[Natural history of hypertrophic obstructive cardiomyopathy in young patients: apropos of 40 cases]. / Histoire naturelle de la myocardiopathie hypertrophique obstructive du sujet jeune: à propos de 40 observations.

This retrospective study analysed the outcome of children with hypertrophic obstructive cardiomyopathy. Between 1969 and 1992, 40 patients all under 20 years of age (mean = 10.9 +/- 6.2 years) were followed up for this condition. There was a positive family history of hypertrophic cardiomyopathy and/or sudden death in 21 cases (53%); 21 (53%) were symptomatic: > NYHA Stage II dyspnoea (n = 13); chest pain (n = 8); syncope (n = 7) or palpitations (n = 4). Thirty-two patients were treated by betablockers, 1 by verapamil, 2 by amiodarone (associated with propranolol in 1 case); 7 patients underwent surgery and 6 others, asymptomatic, had no specific treatment. The mean follow-up period was 10.2 +/- 6.2 years with no drop-out: 13 patients died (1 non-cardiac death, 2 of unknown causes and 10 of cardiac causes, including 8 sudden deaths) giving an annual global mortality of 3.2%; the actuarial 5 and 10 year survival rates were 90 and 85% respectively. The 27 survivors were compared with the 13 patients who died: of the 11 clinical and paraclinical criteria examined, only a previous history of syncope correlated with global mortality (p = 0.004) and sudden death (p = 0.0008).

Pregnancy and prosthetic heart valves: a French cooperative retrospective study of 155 cases.

A French cooperative retrospective study analysed 155 pregnancies in 103 women with prosthetic heart valves: 95 mechanical prosthesis (MP) and 60 bioprostheses (BP). Among them 13 MP and 10 BP were bivalvular and four were mixed implants. In all, 182 (108 MP and 74 BP) prostheses were exposed to the risk of pregnancy. Among the 108 MP-bearing patients, 16 thromboembolic accidents (TEA) were recorded: 10 thromboses in 13 mitral, two aortic and one pulmonary MP. TEA were four times more frequent under oral anticoagulant therapy. Among the 74 BP, seven suffered premature valve failure. Ninety-nine infants were born to 50 MP-bearing women (53%) and 48 BP-bearing patients (80%) (P < 0.001). Twenty miscarriages were reported; they occurred more often under anticoagulant treatment (17%) than without it (4%) P < 0.02). Coumarin-induced embryopathies were rare (only one definitively identified). Because pregnancy with an MP under anticoagulant therapy is dangerous for the mother and may effect the fetus, the therapeutic indications for women of child-bearing age must be taken into consideration. In a women already with an MP at the time of conception, the duration of heparin therapy should be limited to the following two periods: from the 6th to the 12th week (coumarin-induced embryopathies) and during the last 2 weeks of gestation (haemorrhages during delivery and the neonatal period).

[Pregnancy in patients with heart valve prosthesis. A French retrospective cooperative study (155 cases)]. / Grossesses chez les porteuses de prothèses valvulaires. Etude coopérative rétrospective française (155 cas).

A French cooperative study of 155 pregnancies in 103 women with valvular prosthesis (95 mechanical, 60 bioprostheses including 27 bivalvular prostheses) is reported. Therefore, a total of 182 prostheses (108 mechanical and 74 bioprostheses) were exposed to the risk of pregnancy. The maternal outcome in the group of 108 mechanical prostheses was complicated by 16 thromboembolic events (TE) including 10 prosthetic valve thromboses which required emergency valve replacement in 4 cases, 6 systemic TE in 13 mitral, 2 aortic and 1 pulmonary mechanical prostheses. The TE were four times more frequent in patients on heparin than in those on oral anticoagulants. There were 4 deaths, 3 among the 10 prosthetic valve thromboses (one reoperation, two sudden deaths). Seven of the 74 bioprostheses were reoperated for degeneration on average 5.9 years after the initial operation but there were no deaths or TE. The outcome of pregnancy was 99 children (63%), 49 of which were born to mothers with mechanical prostheses (53%) and 50 to mothers with bioprostheses (80%) (p < 0.001). Seven of the children were born prematurely, all mothers being on anticoagulant therapy. The birth weight was over 400 grams heavier (3 kg versus 2.6 kg) in the bioprosthesis group (p < 0.05). The 20 spontaneous abortions (13%) were more common in patients on anticoagulants (17%) than in those without (2%) (p < 0.02). Congenital defects due to oral anticoagulants were rare (one certain case). There was one case of phocomelia, an abnormality which has never been described in this context. The 36 remaining pregnancies were still deaths (N = 5), abortion due to maternal death (N = 4), maternal complications (N = 8), therapeutic (N = 9) or voluntary abortions (N = 10) (28 mechanical and 8 bioprostheses).(ABSTRACT TRUNCATED AT 250 WORDS)

Anomalous origin of left coronary artery from the pulmonary artery: evolution of left ventricular function and perfusion after surgery in a 44-year-old man.

Anomalous origin of left coronary vessel from the pulmonary artery is an almost universally fatal form of congenital heart disease unless appropriate corrective surgery is performed at an early age. A case was diagnosed in a symptomatic 44-year-old man who presented an impairment of systolic left ventricular function. The abnormality was successfully treated with ligation of the left coronary artery combined with left internal mammary artery anastomosis. Left ventricular function improved dramatically six months after surgery, both at rest and during exercise. Such an evolution suggests that surgery may be indicated not only in infants but also in adults with this congenital heart abnormality.

[Prognosis of chronic non-dystrophic and surgically treated aortic insufficiency]. / Pronostic de l'insuffisance aortique chronique non dystrophique et opérée.

Between January 1972 and March 1983, 123 patients with isolated non dystrophic aortic insufficiency underwent aortic valve replacement. During a mean follow-up period of 4.3 years per patient, 25.6 p. 100 of the patients died, including a 4.1 p. 100 hospital death rate. The actuarial survival rate was 74 p. 100 at 5 years and 62 p. 100 at 10 years. The main causes of mortality were heart failure, dysrhythmias and sudden death. A study of prognostic factors based on the preoperative data showed that clinical, radiological, electrocardiographic and haemodynamic signs were informative. In agreement with other authors, we found that preoperative cardiac dilatation and left ventricular dysfunction were predictive of a poor prognosis.

[Prognosis of surgically corrected chronic aortic insufficiency]. / Pronostic des insuffisances aortiques chroniques opérées.

It is difficult to determine the surgical indications of asymptomatic chronic aortic regurgitation (AR). This study was undertaken in 205 patients with pure AR, operated between 1970 and 1982: 136 patients were symptomatic (Classes III and IV of the NYHA, +/- cardiac failure +/- angina, mean age: 49.5 years). Sixty nine asymptomatic patients (Classes I and II of the NYHA without cardiac failure or angina, mean age: 42 years). The prognostic value of 58 variables was studied in these two groups. The mortality during the first postoperative month was 14% in the symptomatic patients and the 5 year survival rate was 68.7%; this was significantly lower in patients with ECG changes of systolic left greater than or equal to ventricular overload, with radiological cardiomegaly (cardiotolerance index 0.60) with calcific aortic valve disease, with raised arterio-venous difference and/or low cardiac output, and with a low ejection fraction. In the asymptomatic group, the hospital mortality was zero and the 5 year survival rate 86.8%. This was significantly decreased in patients with an increased PR interval and a low cardiac index. It is possible that the small number of patients did not demonstrate the predictive value of left ventricular function in asymptomatic patients (a hypothesis suggested by the causes of secondary mortality which were the same in both groups of patients).(ABSTRACT TRUNCATED AT 250 WORDS)

[Hypereosinophilic syndrome with endomyocardial involvement in children. Critical analysis of the literature apropos of a case]. / Le syndrome hyperéosinophilique avec atteinte endomyocardique chez l'enfant. Analyse critique de la littérature à propos d'un cas.

A 6 years old girl presented with hypereosinophilia of 7 months duration and cardiac failure from restrictive myocardiopathy, leading to death in 3 years 6 months. Transient blast cells were found. Sixteen previously reported pediatric cases of hypereosinophilia of unknown cause with cardiac involvement are studied. The meaning of eosinophilic leukemia is discussed, the frequency of cardiac damage and the role of hypereosinophilia in the mechanism of cardiac injury are emphasized. Therapy may stabilize or reverse cardiac involvement. In the cases with intractable congestive failure, surgical treatment must be recommended.

[Congenital abnormalities of origin or distribution of the coronary arteries arising from the aorta]. / Anomalies congénitales d'origine ou de distribution des artères coronaires naissant de l'aorte

The authors report 16 cases of this type, without associated cardiac abnormality, seen in the course of 3000 coronary arteriographies in adults. The diagnosis has been made on the findings at selective coronary arteriography, completed eight times by one totalinjection above the sinus. A classification of distribution anomalies and of cases of hypoplasia of one coronary artery is put forward, based on the number of ostia. The role which these anomalies might play in the genesis of myocardial ischaemia is discussed, and was formally considered responsible in two cases. It has not been demonstrated that they play a part in the development of atheromatous stenosis of the coronary arteries. It is vital that these anomalies should be recognized preoperatively.