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Biomedical subjects

C Pernot

Publications and source records attributed to C Pernot.

At least 109 records · Page 6Linked to original sources

[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↗

[Cerebral complications of congenital cardiopathies].

In children with congenital heart disease, cerebral vascular accidents may have a variety of causes. Beside subacute endocarditis, complications of heart surgery, emboli and hypertensive accidents in some rare non-cyanotic cardiopathies, emphasis must be laid on the complications of chronic anoxia in cyanotic cardiopathies, anoxic spells, ischaemic cerebral accidents and abscesses of the brain. The latter have become rare and should mostly be feared in older children with inoperable cyanotic cardiopathy. Cerebral vascular accidents proper are a greater cause for concern, since they are not uncommon--especially in young children with Fallot's tetralogy or complete transposition of the great vessels awaiting surgery--, have serious sequelae and occur without warning. Their mechanism, and therefore their prevention, differs according to age. They are particularly dangerous in infants and in children below the age of 4. The only effective prevention is early and complete surgical correction of the cardiopathy.

Brain Abscess↗

[Outcome and monitoring of children following cardiac surgery].

The spectacular advances in the surgery for congenital heart disease in children and infants have led to the appearance of a new pathology, late complications which warrant a systematic cardiological surveillance, to allow the patient to lead as normal a life as possible, compatible with the potential sequelae of this type of surgery. These complications arise essentially after ventricular open heart surgery, especially for tetralogy of Fallot and for interventricular communications. The ventricular function may be altered, even if the functional status appears to be good, so it must be systematically monitored, in particular by isotope methods. Arrhythmias and conduction disorders require special attention as they are often delayed in their appearance and can be serious, even causing sudden death, hence the value of electrophysiological investigations and, more especially, Holter monitoring and stress tests. The problems are even more complex when the operation was only aimed at a physiological correction, especially after operations for complete transposition of the great vessels and so-called "ortho-terminal" operations for tricuspid atresia or a single ventricle. In these cases, as well as ventricular dysfunction, we also see arrhythmias which can sometimes be severe, requiring anti-arrhythmic treatment and sometimes electro-systolic pacing.

Cardiac Surgical Procedures↗

[Chromosome 11 and cancer].

Two cases with chromosome 11 anomaly related to cancer are reported. The first one has a pericentric inversion (inv. p14 q12) with sympathoblastoma and Ondine's curse. The second one has a deletion (11p13) with aniridia and catalase deficiency but without Wilms tumor at two year of age. Retinoblastoma, nephroblastoma and sympathoblastoma may be related to genome modification. The mechanism of oncogenesis are discussed.

Abnormalities, Multiple↗

[Assessment of the branches of the pulmonary artery by 2-dimensional echocardiography].

Sixty three cases of Fallot's tetralogy aged from 1 month to 30 years old, were studied by 2D echocardiography to evaluate the diameter of the pulmonary arteries and to detect stenosis of the main pulmonary arteries. The right pulmonary artery was visualised clearly enough to be measured in all 63 cases whereas the left pulmonary artery could only be adequately recorded in 58/63 cases. The junction of the two pulmonary arteries was confirmed by 2D echo in 61/63 cases; in two cases, the left pulmonary artery was not connected (2/63), confirmed at angiography and surgery. Six stenoses of the pulmonary arteries, confirmed surgically (6/7), were detected by 2D echo but there were also 3 false positive results. The pulmonary arteries were measured from suprasternal views; the values obtained ranged from 3 to 15 mm. There was a good correlation with the angiographic measurements (R = 0.81 for the right pulmonary, and R = 0.82 for the left pulmonary arteries). Good correlations were also observed between the peroperative and 2D echo measurements (R = 0.84 for the right pulmonary; R = 0.77 for the left pulmonary artery). 2D echocardiography is a non-invasive reliable technique for visualising the pulmonary arteries and their origin, for measuring the calibre of these vessels and for detecting severe proximal pulmonary artery stenosis.

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↗

[Giant congenital paravertebral arteriovenous fistulas in children].

Six cases of giant congenital paravertebral arteriovenous fistula in children are presented. In one case the fistula was jugulocarotid, in another case between the vertebral artery and jugular vein. Two were cases of fistulae in the lower thoracic region, and the last two were subclavian fistulae, one of which caused the death of the neonate. The principal embryologic and clinical features of these anomalies are described, and the choice between treatment by surgery or embolization discussed in the light of the new materials available for the latter procedure.

Aorta↗

[Thymolipomas simulating cardiomegaly].

With reference to three personal observations, the authors discuss the problems raised by the diagnosis of thymolipomas, which are benign asymptomatic tumors discovered on a plain chest film and simulating cardiomegaly. On careful analysis of a high voltage plain chest film, the pulmonary vasculature can be detected through the cardiomediastinal opacity. Total body scan is also useful for establishing diagnosis before surgery.

Aged↗

[Radiological anomalies in the Noonan syndrome (author's transl)].

In the Noonan syndrome various anomalies can be demonstrated radiologically. Of particular interest are cardiovascular lesions, such as atypical pulmonary stenosis, hypertrophic cardiomyopathy and the lymphangiectasias. Skeletal anomalies can be found, including retardation of bone maturation and sternal abnormalities. Other anomalies concern the urinary tract (pyeloureteral stenosis) and the digestive system. Radiological diagnostic procedures allow to disclose various elements of this polymalformative syndrome. They should be performed whenever suggestive clinical signs are present, e.g. hypertelorism, gonadal dysgenesis, down-slanting palpebral fissures and shield-like chest.

Bone and Bones↗