[Double-chamber right ventricle, aortic subvalvular stenosis and interventricular septal defect. Apropos of 12 cases].

The authors report 12 cases of double-chamber right ventricle associated with discrete subaortic stenosis and ventricle septal defect. The statistics derived from 3,292 surgical reports of congenital heart diseases operated on at the Marie-Lannelongue Surgical Center over an 8 years period show that this association is 7 times more frequent than the law of chance. Twenty-two per cent of double-chamber right ventricles had an associated discrete subaortic stenosis and, in 9% of cases of subaortic stenosis a double-chamber right ventricle was observed.

[Outcome of patients with Eisenmenger syndrome. Apropos of 62 cases followed-up for an average of 16 years].

In an era when heart-lung transplantation offers a therapeutic option for patients with Eisenmenger's syndrome, it is important to assess the natural history of this condition. With this objective the authors studied 62 patients followed-up by the same cardiologist. The average follow-up period was 16 years, but 22 patients were followed up for over 20 years.

[Valvotomy and congenital aortic valve stenosis. Long-term results].

Twenty nine patients (average age 11 years) underwent valvotomy for congenital valvular aortic stenosis from 1967 to 1983. The medium and long-term results were analysed retrospectively: 14 children have been reoperated; 11 for restenosis and 3 for aortic regurgitation after an average period of 11 years. Thirteen of the other 15 patients have been regularly followed-up for about 10 years: there are 7 good results, 3 average results and 3 restenoses.

[54 cases of infectious endocarditis seen in 32 years in a population of 2038 congenital heart diseases].

A longitudinal study of a 32-year period (1954-1987) involving 2038 patients with congenital heart defects followed by the same physician yielded 54 cases of infective endocarditis (IE). Complex cyanogenic cardiopathies were particularly exposed to the risk of infection (8.2 IE for 1000 patient-years), then came ventricular septal defects (2.

[Tetralogy of Fallot. 20 years' experience in 144 cases].

Between 1968 and December 1987, 144 patients with tetralogy of Fallot were examined at La Pitié Hospital, Paris. 76 were female and 68 male, with a mean age of 8.1 years at the first visit.

[Atrial septal defect and cyanosis. Apropos of 6 cases related to abnormal drainage of the inferior vena cava into the left atrium].

The authors report six cases of atrial septal defect (ASD) associated with abnormal drainage of the inferior vena cava into the left atrium responsible for right-to-left shunting, without pulmonary hypertension. The abnormal drainage could be due either to an anatomical malposition of the inferior vena cava opening into the left atrium, or to an abnormal blood flow from this vein, normally located through a low ASD, under the influence of anatomical, mechanical and haemodynamic factors. Clinically, all patients presented with light cyanosis and with the usual signs of ASD.

Congenital valvular aortic stenosis in adults.

Based on a study of 20 patients operated on between the ages of 21 and 38 for congenital valvular aortic stenosis, the distinguishing features of congenital aortic valvular stenosis in adults are reviewed: valvular calcification (75%), valvular dome rarely present (10%), usually moderate cardiac disability (70%), diminished or inaudible second heart sound (50%), associated diastolic murmur (75%), electrocardiographic left ventricular hypertrophy (70%), and an infrequent protosystolic click (30%). Surgery is necessary for symptomatic patients. If patients are asymptomatic, surgery is decided after measuring the left ventricle-aortic pressure gradient by continuous Doppler wave study, or by cardiac catheterization.

[Origin of the common pulmonary vein, septation of the primary sinus venosus atrial situs and theory of the "sinus man"].

Situated at the entry to the heart, the sinus venosus regulates at an early stage the distribution of the veins. Originally symmetrical, it receives on either side an omphalomesenteric vein, a common cardinal vein (duct of Cuvieri, ductus cuvieri) and a common pulmonary vein. This symmetrical pattern disappears with the obliteration of the rough right pulmonary vein and the invagination of the left ductus cuvieri into the sinusal cavity.

[Percutaneous transluminal angioplasty in complex congenital cardiopathies. Apropos of 2 cases].

Percutaneous transluminal angioplasty (PTA), already widely used in stenosis of peripheral, renal or coronary arteries, has now been extended to congenital heart diseases. Thus, in pulmonary or aortic orificial stenosis this simple and fairly safe method has proved to be a suitable alternative to surgery. In other cases it may be used for tiding the patient over a critical period pending surgery.

[Dissecting aneurysm of the extracranial vertebral arteries and pregnancy].

A 32-year-old woman was at the end of her first and-until then normal-pregnancy, when she abruptly experienced a severe neck pain. Delivery was provoked two days later but a week after she suddenly developed a left lateral medullary syndrome. Angiography disclosed a severe diffuse and irregular narrowing of both vertebral arteries in their second and third segments, typical of dissecting aneurysm.

[Eisenmenger's syndrome and pregnancy].

The consequences of pregnancy, abortion or tubal ligature were studied in 11 women with the Eisenmenger syndrome. Seventeen cases of pregnancy and 7 tubal ligatures were reviewed. In the 4 pregnancies continued of term and preceded by cardiac catheterisation, there were two maternal deaths on the 2nd and 7th day of the postpartum period due to pulmonary embolism confirmed in one case and suspected in the other.

Migration and torsions of the conotruncus in the chick embryo heart: observational evidence and conclusions drawn from experimental intervention.

It seems difficult to escape the conclusion that conotruncal migration and torsions do occur: The amplitude, direction and timing of these movements can be accurately tracked and they can be experimentally arrested. The movements have a significant function: Associated with the partitionings, they regulate the outlet ventricular distribution at two levels, permitting an adjustment between the ampullae and conus (a function of migration) and between the proximal and distal conal segments (via torsions). Their defects result in malalignments--double outlet right ventricle and transposition of the great vessels, respectively.

[Ebstein's disease associated with Fallot's tetralogy. Apropos of a familial case, review of the literature, embryologic and genetic implications].

Ebstein anomaly is a rare congenital cardiac malformation, representing 0.3 p. 100 of all congenital heart disease.

[Single ventricle and malposition of the dorsal part of the interampullary septum. Experimental study on the chick embryo heart].

After the blood has passed through the atrioventricular orifice or orifices or the atrioventricular canal when present, it is distributed to the ventricular ampullae. This distribution depends on the position of the dorsal component or dorsal horn of the interampullary septum with respect to the atrioventricular canal. The position of dorsal horn seems itself to depend on unequal or differential growth of the basal part of the ventricular ampullae.

[Measurement of arterial pressure in children. Influence of the resting time, inflatable surface and type of arm-band].

Measurements of arterial pressure in children should be standardized as much as possible. The influence on readings of three factors--pre-measurement resting time, area of the inflatable surface and type of cuff--was investigated in 151 children divided into several homogeneous groups. The study showed that reliable values can only be surface area, it would appear that no modern apparatus provides accurate readings for all age groups.

[Subvalvular aortic stenosis in adults. Apropos of 31 cases].

The aim of this cooperative study was to analyse the clinical profile of subvalvular aortic stenosis (SVAS) in adults. Thirty one cases were collected : patients aged 17 to 60 years (average 34 years). The diagnosis was confirmed in all patients either at surgery (30 cases) or at autopsy (1 case).

[Coronary arteries and their variations. An embryological explanation].

The main coronary arteries originate from three vascular circles: The atrioventricular circle, which forms the right coronary artery and the circumflex branch of the left coronary artery; the interampullary circle, which gives rise to the anterior and posterior descending coronary arteries; and the conotruncal circle, also known as the circle of Vieussens, which communicates with the lumen of the truncus arteriosus by way of the coronary ostia and which also anastomoses with the other two coronary arterial circles, thereby establishing the definitive coronary arterial circulation. A diagram facilitates an easy representation of the numerous variations of the coronary arteries that have been described.

[Myocardium-cardiac jelly dissociation and conal torsion. A study on the chick embryo heart].

Conal twisting seems to result from dissociation between the cardiac jelly and the deep myocardial interface like the lining in the sleeve of a jacket. The conal ridges are the natural markers of the jelly and endocardium. They are responsible for septation of the conus and enable the twisting to be observed and measured.

Familial congenital heart disease: how are the various types related?

The distribution of congenital heart lesions was studied in 238 families with at least 2 affected members. A statistical analysis was performed. Concordant lesions were found in 48% of the affected first degree relatives and in 28% of the affected second and third degree relatives.