patients were admitted for symptoms (syncope, cardiac failure, or seizures), abnormal auscultation with an irregular heart rate or bradycardia, or because of a family history of a long QT syndrome. All infants had a long QTc, ranging from 0.46 to more than 0.70 second. Eight patients who had a QTc over 0.60 second developed severe ventricular arrhythmias (torsades de pointes, ventricular tachycardia) or second-degree AV block. Twelve of 15 were treated with beta-blocking agents, combined with ventricular pacing in five cases. Four infants died in the first month of life; they all had a very long QT interval and had experienced ventricular arrhythmias and AV block. Six children are still being treated with beta-blocking agents for the long QT syndrome and are doing well. In five infants, electrocardiographic abnormalities were transient and the QT interval returned to normal within 1 year. Therefore (1) prolongation of the QT interval in neonates may be transient or may represent an early form of the long QT syndrome and (2) the length of the QT interval may provide data on prognosis: those with a QTc less than 0.50 second returned to normal; those with a QTc greater than 0.60 second were associated with severe arrhythmias and four of eight infants died.

Adrenergic beta-Antagonists↗

[Rhythmogenic cardiomyopathies of atrial origin in children. Myth or reality?].

Incessant, rapid, supraventricular tachycardia may be complicated by cardiac failure with ventricular dilatation and hypokinetic wall motion on echocardiography: so-called tachycardia-induced cardiomyopathy. The diagnosis is simple when the cardiac rhythm is not sinus rhythm. The authors report the cases of 4 children aged 7 months to 12 years, referred for diagnosis and treatment of apparently primary cardiomyopathy. The findings of spontaneous or vagally-induced atrioventricular conduction defects, a permanently rapid atrial rhythm though influenced by 24 hour variations, or periodic abnormal rate increases, suggested myocardial dysfunction due to an ectopic atrial tachycardia. This was an essential step in management as the control of the tachycardia by amiodarone or betablocker therapy resulted in regression of symptoms and normalisation of left ventricular function. However, some atrial tachycardias are very resistant to medical treatment and, in such cases, there should be no hesitation in using more radical approaches, surgery or ablation, even and especially in patients with severe cardiac failure. In conclusion, apparently primary dilated cardiomyopathy in children may be due to chronic atrial arrhythmia and it is essential to perform at least Holter monitoring in order not to miss this diagnosis.

Adrenergic beta-Antagonists↗

[Congenital long QT syndrome. Mid-term prognosis].

Twenty-five cases of the congenital, idiopathic long QT syndrome, occasionally diagnosed at birth and persisting after the 4th day of life, were analysed retrospectively to determine the medium-term prognosis and to identify risk factors. The age of the patients varied: 13 neonates including 10 of less than 5 days of age, and 12 children 1 to 12 years old. Eleven patients belonged to families with long QT syndromes. The recruitment also varied: syncope or near syncope (9 cases), bradycardias or tachyarrhythmias (9 cases) or by systematic familial enquiry (7 cases). The corrected QT interval (QTc) was greater than 0.44 s in all patients, and 17 patients had conduction (atrioventricular or intraventricular) defects and/or ventricular arrhythmias (tachycardia, torsades de pointe or fibrillation). All except two older children were prescribed betablocker therapy and in 6 cases of resistant arrhythmias a pacemaker was implanted. There were 6 deaths: 4 deaths occurred in neonates who had the longest QT intervals (greater than 0.65 s) complicated by conduction defects and tachyarrhythmias. The other 2 fatalities were in older children who both died during syncope. The 19 survivors were followed up for 3 months to 16 years: 4 children with the shortest QTc values (0.44 to 0.48 s) have completely recovered; 2 neonates treated for 9 months and then weaned off therapy without any complications and 2 older children who were not treated. The other 15 cases are all under treatment but do well and have had no syncopal episodes during follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenergic beta-Antagonists↗

[QT prolongation and circulatory arrest after an injection of erythromycin in a newborn infant].

A 8 day-old full-term newborn showed severe cardiac disturbances after intravenous injection of erythromycin. The neonate, suspected of having Chlamydia pneumonitis because of tachypnea and rhinitis, had been given 5 injections of erythromycin without clinical effect. Pallor, vomiting and bradycardia developed a few minutes after the 6th injection, and ECG showed ventricular arrhythmia, prolonged QT interval and an atrioventricular block. The infant died in intensive care unit. This case and the analysis of other published cases of cardiac disturbances following the parenteral use of erythromycin, indicate the potential arrhythmogenic risk of this drug. It is suggested that newborns treated with erythromycin should be monitored by ECG.

Erythromycin↗

Evolving concepts in the management of congenital junctional ectopic tachycardia. A multicenter study.

We reviewed the records of 26 infants with congenital junctional ectopic tachycardia (JET) from seven institutions to examine the evolution in the management of this tachycardia that is difficult to treat. JET was defined electrocardiographically as an incessant tachycardia with normal QRS morphology and atrioventricular (AV) dissociation. The ventricular rate ranged from 140 to 370 beats/min (mean, 230 beats/min); 16 of 26 patients had cardiac failure. Treatment success was defined as a stable decrease in the rate of JET, below 150 beats/min; partial success was a significant decrease of JET rate with alleviation of symptoms. All patients received digoxin with no significant effect. Propranolol was given to 16 patients, with two successes and one partial success. Combinations of other conventional agents were used in 11 patients with two successes; 14 patients were treated with amiodarone, which resulted in eight successes and three partial successes; three patients died suddenly on medical treatment (amiodarone, one patient; propranolol, one patient; or amiodarone plus propranolol, one patient); sudden AV block was a possible cause and consequently, two later patients had pacemaker implantation as well as medical treatment. His catheter ablation was successfully performed twice but contributed to death in a newborn; three surgical His ablations were performed for intractable JET with two successes and one death. The overall mortality was 35%. Among survivors, treatment has been stopped without any complications in five patients ranging in age from 10 months to 8 years (mean, 3.5 years). It seems that amiodarone alone is the best drug for treatment of congenital JET; necessity for permanent pacing remains unsettled. His ablation should be reserved only for intractable JET.

Amiodarone↗

[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↗

[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↗

[Partial atrioventricular block and prolonged QT interval in 4 premature infants receiving diphemanil].

Four premature infants presenting with episodes of bradycardia in the first weeks of life were given diphemanil. One of them received an overdose accidentally. Paradoxically, this induced a permanent bradycardia leading to the discovery of a grade II A-V block as well as a prolonged QT interval. Discontinuation of the drug resulted in a prompt normalization of these changes. It is felt that this anticholinergic therapy may have caused a prolongation of the QT interval and, therefore, a partial A-V block in case of sinus tachycardia. Thus, such a therapy should not be given to young premature infants without having checked the QT interval on a ECG tracing and having made sure that it is adapted to the actual heart rate. It is also advised to reduce usual doses.

Bradycardia↗

[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↗

Anomalous left coronary artery arising from the pulmonary artery in infancy: is early operation better?

From January 1975 to January 1987, 21 consecutive infants aged less than six months (mean (SD) 2.6 (1.2] were admitted with anomalous origin of the left coronary artery from the pulmonary artery. In the first 12 patients, who were seen up to 1982, operation was performed after the age of one year (mean (SD) 29 (29) months) (group 1). The next nine infants, seen from 1983 to 1987, had their operations within a few weeks of the onset of symptoms (mean (SD) age 4.8 (1.4) months) (group 2). In group 2 the left coronary artery was relocated into the aorta, whereas in group 1 there was additional resection of the left ventricular wall or mitral valvoplasty or both. At presentation there were no differences in age, clinical condition, heart enlargement, and echocardiographic left ventricular dysfunction between groups 1 and 2. Seven of the 12 patients in group 1 died, five while they were awaiting operation (three died suddenly at home) and two at operation. The five survivors are doing well 6.4 (3.1) years after operation with normal left ventricular function which improved slowly over several months after operation. Two of the nine patients in group 2 died; both deaths occurred at or soon after operation. The seven survivors are doing well 1.8 (0.9) years after operation. In three, left ventricular function recovered within three weeks; and there was even partial or total regression of the Q waves in the supposedly necrotic areas. In the remaining four the pattern of improvement in left ventricular function resembled that in group 1. Operation should be undertaken early in infants with anomalous left coronary artery arising from the pulmonary artery because the procedure is relatively safe, prevents a high natural mortality, and offers a better chance of a faster recovery of left ventricular function.

Age Factors↗

Fate of pulmonary artery after anatomic correction of simple transposition of great arteries in newborn infants.

From April 1984 to April 1987, surgical anatomic correction was performed in 86 newborn infants, 2-23 days old (6.8 +/- 3.6 days, mean +/- SD) with simple transposition of the great arteries. In all patients, the pulmonary artery was reconstructed by end-to-end anastomosis according to the Lecompte maneuver, including eight patients with side-by-side position of the great arteries. Three different approaches were used. In the first 10 patients (group 1, six survivors), two separate patches of preserved tanned pericardium were used to reconstruct the pulmonary artery, whereas in the next 15 patients (group 2, 13 survivors), a single patch of the same material was used, and in the last 61 patients (group 3, 56 survivors), surgery was performed with a single patch of fresh autologous pericardium. Among the 75 survivors, 68 (including six in group 1, 12 in group 2, and 50 in group 3) were followed serially for at least 6 months (6-48 months, 26 +/- 9 months) with sequential noninvasive evaluations. At follow-up, all were asymptomatic with normal growth. Two patients with severe pulmonary artery stenosis (group 1) were successfully reoperated on. Four infants with moderate pulmonary artery stenosis have been followed medically and have had stable right ventricular pressures. The last 62 patients have normal or near-normal right ventricular pressures. The spatial relation of the great arteries did not affect the quality of the results. Group 1 had clearly the worst results.(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Surgical↗

[Cardiac transplantation in the infant and young child. Preliminary results].

Between January and December, 1987, a programme of heart transplantation in paediatrics was designed and carried out in 9 children by the medical and surgical teams of the Necker/Enfants Malades-Laënnec hospitals group, Paris. Six of the patients were infants of less than 2 years (4 were under one year), and the oldest child was 10 years old. All patients seemed to be condemned to an early death either because their congenital heart disease was beyond the resources of conventional surgery (6 cases) or because their dilated cardiomyopathy was refractory to all medical treatments. Three children died at the end of the operation or a few days afterwards, due to poor quality graft (1 case), fulminating bacterial superinfection (1 case) or intractable pulmonary hypertension (1 case). The remaining 6 children are now living as normally as possible in their respective families. The long-term immunosuppressive treatment consists of cyclosporine and azathrioprine; corticosteroids are only used at the very beginning of treatment or in case of graft rejection. Only two episodes of rejection, confirmed by endomyocardial biopsy, were observed in the same patient during the first postoperative month. Biopsy was never performed systematically in order to spare the patient's vein, and the diagnosis of rejection was suspected on clinical grounds.(ABSTRACT TRUNCATED AT 250 WORDS)

Biopsy↗

[Development of aortic valve stenosis in infants and children. Apropos of 66 cases hospitalized from 1973 to 1984].

Sixty-six children aged over 6 months hospitalized for isolated aortic valve stenosis without cardiac failure but with a transvalvar systolic pressure gradient of more than 40 mmHg were followed up and re-evaluated for a mean period of 5.4 +/- 3.5 years. Twenty-two children who were asymptomatic, had no ST-T changes and had a less than 60 mmHg gradient were not operated upon; they remained stable over a mean period of 5.4 +/- 3.4 years; their gradient was not significantly modified (53 instead of 51 mmHg) and none of them underwent surgery. Forty-four children with symptoms and/or a more than 60 mmHg gradient underwent valvotomy under extracorporeal circulation without mortality. Surgery was effective against the major symptoms in all cases, and it reduced the gradient from 75 +/- 25 to 36 +/- 18 mmHg at the expense of aortic regurgitation in 7 cases, 2 of them with significant haemodynamic repercussions. During the follow-up period (5.8 +/- 3.7 years) 1 patient died of bacterial endocarditis, 5 were reoperated upon (with prosthesis in 4) without mortality and with 4 good results, and 1 patient underwent valvoplasty which proved partially effective but resulted in moderate aortic regurgitation. All other children are now doing well; 29 are completely asymptomatic and without ST-T changes at rest or during exercise. We conclude that aortic valve stenoses with moderate gradient are very stable at mid-term and that surgical valvotomy is a generally effective and low-risk procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Switch operation for transposition of the great arteries in neonates. A study of 120 patients.

From March 1984 to January 1987, anatomic surgical correction was performed on 110 newborn infants (2 to 23 days old, mean 7.8 +/- 3.5, standard deviation) with simple transposition of the great arteries and 10 additional neonates (7 to 30 days old, mean 17.9 +/- 8.3, standard deviation) with transposition and a large ventricular septal defect. All had preoperative catheterization. Ninety-six percent of the patients underwent balloon atrial septostomy and 90% received prostaglandin E1 infusion until the time of the operation. The anatomy of the coronary arteries according to the Yacoub classification was as follows: type A, 82 patients; type B, 5 patients; type C, 4 patients; type D, 23 patients; and type E, 6 patients. Continuous hypothermic bypass with no circulatory arrest was used for all patients except two. Myocardial protection was ensured by crystalloid cardioplegia. Coronary artery relocation was performed according to the Yacoub technique with some modifications, and pulmonary artery reconstruction was done according to the Lecompte maneuver in all patients, even when the great vessels had a side-by-side relationship. The proximal pulmonary artery was reconstructed with two circular patches for the first 10 patients and with a single large posterior patch for the last 110 patients. Tanned heterologous pericardium was used for the first 25 patients and autologous native pericardium for the last 95 patients. The perioperative mortality rates were 8.3% for the entire series and 5.4% for the last 110 patients, with no deaths in the group having transposition plus ventricular septal defect. Late death from acute myocardial infarction occurred in two patients in the second month after operation. No patient was lost to follow-up, which ranged from 2 to 46 months (mean 16 +/- 11.2, standard deviation). The follow-up included sequential noninvasive evaluations and 32 catheterizations performed 10 to 18 months postoperatively. Two patients were reoperated on for pulmonary stenosis caused by retraction of the two heterologous pericardial patches, but neither died. Six others have mild to moderate pulmonary stenosis. Two patients have trivial aortic regurgitation. None have aortic dilatation or supravalvular aortic stenosis. The 108 survivors have no cardiovascular symptoms. They all are in sinus rhythm, have normal left ventricular function, have no ischemic problems, and receive no medication.

Coronary Vessels↗

Restrictive cardiomyopathy in children. Ultrastructural findings.

Restrictive cardiomyopathy is usually related to fibrosis of the endocardium or to an infiltrative disorder. However, in few cases, it can be due to isolated pathology of the myocytes but such alterations are not well characterized. This paper reports the disease in two 7 year old patients. There was severe venous congestion and catheterisation revealed increased end diastolic pressure in the ventricles. Both pericardial and myocardial biopsies were performed, as the clinical and haemodynamic data were indistinguishable from constrictive pericarditis. The structure of the pericardium was normal. The endocardium was not thickened. The interstitium of the myocardial tissue was not increased. Electron microscopic examination revealed intracellular masses of disorganized myofilaments. These large deposits may have produced decrease compliance of the myocytes and of the ventricular walls.

Biopsy↗