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F Marçon

Publications and source records attributed to F Marçon.

At least 19 recordsLinked to original sources
PubMed2006

[Implantable cardioverter defibrillators in pediatric patients: results in a series of 33 cases].

UNLABELLED: Implantable defibrillator is the recognized treatment of sudden cardiac death. Miniaturization of the devices allows implantation in children. METHODS: This multicentric retrospective study analyzed data of 33 children aged 18 years and less who were implanted from 1990 to 2005. RESULTS: Age of patients are 10 to 18 years, 20 patients were implanted after a resuscitation of sudden death, 10 after a syncope, 2 after a ventricular tachycardia and 1 in a prophylactic way. Tachycardias on primary electrical disease are most frequent (46%). Hypertrophic cardiomyopathy accounts for 22%, DAVD for 14%, congenital cardiopathies for 12%. Seventeen patients received appropriate shocks and 14 patients had inappropriate shocks. There were two unexplained deaths. Five leads fractures and two device infections were noted. CONCLUSION: Implantable defibrillator is an effective treatment for children high-risk of sudden death. Occurrence of inappropriate shocks due to sinusal tachycardia, infections and leads fractures are frequent.

Adolescent↗
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

[Paediatric cardiac surgery and autoevaluation: risk score, complexity score and graphic analysis].

The creation of a paediatric surgical unit requires autoevaluation in order to: assess the quality of the results with respect to recognised international standards, answer the family's questions about the results obtained and adhere to criteria of accreditation Between January 2003 and December 2004, 201 consecutive patients, children (N= 164) or operated for adult congenital heart disease (N= 37) were treated. No patient was excluded. The RACHS-1 risk score, the ARISTOTLE scores of complexity and performance and the CUSUM and VLAD graphic analyses were applied to the study of hospital mortality. An original "variable performance-adjusted display" (VPAD) graphic analysis was performed to show up any possible variations of performance. Paediatric hospital survival was 97.56% (95% CI: 93.9 - 99.1). The paediatric complexity and performance scores were 6.79 +/- 0.22 and 6.62 respectively. In the absence of statistical significance in this field of autoevaluation, graphic analyses indicated the performance of our unit with no "learning" curves. Graphic scores and analyses allow assessment of the function of a paediatric cardiac surgical unit and the variations of complexity with respect to time, before the appearance of statistical significance. The ARISTOTLE complexity and performance scores and their adaptation in VPAD seem to be more reliable and discriminating than the RACHS-1 score.

Adolescent↗
PubMed2005

[Complications of prostaglandin E1 treatment of congenital heart disease in paediatric medical intensive care].

The authors undertook a retrospective study of the modes of prescription, the tolerance and efficacy of prostaglandin E1 in 62 consecutive neonates with congenital heart disease (average Age 1.6 days: 35 boys: weight: 3.1 +/- 0.6 Kg) admitted to the paediatric intensive care unit of Nancy University Hospital between 1998 and 2002. The infusion time and cumulative dosage were 134 +/- 112 (6-480) hours and 111 +/- 94 (4-396) microg/Kg respectively. The side effects that were observed were: Apnoea (19%), abdominal distension (16%), bradycardia (13%), enterocolitis (6.5%), hypotension (6.5%), vomiting (5%), fever (1.6%) and skin rash (1.6%). Gastrointestinal disturbances are associated with a low body weight (p<0.04), to prolonged treatment (p<0.02) with no influence of initial or cumulative dosages (P=NS), with respiratory assistance (p<0.03) and longer hospital stay (p<0.01). Hypotension was commoner in cases of poor neonatal adaptation. Mortality was correlated with severe initial acidosis (p<0.02), a low Apgar score, the initial prolonged use of high doses of prostaglandin (p<0.04), and the presence of severe valvular aortic stenosis or hypoplasia of the left heart (p<0.002). The authors conclude that treatment with prostaglandin is effective in the majority of cases despite the use of low maintenance doses (0.01 microg/Kg/min). Gastrointestinal disturbances favourised by the perinatal context, the cardiac disease, and prolonged treatment are significant factors for morbidity and mortality. The beneficial role of early neonatal enteral feeding was not demonstrated in this high risk population.

Alprostadil↗
PubMed2004

[Grave's disease in children with 22q11 deletion. Report of three cases].

Hypothyroidism is a well recognized complication of 22q11.2 deletion syndrome. Auto-immune hyperthyroidism is less common. We report three patients with a 22q11.2 deletion and Graves' disease diagnosed at age 17, 14 and 11 years, respectively. The clinical and biological presentation was typical for auto-immune hyperthyroidism. Graves' disease should be periodically sought during the follow-up program of patients with 22q11.2 deletion syndrome.

Adolescent↗
PubMed2004

[Kawasaki disease in newborns and infants: refractory forms to immunoglobulin therapy].

UNLABELLED: We studied 52 consecutive patients with Kawasaki disease hospitalized (1984 -2003) during the acute phase (mean age 2.5 + 2.4 years; range 0.3 to 16 years, 34 males, 18 cases with coronary aneurysms, median follow-up 6.7 years), and identified a subgroup presenting a refractory subtype to immunoglobulin therapy. RESULTS: forty-nine infants benefited from a first regimen of immunoglobulins, 8.4 + 6 days following the onset of symptoms. Eleven infants (1.4 + 1.2 years, range 0.3 - 4.3 years, median 1.7 years) were non-responders, with coronary aneurysms in 8 cases (giant aneurysms (>8 mm) in 4 cases). These 11 infants were treated a second time by immunoglobulins, but 6 cases (1.8 + 1.6 years, with two cases of severe ventricular dysfunction and 2 cases of fatal myocardial infarction) required an additive therapy with (oral or IV route) corticosteroids (2) and cyclophosphamide bolus (4) with or without repetitive plasmapheresis (4). Non-responder patients had their treatment onset later (p<0.0003) using higher dosages (p<0.005), a longer delay for fever or biological signs correction (p<0.02), a worsening of coronary lesions (p<0.05) with more coronary secondary aneurysms (p<.005). The aneurysms, more frequent at the second phase of the disease (p<0.0001) are associated with: a younger age (p<0.03), a lower weight (p<0.02), a later onset of treatment (p<0.03), prolonged fever or inflammatory syndrome (p<0.05), higher level of fibrinogene (p<0.02). The overall mortality (5.7%) is correlated with giant aneurysms (p<0.001), myocardial ischemia (p<0.0001), heart failure (p<0.0001), and lack of early response to treatment (p<0.003). CONCLUSION: immunoglobin therapy can be repeated. In case of severe forms, the use of corticosteroids, cyclophosphamide and plasmapheresis may be proposed.

Adolescent↗
PubMed2004

[Late presentation of type A aortic arch blockage with a large type I aortopulmonary window].

We report the case of a 39 day old infant, hospitalised for congenital cardiopathy associated with type A blockage of the aortic arch with a large type I aortopulmonary window. The infant was in cardiogenic shock with pulmonary systemic hypertension and a tightly stenosed arterial canal (< 2 mm). With no possibility of re-opening the arterial canal under PGE1 at this stage, complete repair was performed as an emergency. After section of the aortopulmonary window, it was closed on the pulmonary side with a patch of autologous pericardium. Repair of the aortic arch was performed without prosthetic material, under selective cerebral perfusion to protect the brain parenchyma, after mobilisation of the descending thoracic aorta, which was anastomosed directly with the distal part of the window and aortic arch. Recovery was uncomplicated, with no residual lesion at 6 month post-operative follow up. The late clinical presentation of this patient shows the effect of medical management without prior catheterisation, with operative techniques minimising peri-operative tissular ischaemia and conserving aortic and pulmonary growth potential.

Abnormalities, Multiple↗
PubMed2003

[General anaesthesia for Caesarean section in a patient with pulmonary atresia and an intact intraventricular septum].

We report the case of a 22-year-old primigravida patient presenting pulmonary atresia with intact ventricular septum, a rare congenital heart disease. Caesarean delivery was performed at 32 weeks of gestation because of moderate maternal function deterioration and foetal growth restriction. General anaesthesia was used because of the presence of lumbar Harrington rods. The administration of etomidate, celocurine and a continuous perfusion of remifentanil right from induction ensured haemodynamic stability and a rapid emergence and recovery.

Adult↗
PubMed2003

[Isolated congenital complete atrio-ventricular block].

The objective of this work was to study the long term evolution of a retrospective series of 54 patients affected with congenital isolated complete atrio-ventricular block (CAVB) and to analyse the value of the different methods used for surveillance. Our series included 54 patients affected with isolated CAVB, without associated cardiopathy, diagnosed at an average age of 5.3 +/- 5.5 years, of which 9 were in utero. The average duration of follow up was 14.5 +/- 9.6 years. During the evolution, a cardiac stimulator was placed in 41 patients (76%) at a relatively late average age of 13.3 +/- 9 years, significantly lower for CAVB diagnosed before the age of 1 year (9.6 +/- 7.4 years) than for those diagnosed after the age of 1 year (16.2 +/- 9.2 years) (p < 0.02). The approach was endocavitary in 39 cases and epicardial in 2 cases. The only 2 deaths in our series (4%) concerned 2 patients of 18 and 26 years already fitted with a stimulator. Three patients progressed to severe dilated cardiomyopathy despite implantation of a stimulator. The decisive arguments for implantation of a PM were clinical (11 patients), Holter ECG (25 patients), stress test (17 patients), electrophysiological investigation (5 patients), echocardiography (3 patients) and surgical intervention (2 patients). In conclusion, our study confirms the good prognosis of isolated congenital complete atrio-ventricular block, but underlines the possible progression in rare cases in spite of stimulation towards dilated cardiomyopathy for which the aetiology remains uncertain. Three quarters of the patients required a stimulator at a somewhat late age.

Adolescent↗
PubMed2003

[Cardiac stimulation in the infant and young child].

This study evaluates the problems and the evolution of cardiac stimulation in infants (aged < 3.5 years) by comparing the endocavity and epicardial routes in a retrospective series of 37 patients. Thirty seven patients aged 1.2 +/- 0.9 years treated with epicardial (n = 19) or endocavity (n = 18) stimulation were followed for 10.9 +/- 6.4 years (0.75-24). The 2 patient groups did not differ in age or weight. Four patients were lost to follow up, and 1 died. The functional duration of the first stimulator was not significantly different if the initial approach was epicardial or endocavity. The endocavity probes were introduced by venous denudation in 15 cases and by subclavian puncture in 3 cases. Fourteen of the 19 children fitted by the epicardial route went on to endocavity stimulation, of which 10 were at the first replacement. None of the 18 patients fitted by the endocavity route went on to epicardial stimulation. Out of 11 endocavity probe replacements and 9 atrialisations, the homolateral venous approach was always possible except in 2 cases. In conclusion, the results for the epicardial and endocavity routes are comparable. For technical reasons (calibre of the veins, size of the stimulator) it would appear reasonable if the endocavity route was used, making do initially with a mono chamber stimulation. The advances in the epicardial electrodes abolishes the major handicap (threshold elevation) of this approach which can be advocated when double chamber stimulation seems preferable.

Cardiac Pacing, Artificial↗
PubMed2002

[Twin-twin transfusion syndrome: report of two cases with hemodynamic complications].

CASE REPORTS: We report two cases of cardiac dysfunction in twin-twin transfusion syndrome (TTTS) evaluated with serial echocardiography. Two cases of TTTS were referred at 27 and 26 weeks. At delivery at 31 weeks, the first recipient twin had evidence of severe cardiac dysfunction with decreased ventricular function and transient systemic hypertension. There was polycythaemia. Favorable outcome was observed after treatment with arterial vasodilating (nicardipine) and inotropic agents (dobutamine, enoximone), and reduction of haematocrit. At 28 weeks the other recipient twin had cardiac dilatation with hypokinetic myocardium. These alterations were cured by dobutamine. CONCLUSION: These cases show that even severe cardiac dysfunction may be reversed after birth unlike in utero natural evolution.

Adult↗
PubMed2002

[Sports and congenital heart disease in the adult].

The practice of sporting activities is not only allowed, but also should be recommended for most adult patients affected by congenital cardiopathy. This permissive attitude should be based on a good understanding of congenital cardiopathy, whether operated or not, and its foreseeable evolution in adulthood. Aptitude for sport should be supported by a precise evaluation of the patient's functional, rhythmological and haemodynamic status at rest and under effort. Regular monitoring is often justified, and is greatly facilitated by the quantity and quality of modern non-invasive surveillance methods, the first of which are Doppler echocardiography and ergometric techniques. The stress test should be readily coupled with a study of ventilatory parameters under effort, as there are frequently disturbances of pulmonary exchange. Non-competitive sporting activity is generally implied for congenital cardiac patients, but there are exceptions in certain cases. Patients should generally be directed towards an endurance sport, undertaken without any spirit of competition, as a function of the results from effort tests (easy confrontation for this type of sport). It is only based on the tolerance of this basal activity that other sports are then authorized. It is illusory to demand systematisation for sporting aptitude in each type of cardiopathy as the situations encountered in practice vary from one individual to another. It is nevertheless possible to define a certain number of guidelines which are suitable for modification for each patient as a function of the clinical history, any associated vascular risk factors, and the patient's motivation. All said and done, absolute contra-indications to sport are rare. Relative contraindications to a type of sport or to a level of sporting activity as a function of the cardiopathy are encountered more and more. Nowadays the selective aptitude for certain sports should be discussed rather than the contra-indications. Under these conditions, sport can only be beneficial for adult congenital cardiac patients, and it plays a part in the prevention of the acquired risk of cardiopathy from which these patients have no escape.

Echocardiography, Doppler↗
PubMed2001

[Role of pediatric cardiologists in the management of neonates with congenital heart disease].

Whether diagnosed before birth or suspected in a newborn, congenital heart diseases rapidly require an evaluation by the pediatric cardiologist. The role of the pediatric cardiologist is threefold: 1) to realize a careful anatomical examination using color Doppler echocardiography; 2) in many cases to perform a palliative or curative therapeutic act based upon interventional catheterization, such as Rashkind atrioseptotomy or percutaneous balloon valvuloplasty; and 3) whenever necessary, to take a rapid decision of cardiac surgery in concert with the cardiac surgeon, and to prepare the child for surgery.

Cardiac Catheterization↗
PubMed2001

Paroxysmal tachycardia in children and teenagers with normal sinus rhythm and without heart disease.

The purpose of this study was to evaluate the value of esophageal programmed stimulation in children and teenagers with normal sinus rhythm ECG and normal noninvasive studies, having palpitations and syncope, and no documented tachycardias. Paroxysmal tachycardias are frequent in children and are often related to accessory connection. These tachycardias are sometimes difficult to prove. Transesophageal atrial pacing was performed at rest and during infusion of isoproterenol in 31 children or adolescents aged 9-19 years (16 +/- 3 years) with normal sinus rhythm ECG and suspected or documented episodes of paroxysmal tachycardia. Sustained tachycardia was induced in 27 patients, at rest in 13 patients, and after isoproterenol in 14 remaining patients. Atrioventricular nodal reentrant tachycardia was found as the main cause of paroxysmal tachycardia (22 cases). Six patients were followed by a vagal reaction and dizziness. These patients had spontaneous tachycardia with syncope. In three other patients, atrial fibrillation was also induced. Concealed accessory pathway reentrant tachycardia was identified in three patients. In two patients, a regular wide tachycardia with right bundle branch block morphology was induced; the diagnosis of verapamil-sensitive ventricular tachycardia was made in a second study by intracardiac study. In conclusion, atrioventricular nodal reentrant tachycardia was found as the main cause of symptoms in children with normal sinus rhythm ECG. Syncope is frequently associated and provoked by a vagal reaction. This diagnosis could be underestimated in adolescents frequently considered as hysterical because noninvasive studies are negative.

Adolescent↗