[Partial atrioventricular block and prolonged QT interval in 4 premature infants receiving diphemanil]. / Bloc auriculo-ventriculaire partiel et allongement de QT chez quatre prématurés recevant du diphémanil.

Four premature infants presenting with episodes of bradycardia in the first weeks of life were given diphemanil. One of them received an overdose accidentally. Paradoxically, this induced a permanent bradycardia leading to the discovery of a grade II A-V block as well as a prolonged QT interval. Discontinuation of the drug resulted in a prompt normalization of these changes. It is felt that this anticholinergic therapy may have caused a prolongation of the QT interval and, therefore, a partial A-V block in case of sinus tachycardia. Thus, such a therapy should not be given to young premature infants without having checked the QT interval on a ECG tracing and having made sure that it is adapted to the actual heart rate. It is also advised to reduce usual doses.

Congestive cardiomyopathy and spasm of coronary microvascularisation in man. Electron microscopic evidence.

This paper reports our observations of right ventricular biopsies taken from a 23 year old patient with a congestive cardiomyopathy. The ultrastructural examination of these samples revealed coronary capillary and arteriolar spasms, as well as an ischemic aspect of the myocytes. The responsibility of the coronary microcirculation in the genesis of dilated cardiomyopathies has been suspected for a long time. It has recently been demonstrated in the Syrian hamster, but it has never been clearly evidenced in man. The case that we present seems to confirm this hypothesis. The fact that the spasm is a transitory phenomenon and that it most probably does not involve all areas of the myocardium simultaneously could explain the difficulty encountered in observing this phenomenon in endomyocardial biopsies.

[Hemodynamic development of the auricular communication of the ostium secundum and sinus venosus type. Study of 1189 patients]. / Evolution hémodynamique de la communication auriculaire de type ostium secundum et sinus venosus. Etude de 1,189 patients.

The haemodynamic data of 1,189 subjects with an atrial septal defect catheterised between 1953 and 1983 at the Marie Lannelongue Surgical Center and at Broussais Hospital was reviewed. Sixty per cent were under 20 years of age and 40 per cent were 21 to 75 years of age. The defect was an ostium secundum type in 89 per cent and sinus venosus in 11 per cent of patients. The commonest associated cardiac malformation was a partial anomalous pulmonary venous drainage which was present in 125 patients. Systolic and mean pulmonary arterial pressures decreased from a value slightly higher than normal between birth and 5 years of age to normal values between 5 and 20 years of age and then increased progressively to maximal values attained after 50 years of age (p less than 0.001). The proportion of patients with raised systolic and mean pulmonary arterial pressures increased with age after 20 years of age (p less than 0.001). A parallel increase in pulmonary blood flow was observed after 20 years of age, which was less marked than the increase in pressures, but nevertheless statistically significant (p less than 0.05). This increase in left to right shunt disappeared after 50 years of age. An increase in pulmonary resistances was observed after 20 years of age and persisted all through life (p less than 0.001). Two successive preoperative catheter studies were performed in 34 patients after a 6.4 +/- 4.7 years interval. In each patient, systolic pulmonary arterial pressures had increased and were significantly higher at the time of the second study (p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

[Surgical closure of interventricular communications before 2 years of age. Long-term clinical and electrocardiographic course]. / Fermeture chirurgicale des communications interventriculaires avant 2 ans. Evolution clinique et électrocardiographique à long terme.

The long-term follow-up of the first 100 children under 2 years of age, presenting with ventricular septal defect (VSD) who underwent surgical closure between 1972 and 1976 at the Centre Chirurgical Marie-Lannelongue, was studied. Only cases with isolated VSD were taken into account except for 18 cases with ductus arteriosus related to operation and 41 cases with atrial septal defect (ASD) or persistent foramen ovale. This study included clinical and electrocardiographic investigations focused on the long-term follow-up of conduction disorders. Clinical tolerance was excellent with only one Eisenmenger's syndrome induced by a residual VSD. Three secondary cases of aortic regurgitation without present functional repercussions were observed. Conduction disorders were considered as unchanged or even improved. There were no sudden death, no secondary atrioventricular block (AVB), no trifascicular block even after one case with transitory immediate postoperative AVB.

[Aortic subvalvular stenosis caused by accessory mitral tissue]. / Sténose aortique sous-valvulaire par tissu mitral accessoire.

Three cases of subaortic stenosis due to accessory mitral tissue are presented. The diagnosis of the cause of obstruction was correctly established in each child before surgery, by 2D echocardiography and cineangiography. Resection of the accessory mitral tissue through a simple aortotomy gave excellent results. The eight previously published cases are discussed, emphasising the importance of accurate preoperative diagnosis. The cause of obstruction is difficult to determine at surgery, which may have disastrous consequences, whilst simple resection of the abnormal tissues leads to a complete and definitive cure.

[2-dimensional echocardiography of congenital obstruction of left ventricle inflow. Apropos of 25 cases]. / Echocardiographie bidimensionnelle des obstacles congénitaux au remplissage du coeur gauche. A propos de 25 cas.

Twenty-five cases of left ventricular inflow obstruction in children aged 4 months to 11 years were investigated by two-dimensional echocardiography and cineangiography. The results of 2D echo were compared with the operative appearances in 15 cases and the pathological findings in three cases. This series included 5 cases of cor triatrium and 20 of congenital mitral stenosis, 3 of which were associated with a supramitral ring. Abnormalities of the mitral papillary muscles were the main cause of congenital mitral stenosis in this series (12 cases); there were 6 cases of single papillary muscle and 6 cases with two closely situated or fused papillary muscles. These abnormalities of the mitral papillary muscles were demonstrated on the transverse left ventricular views obtained from the parasternal or subcostal regions. All the papillary muscle abnormalities diagnosed by 2D echo were confirmed at autopsy or surgery. Cases of mitral stenosis due to short and thickened chordae were demonstrated on parasternal long axis views. Hypoplasia of the mitral ring was visualised on long axis or apical views. The supramitral ring was demonstrated on the parasternal long axis or apical four chamber views, as an abnormal echo situated just above the mitral valve in the left atrium. All three cases in this series were correctly diagnosed by 2D echocardiography. Nineteen of the 20 congenital mitral stenoses were associated with other cardiac malformations (14 coarctations of the aorta, 9 aortic stenoses, 4 ventricular septal defects) which were also detected by 2D echocardiography.

[Outcome of infants operated on for aortic coarctation before 3 months of age]. / Devenir des nourrissons opérés avant 3 mois d'une coarctation aortique.

Between January 1966 and June 1982, 177 infants less than 3 months of age underwent surgical cure of coarctation of the aorta. Seventy-one percent had associated cardiac lesions not including a patent ductus arteriosus. With the cure of the coarctation either by resection anastomosis or angioplasty, 45% underwent associated surgical procedure, mainly pulmonary artery banding. Early mortality was 26% for the last 4 years of the study. Early deaths, significantly correlated with the severity of the preoperative heart failure and the importance of pulmonary hypertension, were not correlated with an associated heart disease. Of the 95 survivors operated on before December 31, 1981 and with a long follow-up, 18 died later on. Late deaths are related with associated cardiac malformations. Thirty-nine (41%) of the survivors had recurrent coarctation. This was correlated with the year of surgery and not with the surgical technique. In cases with coarctation of the aorta surgical cure should not be withheld before age 3 months, when there is heart failure, with or without associated cardiac malformations. The mortality, even in isolated coarctation (6%), the occurrence of sudden deaths after good surgical repair, the frequency of recoarctations lead to suggest that in infants under 3 months of age, careful supervision and conservative medical treatment should be considered, if the coarctation is isolated and well tolerated.

[Assessment of the branches of the pulmonary artery by 2-dimensional echocardiography]. / Evaluation des branches de l'artère pulmonaire par l'échocardiographie bidimensionnelle.

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.

[Paroxysmal Hisian tachycardia and familial sarcoidosis]. / Tachycardie hisienne en salve et sarcoïdose familiale.

We report a His-bundle tachycardia in a 6 year-old patient without evidence of heart disease. Diagnosis and follow-up were assessed by exercise stress testing and repeated long-term electrocardiographic recording. As sarcoidosis was present in the mother and the aunt, this condition was discussed in this child also. However, since there is no absolute proof for this disease, the hypothesis of an antenatal injury of the bundle of His was discussed: this could be secondary to the transplacental transfer of abnormal maternal antibodies, as frequently observed in women with clinical or biologic evidence of connective tissue disease.

[Two-dimensional echocardiography in subvalvular aortic stenosis. Apropos of 20 cases]. / Echocardiographie bidimensionnelle des sténoses aortiques sous-valvulaires. A propos de 20 cas.

Twenty cases of discrete subvalvular aortic stenosis in children aged from 8 months to 23 years were examined by 2-dimensional echocardiography and cineangiography. Fifteen of these patients subsequently underwent open heart surgery to relieve the obstruction. In 18 cases the obstacle was a fixed stenosis (13 type I, 3 type II and 2 type III) and in two cases, the obstacle was provoked by accessory tissues of the anterior mitral leaflet. The echocardiographic incidences used were the left parasternal long axis, the apical LAO-equivalent, and longitudinal and transverse subcostal views. In type I, fixed subvalvular stenosis, one fine abnormal echo was visualised in the left ventricular outflow tract throughout the cardiac cycle immediately below the aortic valve, best seen on apical views. Twelve of the 13 stenoses of this type were demonstrated by 2D echocardiography. In type II stenosis an abnormal thickened echo was visualised in the left ventricular outflow tract at a distance from the aortic cups. The adherence of this abnormal echo to the anterior mitral leaflet was best demonstrated by apical views. All three cases of this type were demonstrated by echocardiography. Type III stenosis was characterised by widespread narrowing of the subaortic region with irregular borders. Obstruction due to accessory tissues of the anterior mitral leaflet were well demonstrated in left parasternal long axis and apical views. They showed an even rounded mass of echos attached to the anterior aspect of the anterior leaflet prolapsing into the left ventricular outflow tract. Both cases in this series were diagnosed by 2D echocardiography. Therefore, 2D echocardiography with apical and subcostal views is a valuable method for diagnosing subvalvular aortic stenosis and for determining its anatomical type.

[Diagnosis and localization of ventricular septal defects by two-dimensional echocardiography. 50 cases]. / Diagnostic et localisation des communications in interventriculaires par l'échocardiographie bidimensionnelle. A propos de 50 cas.

A series of 50 children aged between 1 month and 15 years old, with ventricular septal defects (VSD) were investigated by two-dimensional sector scanning and cineangiography. Fifty other children of the same age group with congenital heart disease without VSD were also investigated by two-dimensional echocardiography and angiography. No VSDs were diagnosed by echocardiography in children with angiographically intact inter ventricular septa. In the group with VSD, two-dimensional echo localised with precision: --36 membranous VSDs (36/36) --5 infundibular VSDs (5/6) --2 isolated muscular VSDs (2/2) --I atrioventricular canal type VAD (I/I) --3 multiple VSDs (3/5). The subcostal view was the most useful for visualising the VSD in 49 out of 50 cases. The parasternal views only showed the VSD in 23 cases, and the apical views only in II cases. The dimensions of the left heart chambers and the movement of the interatrial septum gave an indication of the volume of the left-to-right shunt. Dilatation of the left heart chambers and bowing of the interatrial septum into the right atrium in systole were observed in all cases where QP/QS greater than 2, except when there was an associated atrial septal defect. Two-dimensional echo also detected associated lesions: pulmonary stenosis (9/9), prolapse of an aortic valve cusp (4/5), atrial septal defect (2/2), stradling of the tricuspid valve (I/2). Two-dimensional echocardiographic short axis subcostal views are reliable in the detection and localisation of VSD. Nevertheless, trabecular and apical VSDs are particularly difficult to visualise and the use of pulsed Doppler coupled with two-dimensional echocardiography should enhance the sensitivity in diagnosing this type of VSD.

[Heart with criss-cross ventricles associated with an obstruction of the ejection pathway of the left ventricle and a mitral anomaly]. / Coeur avec ventricules entrecroisés associé à une obstruction de la voie d'éjection du ventricule gauche et à une anomalie mitrale.

A case of criss-cross heart with situs inversus associated with subvalvular aortic stenosis and mitral regurgitation in a 9 year old boy is reported. Surgical correction of the valvular abnormalities gave an excellent clinical and haemodynamic result. A review of the literature shows that this abnormality of rotation of the ventricles coexists with a concordant atrioventricular connection in 70 % cases; the ventriculoarterial connection is only discordant in 12 % cases. The right ventricle is always situated above the left ventricle. The most common associated lesions are usually accessible to surgery; ventricular septal defect (98 % cases); stenosis or atresia of the pulmonary artery (70% cases). However, right ventricular hypoplasia is also common (65 % cases). Six patients underwent surgical correction of the associated lesions with 4 successes and 2 deaths. Surgery is possible in these complex cardiac malformations with good results providing a precise preoperative anatomical diagnosis is made.