Biomedical subjects
Publications and source records attributed to A Vliers.
Based on their experience, the authors are demonstrating that early surgery of cardiac abnormalities is justified. This has become possible, during the last few years, because of a better understanding of cardiac anatomy, metabolism and function in the small child. Results are showing that 75% of operated children are leading a normal life. Further improvements have to be pursued with as final objective the prevention of cardiac abnormalities.
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)
We describe a family in which two generations are affected: two brothers and one of their maternal uncles. One of their two half-sisters (same mother) is also suspected of having the same cardiopathy. This observation confirms the autosomal dominant transmission of the disease and shows its variable expressivity in the family under study.
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).
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.
A psychometric and behavioral evaluation was undertaken in the first 31 patients surviving after intracardiac repair during circulatory arrest and deep hypothermia in the first months of life. I.Q. in these patients were similar to those in normal children albeit that no extrasurgical pathological condition impaired the cerebral potential (dysmaturity). Graphic and spatial structuration problems were present in half the patients around age 5-6 years. These problems disappeared later on. Functional autonomy was decreased at the beginning of school. There was no school absenteism. Participation in gymnastics was normal. One third of patients participated in sports regularly.
The post-operative review of 81 patients operated for a large interventricular communication before the age of two years reveals excellent results: the mean age at operation was 7 months, extracorporeal circulation was performed in 25 patients for a mean duration of 77 minutes and profound hypothermia to 18 degrees C was induced in 56 patients. The atrial approach was used in 30% of cases. The mean follow-up period is 26 months. There was one case of complete bundle branch block, which was subsequently fitted with a pacemaker, 7 cases of bifascicular branch block and a right bundle branch block in the majority of cases. The pulmonary vascular resistance was virtually normal (a mean of 3.2 U.m-2 prior to the operation and a mean of 1.9 U.m-2 after the operation). The left ventricular volume, which was markedly increased in all patients preoperatively, returned to normal with maintenance of a normal ejection fraction. The neuro-psychomotor behaviour was abnormal in 13 of the 63 patients tested. These abnormalities are not related to the correction procedure, but to pre-existing pathology (small birth weight). 52 patients from a series of 63 cases operated for tetralogy of Fallot before the age of 2 years (median age 12 months) were also reviewed. Extracorporeal circulation lasting a mean of 115 minutes was performed in 31 patients, cardiac arrest under profound hypothermia was induced in the other cases, for a mean duration of 61 minutes. The mean follow-up period was 29 months.(ABSTRACT TRUNCATED AT 250 WORDS)
This study concerns 89 cases of isolated transposition with early surgical repair by auricular transposition of the venous returns. Fairly good results were obtained with an operative mortality rate less than 10%, few postoperative complications, and long-term follow-up without significant worsening of the functional capacities of these patients. From this study, the authors state precisely the present indications for treatment of isolated transposition.
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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.
Statistical multivariate and conventional deterministic methods of computerized interpretation of the electrocardiogram (ECG) were compared in the analysis of 1711 pediatric orthogonal ECGs validated by nonelectrocardiographic criteria on the basis of clinical and anatomic diagnoses. Among 642 children catheterized for the evaluation of congenital heart disease, there were 140 patients with left ventricular hypertrophy, 299 with right ventricular hypertrophy, and 203 with biventricular hypertrophy. A group of 1069 obviously healthy school children was studied as a control. The overall accuracy of multigroup ECG diagnosis was 85% and 79% for the statistical and deterministic methods, respectively. The diagnostic performances of both methods expressed in terms of sensitivity and predictive value were the highest for normal children and those with right ventricular hypertrophy and lowest for children with biventricular hypertrophy. The statistical method was more sensitive in the diagnosis of left ventricular hypertrophy (74% vs 64%), right ventricular hypertrophy (86% vs 83%), and biventricular hypertrophy (62% vs 50%). Mutual agreement for a correct diagnosis by the two methods was 83% for normal children and 82% for those with right ventricular hypertrophy but only 61% for children with left ventricular hypertrophy and 39% for those with biventricular hypertrophy. In conclusion, better classification results are obtained with statistical multivariate techniques as compared with conventional deterministic analysis, but both methods of ECG interpretation are complementary and their combination in the same electrocardiographic computer program can improve diagnostic accuracy.
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.
The evaluation of a new computer program for analysis and interpretation of pediatric Frank vectorcardiograms is reported. The program includes extensive age- and sex-dependent criteria based on tables of limits for numerous vectorcardiographic parameters. In 728 catheterized patients, the diagnostic performance for type A statements was tested against independent and objective evidence obtained from hemodynamic and angiographic data. The overall diagnostic accuracy ranged from 75 to 89% without difference between children less than 2 years of age and those greater than or equal to 2 years of age. Sensitivities and specificities of the various diagnoses did not differ much between the 2 age groups. In the younger children, the accuracy of a positive diagnosis of left ventricular hypertrophy, right ventricular hypertrophy, and biventricular hypertrophy was 20, 15, and 32% higher, respectively, than in the older children. The accuracy of the diagnosis "normal" was 28% lower in the younger children. These differences were explained by the higher proportion of pathologic findings in the younger children: 93% versus 74% in the older children. Given the strict methods of the evaluation, the diagnostic accuracy of this pediatric program was considered clinically satisfactory. Program performance appears to be dependent not on patient age but on prevalence of abnormalities in the population analyzed. Further improvement can be expected by making the criteria more adaptable to the composition of the population.
The authors report a case of Libman-Sacks endocarditis of the posterior mitral leaflet in a child treated for disseminated lupus erythematosus for 8 years. Mitral valve replacement was performed. Libman-Sacks vegetations are hardly ever recognised in vivo and have never been previously reported in a child. The incidence of this cardiac complication of lupus erythematosus is probably higher than indicated in the literature. Attentive cardiac examination and systematic echocardiography should be part of routine follow-up of patients with disseminated lupus erythematosus.
His bundle electrograms were recorded in 42 patients with secundum atrial septal defects (ASD) and in 50 controls, aged 1 month to 16 years, in normal sinus rhythm and with normal PR intervals. Intervals PA (P less than 0.01) and PR (P less than 0.02) were found to be significantly prolonged in ASD patients. Analyzing the results according to age, a significant increase for intervals AH (P less than 0.05), HV (P less than 0.001) and PR (P less than 0.001) was noted in the oldest ASD children as compared to the younger ASD children, whereas in controls only the PR interval was found to be significantly prolonged (P less than 0.05) in the oldest subgroup. Under the age of three years, atrioventricular conduction intervals were not significantly different whether or not an ASD was present. However, in older children with ASD as compared to controls, a significant increase was noted for interval PA (P less than 0.03) in children four to eight years old and for intervals HV (P less than 0.01) and PR (P less than 0.001) in children 9 to 16 years old. ASD patients showed a more significant correlation with age for interval PR with a significantly steeper slope of the regression line. Results show the impact of ASD on AV conduction to be age-related, with involvement not only of the PA interval but also of the HV interval.
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A series of 19 neonates with persistent ductus arteriosus associated with right heart pathology such as pulmonary or tricuspid atresia (13 cases), left heart and aortic arch pathology (3 cases) and simple transposition of the great arteries (3 cases) is presented. The efficacity of an infusion of prostaglandins (PG E1, 0,05 /kg/min) in improving the clinical condition of the patients before surgery was confirmed. A rapid improvement in the blood gases (02 saturation) was obtained in right heart pathology. In children with the second group, the efficacity of PG depended mainly on the clinical state and degree of peripheral hypoperfusion before therapy. For this reason, the infusion should be instituted as soon as possible, even before confirmation of the diagnosis. PG were used in a newborn with simple transposition of the great arteries in association with balloon septostomy in order to obtain an earlier result with the latter procedure. The incidence of side effects was higher in this than in other reported series. The protocol of infusion and its surveillance are discussed. Prolonged infusion of PG is not advised as it was probably responsible for oedematous infiltration of the vessel wall in one child.