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Biomedical subjects

F Marçon

Publications and source records attributed to F Marçon.

At least 73 records · Page 4Linked to original sources

[Current role of radionuclide imaging in pediatric cardiology].

Three main nuclear medicine methods are used in paediatric cardiology: sequential first-pass radionuclide imaging of the cardiac cavities, radionuclide equilibrium ventriculography and radionuclide myocardial imaging. Valuable functional information is obtained, and invasive explorations can be avoided in an ever increasing number of cases. Of particular interest is left-to-right shunt measurement which indicates that atrial septal defects must be surgically corrected when the pulmonary/systemic flows ratio (QP/QS) is above 2. This technique is also useful to evaluate the tightness of repairs in ventricular and atrial septal defects. Radionuclide studies of the right and left ventricles may detect dysfunction in one or the other cavity. The left ventricular ejection fraction is reduced in myocardiopathy an in aortic or mitral valve diseases seen at a late stage. The right ventricular function is often abnormal, notably during exercise, after repair of the tetralogy of Fallot and after atrial correction of complete transposition of the great arteries. An altered ejection fraction in patients with single ventricle is also a sign of deterioration. Right ventricular diastolic overload evaluated by radionuclide equilibrium ventriculography correlates with the QP/QS ratio value in atrial septal defects and with the inducibility of ventricular tachycardia by endocavitary pacing in repaired tetralogy of Fallot. Thallium 201 myocardial imaging provides information on myocardial ischaemia, notably that associated with congenital abnormalities of the coronary arteries. Its use had now been extended, albeit with some limitations, to the evaluation of right ventricular systolic overload. Other radionuclide techniques are being developed with new tracers: Kryton 81m for studies of the right ventricle, short-lived radionuclides for first-pass studies, Iodine 123-labelled fatty acids for myocardial imaging. More recently, some substrates, such as deoxyglucose, have been labelled with positron emitters permitting in vivo metabolic studies.

Cardiac Output↗

[Outcome of 195 patients with Wolff-Parkinson-White syndrome].

Between 1974 and 1984, 207 patients with Wolff-Parkinson-White syndrome (WPW) were admitted to our hospital department; 195 of them were followed up for periods ranging from 1 to 12 years (6 years in children, 3 years and 9 months in adults on average); 160 had undergone electrophysiological exploration. Fifty-seven patients were less than 16 years old: 7 died, including 6 with associated congenital heart disease; an asymptomatic 12-year old girl died suddenly while taking part in a sporting event. The signs of WPW disappeared in 5 out of 10 children under 1 year of age. One hundred and thirty-eight patients were older than 15: 15 of them died, but only 3 deaths were related to WPW: one was consecutive to surgery for WPW and one to fulguration; the third patient died of WPW tachyarrhythmia; the refractory period of his Kent's bundle was short, but his compliance with treatment was irregular. We found no correlation between changes in functional symptoms and Kent's bundle refractory period values; paradoxically, the frequency of attacks and resistance to treatment was higher in cases with long refractory period. On the whole, this series confirms that WPW usually is a benign disease. However, the risk of sudden death, of which it offers an example, indicates that all patients with WPW should be evaluated with at least an exercise test and, depending on its results or on the socio-professional context, an electrophysiological exploration.

Adolescent↗

[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↗

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↗

[Congenital subclavian steal syndrome. Apropos of 14 cases. Review of the literature].

Fourteen cases of the congenital subclavian steal syndrome are presented with a review of 99 cases in the literature. This abnormality is usually asymptomatic and a fortuitous finding (clinical: asymmetric blood pressure; radiological: investigation of an associated cardiovascular malformation). The possible malformations are numerous and may be diagnosed by angiography: however, they can be suspected from three simple investigations, clinical examination indicating the side of lower blood pressure, chest X-ray and barium swallow. Seventy one per cent congenital subclavian steal syndromes are associated with a right-sided aortic arch, the steal being then almost always left-sided via a left subclavian artery anomaly. In this group, a left subclavian artery isolated from the aorta represents about a half of the cases. A cardiac malformation is present in one out of two cases and a patent ductus arteriosus uniting the left subclavian artery and the left pulmonary artery is observed in one out of four cases. Twenty nine per cent of congenital subclavian steal syndromes are associated with a left-sided aortic arch. In these cases the abnormalities usually involve the aortic arch (57% of cases) or a right or left subclavian artery. Therefore, in this group, there is no preferential side for the subclavian steal which can be right, left or bilateral. These abnormalities usually only require medical surveillance. When they become symptomatic, vertebro-subclavian revascularisation is justified. The presence of a subclavian steal may have surgical implications: it is important to exclude a subclavian steal before performing a Blalock anastomosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗

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↗

[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↗

[Radioisotopes for the study of right and left ventricular function in surgically treated Fallot's tetralogy].

Radio-nuclide angiography after surgical correction of tetralogy of Fallot (TOF) allows measurement of the ejection fraction, especially of the right ventricle, under basal conditions and on effort, parameters which are difficult to measure by other non-invasive methods. Twenty-two children with a mean age of 12.6 +/- 6.9 years who had undergone complete correction of TOF at a mean age of 6.7 +/- 3.8 years underwent Technetium 99m blood pool studies at equilibrium. The results were compared with those of a control group of children with a mean age of 10.2 +/- 3.3 years. No first passage studies were performed because the frequency of postoperative pulmonary regurgitation does not allow quantification of the shunt due to a possible residual ventricular septal defect. No significant difference was found between the patients and control subjects at rest: LV ejection fraction (66.7 +/- 11 p. 100 vs 63 +/- 7.7 p. 100), RV ejection fraction (50.3 +/- 7.2 p. 100 vs 54 +/- 14.8 p. 100). The response to effort of the right and left ventricles depends on the type of exercise. The LV ejection fraction increased normally whilst the RV ejection fraction showed a lot of individual variation. Equilibrium radionuclide angiography was also used to calculate the ratio of right to left end diastolic ventricular volumes. This ratio indicates the degree of RV diastolic overload when LV diastolic volumes are normal, which was the case in our series. The study group showed a significant increase in this ratio compared to control subjects (1.94 +/- 0.65 vs 1.2 +/- 0.23). Exercise radionuclide imagery should provide more accurate assessments of the surgical results and prognosis of these children providing standardised protocols are used. These investigations are best performed in patients over 15 years old, so as to avoid the technical difficulties related to small size. Resting studies are possible at all ages.

Adolescent↗

[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↗

[Late ventricular tachycardia after surgical correction of congenital cardiopathy. Impact, severity and predictive factors].

A group of 6 patients operated for Fallot's tetralogy (4 cases) or a similar congenital cardiac malformation (2 cases) were studied to assess the prevalence, severity and predictive factors of late post-operative ventricular tachycardia. These 6 patients were compared with a control group of 44 patients undergoing complete correction of Fallot's tetralogy. The age at operation (6 +/- 2 years), the length of follow-up (8 +/- 2.5 years) and surgical technique were comparable in both groups. The clinical parameters and results of non-invasive investigations (resting ECG, Holter monitoring) were compared. The first attack of tachycardia occurred on average 3 years after surgery. A triggering factor was found in 3 cases (a blow on the chest--2 cases--, general anaesthesia--1 case). These postoperative tachycardias are characterised by poor clinical tolerance, a tendency to recur, resistance to therapy and reserved prognosis. They are significantly commoner in patients operated late and in those with a poor haemodynamic result: right ventricular systolic pressure greater than 60 mmHg and recurrence of the ventricular septal defect. The combination of ventricular tachycardia and a poor haemodynamic result is associated with a mortality rate of 33 p. 100. The functional status of the patient, conduction defects and the surgical technique used were not predictive of ventricular tachycardia. The detection of a ventricular arrhythmia on the resting ECG, and, particularly, on Holter monitoring, and the induction of ventricular tachycardia by ventricular stimulation should lead to treatment against malignant ventricular arrhythmias and sudden death. The choice of anti-arrhythmic agent may be guided by endocavitary stimulation techniques to assess its efficacy.

Child↗

[Supraventricular arrhythmia in newborn infants and interatrial septal aneurysm].

Three neonates with relapsing supraventricular arrhythmia, diagnosed in utero in one case, presented with aneurysm of the atrial septum (AAS) found at TM and bidimensional echocardiographic examination. Arrhythmia consisted of atrial flutter 1/1 or 2/1 (1 case) or supraventricular extrasystoles with episodes of supraventricular tachycardia (2 cases). Although AAS not associated with congenital heart disease related with a downstream obstacle on the right or left heart is considered a rare condition, it is likely that echocardiography will disclose it more frequently. Anyhow, it seems worthwhile searching for it in cases with apparently primary supraventricular arrhythmias. Although the precise relationships between arrhythmia and AAS are not yet known, one could suggest that this organic anomaly is susceptible of originating and of maintaining the arrhythmia longer, if not in a more sever fashion, than in neonatal isolated arrhythmia, especially without any preexcitation syndrome. Further studies are necessary for a better understanding of the meaning and mechanism of such an association.

Arrhythmias, Cardiac↗

[Abnormal origin of the anterior interventricular artery from the pulmonary artery responsible for a postinfarction left ventricular aneurysm in a 6-year-old girl].

The isolated abnormal origin of the anterior interventricular artery from the pulmonary artery with the circumflex artery, arising normally from the right coronary artery and implanting in the aorta is much less common than the origin of a common left coronary trunk from the pulmonary artery. The first condition is also better tolerated than the second. In the present case, the clinical tolerance was excellent, but there was, nevertheless, a complicated antero-apical necrosis of a left ventricular aneurysm, which was successfully resected at the same time as the reimplantation of the anterior interventricular artery into the aorta. The patency of the anastomosis was subsequently confirmed.

Child↗

[Central and peripheral coronary-pulmonary fistulae. Apropos of 4 case reports].

The authors report four cases of fistulae between coronary arteries and the main pulmonary artery or its branches. Proximal fistulae, often considered with coronary-cardiac fistulae (between coronary arteries and cardiac cavities, usually in the right heart) probably result from accessory coronary buds originating in the truncus arteriosus, on its pulmonary part, as in anomalous coronary arteries stemming from the pulmonary trunk. Two such cases are reported, including one in a girl with severe congenital mitral insufficiency. Peripheral fistulae probably do not result from a single mechanism, and their congenital or acquired origin is often difficult to determine, especially in adults. Nevertheless, in a 54-year-old female with angina by coronary steal, without any associated pulmonary disease, the fistula is probably congenital. On the other hand, in a boy who had a Blalock operation for Tetralogy of Fallot, the fistula is probably acquired, as part of the collateral circulation which developed after pleuro-pericardial symphysis. Such fistulae usually do not require surgery except in cases of coronary artery steal, which are exceptional in children but not infrequent in adults.

Adolescent↗

[Use of emergency 2-dimensional echocardiography in the diagnosis and maintenance of newborn and nursing infants hospitalized because of poorly tolerated cardiopathy. A prospective study of 82 cases].

The value of 2D echocardiography was assessed prospectively in 82 patients (average age 2.6 months) including 41 newborn babies, in the emergency investigation of cardiac distress. The recording was made before any other investigations or treatment and associated subcostal and supra sternal views, and the results were compared with angiography (64 cases) or anatomical findings (44 cases). A cardiac abnormality was excluded in 5 cyanotic newborn children (persistent foetal circulation) and a precise diagnosis was made in 74 of the remaining 77 patients (94 p. 100). All cases of malposition of the great vessels were recognised (17 cases). The diagnosis was based on the simultaneous recording of the vessels, proximal branches and their ventricular connections. In conditions with severe obstruction of the pulmonary outflow tract (26 cases), 2D echo enabled an exact evaluation of ventriculo arterial concordance, pulmonary confluence and a quantitative assessment of the diameter of the pulmonary artery in 25 patients. Angiography provided little further information, mainly concerning associated lesions (anterior muscular VSDs, abnormalities of the supra aortic vessels). On the other hand, 2D echocardiography was superior to angiocardiography for the study of the atrioventricular values and intracavitary and subvalvular obstruction. 2D echo is a reliable method for emergency assessment of the indication for therapeutic catheterisation (atrioseptostomy). In other cases it allows it to be deferred and guided. The comparison of the results of 2D echo and angiocardiography in this series suggests the possibility of reducing the number of endocavitary investigations in the newborn and infant in the future, even in cases where surgery is being considered.

Angiocardiography↗

[Cardiovascular malformations associated with tetralogy of Fallot. Apropos of a series of 250 cases of tetralogy of Fallot].

This study was undertaken in 39 patients with Fallot's tetralogy associated with one or more cardiovascular malformations. Some common anomalies, simple anatomical variations were excluded (right sided aortic arch, patent foramen ovale). Out of a total of 48 anomalies (6 children had 2, 3 or 4 associated malformations) there were 5 anomalous systemic venous drainages (including 4 supernumerary left superior vena cava), 8 secundum atrial septal defects (including one multiperforated defect), 2 muscular ventricular septal defects, 7 atrioventricular canals (5 in mongol children); 1 tricuspid hypoplasia, 1 absent left pulmonary artery, 1 mitral valve prolapse, 1 obstructive cardiomyopathy, 10 coronary anomalies (including 5 ectopic left anterior descending arteries arising from the right coronary artery), 8 patent ductus arteriosus (arising from the left subclavian in 4 cases of right aortic arch), 3 retro oesophageal subclavian arteries and one congenital subclavian steal syndrome. The incidence of associated malformations was 15 p. 100, but values of up to 30 p. 100 have been reported mainly in anatomical studies. The authors emphasise the diagnostic and therapeutic implications of these associated malformations. In most cases, especially Fallot's tetralogy with an endocardial cushion defect, 2D echocardiography proved to be an essential diagnostic tool. Catheter studies, however, remain necessary, especially for the detection of aortic and coronary anomalies: selective coronary angiography is sometimes required. Difficult surgical problems are mainly encountered when the associated anomaly has not been diagnosed preoperatively. Although the variations of systemic venous drainage are not of great importance, those of the aortic arch can influence the technique of palliative procedures. Muscular VSD must not be missed. Tricuspid hypoplasia may pose a complex surgical problem. Malformations of the left heart which are sometimes unrecognised, aggravate the situation considerably. The association of Fallot's tetralogy and atrioventricular canal can now be corrected under good conditions. Finally, variations in the coronary anatomy must be documented meticulously as they may contraindicate early complete repair.

Angiocardiography↗

[Double isthmic coarctation of the aorta: "tandem" coarctation].

A second case of double coarctation of the thoracic aorta is reported, the first having been observed at the Marie-Lannelongue Surgical Center in an older child. This case was a 3 months infant in which the missed pre- and postoperative diagnosis led to reoperation after control catheter and angiographic studies. These investigations were carried out one month after the first operation because of persistent severe cardiac failure. Surgical cure in two stages consisted in a Waldhausen plastic enlargement procedure and a Crafoord-type resection anastomosis, ensuring the best chances for a good result.

Aortic Coarctation↗

[Importance of the exercise test in the follow-up of surgically treated congenital aortic stenoses].

Sixty four children with isolated congenital aortic stenosis (39 valvular, 16 fixed subvalvular, 4 supravalvular and 5 multiple) were operated at a mean age of 11,5 years. Valve repair was possible in all but three patients who had to undergo valvular replacement. Myotomy was associated in 18 cases (28 p. 100). The mean systolic pressure gradient was 79,9 mmHg (+/- 17,8); there was associated aortic regurgitation in 21 patients but this was minimal except in one case. Twenty children (31 p. 100) had symptoms on effort and the basal ECG showed ST-T wave changes in the left precordial leads in 30 cases (47 p. 100). Several preoperative exercise ECGs were performed in 29 patients without ST-T changes on the resting ECG. The exercise ECG was positive in 15 patients, providing one of the arguments for surgery; a poor blood pressure response to exercise was observed in 12 patients with a negative test. Out of the 28 patients with a positive preoperative exercise ECG, 7 (25 p. 100) went on having a positive result after surgery (p less than 0,05). The maximal heart rate was not significantly higher after surgery but the total work was significantly greater (p less than 0,01) and the increase in systolic blood pressure was even more significant (p less than 0,001). Out of 14 patients undergoing repeat catheterisation for a continuing positive exercise ECG or for ST-T wave changes on the resting ECG, there were 6 residual severe stenoses, 3 severe aortic regurgitations, 3 hypertrophic cardiomyopathies which were obstructive in 2 cases. The exercise ECG is a means of appreciating the consequences of the stenosis which are the cause of the complications (myocardial ischemia and poor blood pressure adaptation). This justifies its use in assessing the surgical indications and for the follow-up of the surgical result. A persistantly positive exercise ECG and continuing ST-T wave changes on the resting ECG are signs of a poor surgical result and hemodynamic revaluation should be considered; besides severe postoperative aortic regurgitation, residual or recurrent stenosis and, above all, asymmetric septal hypertrophy, obstructive or not, are the main causes of poor postoperative results.

Adolescent↗

[Outcome of patients undergoing open-heart surgery for congenital heart disease in early childhood].

Corrective surgery for congenital heart disease is performed on progressively younger children in order to restore normal haemodynamics during infancy and to avoid the disadvantages of palliative operations. The authors consider the long-term outcome of children who have been operated for complete transposition of the great vessels--by physiological correction or by anatomical correction--for tetralogy of Fallot, interventricular communication, atrio-ventricular channel and aortic stenosis. The sequelae and complications of these various operations are reviewed and the authors stress, in particular, the risk of delayed arrhythmias and disturbances of ventricular function. The risk of delayed complications justifies long-term follow-up of all these patients, even those who are leading a normal life, i.e. the majority of them.

Cardiac Surgical Procedures↗