[Cardiac amyloidosis apropos of a case disclosing Kahler's disease].
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Biomedical subjects
Publications and source records attributed to J P Folliot.
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Over a 10 year period, four cases of post myocardial infarction pseudo-left ventricular aneurysm were observed, two directly arising from the infarct (postero diaphragmatic patients had pseudo-aneurysms which arose inferiorly from true anteroapical aneurysms and underwent surgical resection. Apart from the histological features, a certain number of diagnostic signs merit review: --A clinical course marked by a number of phases (myocardial infarction--pericardial syndrome--true aneurysm complicated or not by arrhythmias--period of stabilisation followed by deterioration due to rapid progression of cardiac failure). -- "Angiographic-like" ultrasonic and isotopic methods complement left ventriculography which confirms the parietal aneurysm and may show three very suggestive signs of pseudo-aneurysm: a narrow communication with the true aneurysm; delayed and prolonged filling of the bulge; inferior extension with localization by pericardial adhesions. Surgery is imperative, the main problem being the extent of resection of the true aneurysm. This is related to the rigid or calcific character of the neck of the pseudo-aneurysm.
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Out of a total of 1 470 cardiac catheterisations performed in one year, 5 were motivated by the chance finding of giant negative T waves, greater than 10 mm and maximal in leads V3, V4. The patients were all males with an average age of 50 +/- 3,2 years. Four were asymptomatic and the other had scleroderma. The changes partially regressed on exercise electrocardiography. M mode echocardiography showed normal septal (8,8 +/- 0,25 mm) and posterior wall (8,38 +/- 0,21 mm) thickness and a normal septum/posterior wall ratio (1,02 +/- 0,01). Coronary angiography was normal in all cases. Left ventricular and diastolic pressure was raised after ventriculography (21 +/- 1,34 mm Hg). There was no intraventricular pressure gradient. Left ventriculography in the 30 degrees right anterior oblique plane showed an end diastolic appearance similar to that of the ace of spades and the apex was obliterated in systole. End diastolic volumes (68 +/- 4,7 ml/m2) and ejection fractions (68,6 +/- 5,21) were normal. The wall thickness measured from the angiography was 10,4 +/- 0,81 mm at the mid zone of the anterior wall and 17,2 +/- 0,85 at the apex with a septum / posterior wall ratio of 1,67 +/- 0,08. In three cases, atrial pacing with coronary arterial and venous lactate sampling revealed abnormalities in myocardial metabolism. The final diagnosis was of non obstructive apical hypertrophic cardiomyopathy. The site of hypertrophy explains the electrical changes. The prognosis of this cardiomyopathy is still unknown, one regression and two aggravations of the electrical changes have been observed. continuous 24 hour ECG showed an episode of ventricular tachycardia in 1 patient, which would imply a reserved prognosis.
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The authors report 8 cases of complete atrio-ventricular block (AVB) which came on during bacterial endocarditis. The aortic valve was more frequently affected (6/8). The conduction disorder is necessarily unstable. The prognostic significance of AVB is always very grave--all the patients have died. The valve lesions are often severe. A histological study of the conducting pathways has been carried out. The classically described aneurysm of the membranous septum was not responsable for any cases of AVB in this series. The most frequent cause of the AVB (5/8) was an infiltration of the prenodal area and the A-V nod itself, starting from the posterior aortic cusp and, in one case, from the tricuspid valve. The bundle of His is affected either by extension of the A-V node lesion or by the focus on the right cusp. Strings of inflammatory cells may follow the sheath of the bundle branches. Haematogenous micro-abscesses are sometimes found in the conducting tissues.
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