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Biomedical subjects

L Fermont

Publications and source records attributed to L Fermont.

At least 37 records · Page 2Linked to original sources
PubMed1998

[Intrahepatic arteriovenous fistula. Prenatal diagnosis, physiopathological study and neonatal management]l.

A case of arteriovenous fistula of the liver diagnosed at 30 weeks of gestation is reported. The etiologies of an hypoechogenic structure in the fetal liver are discussed showing the contribution of pulsed wave Doppler and color Doppler to the diagnosis. The clinical evolution towards heart failure led us to examine the pathophysiology of such a lesion. The prenatal management of this arteriovenous malformation is exposed.

Adult↗
PubMed1997

Atrio-ventricular valve dysplasia in 22 newborn infants.

We retrospectively studied the experience of our institution with isolated dysplasia of one or both atrio-ventricular valves in 22 newborn infants. All patients with associated cardiac malformations were excluded. Ten patients exhibited isolated tricuspid valve dysplasia. One patient had tricuspid valve dysplasia and a dysplastic pulmonary valve. In 10 patients, both atrio-ventricular valves were affected. Finally, mitral valve dysplasia was associated with pulmonary valve stenosis in 1 case. Associated syndromes and/or chromosomal anomalies were: Down syndrome (n=2), trisomy 18 (n=1), Noonan syndrome (n=1), Marfan syndrome (n=3), Ehlers-Danlos and Cutis laxa (n=2). Mortality was 27.2% during follow-up (mean 51 months): 3 patients with chromosomal aneuploidies, 2 patients with severe neonatal Marfan syndrome and 1 with Ehlers-Danlos. Complications were: sustained supra-ventricular tachycardia in 3, neonatal staphylococcal tricuspid valve endocarditis in 1, persistent significant valvular disease in 8. In the remaining 9 survivors, the dysplasia of the atrio-ventricular valves persists with absent or mild incompetence. Beside obvious chromosomal anomalies, newborn infants with dysplastic valves should be investigated for manifestations of connective tissue disorders. This may help to identify new pleiotropic syndromes which include valvular dysplasia as one manifestation.

Aneuploidy↗
PubMed1996

Prenatal MRI diagnosis of fetal cerebral tuberous sclerosis.

Tuberous sclerosis (TS) is an autosomal dominant phakomatosis. A high percentage of spontaneous mutations leads to the diagnosis of new cases in normal families. This diagnosis is suspected at antenatal ultrasound on the discovery of multiple cardiac tumors. Antenatal cerebral ultrasound shows a normal appearance in affected fetuses. Eight fetuses with multiple cardiac tumors were studied with antenatal MRI with, in five cases, an abnormal appearance showing hyperintense subependymal and cortical nodules on T1-weighted images. Among the three remaining patients MRI was non-contributive in one due to movement artefact, one had abnormal postnatal MRI consistent with TS and one had a normal postnatal and clinical examination. We conclude that MRI is a valuable tool in making the diagnosis of TS in fetuses with multiple cardiac tumors.

Female↗
PubMed1996

Aortico-left ventricular tunnel in fetuses and infants.

BACKGROUND: Aortico-left ventricular tunnel is a rare congenital abnormal communication between the aorta and the left ventricle presenting in early childhood as aortic regurgitation and cardiac failure. This condition has rarely been reported in fetuses. Operation is the only treatment, and postoperative aortic incompetence could be related to the age or the type of repair. METHODS: We conducted a retrospective, two-institution review, from 1983 to 1995, of aortico-left ventricular tunnel diagnosed in utero and before 6 months of age. RESULTS: Three cases of aortico-left ventricular tunnel were diagnosed in utero by Doppler echocardiography between 22 and 24 weeks' gestation. Prenatal aortico-left ventricular tunnel was associated with severe left ventricular dysfunction, aortic valve anomalies, and fetal hydrops. One death occurred in utero and one immediately after birth, and in 1 case pregnancy was interrupted. In these 3 cases the diagnosis was confirmed by autopsy. Three neonates and 2 infants had the diagnosis of aortico-left ventricular tunnel made after birth and underwent successful surgical repair. At short and midterm follow-up all patients are alive and aortic valve regurgitation is absent or trivial. CONCLUSIONS: This series shows that aortico-left ventricular tunnel covers an anatomic spectrum of lesions. Cases diagnosed in utero by Doppler echocardiography are characterized by severe ventricular dysfunction, associated aortic valve lesions, and poor outcome. Postnatal cases represent the more favorable end of the spectrum, with no associated lesions, and can be repaired without mortality and with good functional results.

Adult↗
PubMed1996

[Anomalies of umbilical vein in the fetus. A case report and review of the literature].

Abnormal connexion of the umbilical vein in the inferior vena cava was diagnosed in a fetus at 18 weeks' gestation. Associated anomalies included nuchal and axillary lymphangiectasia, hypertrophic cardiomyopathy and hepatomegaly. Neonatal death occurred after spontaneous vaginal delivery at 25 weeks. A review of the literature summarizes the diagnostic criteria and associated anomalies.

Abnormalities, Multiple↗
PubMed1994

Prenatal diagnosis of tuberous sclerosis. Use of magnetic resonance imaging and its implications for prognosis.

Tuberous sclerosis (TS) is an autosomal dominant disorder with a high rate of de novo mutation. The real difficulty is to ascertain the diagnosis and to give the neurological prognosis in each case. Prenatal diagnosis of TS is generally based on ultrasonographic signs of multiple cardiac tumours, i.e. rhabdomyomas. Recent progress in magnetic resonance imaging (MRI) enables the diagnosis in a large proportion of cases based on typical brain lesions. It may have a role in the prenatal management of TS, although MRI images seem to underestimate the anatomical findings. Two cases in which TS was diagnosed prenatally are presented with reference to the value of MRI in the prenatal management and comparison with anatomical findings.

Female↗
PubMed1994

Flecainide distribution, transplacental passage, and accumulation in the amniotic fluid during the third trimester of pregnancy.

OBJECTIVE: To study the disposition of flecainide acetate and its transplacental passage (both into the fetus and in the amniotic fluid) during the third trimester of pregnancy. DATA SOURCES: Reference articles and books are identified in the text. A literature review is presented. CASE SUMMARY: Flecainide distribution, transplacental passage, and accumulation into the amniotic fluid were studied in a patient at term presenting with a fetal supraventricular tachycardia diagnosed at 33 4/7 weeks of gestation. The fetal tachycardia was accompanied by cardiac failure with placental anasarca and hydramnios. Flecainide 100 mg po bid was prescribed initially; by the time of delivery, the dosage had been decreased to 50 mg bid. At delivery day (39 5/7 weeks), the pharmacokinetics of total flecainide were studied at plateau. DATA SYNTHESIS: The concentrations of flecainide at birth in fetal and maternal blood and in amniotic fluid were 235.4, 241.2, and 6426.5 micrograms/L, respectively. Calculation of a fetomaternal blood accumulation ratio of 0.97 showed that, at this gestational age, flecainide penetrates the placental membrane easily without accumulation in fetal blood. In contrast, the concentration of flecainide in amniotic fluid was approximately 27-fold that measured in maternal peripheral blood. Our results suggest the following: (1) close to term, the metabolic clearance (fetal hepatic clearance) of flecainide offers a high yield and its excretion by the fetal kidney is efficient; (2) given that amniotic fluid is constantly swallowed, it seems that, in contrast to what is seen in adults (relative oral bioavailability > or = 95 percent), the oral bioavailability of flecainide is possibly low in the fetus at term or close to term; under such circumstances, the drug would accumulate passively within the gestational sac; and (3) an alternative explanation is that the concentration in the fetus is, in part, the result of both transplacental crossing of the drug and reabsorption orally from the amniotic fluid. CONCLUSIONS: The regular therapeutic monitoring of flecainide is necessary and sufficient in the mother as the concentrations found appear to accurately reflect the degree of fetal accumulation. Because previous studies in infants and children have indicated few toxic adverse effects attributed to flecainide, it appears that the risk to a sucking infant of ingesting toxic amounts of flecainide in human breast milk is very low. Finally, the child of the patient described here has normal initial growth and development at the present time. The transplacental penetration of a drug can be considered, according to gestational age and the disorder being treated, as being of no consequence, dangerous, or desirable. Flecainide appears to fall into this last category.

Adult↗
PubMed1993

Aortic arch thrombosis in the neonate.

Two cases of neonatal aortic arch thrombosis are reported. One patient, who had ascending aortic thrombosis, died preoperatively. The other had reoperation and is alive and well at 6 months' follow-up. No obvious cause was found.

Adult↗
PubMed1992

Early palliation of univentricular hearts with subaortic stenosis and ventriculoarterial discordance. The arterial switch option.

The optimal Fontan-type operation greatly depends on appropriate initial palliation. Several surgical techniques have been used in infancy to palliate patients with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis. The two most common are pulmonary artery banding and Damus-Norwood procedures. Palliative arterial switch operation is another surgical option that was used in this early series of seven infants. The principle of this operation is to "switch" the subaortic obstruction into a subpulmonary obstruction; the coronary artery relocation on the large pulmonary trunk creates a harmonious aortic root and the connection of the rudimentary ventricular chamber to the pulmonary artery trunk creates a natural protection of the pulmonary vascular bed through the restrictive bulboventricular foramen. Seven infants with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis underwent a palliative arterial switch operation. All infants had an associated aortic arch obstruction of various degrees, including one with interrupted aortic arch, five with coarctation with severe arch hypoplasia, and one with isolated arch hypoplasia. There were three with double-inlet left ventricle, three with tricuspid atresia, and one with transposition of the great arteries with ventricular septal defect and severe right ventricular hypoplasia. The subaortic obstruction was patent at birth in five patients who underwent a palliative switch operation in the first 2 months of life, and rapidly occurred following a previous neonatal pulmonary artery banding associated with arch repair in two patients who underwent a switch operation at 5 and 8 months of age, respectively. The operation includes aortic arch repair without prosthetic material, an atrial septectomy, and the arterial switch. An associated pulmonary shunt was required in five patients and a pulmonary artery banding in one. There was one early death in a patient with [S,L,L] anatomy and congenital atrioventricular block, leading to an early mortality of 14% (95% confidence limits: 1% to 28%). There was one late death. Four survivors are waiting for a Fontan-type procedure, and one survivor had satisfactory right ventricular growth. Early palliative arterial switch operation offers several advantages: reconstruction of a harmonious aortic root, natural protection of the pulmonary bed through the restrictive bulboventricular foramen, prevention of deleterious myocardial hypertrophy, and arch reconstruction without the introduction of a foreign material. This aggressive technique may provide a satisfactory palliation in infants with univentricular hearts and ventriculoarterial discordance, when the bulboventricular foramen/aortic anulus ratio is less than 0.8 or when the subaortic stenosis is severe enough to be associated with an arch obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)

Abnormalities, Multiple↗
PubMed1992

[Prenatal cardiology: can we treat the fetal heart?].

Antenatal diagnosis of heart disease has become very efficient with the advances in ultrasound technology. Accordingly, antenatal treatment of some heart diseases is possible and this is mainly represented by fetal tachycardia and brachycardia. In fetal tachycardia, medical treatment leads to improvement or total curve in approximately 85% of cases. In contrast, there is no efficient medical treatment for fetal bradycardias. In any case, fetal therapy must fulfill several conditions, namely: transplacental transfer of the drug, innocuity for the mother and the fetus, absence of teratogenic effect and low extracardiac tissular accumulation.

Bradycardia↗
PubMed1990

[Heart tumors in newborn infants and infants. Apropos of 5 cases].

Between February 1985 and March 1987, 5 children underwent resection of primary cardiac neoplasms, 3 of them in the first days of life and 2 before the age of 6 months. Routine echocardiographic follow-up of pregnancies allowed detection of cardiac tumors in 2 foetuses 30 and 36 weeks old. In 3 children the diagnosis was suspected by the discovery of cardiac murmur or congestive heart failure. The first case was an hemangioma, originating from outside of the left ventricular wall, which could be resected without cardiopulmonary bypass (CPBP). The other case was a pseudomyxoma, spreading extensively inside the right atrium. The third case was a rhabdomyoma arising from the pulmonary infundibulum with clinical manifestations of tuberous sclerosis. The last two patients had intraseptal lesions, just above the aortic valve; complete resection was therefore impossible, particularly in one patient with multiple tumors. There was one death related to congestive heart failure. The remaining four survivors were followed up for an average of 18.4 months (+/- 12.9) and all were in functional class I. Echocardiographic follow-up showed evidence of a residual subaortic lesion in one asymptomatic patient.

Age Factors↗
PubMed1990

[Tumors of the heart in newborn infants and infants. Apropos of 5 cases].

Between February 1985 and March 1987, 5 children underwent resection of primary cardiac neoplasms, 3 of them in the first days of life and 2 before the age of 6 months. Routine echocardiographic follow-up of pregnancies allowed detection of cardiac tumors in 2 foetuses 30 and 36 weeks old. In 3 children the diagnosis was suspected by the discovery of cardiac murmur or congestive heart failure. The first case was a hemangioma, originating from outside the left ventricular wall, and was resected without cardiopulmonary bypass (CPBP). The other case was a pseudomyxoma, spreading extensively in to the right atrium. The third case was a rhabdomyoma arising from the pulmonary infundibulum with clinical manifestations of tuberous sclerosis. The last two patients had intraseptal lesions, just above the aortic valve; complete resection was therefore impossible, particularly in one patient with multiple tumors. There was one death related to congestive heart failure. The remaining four survivors were followed up for an average of 18.4 months (+/- 12.9) and all were in functional class I. Echocardiographic follow-up showed evidence of a residual subaortic lesion in one asymptomatic patient.

Female↗
PubMed1989

Extrathoracic heart (ectopia cordis). Report of two cases and review of the literature.

We report 2 cases of extrathoracic heart (ectopia cordis) operated on a few hours after birth at Laennec Hospital, Paris. The first patient had an associated diastasis of the rectus muscles. The second one, in whom diagnosis was made by ultrasound during fetal life, had a normal abdominal wall. In both cases it was possible to accommodate the heart in the left pleural cavity after a wide dissection of the posterior mediastinum. Both babies, however, died soon after the operation. Double outlet right ventricle was found in both. We have reviewed those cases previously described and discuss the importance of prenatal echocardiographic diagnosis in this rare condition.

Female↗
PubMed1987

[Left ventricular function and cardiovascular adaptation to exercise in young sportsmen].

In order to find out whether the expression "sports heart" can be used in children and if so, to give an objective definition of it, we studied a group of 40 young people (mean age 12.2 +/- 1.6 years) subjected to intensive training and practising sports regularly, i.e. about 12 hours of physical activity per weeks on average. The sports practised were mainly tennis among boys and ice-skating among girls. A group of 40 subjects with similar characteristics but no particular physical training was studied simultaneously and served as control. These 80 young people had normal heart structure. All underwent routine cardiovascular examinations (physical, radiological, electrocardiographic), complete one - and two - dimensional echocardiography to evaluate left ventricular function and an exercise-test on ergometric bicycle. No difference was found between the left ventricle of sportsmen and that of controls; in particular, there was neither dilatation nor parietal thickening in sportsmen, so that all functional values were the same. During exercise-tests sportsmen performed better than controls, with significantly longer duration of effort and greater maximal oxygen consumption. However, heart rate at rest, then during exercise and recovery, and variations in systolic blood pressure during exercise and in the 10 minutes which followed were identical in both groups. The only notable difference was a deeper fall in diastolic blood pressure in sportsmen, indicating a better opening of the peripheral vascular bed during exercise. It is concluded that there are few cardiovascular differences between trained sporting children and children with normal physical activity, probably because the relatively short training undergone does not leave sufficient time for most of the adaptation systems to become established.

Adolescent↗
PubMed1987

[Endocarditis of the ventricular septum. Echocardiographic diagnosis].

A 5 year-old child presented with prolonged fever, severe impairment of general condition and a pleuro-pericardium effusion. Echocardiography revealed a "bell-clapper-like tumor" in each ventricle, developed from the ventricular septum. Presence of a staphylococcal sepsis and early disappearance of the echocardiographic signs after antibiotic treatment allowed for diagnosis of bacterial endocarditis with abscess of the ventricular septum. Sinusitis and IgA deficiency were later found to be also present.

Child, Preschool↗