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Biomedical subjects

A Sacrez

Publications and source records attributed to A Sacrez.

At least 91 records · Page 5Linked to original sources

[Course and prognosis of primary hypertrophic cardiomyopathies].

33 patients with hypertrophic cardiomyopathy were followed for a mean duration of 48 months. 29 of them were reviewed; 6 had died, including 4 with a familial form belonging to two different families. No cases of sudden death were observed. The mortality rate was 4 p. cent at 1 year, 11 p. cent at two years and 21 p. cent at 5 years. The clinical course was marked by a functional deterioration in one-third of cases and, on echocardiography, by an increase in the diastolic diameter of the left ventricle and in the thickness of the septum, independent of the clinical course. Subjects from "high risk" families have a very poor prognosis (4 deaths out of 7 patients at an average age of 25). These families present major conductive disturbances on the electrocardiogram and a very marked parietal hypertrophy on the echocardiogram. No other prognosis factor independent of the familial aspect was revealed.

Adolescent↗

[Coronary lesions in myocardial infarction].

The authors analyse the coronary lesions in 285 patients with primary myocardial infarction (164 anterior, 121 inferior infarcts) undergoing coronary angiography an average of 4 months after infarction. The statistical study of the analytical table of the lesions according to severity and site, demonstrated a significant difference in each group (p less than 0,001): --there was a very clear dominance of occlusion of the LAD artery in anterior infarction but severe narrowing (greater than or equal to 70%) was observed mainly on the right coronary and left circumflex arteries; --in inferior infarction, the incidence of occlusion was higher on the right coronary artery and severe narrowing was divided between the LAD and left circumflex arteries. Controlateral, double or triple vessel disease was present in 74% of anterior and 85% of inferior infarcts. There were many more patients with double and triple vessel lesions than with single vessel disease. Residual angina gave no indication of the extension of the lesions in anterior infarction but patients with this complication after inferior infarction had a higher rate of triple vessel disease. Stress testing is exploitable in inferior infarction but did not give any discriminating results. In this series, angina and stress testing only allowed triple vessel disease to be suspected in patients with inferior infarction. A coronary arteriographic study, by showing the severity and controlateral extension of the lesions, comparable in primary anterior and inferior infarction, gives important prognostic information and allows assessment of surgical possibilities.

Angina Pectoris↗

[Myocardiopathy with lipid overload and leukocyte palmityl carnitine transferase (PCT) deficiency].

Histological changes of the skeletal muscle with moderate fatty infiltration and varied abnormalities of the muscle fibres and conjunctivo-vascular tissue were demonstrated in three adult patients with apparently primary cardiomyopathy with dilatation. Leukocyte PCT activity was decreased and plasma carnitine was increased. In two cases, postmortem cardiac biopsy showed a massive accumulation of lipids and an intense progressive fibrosis dissociating the atrial and ventricular cardiac fibres. A disturbance of lipid metabolism could be the cause of this cardiac and skeletal muscle disease, but the reduced PCT activity could be a sign of more diffuse myocardial disease as suggested by the fibrous lesions and vascular abnormalities observed in our cases. These cases emphasise the value of routine investigation of PCT activity in primary cardiomyopathy and of ultrastructural studies of skeletal muscle, even in the absence of clinical involvement, which may show relatively severe changes reflecting the state of the myocardium.

Acyltransferases↗

[Coronary-left ventricular fistulae].

The authors report a case of fistulae between the right and left coronary arteries and the left ventricle. A 58 year old man was investigated for inaugural unstable angina without infarction. Coronary angiography showed multiple communications between the terminal branches of the septal arteries of the left anterior descending, the terminal segments of the left diagonal and the terminal portion of the right coronary artery and the apical region of the left ventricle. Angiography in the ascending aorta confirmed the diagnosis of coronary-left ventricular fistulae. The dilatation and rapid emptying of the entire coronary circulation were indirect signs of increased flow due to the shunt. There was no coronary alternation or stenosis. The rarity of coronaro-cardiac fistulae, especially involving both main coronary arteries and the left ventricle, is emphasised. The symptoms of angina are explained by the coronary "steal" syndrome : the diagnosis is angiographic; rare cases with high flow and a favourable anatomical set up may be managed surgically.

Coronary Vessel Anomalies↗

Unusual familial cardiomyopathy with storage of intermediate filaments in the cardiac muscular cells.

Unusual histological and ultrastructural changes in cardiac muscle cells have been found in 3 brothers with progressive myocardial deficiency. Histologically, this cardiomyopathy was characterized by massive storage of PAS-negative proteinaceous material in most cardiac muscle cells. The electron microscope showed that this material consisted of sinuous filaments, 7-10 nm in diameter, similar to the intermediate filaments normally present in cardiac muscle cells. Filament storage coincided with the disintegration of neighbouring myofibrils, with particular change in Z bands giving rise to rod-like bodies and more complex structures formed by the association of Z band material and sarcoplasmic reticulum (SR) tubules. Filament storage and myofibrillar disintegration always occurred in areas where the SR developed and involuted extensively. Relatively high glycogen accumulation also occurred, in close relation to the SR changes. Discrete SR proliferation, glycogen overload and filament deposits were observed in a few skeletal fibres. These observations suggest that disturbance in the metabolism of desmin (protein subunit of intermediate filaments and a fundamental component of Z bands) might be involved in this type of cardiomyopathy. The influence of a chronic defect in calcium regulation might also be envisaged in view of the marked SR abnormalities.

Adult↗

[Familial cardiomyopathy: a study of two families with myocardial and skeletal muscle biopsies].

Cardiomyopathy was diagnosed in several members of two families. This familial cardiomyopathy showed symmetrical or asymmetrical hypertrophy of the ventricular walls with or without obstruction to the left ventricular outflow tract. Certain forms were asymptomatic and were revealed by the family history and echocardiography. Myocardial and intercostal muscle biopsy was performed for a biochemical and ultrastructural analysis. Different myocardial features were observed in the two families: a large increase in the glycogen deposits in the one, without clinical signs of a glycogen storage disease, and intracellular deposits of a filamentous protein substance in the other.

Cardiomyopathies↗

[Two-dimensional echocardiography of ventricular aneurysm. Study of 38 cases with angiographic correlation].

This is a study of the data supplied by cross-sectional echocardiography in the diagnosis of post-infarction aneurysm. It involves 38 confirmed cases of myocardial infarction. The clinical, electrical, radiological and echocardiographic data (in M mode) were specified. Right anterior oblique ventriculography showed 21 posterior and 17 anterior aneurysms (4 antero-lateral, 5 apical, 8 antero-apical), and 14 mitral regurgitations. Cross-sectional echocardiography using a (30 degrees or 90 degrees) mechanical sector scanner allowed the study of the kinetics of 6 segments following 5 viewing angles: one longitudinal, two transversal, two apical. Each segment was classified according to its shape and motion: akinetic, dyskinetic or aneurysmal, and the papillary muscles of the mitral valve were assessed as normal or pathological (dense and motionless on the echogram). 35 of the 38 aneurysms seen at angiography, were detected by cross-sectional echocardiography; in one case the diagnosis could not be made for technical reasons; in two cases echocardiography was in favour of akinesia. An abnormal papillary muscle was observed in the 14 cases of mitral insufficiency. The causes of error in localisation were considered. In conclusion, cross-sectional echocardiography appears to be an excellent atraumatic procedure for the diagnosis of aneurysms and papillary muscle dysfunction.

Adult↗

[Angina pectoris in a hypertensive patient with left ventricle hypertrophy: echo-angiographic comparisons].

Not every case of angina pectoris occurring in a hypertensive patient is indicative of coronary atherosclerosis. Nine patients with essential hypertension of moderate degree had attacks of angina of sufficient severity to require investigation by arteriography. In these patients, the coronary arteriogram was normal but ventriculography showed hypertrophy of the walls of the left ventricle of restrictive or obstructive type. These appearances were confirmed by echocardiography which also showed hypertrophy of the septum and, in certain cases, confirmed the involvement of the ventricle, while by contrast the electrocardiogram and radiological appearances of the heart were essentially normal. The beta-blockers may have an important part to play in such conditions, and echocardiography is suggested as part of the routine investigation in cases of hypertension.

Adult↗

[Echography after regularization of atrial fibrillation].

An echographic study was carried out on 17 patients before and after conversion of atrial fibrillation to normal rhythm. It showed up the moment of return of atrial contraction as a movement of the posterior wall of the left auricle and a reappearance of the 'a' wave or the anterior cusp of the mitral valve. The contraction can appear immediately after the electric shock, or may be delayed.

Adult↗