[A case report of Williams and Beuren syndrome with associated aggravation of in situ distal pulmonary vascular obstruction due to thrombotic disease]. / Une observation de syndrome de Williams et Beuren avec aggravation de l'obstruction vasculaire pulmonaire distale par maladie thrombotique in situ associée.

We report a case of Williams and Beuren syndrome in which the obstruction of the pulmonary vascular bed, linked with peripheral stenoses of the pulmonary arteries, associated here with an interventricular communication, was aggravated due to the coexistence of in situ thrombotic disease, leading to a progressive hypoxaemia, requiring long-term anticoagulant treatment, and compromising the long-term prognosis. This case report puts into question the so-called favourable prognosis of pulmonary arterial branch stenoses in this malformation syndrome.

[Apparently primary acute heart failure in infants. Long-term outcome]. / Insuffisances cardiaques aiguës en apparence primitives du nourrisson. Evolution à long terme.

Cases of acute cardiac failure which are apparently primary, and occurring in babies or young children have one characteristics clinical picture. There are three etiological agents, which cannot be differentiated clinically: myocarditis, fibroelastosis, and primary non-obstructive cardiomyopathies. When the critical early period, which still carries a high mortality, has been passed, the outlook is relatively favourable. 61 babies or infants of less than 30 months with acute primary cardiac failure were followed up for a period of between 5 and 20 years. 33 of them were considered cured; 22 have minor sequelae (most frequently) left ventricular hypertrophy on X-ray or ECG); 3 have persistent cardiac failure despite treatment; 3 have died after a period of more than 5 years. There is no clinical, X-ray or ECG finding which can predict the longterm outlook in this condition. It is virtually impossible to differentiate between myocarditis and firboelastosis; it seems likely in the majority of cases that we are dealing with different modes of development in the same condition.

[Long-term results of 156 Mustard operations for simple transposition of the great vessels]. / Résultats à long terme de 156 interventions de Mustard pour transposition simple des gros vaisseaux.

156 children with simple transposition of the great vessels have been operated on by Mustard's technique between 1-6-1967 and 31-12-1976, with an immediate overall mortality rate of 8%. In the longterm, the 9% of late deaths and more especially the complications are regrettable; complications were either of severe arrhythmia (almost 30%) leading to 2 late deaths and 7 pacemaker implantations, or venous obstruction relating to problems with the patch (25%). Despite the improved techniques and consequent reduction in surgical mortality and late complications, longterm prognosis is still unsure because of the unknown fate of the intra-atrial partition, and that of the "passive" rhythms which are so commonly found after this type of surgery.

[Hypoplasia of the right ventricle and cyanosis due to left-right atrial shunt within the framework of Turner's syndrome. Surgical closing of the interatrial connection after occlusion testing with balloon catheterization]. / Hypoplasie du ventricule droit et cyanose par shunt droite-gauche auriculaire dans le cadre d'un syndrome de Turner. Fermeture chirurgicale de la communication interauriculaire après test d'occlusion par ballonnet au cours du cathétérisme.

The authors describe the unusual cardiac malformation in a young girl with Turner's syndrome: right ventricular hypoplasia and atrial septal defect, resulting in central cyanosis. A trial of temporary occlusion of the atrial septal defect at cardiac catheterisation before surgical closure suppressed the right-to-left shunt and the cyanosis without significantly changing the hemodynamic parameters. The authors review the types of cardiovascular abnormalities associated with Turner's syndrome and emphasize the value of a temporary occlusion test of defects in certain cyanotic cardiac malformations with atrial right-to-left shunts; it may lead to a relatively simple surgical procedure, as in the case described.

[A case of subannular aneurysm of the left ventricle]. / Un cas d'anévrysme sous-annulaire du ventricule gauche.

The authors present a case of sub-annular aneurysm of the left ventricle in a child of 7. It was associated with aortic incompetence, a ventricular septal defect, and coarctation of the aorta which were discovered during investigation of streptococcal septicaemia. As a first stage procedure, the aortic valve was repaired, the aneurysm was excised, and the ventricular septal defect closed. At a later stage, the coarctation was resected. The question of whether this lesion was congenital or acquired is discussed. After one year, the result remains satisfactory.

[Corrected transposition of the great vessels with stenosis of the left atrioventricular valve in a 40-year-old woman]. / Transposition corrigée des gros vaisseaux avec sténose de la valve auriculo-ventriculaire gauche chez une femme de 40 ans.

The authors reports the detection of a rare and remarkably well tolerated cardiac malformation in a 40 year old woman whose clinical presentation would have suggested an ordinary mitral stenosis, had there not been a number of very unusual signs. Cardiac catheterisation and angiography showed a corrected transposition of the great arteries with stenosis of the systemic atrioventricular valve. This case is used to illustrate the principal malformations observed in association with corrected transposition of the great arteries, especially the common and very variable abnormalities of the systemic atrioventricular valve. The unusual feature of the reported case was the stenotic nature of the valvular lesion and the fact that this was the only associated intracardiac abnormality, simulating, at first sight, a rheumatic mitral stenosis.

[Percutaneous occlusion by a detachable balloon of a fistula between the left coronary and the pulmonary artery]. / Occlusion par voie percutanée au moyen d'un balloon détachable d'une fistule entre l'artère coronaire gauche et l'artère pulmonaire.

A large fistula between the left circumflex coronary artery and the distal branches of the right pulmonary artery resulting in myocardial ischemia in a 52 year old woman with no angiographic atheromatous coronary lesions, was occluded by a detachable balloon introduced percutaneously and positioned at the origin of the fistula. A control angiogram performed one month later showed the balloon in position and the disappearance of the fistula. The Thallium myocardial scintigraphic abnormalities regressed after occlusion of the fistula. This is the second case (the first one in France) of radical non-surgical treatment of a congenital coronary fistula.

[Direct atriopulmonary anastomosis (modified Fontan) for single ventricle and its equivalents]. / Anastomose atrio-pulmonaire directe (Fontan modifié) pour ventricule unique et ses équivalents.

The modified Fontan procedure is being used in an increasing number of complex cyanotic cardiac lesions with pulmonary stenosis. Seven patients aged 11 to 24 years (average 17.5 years) underwent surgery by a technique derived from the Fontan procedure: direct atriopulmonary anastomosis without a tube or valve. The tricuspid valve when patent was closed with a patch. The diagnoses were: single ventricule (4 cases), Taussig-Bing anomaly (2 cases) and tricuspid atresia (1 case). All patients had associated pulmonary stenosis with low pulmonary vascular resistances. The great vessels were in L-malposition in 3 cases. The hospital mortality was nil. Transient atrial fibrillation was observed in 2 cases and was well tolerated clinically. The follow-up period ranges from 2 months to 4 years (average 2.3 years). All patients are acyanotic with no signs of right-sided failure and in sinus rhythm. Control cardiac catheterisation and angiography were performed in 6 cases and showed good function of the anastomosis and a mean atrial pressure of 14 mmHg. Direct atriopulmonary anastomosis offers a very acceptable surgical solution to certain forms of single ventricle or equivalent with low pulmonary pressures. The short and medium term results seem to be better than those of intraventricular repair.

[Complete correction of Fallot's tetralogy in infants. Preliminary results]. / Réparation complète de la tétralogie de Fallot chez le nourrisson. Résultats préliminaires.

Complete repair was undertaken in 28 infants with severe forms of tetralogy of Fallot because severe cyanosis or anoxic spells. Age at operation ranged from 8 days to 11 months (mean: 4,4 months). Among the 24 cases without any other intracardiac defect, there were 6 early deaths related in 4 cases to a wrong indication or a technical error. Re-investigation was performed in 14 out of the 18 survivors from 3 weeks to 6 months post-operatively: the result is good in 10 cases, fair in 1 case, and poor in 3 cases requiring twice a reoperation with 1 further death and 1 good final result. More accuracy in selecting the surgical patients and improvement in operative techniques should result in a further decrease of our early mortality rate. Primary complete repair should therefore become a substantial alternative to the palliative shunts which results seem to be hazardous in the more severe types of the defect.

[Exudative enteropathy and residual tricuspid insufficiency following repair of tetralogy of Fallot. Regression following tricuspid valvuloplasty]. / Entéropathie exsudative et insuffisance tricuspidienne résiduelle après réparation de tétralogie de Fallot. Régression après valvuloplastie tricuspidienne.

Chronic right ventricular failure was observed in a young female operated at 25 years of age for Fallot's tetralogy despite surgical refixation of the patch closing the ventricular septal defect which had previously worked loose. The signs of right ventricular failure were due to severe tricuspid incompetence, confirmed at catheterisation and selective right ventricular angiography. They were associated with major hypoproteinaemia which was not due to urinary loss nor hepatic dysfunction, but which was attributed to an exudative enteropathy. The correction of the valvular defect by valvuloplasty was followed by rapid correction of the biological abnormality. The authors review the relationship between serum proteins and cardiac disease, discuss those cardiac affections with known associations with exudative enteropathy, and also the possibilities of reversing the biological abnormality after surgical cure of the causal cardiac lesion.

[A platypnea-orthodeoxia syndrome]. / Un syndrome platypnée-orthodéoxie.

We report the case of a 68 years old patient with platypnea orthodeoxia syndrome who has been clinically suspected on cutaneous saturation position's variation. It has been confirmed by transthoracic and transesophageal echocardiography (TEE). TEE showed the size of patent foramen ovale (PFO), visualised the right to left shunt. A right heart angiography confirmed echocardiographic data and allowed successful closure by a Cardioseal percutaneous transcatheter button device implantation. Symptom's disappearance and a TEE control after 2 months confirmed the success of the procedure.

[Median-term results of the Ionescu-Shiley valve bioprosthesis (98 patients)]. / Résultats à moyen terme de la bioprothèse valvulaire de Ionescu-Shiley (98 patients).

Between April 1979 and September 1981, 98 patients have undergone valve replacement with an Ionescu-Shiley bovine pericardium xenograft (54 men and 44 women, average age 53 years, range 13 to 76 years). Fifty-three patients underwent aortic valve replacement (AVR); 28 had a mitral valve replacement (MVR) and 17 had double valve replacement (MAVR). Nine patients (9%) were operated as an emergency for endocarditis or for acute malfunction of a previously implanted prosthesis. The hospital mortality was 9% (AVR: 7%, MVR: 7%, MAVR: 17%). The eighty nine survivors were followed up for 6 to 37 months (average: 21 months). All patients were examined or contacted directly (as were the treating cardiologists) during the 2nd trimester of 1982. Eight patients were lost to follow-up. Six patients died during follow-up (6,7%) 3 to 22 months after surgery. The valvular prosthesis was the cause of death in 2 cases. Although only 37 patients (41%) were maintained on long-term anticoagulant therapy, there were no cases of thrombosis of the valve and the incidence of embolic complications was 0,9% per patient year in AVR and 2,6% per patient year in MVR. Four patients (2,6% per patient year) developed endocarditis on their prosthesis and 3 were cured by medical treatment alone. One patient was reoperated 1 year after the initial operation. One case (0,7% per patient year or 0,5% per valve year) of premature calcification was observed (18th month) in a 16 year old boy who was reoperated as an emergency without any success.(ABSTRACT TRUNCATED AT 250 WORDS)

[Myocardiopathies secondary to cardiovascular diseases: systolic strain and diastolic overload (author's transl)]. / Myocardiopathies secondaires aux affections cardio-vasculaires: contraintes systoliques et surcharges diastoliques.

All cardiovascular malformations compromise ventricular function and lead to alterations in the myocardial structure. Most of these are adaptive reactions. However, in some cases, this reaction overreaches its object and leads to an authentic myocardiopathy. This myocardiopathy is usually hypertrophic in obstacles which result in systolic overload of the left ventricle (coarctation peri-orificial stenosis of the aorta, systemic hypertension) or of the right ventricle (pulmonic stenosis), or congestive in diastolic overload by left-to-right shunts or valvular insufficiency. These myocardiopathies may prevent recognition of the signs of the underlying heart disease thereby leading to diagnostic errors and delay in therapy. They may constitute the main factor of an operative decision.

[Acute infectious myocarditis (author's transl)]. / Myocardites aiguës infectieuses.

Among the primary myocardiopathies resulting from a known cause, acute infectious myocarditis plays an important part. This condition, usually interstitial myocarditis of viral etiology, occurs predominantly in the infant under two years of age. They are characterized by a hypotrophic hypokinetic myocardiopathy which is very hard to differentiate from endocardial fibroelastosis. Fibroelastosis is probably only one possible result of the course of certain myocarditis'. However, on the whole, prognosis is good and the children recover. Typhoid fever and diphtheria may also compromise myocardial function.