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We present the results of surgical treatment of eight patients operated in the service of osteoarthritis scapho-trapézo-trapézoidienne (STT) by resection of the distal pole of the scaphoid under arthroscopic. Studies have already shown the good results of this technique in open surgery. This is a retrospective study of eight patients of mean age 70 years. They had an isolated STT osteoarthritis, which is not relieved by medical treatment. They were operated between 2004 and 2007 by the same surgeon and were reviewed at a mean of 13 months by an independent examiner. The evaluation was focused on pain, range of motion and grip strength. A radiological assessment was conducted to examine a possible carpal mal-alignment. Two patients were excluded because they had received a trapeziectomy for rhizarthrosis associated. The pain had decreased in seven cases with complete disappearance in six cases. The force had been increased or stabilized in seven cases and the mobility is the same in six cases. We got six very good results, one good result and one bad result without aggravation. Furthermore, no patient had been taken, nor any complications noted. The good results obtained with this minimally invasive outpatient surgery, encourages us to offer first-line therapy in patients with isolated STT osteoarthritis is not relieved by medical treatment. The resection of the distal pole of the scaphoide under arthoscopy gives less burdensome and less complication that the arthrodesis, while providing very good results.

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).

The authors report the case of a woman with thrombosis of a tricuspid Saint Jude prosthesis during the fourth month of pregnancy. A first course of thrombolytic therapy with rt-PA reestablished normal prosthetic valve function but was followed by a threatened abortion and severe uterine haemorrhage. An early rethrombosis of the prosthetic valve led to interruption of the pregnancy after failure of a second course of thrombolysis, and to replacement of the tricuspid valve prosthesis. This case illustrates the problems of pregnancy in women with mechanical cardiac prosthetic valves and the difficulties of treatment.

The authors undertook a retrospective study of 69 cases of infective endocarditis (IE) in 68 children treated from 1971 to 1992. The comparison between two groups (Group I comprising 34 patients treated between 1971 and 1981; Group II comprising 34 patients treated between 1982 and 1992) based on a review of the literature showed that the natural history of paediatric IE has changed during these two decades: a slight increase in the incidence in young children. The sequellae of rheumatic heart disease play no role in determining IE in France. Congenital heart disease plays a major role (72% of cases) with increasing numbers having undergone surgical treatment for more complex lesions. Mitral valve prolapse has become a more common cause with multiple portals of entry, predominantly buccal and oto-rhino-laryngeal. Blood cultures are positive in 75% of cases, the commonest organisms being Streptococci and Staphylococci, but the frequency of uncommon pathogens is increasing. Echocardiography plays a major role in the diagnosis and inventory of IE (vegetations demonstrated in 64% of cases in Group II). Although mortality is progressively decreasing (3% in Group II) because of more frequent surgical indications (32% in Group II) and more severe sequellae: only 27% of children in Group II were cured without sequellae or aggravation of their previous cardiac lesion.

The aim of this study was to assess the long term prognosis of congenital atrioventricular block (AVB). From 1965 to 1990, 42 cases of congenital AVB (22 antenatal or natal diagnoses and 20 presumed congenital AVB according to Yater's criteria). The AVB was isolated in 28 cases and associated with cardiac disease in 14 cases (8 of which were corrected transposition of the great arteries). The average age of the patients was 14 years (range 32 years to 18 months) at the time of the study. There was a clear female predominance (64%). Maternal connective tissue disease was present in 18% of cases (in the group of children born after 1977 when maternal connective tissue diseases was systematically looked for). Cardiac failure was present in 10 cases (8 with associated AVB); syncope and sudden death were observed in 11 cases. The indication for pacemaker therapy was the presence of poor prognostic factors: syncope, poorly controlled cardiac failure, low heart rate, increased QRS duration, prolonged QTc, infrahisian AVB, long pauses or arrhythmias on Holter monitoring. The only significant prognostic factors in this series were a previous history of syncope, increased QRS duration and a QTc of over 0.45 seconds. Fourteen patients were paced (endocavitary pacing only from 1981), usually in the DDD mode: 8 for syncope, 2 for cardiac failure, 4 for a poor prognostic factor.(ABSTRACT TRUNCATED AT 250 WORDS)

Determination of plasma delta-aminolaevulinic acid levels is of interest for the diagnosis of disorders of heme biosynthesis. We describe a reliable method for the determination of this parameter, based on a modification of the original method of Mauzerall and Granick. Analytical criteria are defined and the reference range used, established using data from 40 subjects, was 0.30 to 1.20 mumol/l. Results in patients with porphyria or lead poisoning and in those treated with haemodialysis or anticonvulsants indicate that this determination could be used principally in the diagnosis and management of acute porphyrias and in the early laboratory diagnosis of lead poisoning.

In recent years, thanks to a better understanding of the pathophysiology of congestive heart failure and progress made in the pharmacology of cardiovascular drugs, new therapeutics have been advocated in the treatment of congestive heart failure. Among them, converting enzyme inhibitors are the most useful. However, the classical association of digoxin-furosemide and general measures remains a very effective first-choice treatment in most cases. Only in particular situations, such as cardiomyopathy and decompensated atrio-ventricular insufficiency, should priority be given to converting enzyme inhibitors. Phosphodiesterase inhibitors are essentially used within the context of post-cardiac surgery intensive care. Beta-blockers which have been recently proposed for treatment of adult patients must not be used, as there is still no data available on their effectiveness and tolerance in pediatric patients.

A retrospective study of 150 children under 3 months of age who underwent repair of coarctation of the aorta in the same center (between 1972 and 1987) was undertaken to assess the problems posed by surgery without cardiac catheterisation or angiography. The patients were divided into two groups: Group A, comprising 104 children operated after invasive investigations, and Group B, comprising 46 children operated on Doppler echocardiographic data alone. The two populations were comparable and "hypoplastic aortic arch" type coarctation was present in over 60% of cases. However, there were more ventricular septal defects in Group B (67.5%) than in Group A (49%). There were no significant diagnostic errors in Group B (one case of interrupted aortic arch diagnosed at surgery). The 1 month survival was the same in the two groups (82%). The indications of pulmonary artery banding were less frequent in Group B although there were more ventricular septal defects in these patients. These results confirm the value of Doppler echocardiography in the context of urgent surgery of congenital heart disease.

This review is a retrospective study of the cases of 103 patients under 20 years of age with idiopathic dilated cardiomyopathy and represents the whole experience of the Department of Paediatric Cardiology of the Hospital of Cardiovascular Disease, Lyon, between January 1970 and December 1988. The aetiological investigations were constantly negative: hypertrophic, restrictive and secondary cardiomyopathies were excluded. The clinical, radiological, electrocardiographic and echocardiographic data of each patient was noted at the time of admission and at the end of the clinical course. There were many more infants in this series. The onset of the disease was usually sudden. All patients were given medical treatment. The study period ranged over 19 years. Forty one patients (39.8%) died mainly of terminal cardiac failure. Sixty two patients are still alive, of whom 39 are completely cured, 2 have undergone cardiac transplantation, and 21 have a variable degree of chronic invalidity. The six months survival was 77.5%; the 1 year survival was 70% and the 5 year survival was 60%. Over one third of deaths (36.6%) occurred during the first month, 56% during the first 3 months and 70% during the first year. A statistical analysis comparing the initial criteria to death by the chi 2 test revealed the following prognostic factors: age at presentation (32.9% of deaths in patients under 2 years of age compared to 56.6% in the group over 2 years of age, p less than 0.025) and a family history of myocardial disease. The other clinical, radiological and echocardiographic parameters had no predictive value in this series and it was not possible to identify potential candidates for cardiac transplantation.

Based on 11 personal cases a large review of the literature (188 cases), the authors studied the frequency and types of cardiac anomalies in Di George syndrome. A cardiac anomaly was found in 86% of published cases. Interrupted aortic arch (27%), truncus arteriosus (27%) and tetralogy of Fallot (22%) are the most frequent anomalies. Interrupted aortic arch and truncus arteriosus, being rare congenital heart diseases, their discovery in a neonate must lead to a systematic search for Di George syndrome.

Retrospective studies have been conducted in Lyon (33 patients) and Montreal (24 patients) in order to compare the results of transventricular valvotomy (TVV, 20 cases) and aortic valvotomy with cardiopulmonary bypass (CPB, 37 cases) in neonatal critical aortic stenosis. Clinical, echocardiographic, catheterization and operative data were analyzed in order to determine prognostic factors. Mortality rate was 59%: 30/34 perioperative deaths in the first month, and 4 late deaths after a reintervention for severe residual obstruction. Long term follow up was available for 23 patients (41%) for a 2 to 16 year period (mean 7.5). Five patients (7%) required a reintervention six years after the initial operation. Two of them required valve replacement. Eighteen patients (31%) surviving the initial operation, displayed a satisfactory result, being free of symptoms, endocarditis, reoperation and sudden death. Factors that influenced the outcome included severe heart failure, a left ventricular end-diastolic diameter below 14 mm, an aortic valve annulus below 8 mm, and a poor shortening fraction. Factors that did not influence the prognosis were age, pulmonary hypertension, and the anatomic type of the valve. Trans-aortic valvotomy with CPB was associated with a smaller operative mortality and a better long term result than TVV.

Phrenic stimulation is reserved for the rare patients with central neurogenic apnea due to isolated upper spinal cord injuries. Presenting a series of ten cases the authors describe the indications for phrenic stimulation, the technique of implantation and the conditions for diaphragmatic training by the use of stimulation by low frequency trains allowing for an increase in the number of low muscular fibers. The continuation of accompanying neurological rehabilitation and nursing of these severely handicapped patients is not to be neglected because of the length of the training protocole as the aim is their return home. This technique, therefore, seems particularly suitable for application in a few specialized centers where research for material totally implantable and adapted to the physiological requirements of the patient can be conducted parallel to treatment.

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)

Two cases of right pulmonary artery to left atrium in the newborn are described. Both patients presented with refractory hypoxemia cardiomegaly, hypovascular lung fields. Moreover one patient presented with continuous murmur and localised X-ray shadow. Echocardiographic left atrium enlargement suggested diagnosis and angiography confirmed it. Spontaneous improvement in one child is due to a decrease in pulmonary vascular resistance and has allowed the patient to attain an age and a weight in which surgical ligation has been performed more safely.

A case of severe Fallot's tetralogy is presented; there was angiographic evidence of progression towards pulmonary atresia. The relationship between Fallot's tetralogy and pulmonary atresia with open septum is discussed, as is the variability of the pulmonary circulation found in cases of pulmonary atresia with septal defect, and the treatment is also discussed.

Two cases of multiple coronary aneurysms are reported in patients aged 15 and 25 years. The clinical presentation in both cases was myocardial infarction. The diagnosis was confirmed by the demonstration of rounded calcification at the border of the aneurysms on chest X-Ray, and multiple aneurysmal dilatations on selective coronary angiography. These two cases and the other 16 found in the medical litterature allow analysis of the clinical signs of idiopathic coronary aneurysms, generally these of acute coronary insufficiency. Two-dimensional echocardiography seems to be the most effective means of detecting this type of pathology. The incidence of coronary aneurysms during infantile periarteritis nodosa, and in Kawasaki's syndrome, very similar conditions affecting infants and children, suggest that these two diseases may play a role in the formation of coronary aneurysms in adolescents and young adults, which would therefore be sequellae of an inflammatory arteritis of childhood.

Sometimes angina pectoris with normal coronarography can be explained by several enzymatic disturbances. These are as follow: -- a marked decrease of serum lactate-deshydrogenase 1 and 2 isoenzymes, -- the absence of myocardial lactate-deshydrogenase isoenzymes, --the decreased affinity for substrate lactate of residual lactate-deshydrogenase in serum, -- the increased production of 2,3 diphosphoglycreate.

TCA is an original method of myocardial revascularisation which, when successful, enables coronary bypass surgery to be avoided. However, a certain number of failures and complications are inherent to the development of a new therapeutic technique. The object of this study is to analyse the evolution of our results with this technique to determine which factors changed as our experience increased. The methodology used was that initially described by A. Gruntzig. TCA was carried out 72 times on the left anterior descending, 20 times on the right coronary and 8 on the left circumflex coronary artery (72 single vessel, 28 multivessel disease). The primary success rate was 75% (72% for the first 50 and 78% for the second 50 procedures). Coronary bypass surgery was required within the first 24 hours of TCA in 7% of cases. Myocardial infarction (Q wave changes) was observed in 5% of cases. One patient died on the third day (occlusion of an aorto-coronary venous graft). In retrospect, taking into account the experience gained and the development of the material now available, a certain number of failures and complications could probably have been avoided. However, a certain number of complications seems to be unavoidable. TCA remains an inviting but not infallible alternative in the treatment of coronary artery disease and should be included in the medico-surgical management of this condition.

Since the recent introduction of percutaneous balloon valvuloplasty, there has been a renewed interest in anatomical studies. This study was based on a retrospective analysis of 100 autopsy reports and 269 surgical reports from adult patients with AS. Valvular calcification, which was always found over the age of 50 years, plays an important part in the origin of aortic stenosis (AS). Congenital or acquired aortic valvular lesions are a common pre-condition for calcified AS. Three anatomical types were found: (i) calcified bicuspid valves with anterior and posterior cusps (more frequent than a left and a right cusp) were found in 41% of autopsy reports and 40% of surgical reports. The resultant rigidity due to calcification makes the valve stenotic; (ii) post rheumatic calcified AS with strong fusion of the commissures and calcified cusps was found in 30% of autopsy reports and 8% of surgery reports, and (iii) degenerative calcific aortic stenosis was the most frequent form found over 70 years of age. The sinuses of Valsalva were filled with calcium deposits. The three commissures were apparently free, but cusp fusion was found on the ventricular aspect of the valve (29% of autopsy cases and 52% of surgical reports). Percutaneous balloon valvuloplasty is more efficient in this anatomical type.

This study takes stock of the current results of physiological correction (Mustard's or Senning's operation) in simple transposition of the great vessels, at a time when anatomical corrections, or detranspositions, are developing. Between January 1, 1974 and December 31, 1984, 168 neonates with simple transposition of the great vessels were operated upon and followed up for a mean period of 3.67 years (up to 11 years and 10 months). Thirty of them died before correction (12.7% mortality rate with palliative surgery) and 15 immediately after corrective surgery (11.2%). Among the 111 children who survived corrective surgery, the results were satisfactory in 62.7%, fair in 17.8% and poor in 10.2%; the late mortality rate was 9.3%. Post-correction morbidity mainly consisted of mechanical complications suspected in one-third of the patients (with 3 consecutive deaths) and heart rhythm disorders (50% of patients in this series had abnormal Holter recordings), with predominance of atrial rhythm disorders (regression of sinus rhythm was 3.82% per annum). This, after correction the survival curve underwent an actuarial regression of 1.31% per annum. This primary and secondary morbidity and mortality justifies a switch to anatomical corrections the results of which remain to be fully evaluated.

Between September 1st 1974 and June 1st 1976, 12 infants under the age of 6 months have been operated on by a dacron patch aortoplasty for coarctation of the aorta. The associated lesions were a patent ductus arteriosus in each case, a VSD in 8 cases, and a trans-position of the great arteries in 5 cases. A pulmonary artery banding was performed with the aortoplasty in 8 cases, and a Blalock-Hanlon operation in one instance. There were two operative deaths (17 p. 100), amongst them one in a 1400 g premature infant, and a late death (3rd month). Two infants have a clinical aspect of recoarctation. In four infants, the post-operative hemodynamic and angiographic study carried out before the treatment of associated intra-cardiac lesions, shows a good result of the coarctation repair. This particular technique, although not performed as a routine in the infant with coarctation of the aorta, seems to be of interest in the most severe forms of the disease, with diffuse isthmus narrowing and intra-cardiac defects.

The authors report on 2 cases of congenital aneurysms of the coronary arteries and review the published literature. A total of 17 cases were found, which respond to their criteria fo; congenital coronary artery aneurysms discovered in patients after selective arteriography investigation. One of their cases was found to have aneurysms of the hepatic and distal renal arteries (vascular dysplasia?).