[Alveolar capillary dysplasia with misalignment of pulmonary veins: a fatal cause of refractory neonatal cyanosis]. / Dysplasie alvéolo-capillaire avec mésalignement des veines pulmonaires : une cause de cyanose néonatale réfractaire létale.

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare neonatal pathology that combines refractory hypoxemia with severe pulmonary arterial hypertension, and leads to death every time. Histologic examination of lung tissue confirms the diagnosis and is characterized by a decreased number of pulmonary capillaries, immature lobular development, and abnormal proximity between pulmonary arteries and veins. This abnormal proximity is responsible for an arteriovenous shunt. We report five cases confirmed by postmortem histology, which occurred over 14 years in Lower Normandy (France). The cumulative incidence is therefore of 1.8 for 100,000 births. In these five cases, the first symptoms appeared during the first hour of life and death occurred before 24h in four of five cases. The patient with the longest survival had mild histological lesions and delayed emergence of the first symptoms. Genitourinary and gastrointestinal anomalies were associated with ACD/MPV in two cases, and bilateral pulmonary hypoplasia in three cases. Optimized invasive ventilation, pulmonary vasodilators, vasoactive drugs, and pulmonary surfactant did not improve survival. Extracorporeal membrane oxygenation (ECMO) was not used. We present a review of the literature on ACD/MPV, a clinical and histological entity little known to both clinicians and pathologists, whereas a premortem diagnosis is possible and genetic counseling in affected families can be suggested.

[Verapamil intoxication: beware of the delayed effect]. / Intoxication aigüe au vérapamil : attention à l'effet retard.

Intoxication with calcium channel inhibitory drugs is rare but mortality rates reach 10 %. We report the case of a 5-year-old girl who had ingested five 240-mg tablets of extended-release verapamil (VLP) and a tablet of bromazepam. Thirty hours after the ingestion she had a vasoplegic shock, heart conduction disorders, and metabolic complications. She was treated in pediatric intensive care with continuous epinephrine and insulin and recovered completely 60h after the ingestion. This case underlines the danger of calcium channel blocker overdose, increased by the extended-release mechanism: the drug effect is to slow down gastric motility - which explains the latency of the symptoms - but this also increases the drug absorption, inducing a vicious circle. These mechanisms in calcium blocker intoxication need to be kept in mind in order to decrease the mortality of such accidents.

Longitudinal left ventricular strain impairment in type 1 diabetes children and adolescents: a 2D speckle strain imaging study.

AIM: Type 1 diabetes (T1D) involves complex metabolic disturbances in cardiomyocytes leading to morphological and functional abnormalities of the myocardium. The relationship between T1D and cardiac structure and function in children is not well established. Our study investigated whether T1D is associated with early subclinical myocardial disturbances in children and adolescents, and whether the state of metabolic control and diabetes duration are influential factors. METHODS: Standard echocardiography, tissue Doppler imaging (TDI) and two-dimensional (2D) strain imaging were prospectively performed in 100 T1D children (age: 11.3 ± 3.6 years, 52 boys) and compared with 79 controls. RESULTS: The diabetic and control children were comparable with respect to age, gender, heart rate and blood pressure. There were no significant differences between the two groups in left ventricular (LV) ejection fraction, LV remodelling and TDI parameters. Conventional mitral Doppler demonstrated significantly fewer diastolic filling abnormalities with an early filling wave in the diabetes group. Global longitudinal strain (GLS) was also significantly lower in the T1D children, while circumferential strain and radial strain did not differ. GLS correlated with HbA1c (r=0.52; P<0.01), but there was no correlation with diabetes duration. CONCLUSION: Our results suggest that LV longitudinal myocardial deformation is decreased in young patients with T1D, and glycaemic control may be the main risk factor for these changes. Further follow-up is now necessary to precisely determine the clinical significance of these myocardial changes detected by 2D strain imaging in T1D children.

Three dimensional echocardiographic assessment of multiple rhabdomyoma in newborn.

BACKGROUND: Cardiac rhabdomyomas represent the most common primary cardiac tumour in children and are strongly associated with tuberous sclerosis complex. RESULTS: We reported a newborn for whom three-dimensional echocardiogram, with multiplane mode, real-time imaging, full volume and i-slice view, allowed detailed visualisation of multiple highly echogenic and well-circumscribed cardiac rhabdomyoma. CONCLUSIONS: Three-dimensional imaging allowed a better definition of the tumour characteristics and provided a better delineation of the spatial relationship of the mass with a tomographic perspective. Three dimensional imaging may facilitate a possible operative planning and should be included in cardiac mass evaluation and follow-up.

[Tricuspid valve endocarditis after umbilical piercing]. / Endocardite tricuspide après un piercing ombilical.

We report a case of a 16 years old girl who was affected by a septicemia with a septic arthritis of the wrist and a tricuspid endocarditis, 3 months after a navel piercing. The blood culture and the liquid of puncture showed Staphylococcus aureus. A treatment prolonged by intravenous antibiotics permitted a progressive regression of endocarditis, without surgical intervention. The piercing must be achieved in conditions of strict asepsis, it must be advised against to the patient with congenital heart disease. In case of realization of a piercing an antibiotic prophylaxis must be achieved, while using preference pristinamycin in case of cutaneous piercing and amoxicillin in case of mucous piercing. Indeed, infective endocarditis after body piercing are more often brought back with a generally sterner evolution that in our observation.

[Fulminant influenza type A associated myocarditis: a fatal case in an 8 year old child]. / Myocardite fulminante à virus Influenza A: à propos d'un cas fatal chez une enfant de 8 ans.

Fulminant Influenza type A-associated myocarditis are very rare in children. The clinical presentation is non specific, like flu illness, cardiogenic shock or sudden cardiac arrest. We report the case of a eight years old girl with a fulminant Influenza A-associated myocarditis with a fatal evolution despite the use of an extracorporeal membrane oxygenation (ECMO). The aim of this observation is to remind that influenza in children, usually considered as a benign illness, can exceptionally be complicated by a fulminant myocarditis. Because the possibility to recover a full myocardial function, the persistence of severe heart failure despite the medical treatment should conduct rapidly to a mechanical circulatory assistance.

[Acute myocarditis with Toxoplasma gondii. A case report in a newborn]. / Myocardite aiguë à toxoplasme. A propos d'un cas survenu chez un nourrisson.

Acute myocarditis with Toxoplasma Gondii in immunocompetent patients is rare and the paediatric cases touch old children. These myocarditis lead sometimes cardiac insufficiency and sometimes mimic a myocardial infarction. The evolution is often favorable, even when there is no pest-destroying treatment. We report a case of myocarditis toxoplasma Gondii with which has occurred in a 11 month and half old infant, whose evolution was favorable with a symptomatic and pest-destroying treatment. The interest of this observation is related to the scarcity of acute myocarditis caused by toxplasmosis in infant without immunoinsufficiency.

[Preoperative mortality in transposition of the great vessels]. / Mortalité préopératoire de la transposition isolée des gros vaisseaux.

UNLABELLED: The aim of the study was to report the incidence and causes of preoperative deaths in isolated transposition of the great vessels and to describe the clinical findings in these neonates. PATIENTS AND METHODS: In five French centers of pediatric cardiology, data of all the neonates with isolated transposition of the great vessels who died before arterial switch operations between January 1986 and June 1996 were obtained from reviewing hospital files, echocardiography records and autopsy reports. RESULTS: Among 199 neonates with transposition of the great vessels, 20 (9.9%) died before surgery. The death was related to intracranial haemorrhage in one premature neonate, severe and early hypoxemia in 13 full-term patients (group A) and later sudden collapse in six patients (group B). In group A, the symptoms occurred within 20 minutes after the birth and included cyanosis (n = 12), acute respiratory distress (n = 8), and shock (n = 4). Despite assisted ventilation (n = 13), bicarbonate infusion (n = 12), prostaglandin E1 (n = 7), inotropic drugs (n = 5) and balloon atrioseptostomy (n = 7), death occurred at the median age of five hours. The patent foramen ovale was absent or tiny in ten patients, normal in one patient and not specified in two patients. The ductus arteriosus was patent in ten patients and not specified in three patients. In group B, the neonates were initially in a good hemodynamic condition. Unexplained death occurred between two and five days after the birth: one infant with a large patent foramen ovale did not receive prostaglandin E1, four patients died a few hours after an angiographic study or a balloon atrioseptostomy was performed in a catheterization laboratory, and one child suffered from a cerebral anoxia due to a tightened cord. CONCLUSION: We conclude that the high preoperative mortality rate in isolated transposition of the great vessels is mainly due to absent or small atrial shunt. These findings suggest that only prenatal diagnosis of transposition of the great vessels with immediate balloon atrioseptostomy could avoid a fatal outcome.

[Cardiac involvement during classic hemolytic uremic syndrome]. / Atteinte cardiaque au cours du syndrome hémolytique et urémique typique.

BACKGROUND: Cardiac involvement rarely occurs in classic hemolytic uremic syndrome (HUS); it is often fatal. CASE REPORTS: The first patient, a 21-month-old boy, developed classic HUS with acute renal failure. Peritoneal dialysis was performed for 20 days. On the 10th day of dialysis, myocardial infarction occurred, probably related to coronary thrombus. The patient was given heparin and antibiotics because of an unexplained fever. The outcome was favorable despite antero-apical cardiac necrosis, and moderated chronic renal failure. The second patient, a 24-month-old girl, also showed a classic HUS, which required peritoneal dialysis for 10 days. Dilated cardiomyopathy with cardiac failure appeared on the 4th day of dialysis, not related to the volume overload and metabolic consequences of the acute renal failure, such as systemic hypertension or ineffective dialysis. On the 5th day of dialysis neurological involvement appeared. Neurological, cardiac and renal outcome was favorable. The third patient, a 25-month-old girl, developed a classical HUS, requiring peritoneal dialysis for 25 days. No cardiac insult appeared during the acute phase of the disease. After dialysis, the child had chronic renal failure (creatinine clearance: 15 mL/min/1.73 m2). Dilated cardiomyopathy appeared 3 months later, without definite etiology. The outcome was favorable with digoxin treatment. CONCLUSION: A cardiac involvement should also be searched for in the acute phase of HUS and several months later.

[Glucocorticoid-induced hypertrophic cardiomyopathy in premature infants: apropos of 4 cases]. / Myocardiopathie hypertrophique induite par les glucocorticoïdes chez le prématuré: à propos de quatre cas.

BACKGROUND: The steroidal treatment used to prevent bronchopulmonary dysplasia (BD) in the preterm babies may be the cause of several complications, one of them being hypertrophic cardiomyopathy. CASE REPORT: Four infants developed hypertrophic cardiomyopathy during glucocorticoid (dexamethasone and/or betamethasone) treatment for bronchopulmonary dysplasia. In one of them, septal hypertrophy led to left ventricular outflow tract obstruction and congestive heart failure. All four were premature infants born after 2 weeks of gestation and weighing 780 to 1,080 g. The first echocardiographic changes appeared between the 4th and 15th day of the glucocorticoid course when the cumulated dose was respectively 1.82-1.87-3.51 and 3.86 mg/kg. Hypertrophic cardiomyopathy resolved completely between 2 and 4 weeks after cessation of the treatment. CONCLUSION: The glucocorticoid dosage to prevent BD should be reduced to 0.3 mg/kg/j and the myocardial function should be monitored by repeated echocardiograms during the first 15 days of treatment.

Operation for atrial septal defect through a right anterolateral thoracotomy: current outcome.

BACKGROUND: Operative closure of atrial septal defect is today considered a high-benefit and low-risk operation. Patients are often young and sensitive to the cosmetic results of the procedure. The midline scar of median sternotomy may be unsightly and can provoke dissatisfaction and psychological distress. For cosmetic reasons, an alternative operative approach, such as right anterolateral thoracotomy, can be proposed, with better aesthetic results and without increasing operative risks. METHODS: Our study reviews retrospectively the long-term results of a consecutive series of 56 young patients in whom the atrial septal defect was closed through a right submammary approach. The indication for this approach was isolated atrial septal defect in female patients with complete development of the breasts. RESULTS: In-hospital morbidity included three postpericardiotomy syndromes with one operative drainage for a moderate pericardial effusion (subxiphoid approach); 6 patients had supraventricular tachycardia in the early postoperative period. One patient presented with a symptomatic supraventricular arrhythmia and was treated medically for atrial flutter or fibrillation. Follow-up ranged from 12 to 240 months and included 41 of 57 patients. There were no early or late deaths. All patients were in normal sinus rhythm and free of symptoms, in New York Heart Association functional class I. Electrocardiography results showed 4 patients with first-degree atrioventricular block and 5 with complete right bundle branch block. Echocardiographic study results showed 3 patients with a trivial residual shunt. There were no other late complications. Breast volume and symmetry and the character of the scar were evaluated objectively by a physician and subjectively by a multiple-choice questionnaire completed by the same patients. The answers suggested that the patients' subjective impressions were at least commensurate with the objective findings. Most of the patients perceived the cosmetic results as good or excellent. No serious psychological problems related to the scar were found. CONCLUSIONS: Right thoracotomy incision is a safe alternative approach to median sternotomy to repair isolated atrial septal defect in young female patients.

[Late supraventricular arrhythmia complicating Fontan or cavopulmonary type procedures. Apropos of 7 cases]. / Troubles du rythme auriculaire tardifs compliquant les opérations de type Fontan ou de type cavopulmonaire. A propos de 7 observations.

The authors report 7 cases of late arrhythmias after atriopulmonary (5 cases) or total cavopulmonary (2 cases) bypass procedures. There were 6 cases of atrial flutter and one case of atrial tachycardia. The condition presented with cardiac failure in 5 cases. In 2 patients, atrial flutter caused syncope or dizziness. The arrhythmia was reduced by atrial stimulation (3 cases) or by cardioversion (1 case). Prevention of recurrence with oral amiodarone was effective in all cases but was responsible for secondary effects in 4 cases. In one patient, recurrence of atrial flutter was complicated by right atrial thrombosis with cerebral embolism. Five patients were reoperated after cardiac catheterisation and angiography. Surgery consisted of resection of a stenosis of the anastomosis in one case, and the transformation of atriopulmonary anastomosis into a total cavopulmonary bypass because of a very dilated right atrium without stenosis in 4 patients. The immediate postoperative period was complicated by a recurrence of the arrhythmia in 3 children not treated by antiarrhythmic therapy. At long-term, one patient died 6 months after withdrawal of amiodarone therapy of recurrence of atrial flutter. Five of the 6 survivors are treated with amiodarone or a betablocker; 3 have had pacemaker implantation for severe bradycardia. Late atrial arrhythmias complicating atrio- and cavopulmonary bypass procedures carry a risk of cardiac failure and sudden death. When diagnosed, the patient should be investigated for stenosis of the anastomosis but severe dilatation of the right atrium is often the only finding. After restoration of sinus rhythm, maintenance antiarrhythmic therapy should be continued indefinitely.

Severe lupus with infectious thyroiditis and lethal cardiomyopathy.

Clinical cardiomyopathy is an uncommon complication of systemic lupus erythematosus (SLE) and intracavitary thrombosis is rare. We describe a patient with active SLE who developed rapidly progressive cardiomyopathy, the fatal course of which was complicated by an intracavitary thrombus. Repeat cardiac echography studies and the endomyocardial biopsy proved to be helpful in diagnosing the lupus myocarditis and aided the regulation of therapy. Furthermore, the patient presented an acute suppurative thyroiditis never before described, to our knowledge, in SLE.

[Percutaneous aortic valvuloplasty in children, non invasive evaluation and criteria of success]. / La valvuloplastie aortique percutanée chez l'enfant, évaluation non invasive et critères de succès.

The results of percutaneous aortic valvuloplasty in 15 children (average 8.4 years) were evaluated by echocardiography and non-invasive criteria of the indication of this procedure were defined. The aortic valve was unthickened in 7 patients (Group I) and dystrophic in the other 8 (Group II). Valvuloplasty decreased the transvalvular pressure gradient by 47 +/- 33%. Aortic regurgitation was aggravated in 4 patients. The reduction in pressure gradient was significantly greater in Group I than in Group II (64 +/- 19% vs 31 +/- 35%, p < 0.05). In Group I, in contrast to Group II, the results remained stable after an average follow-up of 14.5 months. When the maximal instantaneous pressure gradient on Doppler examination was < 80 mmHg, the peak-to-peak gradient at catheterisation was on average 21% less, whereas when the Doppler gradient was 80 mmHg or more, the percentage difference was only 8%. The myocardial mass index was over the 95th percentile in 7/8 patients with gradients > 80 mmHg whilst it was only increased in 1 patient with a gradient < 80 mmHg. Electrocardiographic LVH was observed in 6 of the 8 patients with a gradient > 80 mmHg but in none of the others. Although valvuloplasty reduced the transvalvular pressure gradient, the results were much less satisfactory in the dystrophic valves. The following indications were proposed for this techniques: a Doppler maximal pressure gradient > or = 80 mmHg, associated with at least one criterion of left ventricular hypertrophy.

[A severe form of vitamin D deficiency with hypocalcemic cardiomyopathy]. / Une forme sévère de carence en vitamine D avec cardiomyopathie hypocalcémique.

The authors report on a case of cardiomyopathy with congestive heart failure in an infant with severe hypocalcemia related to vitamin D deficient rickets. The heart failure was successfully treated with calcium gluconate and vitamin D, associated with dobutamide.

[Correlations between the different criteria of late potentials and results of programmed ventricular stimulation after myocardial infarction]. / Corrélation entre les différents critères de potentiels tardifs et les résultats de la stimulation ventriculaire programmée au décours de l'infarctus myocardique.

Five to ten per cent of survivors of acute myocardial infarction die within two years. The majority of these deaths are sudden and are attributed to a lethal ventricular arrhythmia. This is usually ventricular tachycardia degenerating to ventricular fibrillation. These post-infarction tachycardias are generally due to reentry. They require an anatomic arrhythmogenic substrate, a zone of delayed conduction. This can be detected as late potentials on signal averaged ECG. The triggering of a significant ventricular arrhythmia by ventricular stimulation is closely correlated to the occurrence of a severe ventricular arrhythmia in the months following infarction. Programmed ventricular stimulation could, therefore, help to identify patients requiring close follow-up and/or preventive antiarrhythmic therapy, but cannot be offered to all patients because of its invasive nature. Seventy nine post-infarction patients were studied prospectively. All underwent coronary angiography, signal averaging electrocardiography, and programmed ventricular stimulation at least 15 days after infarction. Fifty five patients had at least one criterion of late potentials (QRS duration > or = 110 ms and/or amplitude of the last 40 ms < 27 microV and/or duration of potentials of under 40 microV > 37 ms). Twenty four patients had no late potentials. The results of programmed stimulation were estimated to be positive when sustained or unsustained monomorphic ventricular tachycardia was triggered, and negative when ventricular fibrillation, ventricular flutter unsustained polymorphic ventricular tachycardia or no arrhythmia could be induced. Programmed ventricular pacing triggered 15 significant events, 17 unsustained polymorphic ventricular tachycardias, 13 ventricular flutters and 11 ventricular fibrillations. The exploration was negative in 23 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Usefulness of oral sotalol for the treatment of junctional ectopic tachycardia.

An infant with junctional ectopic tachycardia diagnosed at three weeks of age was treated with oral sotalol. At the first echocardiogram the shortening fraction was decreased. After one day of treatment the ventricular rate decreased from 230 to 130 beats per minute. Five months later sinus rhythm was restored, shortening fraction was normal and sotalol was withdrawn without relapse.

Effectiveness of oral sotalol for treatment of pediatric arrhythmias.

Sotalol is a beta blocker with class III activity. Few investigators have reported its use in pediatric patients. From August 1985 to May 1990, 66 patients (mean age 8.7 years; range 9 days to 24 years), including 14 infants aged less than 3 months, were treated with oral sotalol alone (n = 46) or in association with digoxin (n = 20). Supraventricular reentrant tachycardia was present in 38 patients (20 with documented preexcitation), atrial flutter in 10 and atrial ectopic tachycardia in 7. Three patients had other types of supraventricular tachycardia. Tachycardia was of ventricular origin in 6 patients and both of supraventricular and ventricular origin in the remaining 2. Mean dose of oral sotalol was 135 mg/m2/day given in 2 doses. Congenital heart disease was present in 28 patients, 14 with previous cardiac surgery, mostly at the atrial level. Prior treatment with 1 or more antiarrhythmic agent had been unsuccessful in 83% of patients. Mean duration of treatment was 13.3 months (range 2 months to 5 years). Overall, treatment was successful in 79% of cases. Highest rate of success was observed in patients with supraventricular reentrant tachycardia with or without preexcitation (89%) and in those with atrial ectopic tachycardia (85.5%). Atrial flutter could be controlled in 60% of cases. Sotalol seemed less effective in ventricular tachycardia with a complete control of the arrhythmia being achieved in only 17%; however, it decreased the number of runs of ventricular tachycardia and the number of ventricular premature complexes in 50% of patients. There were no adverse effects in 89% of patients.(ABSTRACT TRUNCATED AT 250 WORDS)