[Role of right-to-left atrial shunt in exercise tolerance of patients with Ebstein anomaly]. / Rôle du shunt droit-gauche atrial dans la diminution de la tolérance à l'effort de la maladie d'Ebstein asymptomatique.

The aim of this study was to assess cardiorespiratory tolerance to exercise in children with non-operated, paucisymptomatic and untreated froms of Ebstein's anomaly. The authors undertook a prospective study in 11 children, mean age 9.6 years, who had lung function tests, cardiorespiratory exercise stress tests (bicycle ergometry N = 8, treadmill N = 3) and contrast echocardiography. All parameters of spirometry were normal. Contrast echocardiography showed a right-to-left interatrial shunt in 7 children (group 1) whereas the remaining 4 children had no shunt (group 2). The resting oxygen saturation was 97.4 +/- 2%, with no difference between the two groups. On the other hand, oxygen saturation at peak VO2 (VO2 max) was 90 +/- 9.5%, significantly lower in group 1 than in group 2 (85.7 +/- 2.2% vs 98.2 +/- 1.2%; p = 0.03). In group 1, the VO2 max was correlated to oxygen saturation (r = 0.98; p < 0.001, N = 6). The oxygen desaturation was correlated with presence of a right-to-left interatrial shunt (p = 0.01). The reduced exercise tolerance of non-operated, paucisymptomatic children with Ebstein's anomaly is due to a right-to-left interatrial shunt. In patients with poor exercise tolerance, contrast echocardiography is advised for the detection of these atrial shunts.

Cardiorespiratory exercise tolerance in asymptomatic children with Ebstein's anomaly.

The aim of the study was to evaluate cardiorespiratory exercise tolerance in asymptomatic children with Ebstein's anomaly. Eleven children with a mean age of 9.6 years were prospectively studied by spirometry, cardiopulmonary exercise testing (bicycle ergometer n = 8, treadmill test n = 3), and contrast echocardiography. A right-to-left atrial shunt was detected by contrast echocardiography in 7 children (group 1), whereas no shunt was found in 4 (group 2). VO2 max was decreased [84.5 (SD = 16.8)] and was strongly correlated to oxygen saturation in group 1 (p < 0.0001). Oxygen saturation at peak uptake was significantly decreased compared to baseline [97.4 (SD = 2.0) vs 90% (SD = 9.5%), p = 0.02] and was significantly lower in group 1 than in group 2 [85.7 (2.2) vs 98.2% (SD = 1.2%), p = 0. 03]. Oxygen desaturation was related to a right-to-left atrial shunt (p = 0.01). Decreased VO2 max was also correlated to the small size of the left ventricle (p = 0.05). We concluded that decreased exercise tolerance in children with asymptomatic Ebstein's anomaly is related to a right-to-left atrial shunt and to a small left ventricle. In case of poor exercise tolerance, a contrast echocardiography should be performed to detect an atrial septal defect.

[Aptitude to physical effort in children with operated tetralogy of Fallot]. / Aptitude à l'effort physique des tétralogies de Fallot opérées.

BACKGROUND: Long-term follow-up of patients after total surgical correction of tetralogy of Fallot indicates that they have a smaller working capacity than controls, with an increased incidence of late onset complete heart block and sudden death. These abnormalities may be less frequent when surgery is undertaken at an early age. POPULATION AND METHODS: A cardio-pulmonary stress test was performed on 18 patients aged 8 to 20 years who had undergone correction of tetralogy of Fallot when they were 3 months to 7 years (mean age: 3 years). The basic ventilatory tests, exercise ventilatory tests and gas exchange were also performed. RESULTS: The cardiopulmonary stress test was maximal in 16 of the 18 cases. No stress-induced PVCs, or chronotropic insufficiency was found despite a basic long PR interval in 4 cases. A restrictive ventilatory syndrome was seen in 4 cases with low respiratory reserve at exercise (defined as the ratio between maximal observed ventilation and maximal theoretical ventilation, VEMS x 40). The respiratory function was normal in 14 cases with an aerobic capacity of over 40 ml/kg/min. CONCLUSION: Patients with a normal chronotropic function and preserved aerobic capacity show no post-operative restriction or cardiac of pulmonary exercise capacity.

[Aptitude for sports in case of congenital heart block. Apropos of a case]. / L'aptitude à l'exercice physique en cas de bloc auriculo-ventriculaire congénital. A propos d'un cas.

Cardiorespiratory adaptation of congenital atrioventricular block is not well known. The authors present the case of a 20 year old patient with excellent muscular force and maximal aerobic capacity despite severe chronotropic insufficiency on exercise, the heart rate not exceeding 80/mn. This condition may be explained by increased peripheral oxygen uptake and an increased oxygen transport due to high hemoglobin level.

[Essential thrombocythemia and hypertension as a result of stenosis of the renal artery]. / Thrombocytémie essentielle et hypertension artérielle par sténose de l'artère rénale.

Primary thrombocythemia may cause vascular thrombosis; it has been rarely involved in coronary atherosclerosis with myocardial infarction. We report three cases of renal arteries atherosclerosis occurring in association with primary thrombocythemia. These cases are three young women (20, 40 and 42 years old) with severe hypertension secondary to atherosclerosis with stenosis of renal arteries, one or both sided, and in association in one case with diffuse arterial stenosis. Systematic investigation revealed thrombocytosis with latent myeloproliferative syndrome of megacaryocytic colony. Thrombocytosis was previously present as attested by a blood count one year before (in one case) and by long-term peripheral vasomotor troubles, electively improved by aspirin (in two cases). In none of these three cases, vascular risk factors, nor hereditary vascular diseases were present. So we assume that platelets high levels are responsible for this early atherosclerosis, in keeping with the well-know role of platelets in atherosclerosis pathogenesis. Platelets investigations must be done in case of renovascular hypertension, occurring without any classical vascular risk factors.

[Atrio-ventricular disorders following surgery for congenital heart diseases with septal defect (author's transl)]. / Troubles de conduction auriculo-ventriculaire après chirurgie des cardiopathies congénitales comportant un défaut septal.

Post-operative results in congenital heart diseases with septal defect are often darkened by the occurence of disorders in atrio-ventricular conduction. The incidence of these disorders is dependent upon the location of the septal defect, the anatomy of conduction pathways and the operative technique. Intracavitary recording of His bundle activity leads to a more precise localisation of the trouble. Such disorders are more frequent after cure of Fallot's tetralogy than after closure of an interventricular communication. They are rare after closure of an interatrial septal defect and more common after closure of the atrioventricular canal, where they are of atrial node type. The occurrence of an atrioventricular block, even transient, is of poor prognosis, and in case of proximal lesion the insertion of a pacemaker must be considered. The high incidence of conduction disorders justifies continuous per-operative recording and prolonged surveillance, if necessary by Holter's method. Locating the conductive tissue during surgery reduces the frequency.

[Arterial pressure during exertion in patients operated on for aortic coarctation]. / Etude de la tension artérielle à l'effort des coarctations aortiques opérées.

19 patients were studied after operation for coarctation of the aorta. The average age at operation was 13.7 years. Crafoord's operation was performed in 16 cases and in the other three an aortic patch was necessary. The average post-operative period was 4.7 years. 14 cases were classified as good results: normal femoral pulses and arterial blood pressure. The other 5 cases were considered as unsatisfactory: 2 mediocre results with normal arterial blood pressure but diminished femoral pulses and oscillometric indices; 3 poor results with hypertension at rest. On exercise with bicycle ergometry the following results were obtained: the systolic blood pressure rose in a comparable manner to that observed in normotensive individuals of the same age on exercise except in 2 cases. These two patients had an aortic patch and one had a residual intraaortic pressure gradient. The pronostic significance of these findings is not known. However the use of an aortic patch is often associated with abnormal blood pressures at rest or on exercise. The fact that the majority of patients operated for coarctation of the aorta have normal blood pressure profiles on exercise should be emphasised.

[Dwarfism and constrictive pericarditis. Apropos of a case of so-called Mulibrey dwarfism with hypereosinophilia]. / Nanisme et péricardite constrictive. A propos d'un cas de nanisme dit "Mulibrey" avec hyperéosinophilie.

A child of 10 was admitted to a cardiology unit with adiastole. The marked degree of dwarfism and anatomical features were characteristic of the so-called 'Mulibrey' dwarfism. Surgical intervention confirmed the constrictive pericarditis which is normally found in this condition. Unfortunately, the adiastole persisted which, taking into account the marked eosinophilia, made an associated endomyocardial fibrosis seem likely. The distinctive features of constrictive pericarditis and of the constrictive type of cardiomyopathy are recalled.