The natural history of cardiac rhabdomyoma with and without tuberous sclerosis.

The aim of the present study is to contribute to the knowledge of the natural history of cardiac rhabdomyoma in children with and without tuberous sclerosis. In a retrospective study, 33 children with cardiac rhabdomyoma were collected from three pediatric cardiology centres. In 30/33 patients tuberous sclerosis was associated. High prevalence of cardiac rhabdomyoma was found in infancy, with 21/23 detected before the age of 1 year, and 11/33 before 1 month of age. Cardiac manifestations were present in 19 patients: cardiac rhythm disturbances were detected in 13; in 6/33 a Wolff-Parkinson-White syndrome was documented, of which 4 presented paroxysmal arrhythmias. Obstructive or regurgitative phenomena were present in 5; and in 2 patients surgical removal proved necessary. With the exception of one tumoural mass in the right atrium, all 77 tumours were located somewhere in the ventricles, including at atrio-ventricular valve level. Because of spontaneous regression of most of the tumoural masses, treatment should at first be symptomatic, while surgical removal is required only in life-threatening conditions, as documented in 2 of our 33 patients.

Early balloon dilatation of the pulmonary valve in infants with tetralogy of Fallot. Risks and benefits.

BACKGROUND: Balloon dilatation, an established treatment for pulmonary valve stenosis, remains a controversial procedure in tetralogy of Fallot. METHODS AND RESULTS: Balloon dilatation of the pulmonary valve was performed in 19 infants with tetralogy of Fallot. Its effects on the severity of cyanosis, the growth of the pulmonary valve and pulmonary arteries, and the need for transannular patching were evaluated. Clinical, echographic, angiographic, hemodynamic, and operative data were analyzed. The procedure was safe in all, without significant complications. After balloon dilatation, systemic oxygen saturation increased from a mean value of 79% to 90%. This increase proved to be short-lasting in 4 patients, who required surgery before the age of 6 months. Balloon dilatation increased pulmonary annulus size in each case, from a mean value of 4.9 to 6.9 mm (P < .001). This gain in size remained stable over time, with a mean Z score of -4.8 SD before dilatation, -3.1 SD immediately after the procedure, and -2.7 SD at preoperative catheterization (P < .001). Pulmonary artery dimensions remained unchanged immediately after balloon dilatation but increased at follow-up from a Z score mean value of -2.5 to -0.06 SD and from -2.2 to 0.04 SD for right and left pulmonary arteries, respectively (P < .001). At the time of corrective surgery, the pulmonary annulus was considered large enough to avoid a transannular patch in 69% of the infants. This represented a 30% to 40% reduction in the need for a transannular patch compared with the incidence of transannular patch expected before balloon dilatation. CONCLUSIONS: Pulmonary valve dilatation in infants with tetralogy of Fallot is a relatively safe procedure and appears to produce adequate palliation in most patients. It allowed the growth of the pulmonary annulus and of the pulmonary arteries, resulting in a mean gain of 2 SD for those structures.

[Percutaneous embolization of a pulmonary arteriovenous fistula in a child]. / Embolisation percutanée d'une fistule artérioveineuse pulmonaire chez un enfant.

BACKGROUND: Pulmonary arteriovenous fistula is a rare vascular abnormality in children that is usually treated by lobectomy. This report describes a case that was improved by coil embolization. CASE REPORT: A thoracic X-ray was routinely taken before amygdalectomy in a 6 year-old girl. It showed a consolidation area in the left superior lobe that persisted despite treatment with erythromycin for 3 weeks. Bronchoscopy was normal, but study of blood gases showed a refractory hypoxemia with moderate cyanosis and hemoglobin at 16.2 g/dl. Right-heart catheterization and angiography showed a pulmonary arteriovenous fistula that was treated by coil embolization. A control investigation 3 months later showed normal arterial oxygen saturation, hemoglobin at 14.5 g/dl and reduced pulmonary condensation. CONCLUSION: Pulmonary arteriovenous fistula may show few symptoms. Coil embolization appears to be a useful initial non-aggressive treatment.

Morphometrical quantification of brain edema related to experimental multiple micro-infarcts in mice: assessment of neurotropin effect.

Cerebral micro-infarcts were induced in mice by injecting a standardized suspension of latex microbeads into one internal carotid artery. The animals were sacrificed after either 2 or 4 days. The importance of the lesions induced in each brain was morphometrically quantified on stratified samples of microscopic serial sections, whereas edema was evaluated in the same sections by comparing the section area of the injected hemisphere with that of the non-injected side. Lesion density and brain edema were found to significantly correlate. Neurotropin, an inhibitor of kinin release, was curatively administered twice a day to 20 experimental animals which were compared to 20 saline-treated counterparts. In the neurotropin-injected mice, after 4 days the global amount of cerebral edema was significantly smaller than that of the controls and the slope of the lesion/edema regression line was reduced, indicating a lower amount of edema for a given lesion. These findings support the theory that the kallikrein-kinin system plays a major role in the development of the vasogenic late phase of ischemic brain edema.

Doppler and 2D echocardiographic diagnosis of congenital coronary artery fistulae to the right cardiac chambers: report of 3 cases.

We present three cases of coronary artery fistulae to the right cardiac chambers. The first was a 2-day-old neonate in congestive heart failure: 2D and Doppler echocardiography revealed a dilated proximal left coronary artery and a fistulous connection to the right atrium. The other two patients, respectively 4 and 3 years old, were asymptomatic and presented with a continuous heart murmur: a left coronary artery fistula into the right ventricle was detected by ultrasound in one, and a dilated proximal right coronary artery in the other. The diagnosis was confirmed in all three patients. The first patient was operated upon at 18 months of age; the second patient is awaiting surgery, and in the third patient the fistula was ligated at the age of 3 years. The possibility of ultrasound diagnosis without invasive procedures is suggested.

Neurotropin effects on stress and performance of airline pilots in simulated flight conditions.

Effects of Neurotropin, an analgesic and antiallergic drug, have been compared to those of an inert, undistinguishable placebo in a double-blind study on twelve experienced Sabena (Belgian national airline) Boeing-737 captains who were requested to operate in unexpected scenarios of emergency and ILS instrument approaches in a flight simulator of their company. The test determined, in both medication groups, a significant increase in heart rates reflecting stress in these experimental conditions. The time course variations of heart rate showed values significantly lower in the Neurotropin group in comparison to the placebo group. Nervosity and workload were subjectively determined by pilots' self-assessment and workload was found lower under Neurotropin. Errors and quality of work, as evaluated by the Sabena Chief Instructor, were not significantly different in both groups. Instrument performances were recorded and analyzed by computer and the concordance between real and ideal flight paths was significantly better in the Neurotropin group. The observed Neurotropin effects of reducing the manifestation of stress and maintaining the pilots' performances might be due to a modulation of CNS monoamines, similar to that observed in animals submitted to Specific Alternation of Rhythm in Temperature (SART) stress model. This finding could be therapeutically relevant to the aggravation by anxiety of chronic pain and allergy.

Action of neurotropin on cold-induced pain in normal volunteers: a double-blind placebo study.

The analgesic action of neurotropin, a biological compound widely used in Japan with a record of very limited side-effects, was tested in 8 Caucasian normal volunteers. For the pain test, the subjects were requested to immerse the right hand in ice-cold water and to report the appearance of the following sensations: pain threshold (PThr) and tolerance to pain (PTol). Pain sensitivity range (PSR) was calculated by difference between PThr and PTol expressed in s from beginning of immersion. Neurotropin tablets or an indistinguishable inert placebo were administered according to a randomized double-blind cross-over design and their influence in the pain test was investigated during 2 daily sessions. The effects of both treatments were assessed by calculating the difference between initial and post-medication values for each pain response parameter. Under placebo the means of all effects were found to be negative, reflecting the appearance of hyperalgesia under repetitive pain conditions, with neurotropin, the effects on PTol and PSR were positive and significantly different from those of placebo. These results are discussed in terms of possible mechanisms of action: neurotropin analgesic activity could be linked to its kinin release-inhibiting properties or to a reduction in vaso-constriction and hyperalgesia related to an effect on catecholamines.

Surgical treatment of subvalvular aortic stenosis. Long-term results.

From 1966 till May 1988, 53 patients underwent surgery for fixed subaortic stenosis. Subvalvular obstruction was isolated in 27 patients (Group I) and associated with aortic valve lesions in 26 (Group II). A membranous stricture was documented in 5 patients and a fibromuscular ring in 48. Excision of the ring and myectomy were performed in all patients, and an associated aortic valve replacement or reconstruction in 7 and 4 patients respectively. There were no hospital deaths. Follow-up evaluation in 50 patients ranged from 6 months to 22 years. Eight patients had to be reoperated upon (1 from group I, 7 from group II): aorto-ventriculoplasty was performed in 3, aortic valve replacement with redo myectomy in 3 and mitro-aortic valve replacement in 2. One of them had 2 reoperations. Functional status at the time of the last outpatient visit was most satisfactory. Continued evaluation remains necessary as obstruction may reappear despite the absence of symptoms.

Psychological effects of Neurotropin in man under normal conditions and after sleep deprivation: a double-blind placebo study.

Psychological effects of Neurotropin--a widely used analgesic in Japan--have been evaluated in a double blind cross-over study on volunteers belonging to the nursing staff of a hospital under sleep deprivation or not. Each subject was asked to record his feelings by self-assessment on scales registering the following states: fatigue-/rest+, mental stress-/relaxation+, cold sensation-/comfort+, dissatisfaction-/satisfaction+. Neurotropin was shown to be devoid of demonstrable psychological effects in the subjects under normal sleeping conditions. After sleep deprivation, Neurotropin globally determined significantly more psychological evolutions towards positives+ and less towards negative- mood than inactive placebo. From the self-assessed feelings, euphoria was unaffected, whereas Neurotropin significantly reduced fatigue and improved relaxation and comfort. These results are compared with the observations made on animals submitted to Specific Alternation of Rhythm in Temperature (SART) where Neurotropin relieves such symptoms as hyperalgesia. This model for experimental pain study corresponds to a depletion of brain serotonin and catecholamines. The effects of Neurotropin on exhausted volunteers could, as in the SART model, be due to an inhibition of monoaminergic depletion. The relevance of this finding to some association of chronic pain and psychogenic factors is to be considered.

Orthotopic heart transplantation eleven years after left pneumonectomy.

Orthotopic heart transplantation was performed in a 21-year-old medical student 11 years after left pneumonectomy for a rhabdomyosarcoma. The cardiomyopathy was the result of the administration of doxorubicin (Adriamycin). The surgical procedure was largely facilitated as a result of an in-hospital donor and the absence of major adhesions. The early postoperative course was mainly uneventful. The patient is doing well 9 months after operation, without any episode of rejection or infection.

[Congenital heart diseases and obstructive pulmonary vascular diseases in Down's syndrome. Apropos of 142 children with trisomy 21]. / Cardiopathies congénitales et maladie vasculaire obstructive pulmonaire dans le syndrome de Down.

Trisomy 21 accounts for 3 p. 100 of reasons for admission to the Paediatric Cardiology unit of the St Luc University Clinics, Brussels. In a series of 142 cardiac children with trisomy 21 evaluated by catheterization between 1969 and 1987, 54 p. 100 of the cardiac malformations observed consisted of persistent common atrioventricular canal (complete in 45 p. 100 of the cases). The other heart diseases were ventricular septal defect (23 p. 100), atrial septal defect of the ostium secundum type (10 p. 100) and tetralogy of Fallot (9 p. 100). In 40 p. 100 of the patients other cardiovascular abnormalities were associated with these predominant intracardiac shunts. These findings were in agreement with those usually reported in the literature. At the time of investigation (mean age 24 months), pulmonary vascular resistance had already reached a pathological level in 88 p. 100 of the cases. Oxygen tests only slightly improved these results, which suggested that the conditions were favourable to the early development of a pulmonary obstructive vascular disease in Down's syndrome, thus darkening the prognosis of congenital heart disease in mongoloid children. In this series to overall mortality rate of corrective surgery was 23 p. 100. The risk was maximum in infants aged less than 3 months with severe and rapidly symptomatic lesions. The outcome in patients successfully operated upon was satisfactory, with benign residual lesions.(ABSTRACT TRUNCATED AT 250 WORDS)

Long-term follow-up of the Senning operation for transposition of the great arteries in children under 3 months of age.

Twenty-six patients aged 6 days to 3 months (mean 57 days) underwent a Senning procedure for transposition of the great arteries. Twenty-two had intact ventricular septum and four had a small ventricular septal defect. They were followed up for 1 month to 8 years (mean 4 years). There were no late deaths. At late examination, 25 patients were asymptomatic and there was no clinical or echographic evidence of caval or pulmonary venous obstruction. Growth was normal in all but two patients. Neurologic assessment was abnormal in eight patients. The electrocardiogram showed sinus rhythm in 22 patients and asymptomatic arrhythmias in four. Twenty-three patients underwent cardiac catheterization and angiographic studies 2 to 72 months postoperatively (mean 15 months), which demonstrated effective left and right atrial contraction. An atrial shunt was noted in one patient and a ventricular shunt in one. Two infants (8%) had a residual left ventricular outflow tract obstruction (gradients of 26 and 37 mm Hg). Two had mild superior vena caval obstruction (gradients of 4 and 5 mm Hg). We conclude that the Senning procedure can be performed in early infancy with good results and a low incidence of late complications.

[Left ventricle in Noonan's syndrome. Electro-vecto-echo and angiocardiographic aspects]. / Le ventricule gauche dans le syndrome de Noonan. Aspects électro-vecto-écho et angio-cardiographiques.

The electrocardiographic features of Noonan's syndrome have been known for several years, but the discordance between these electrical findings and the underlying haemodynamic disorders remains unexplained. In an attempt to elucidate the genesis of electrical abnormalities, we present here a retrospective study of 14 children with Noonan's disease, aged from a few days to 16 years and evaluated by electrocardiography, vectography, one- or two-dimensional echocardiography, angiography and His bundle electrophysiology. The electrocardiographic abnormalities observed concerned ventricular depolarization and intracardiac electric conduction with, notably, a QRS axis directed towards the right upper part of the electric field and a first degree infra-hisian atrioventricular block (His bundle potentials). Vectography showed in some cases an image of inferior pseudo-necrosis due to the absence of initial inferior forces; this image is highly characteristic. In other cases the QRS loop showed an image of left segmental block which is unusual in this type or cardiac pathology (pulmonary stenosis with or without atrial septal defect of the ostium secundum type).

Echocardiography in neurological disorders.

Numerous neurological disorders are associated with myocardial disease or involve the cardiovascular system. Echocardiography thus proves quite helpful in the evaluation and management of patients with problems such as hydrocephalus, tuberous sclerosis, Friedreich ataxia, mitochondrial encephalo-myopathies, Werdnig-Hoffmann disease, convulsive disorders, syncope, central nervous system infections, etc.

Intraoperative echocardiographic control during ventriculoatrial shunt placement. Technical note.

A method for positioning the cardiac end of a ventriculoatrial shunt with the aid of echocardiography is described. This simple procedure has resulted in safe and accurate shunt placement in infants.

[Severe hypertrophic myocardiopathy in newborn infants. Intra-erythrocyte calcium and the effect of lidoflazine, a calcium-channel inhibitor. Apropos of 2 cases]. / Myocardiopathie hypertrophique sévère du nouveau-né. Calcium intra-érythrocytaire et effet de la lidoflazine, inhibiteur d'entrée calcique. A propos de deux cas.

Two cases of severe hypertrophic obstructive cardiomyopathy in the neonate are reported. The first case was poorly tolerated because of predominant stenosis of the right ventricular outflow tract causing right to left shunting through a patent foramen ovale. The second case presented with severe syncope at 6 weeks of age. The first patient was treated with propranolol without any success. Regression of the clinical and electrocardiographic signs was obtained in both cases with lidoflazine, within a few weeks. Red blood cell calcium concentrations were abnormally high in both patients before treatment and returned to normal levels with clinical and echocardiographic improvement. Abnormal accumulation of intracellular calcium in the myocardium is a possible mechanism of this cardiomyopathy. The relation between this type of accumulation and the effects of catecholamine discharge are recalled. The possible initiating role of an enzymatic abnormality of calcium entry is discussed: the chaotic geometric alignment of the myocardial fibres, characteristic of this type of hypertrophy, would therefore be a secondary phenomenon. Once constituted, the hypertrophy would then become irreversible. However, it may be possible to reverse it in the neonate by calcium antagonists as suggested by these two cases.

Myxomatous degeneration of the mitral valve in a child with Turner syndrome and partial anomalous pulmonary venous return.

This a report of myxomatous mitral valve degeneration in a child with Turner syndrome. The diagnosis was first suspected at 7 months of age. At 5 years of age, ultrasonic and angio-cardiographic confirmation prompted a successful mitral valve replacement with a Starr prosthesis. The child also had partial anomalous pulmonary venous return and a dysplastic right kidney. The child has remained well for 4 years after surgery.