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Biomedical subjects

A M Worms

Publications and source records attributed to A M Worms.

At least 55 records · Page 3Linked to original sources
PubMed1990

[Cardiac rhabdomyoma in children and Bourneville's tuberous sclerosis].

Cardiac rhabdomyomas are rare congenital tumours resulting from an early dysembryoplastic disorder of organogenesis. They are generally benign hamartomas which may be the first manifestation of a phakomatosis, tuberous sclerosis (TS) (Bourneville disease), present in over half of cases. The cases of 11 children with cardiac rhabdomyomas are reported. All of them also had extracardiac lesions of TS. Their ages ranged from 1 day to 6 years of age. In one case, the diagnosis was made antenatally by foetal echocardiography. The clinical expression may be very serious when there are hemodynamic disturbances resulting from an obstructive syndrome (N = 3) or arrhythmias (N = 2). Nevertheless, the cardiac evolution is usually favorable (N = 9) in contrast to the neuropsychiatric outcome which is generally catastrophic (N = 8). The diagnosis is made by two-dimensional echocardiography and magnetic resonance imaging. Surgical resection of the tumours is reserved for forms complicated by cardiac failure due to intracardiac obstruction (N = 2) of life-threatening resistant arrhythmias. Genetic counseling should be directed towards prevention of TS because it is transmitted in an autosomal dominant mode. However, most of the cases are sporadic. Echocardiography should be systematic in all children with TS and is also recommended for members of their families, even those apparently unaffected, in order not to miss paucisymptomatic forms of TS.

Adolescent↗
PubMed1989

[Malignant quadrivalvular dysplasia of Marfan syndrome in a neonate].

The authors report the case of a neonate who died early of refractory heart failure and presented with all the characteristics of Marfan's syndrome in its early form with articular retractions. There was clinical evidence of mitral, tricuspid and major aortic regurgitation. Two-dimensional and doppler echocardiography confirmed the prolapse of highly dystrophic valves and the quadrivalvar regurgitation. Typical lesions of the connective-elastic tissue were found at autopsy. The occurrence of malignant Marfan's syndrome in neonates seems to be exceptional; the syndrome may take the rare "en contractures" form. Death is not always ascribable to the cardiac pathology, this pathology being often less generalized than in our case which seems to be the first one where such diffuse and massive valval lesions have been observed. This particular form was comparable to the "congenital polyvalvular disease" described by Bahrati and Lev, except that the skeletal and visceral abnormalities of Marfan's syndrome are absent in that disease.

Aortic Valve↗
PubMed1989

[Percutaneous transluminal angioplasty of stenosis of the pulmonary arteries after surgical repair of tetralogy of Fallot].

Post-surgical stenoses in patients operated upon for tetralogy of Fallot create difficult therapeutic problems. Between November 1985 and November 1988, we performed 17 percutaneous transluminal angioplasties in 13 children aged from 5 to 15.1 years (7.7 years) for stenosis of pulmonary artery branches after correction of tetralogy of Fallot. Group I consisted of stenoses consecutive to palliative anastomoses: 6 dilatations of 5 stenoses in 4 children. Group II concerned stenoses of the pulmonary artery at the top of widening patch on the pulmonary trunk of bifurcation: 5 dilatations of 5 stenoses in 4 children. Group III was made of congenital stenoses sometimes located upstream of a widening patch which made them worse: 6 dilatations of 6 stenoses in 5 children. Angioplasty was performed after catheterization and angiocardiography, the diameter of the balloon being 1.2 to 4-fold that of the stenosis (mean 2.65). There was no complication. In 7 cases the diameter of the stenosis increased by 27 to 96 p. 100. In 2 of these cases, this increase produced no change in pulmonary pressures and perfusion. In the remaining 5 cases, regarded as successful, the diameter of the stenosis increased from 5.7 mm to 9 mm on average, and the right ventricular pressure fell from 62.4 mmHg to 48.8 mmHg; the gradient was reduced by 24 to 50 p. 100 in 4 cases and the lung perfusion was improved in the 5th case. The proportions of successful angioplasties were: 2 out of 6 in group I, zero out of 5 in group II and 3 out of 6 in group III.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1989

[Percutaneous angioplasty of recoarctations of the aorta. Apropos of 11 cases].

Between January 1986 and October 1988, 11 young patients aged from 3 months to 29 years (10.6 years) underwent percutaneous transluminal angioplasty for recoarctation of the aorta. The procedure was performed 3 months to 21 years (6.7 years) after the initial operation for coarctation (9 cases: 8 terminoterminal sutures and 1 Waldhausen operation) or for complete interruption of the aortic arch (2 cases: direct terminoterminal suture with banding of the pulmonary artery in 1 case and closure of an aorto-pulmonary fistula in the other case). The diameter of the balloon selected was at most 1 to 1.5 mm wider than that of the smallest diameter of the aorta upstream or downstream of the stenosis, as measured by prior aortography. Adverse events recorded were thrombosis of the femoral artery in a 1-year old child, transient subendocardial lesion wave during dilatation in another child, and regressive left bundle branch block in a third patient. In one patient a small aneurysm developed at the site of dilatation: it was perfectly stable after 8 months. A satisfactory result was obtained in 4 cases, with a 52 to 95% increase in diameter of the stenosis and reduction of the gradient which fell from 56 mmHg on average to 18.25 mmHg. One child was lost sight of; in the remaining 3 patients the result remained stable after 6 to 20 months. A partial result (widening of the stenosis without change in gradient) was obtained in 2 cases. Five of the 11 cases were failures.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1989

[Cayler's cardio-facial syndrome. Apropos of 19 cases].

Hypoplasia of the depressor angulae oris muscle can be detected in the crying newborn by an asymmetry of the facies, and it must be differentiated from facial palsy. Asymmetry of the facies during crying spells associated with a congenital cardiac abnormality constitutes the Cayler's cardiofacial syndrome. Nineteen cases of this syndrome are reported. A wide spectrum of congenital heart defects of varying severity was observed, ventricular septal defect being the most common along with frequent malformations involving systems and organs other than the heart.

Adolescent↗
PubMed1989

[Noonan's syndrome and its cardiovascular dysplasia. Apropos of 64 cases].

The multiple malformation syndrome described by Jacqueline Noonan in 1963 is one of the most commonly encountered syndromes in pediatric cardiology. The series presented here comprises 64 cases, 20% of which were familial. The morphotype can easily be recognized, but the variations and changes with age imply a detailed analysis of a wide range of discriminatory features. Cardiovascular lesions are very common and very specific, diffuse but above all characterized by "atypical" pulmonary stenosis, with dysplastic valves and a cardiomyopathy of the left ventricle, which is very particular, but also encountered in other polymalformation syndromes. Aortic lesions and dysplasia of other valves are not infrequent. The genetic mechanism is as yet unknown; at present, only certain hypotheses can be proposed.

Cardiomyopathy, Hypertrophic↗
PubMed1988

[Percutaneous transluminal valvuloplasty in aortic valve stenosis in children].

Percutaneous transluminal balloon valvuloplasty has been used in children since 1983 to treat tight aortic valve stenosis. Although more than 100 cases have been published, its indications compared with those of surgical valvulotomy have not yet been clearly defined. Twelve patients with tight aortic valve stenosis underwent transluminal balloon valvuloplasty: 3 were infants whose ages ranged from 1 month and 24 days to 3 months and 23 days (mean 80 +/- 13.7 days), and 9 were children aged from 4 to 17 years (mean 10.44 +/- 4.61 years). Aortic regurgitation grade I (5) or grade III (1) was present in 6 cases. An 8-year old child had previously undergone surgical aortic valvulotomy simultaneously with repair of aortic coarctation. Other associated malformations were congenital grade III mitral regurgitation in 1 case and Turner's syndrome in 1 case. Dilatation was performed with one or two successive balloons in 11 patients and with two balloons concomitantly in 1 patient. The diameter of the balloon (or the sum of those of the two balloons) was 77 to 112 percent of the diameter of the aortic annulus, but in one case this figure rose to 119 percent after failure of dilatation with a smaller balloon. One infant died at the end of the dilatation, due to perforation of the left ventricle. Another infant developed inferior myocardial infarction which followed a favourable course and left minimal sequelae.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Valve Stenosis↗
PubMed1987

[Cardiovascular dysplasia in Noonan's syndrome. Apropos of 64 cases].

The multiple malformation syndrome with characteristic facies described by Jacqueline Noonan in 1963 is one of the most commonly encountered syndromes in paediatric cardiology. The series presented here comprises 64 cases, almost a quarter of which were familial (6 families). The morphotype (Turner phenotype with hypertelorism) can easily be recognized, but the variations, progressivity and resemblance to some similar syndromes imply a detailed analysis of a wide range of discriminant features. Short stature and webbed neck are less frequent than in Turner's syndrome, while mental debility and, mostly, cardiovascular lesions are much more frequent (the latter are present in 50-60% of the cases). The most typical cardiovascular lesion is pulmonary valve stenosis with dysplastic leaflets, or "atypical pulmonary stenosis syndrome", characterized by a curious electrical axis and above all, by left ventricular myocardiopathy with very peculiar deformations at ventriculography and 2D-echocardiography. In our series, myocardiopathy was even more frequent than dysplastic pulmonary valve stenosis (71.8% versus 64%). The dissemination of dysplastic lesions must be emphasized. They involve the aorta much more often than is usually reported (one-third of the cases); lesions of the lymphatic system are less frequent but may be severe. Occasionally, entirely different heart diseases, such as Fallot's tetralogy, are encountered. Owing to the relationship between its lesions and those of other multiple malformation syndromes, notably those of the phakomatosis group, and to the possibility of borderline cases with these syndromes, or even with other dystrophies, such as Williams-Beuren dystrophia, the cardiovascular dysplasia of Noonan's syndrome must be classified within the vast group of histodysplasias which are embryonic diseases of the "layers" or "neuro-ecto-mesodermoses" of unknown genetic mechanism.

Adolescent↗
PubMed1986

Is the aorta truly dextroposed in tetralogy of Fallot? A two-dimensional echocardiographic answer.

The embryogenesis of tetralogy of Fallot is still much debated. In particular, the dextroposition of the aorta is not considered by all pathologists as a genuine abnormality in this congenital heart disease but rather as a false impression due to an exaggeration of the normal overriding caused by dilatation of the aorta secondary to abnormal hemodynamics. We used two-dimensional echocardiography to examine the spatial position of the aortic root in 22 patients with tetralogy of Fallot (aged 5 days to 24 years, mean 6.4 years) and in 23 normal subjects (aged 1 month to 27 years, mean 7.6 years). Using the parasternal short-axis view, we determined the percent rightward displacement of the aortic root in relation to the plane of the atrial septum, and the relationship between the aortic cusps and the atrial septum. We measured the value of the angle luminal diameter, which was defined as the angle between the plane of the atrial septum and the plane of the left coronary-noncoronary commissure and leaflet appositional plane. In the control group, the aortic root was displaced to the right by only 23.6 +/- 7.6%; the atrial septum crossed the posterior aspect of the aortic root at the middle (n = 19) or at the right half of the posterior cusp (n = 4), and the angle luminal diameter had a value of 43.3 +/- 8.8 degrees. In the 22 patients with tetralogy, the percent rightward shift of the aortic root was augmented to 55.5 +/- 9% (p less than .001) and the atrial septum was related to the posterior commissure in 14 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1986

Roentgen aspects of isolated pulmonary valvular stenosis on chest plain films.

Isolated pulmonary valvular stenosis shows a typical radiological pattern in 70% of the cases, including bulging of the left middle and the right inferior segments, a convex left inferior segment and an upwards shift of the apex. In quite a lot of cases (28%), only one of the four above mentioned criteria, are found, mainly the bulging of the left middle segment. There is a small percentage (6%) of cases with an atypical radiological pattern or showing total normality (5%). There is a clear connection between the cardio-thoracic index and the degree of the stenosis but there is no connection between the severity of the stenosis and the degree of bulging of the left middle segment. However the plain chest film has no value for prognosis (evaluation of the degree of the stenosis) nor to follow the post operative course of this frequent congenital abnormality.

Adolescent↗
PubMed1985

Assessment of right ventricular outflow tract in children by two-dimensional echocardiography using a new subcostal view. Angiocardiographic and morphologic correlative study.

Evaluation of the right ventricular (RV) outflow tract in congenital heart disease is extremely important for surgical management. Therefore, the value of 2-dimensional echocardiography (2-D echo) to assess the RV outflow tract was studied using a new approach: the subcostal elongated right oblique view. Twenty normal children and 49 children with congenital heart disease, aged 1 day to 11 years, were studied. Significant pulmonary infundibular obstruction was present in 22 patients with conotruncal malformations. To obtain the subcostal elongated right oblique view from the short-axis view at the aortic valve level, the transducer was slightly rotated clockwise with an anterior angulation of about 30 degrees so that the ascending aorta was seen in its long axis, providing an image similar to that obtained by a right ventriculogram in the elongated right anterior oblique view. The deviation of infundibular septum was appreciated by measurement of the angle alpha, defined by the long axis of the infundibular septum and the plane of aortic cusps. This view could be obtained in 64 patients (92%). In correlation with angiographic or anatomic data, the subcostal elongated right oblique view permitted recognition of several types of RV outflow tract: type I--normally formed RV outflow tract; type II--disorganized RV outflow tract with obstruction (alpha less than 90 degrees); type III and IV--disorganized RV outflow tract with obstruction (alpha greater than 90 degrees). This view could visualize the crista supraventricularis in type I, but also the anatomic components of RV outflow tract that may contribute to obstruction in the other types: infundibular septum, septoparietal trabeculations and trabecula septomarginalis.(ABSTRACT TRUNCATED AT 250 WORDS)

Angiocardiography↗
PubMed1985

[Selective coronary angiography in children. Technic and indications].

Non-selective opacification of the coronary arteries is often adequate for practical purposes in children, especially for studying the coronary circulation before operating congenital heart disease. However, selective coronary angiography is essential to demonstrate some abnormalities of distribution poorly visualised by the non-selective method and especially in cases of coronary anomalies or acquired coronary disease before procedures of reimplantation of revascularisation. The indications must be carefully considered and the technique particularly adapted using paediatric catheters and trained personnel skilled in retrograde arterial catheterisation in children. 51 selective coronary angiographies were carried out in 47 children 1 to 13 years old, including 12 children under 3 years of age. In 33 cases (23 Fallot's tetralogy the object was to detect coronary abnormalities not observed on non-selective opacification (3 LAD arteries arising from the right coronary, 1 single coronary artery) or to determine their anatomical relationships in complex cardiac malformations (5 cases). There were 5 cases of isolated congenital or acquired coronary disease with signs of ischaemic heart disease in 3 cases (LAD arising from the pulmonary artery and antero-septal infarction, calcified aneurysms and antero-lateral infarction in Kawasaki disease, pseudoxanthoma elastica with multiple coronary stenosis and severe angina). The investigation was indicated for complications of cardiac surgery in 2 children and in 4 cases selective coronary angiography was performed to assess the results of coronary reimplantation.

Adolescent↗
PubMed1985

[Malformations of the tricuspid valve associated with Fallot's tetralogy. Apropos of a series of 224 surgically treated Fallot's tetralogies].

This study was based on 7 children aged 20 months to 13 years with tetralogy of fallot (TOF) and tricuspid valve defects. Cases of endocardial cushion defects (8 cases of atrioventricular) were excluded. Three types of tricuspid valve defect were observed: 4 tricuspid valve prolapse (with one associated mitral valve prolapse); 2 accessory tricuspid valves passing through a ventricular septal defect to prolapse in diastole under the aortic valve; 1 Ebstein anomaly. The prevalence of tricuspid valve defects associated with TOF 3 p. 100 in this series. The diagnosis can: usually be made by 2D echocardiography. Tricuspid valve prolapse (usually the septal leaflet) is visualised in the apical 4 chamber view. The passage of accessory tricuspid tissue across the VSD into the left ventricular outflow tract is visible in the parasternal long axis or subcostal long axis views. Ebstein anomaly can be demonstrated in apical 4-chamber views showing the abnormally apical site of insertion of the septal leaflet of the tricuspid valve and "atrialisation" of part of the right ventricle. Tricuspid valve defects did not pose any special surgical problems during complete repair of TOF but this series did not include any cases of tricuspid hypoplasia or parachute tricuspid valve which have been reported in the literature and which do complicate surgery. Tricuspid valve and subendocardial cushion defects should be looked for systematically in patients with TOF undergoing 2D echocardiography.

Adolescent↗
PubMed1985

[Outcome of children operated on for congenital cardiopathy before 2 years of age. Foreseeable sequelae at adulthood].

A brief description is presented of the long-term follow-up of four commonly encountered cardiopathies undergoing early surgical correction. Sequelae are not increased and probably even less frequent when compared to children undergoing surgery after two years of age. It is still too early, however, to establish whether or not certain manifestations, such as rhythm or conduction disturbances and ventricular dysfunction will develop at a later date. The prognosis of coarctation of the aorta depends to a great extent on other associated lesions which can become serious over time and require additional surgical procedures. In the past, early surgery for large ventricular septal defects was poorly tolerated, but now the prognosis is particularly favorable. Persistent hemodynamic abnormalities such as stenoses of the pulmonary outflow tract can worsen the prognosis in tetralogy of Fallot. In addition, it is especially important to recognize subsequent ventricular dysrhythmias due to their potentially serious nature. The most common procedures for transposition of the great arteries consist of auricular transposition of the venous returns which may later cause rhythm disturbances or venous stenoses. The long-term functional capacity of the right ventricle as a pump for the systemic circulation is not known. A general outline of follow-up care is presented.

Adult↗