["Degenerative" myocardiopathies in childhood (author's transl)].
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Biomedical subjects
Publications and source records attributed to L Fermont.
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188 infants presenting with ventricular septal defects associated with pulmonary hypertension underwent early surgical treatment. Overall death rate was 10% (6% since 1979 and even 0% in case of single defects operated on between the ages of 6 and 12 months). Surgical results were excellent in 48 controlled cases operated on during the 1st year of life. When surgery was performed later, risk of residual pulmonary hypertension remained in cases with equal pressure levels. Our results suggest that all the infants who do not respond properly to medical treatment must be operated on and that those with a major pulmonary hypertension have to be operated on during there second semester of life.
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Massive hypertrophic cardiomyopathy was revealed by echocardiography in a diabetic mother's newborn infant with early severe cardiovascular failure. Clinical and echocardiographic improvement occurred with symptomatic treatment excluding digitalis.
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Four types of supraventricular tachycardia were identified in a retrospective study of 71 babies who presented under the age of 3 months. The arrhythmia originated in the atrium in the first two types (25% of cases, atrial tachycardia 10 cases, chaotic atrial tachycardia 7 cases). In the other types the arrhythmia originated at the atrioventricular node. Reciprocal rhythms were most common (62%) and those starting in the bundle of His the least common (5%). It is relatively easy to distinguish the different types which are important because treatment may vary. Overall 75% recover completely, 11% have sporadic recurrences and 10% have a chronic arrhythmia. The junctional varieties have the worst prognosis.
Four cases of cor triatriatum are reported in 6 weeks to 23 months old infants. Two were associated with partial anomalous pulmonary venous connection and one with a mild aortic stenosis. Two were successfully operated on and are now normal children: one of these underwent surgery at 3 months with an hemodynamic control one year later. The two others died: the youngest because of an inadequate overloading transfusion, the other soon after pulmonary angiography. Problems in diagnosis and treatment of this rare but very tractable condition are discussed.
OBJECTIVE: To study the fetal response to prenatal therapy in non-hydropic fetuses with supraventricular tachycardia (SVT) as a function of fetal haemodynamic status at presentation. STUDY DESIGN: Retrospective study. MATERIAL AND METHODS: Between 1990 and 2000, 40 non-hydropic fetuses presented with SVT. Twenty-eight had reciprocating SVT and 12 had atrial flutter. Ten fetuses had significant tricuspid valve regurgitation. All fetuses were treated prenatally. The main outcome measurement was fetal response to therapy as assessed by the rate of prenatal SVT reduction and by the mean time interval to sinus rhythm restoration. RESULTS: The mean gestational age at presentation was 29 +/- 4.9 weeks. Overall, there were 39 live births and 1 intrauterine death. Reduction of SVT was achieved prenatally in 32 cases (80%). Among the 30 cases without tricuspid regurgitation, prenatal conversion to sinus rhythm was achieved in 27 cases (90%) with a mean time interval of 7 days. Among the 10 fetuses presenting with tricuspid regurgitation, the rate of prenatal conversion was significantly lower (5/10) and the mean time interval to conversion was significantly longer (24 days; p = 0.04, Mann-Whitney test). In the subgroup treated by digoxin as first-line therapy (n = 32), the interval to sinus rhythm restoration was also significantly higher in the presence of tricuspid regurgitation, with a slightly but not significantly lower reduction rate. CONCLUSION: The response to prenatal therapy may be poorer in cases presenting with tricuspid regurgitation.
In spite of active perinatal management, twin-twin transfusion syndrome (TTTS) remains a severe disease with a high risk of neonatal mortality and morbidity. TTTS initially results from an unbalanced blood flow from a donor to a recipient twin. However, its pathogenesis remains unclear, although cardiovascular disturbances and regulation of fetal volemia and diuresis seem central in this syndrome. Previously, we demonstrated that the renin-angiotensin system (RAS) was up-regulated in donor twins as a consequence of hypovolemia, and down-regulated in recipients. This was the first evidence of the implication of the RAS in TTTS. We hypothesize that the RAS plays a key role in the pathogenesis of TTTS. In the donor, RAS up-regulation aggravates oligohydramnios and may increase arterial resistance, which could contribute to placental dysfunction leading to intrauterine growth restriction. In the recipient, paradoxical RAS activation, due to transfer of effectors such as angiotensin II through placental shunts, could explain fetal vascular disturbances and cardiomyopathy. According to our hypothesis, TTTS would appear similar to the classical model of hypertension referred to as '2 kidneys-1 clip' with a donor twin, comparable to the clipped kidney, intoxicating its cotwin, comparable to the normal kidney.
To improve the outcome of severe twin-to-twin transfusion syndrome with 1 hydropic fetus and to prevent ischemic sequelae in the survivor, we developed a technique of selective feticide by vascular embolization of the most severely damaged twin. Acute second trimester polyhydramnios occurred in 4 biamniotic monochorial twin pregnancies, with 1 fetus normal on ultrasound but the other severely damaged by hydrops and hypertrophic hypokinetic cardiomyopathy. The hydropic fetus underwent embolization using a bolus of histoacryl injected into the umbilical vein and fetal heart under ultrasound guidance. In 1 triplet pregnancy with a set of monochorial fetuses, premature labor occurred at 26 weeks, 2 weeks after embolization, and there were 2 neonatal deaths. The 3 other cases resulted in the birth of a normal infant at 31-37 weeks of gestation. This suggests that in twin-to-twin transfusion syndrome with severe polyhydramnios and hydrops of 1 fetus, embolization may salvage the other twin.
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Fetal echocardiography, including simultaneous and ventricular M mode scans, allows the diagnosis of arrhythmias by studying the relationships between atrial and ventricular contractions. It also assesses cardiovascular tolerance by looking at pericardial or peritoneal effusion, left ventricular contraction and by measuring blood velocity in systole and diastole in umbilical arteries by Doppler techniques. We report here our experience on 221 arrhythmias found out of 1344 fetal echocardiographies performed since 1983. Bradycardia without atrio-ventricular dissociation in otherwise healthy fetuses were excluded since they are physiologic when moderate and transient. In addition, 180 out of our 221 cases had transient premature beats and were also excluded. It remained thus only 41 cases with true significant abnormalities of the fetal rhythm or conduction. Bradycardia with atrio-ventricular block and low ventricular rate was found in 15 fetuses. Ventricular rate was the first risk factor, since the tolerance was poor under 60 beats per minute (b/m). In this group, 9 cases were associated with cardiac malformations: mainly atrio-ventricular discordance, atrio-ventricular canal, double inlet ventricle. In the 6 other, the heart was normal and in only one, an immunologic disorder was found in the mother. Cardiovascular tolerance and gestation age were the two determinants for deciding on early delivery. In all cases, the newborn was referred to a pediatric cardiology center where specific measures (artificial pacing or simple follow-up) were undertaken.(ABSTRACT TRUNCATED AT 250 WORDS)
Interruption of the aortic arch, associated with curable cardiovascular anomalies (8 times out of 10) was observed over a period of 8 years in 50 children aged less than 10 days in 80% of cases. They presented with congestive heart failure, with variable degree of shock in most cases, suggesting the diagnoses of left ventricular hypoplasia or coarctation syndrome. Diagnosis was established with ultrasound and angiocardiography. Evolution was lethal in 21 children who were not operated on. 20 children were operated on: before treatment with prostaglandin E1, 9 attempts ended up in 78% immediate mortality and 89% global mortality; after treatment with prostaglandin. E1, these figures were respectively reduced to 45% and 55% (20 cases). This malformation should therefore be recognized as an emergency and then treated with prostaglandin up to surgery.
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