Usefulness of technetium-99m pyrophosphate myocardial scintigraphy in amyloid polyneuropathy and correlation with echocardiography.
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Biomedical subjects
Publications and source records attributed to M Blondeau.
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Technetium 99m cardiac scintigraphy as practiced at present for diagnosing amyloisodid only provides a visual semi-quantitative assessment of uptake of the isotope. To improve the diagnostic accuracy of the method, the authors evaluated prospectively a personal technique of scintigraphy quantification based on early images obtained at the 20th minute in 15 patients with neuropathic amyloidosis. Doppler echocardiographic studies indicated that 9 patients had cardiac involvement whilst 6 were free of cardiac amyloidosis. The index of isotopic uptake (ratio of cardiac/abdominal uptake) was 0.44 to 1.58 in the first group and 0.09 to 0.31 in the second group. The correlation between the scintigraphic index and interventricular septal or posterior wall thickness measured by echocardiography was poor. These results obtained in 15 patients with neuropathic amyloidosis suggest that the scintigraphic index measured at the 20th minute is discriminatory and allows identification of those patients with cardiac involvement. On the other hand, the correlations with echocardiographic wall thickness are poor. Technetium 99m cardiac scintigraphy with this technique of quantification is a useful tool for diagnosing cardiac amyloidosis, especially when echocardiography is difficult to interpret.
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A 52 year old patient presenting with spontaneous anginal chest pain for 4 days was admitted to hospital for a more intense and prolonged chest pain associated with signs of left ventricular failure (gallop, pulmonary crepitations, hypoxemia). Coronary angiography showed marked septal hypokinesia and spontaneous localised spasm of the left anterior descending and marginal arteries with a variable degree of luminal narrowing of the other segments of these two arteries and of the right coronary artery. These changes regressed after intracoronary injection of molsidomine. The signs of left ventricular failure disappeared in 48 hours. The wall motion abnormality, monitored by 2D echocardiography, regressed slowly over 3 days. On the other hand, the electrocardiogram, which showed anterior wall subendocardial ischaemia with prolongation of the QTc interval during the spasm, remained abnormal for a long time. Therefore, in the absence of organic heart disease, coronary spasms associated with vasoconstriction can induce a sufficiently severe and durable alteration of left ventricular function to create clinical signs of cardiac failure and profound and prolonged ST-T wave changes on the electrocardiogram.
Six Drosophila melanogaster tumor suppressor genes causing malignant or benign tumors in specific cell types are described. The wild-type alleles of these genes are instrumental in the differentiation of particular cell types. In the homozygous state, recessive mutations in the genes interrupt the differentiation of the cells and thus cause their uncontrolled, autonomous, lethal proliferation. The tumors show all major characteristics of malignant and benign neoplastic growth. Genomic sequences of four of the genes have been identified and are currently being characterized.
The purpose of this prospective randomized trial was to compare the efficacy of propranolol and amiodarone in suppressing ventricular arrhythmias during the first 6 months following myocardial infarction (MI). 97 patients were treated with either amiodarone (n = 48) or propranolol (n = 49) starting on the 9th day following MI. Holter monitoring was carried out on four occasions: on D7, D21, D90 and D180. There was no statistical difference in the incidence of 'major' arrhythmias (an average of at least 10 ventricular premature complexes (VPCs) h-1, multiform or paired VPCs or runs) between the two groups on D7. A significant difference in favour of amiodarone became apparent at D180 (P = 0.04). Patients were also classified according to whether treatment failed or was successful. 'Success' was recorded when arrhythmias remained minor or became minor (less than 10 uniform VPCs h-1) and 'failure' when arrhythmias remained major or became major, or when patients were withdrawn because of side-effects, or lost to follow-up. The difference remained in favour of amiodarone (P = 0.03 at D21; P = 0.05 at D90; P = 0.06 at D180). Evaluation of the percentage reduction in the number of VPCs at D21, D90 or D180 compared with D7 showed superiority of amiodarone at D90 (P less than 0.01) and D180 (P less than 0.04). In this study, the overall effect of amiodarone on ventricular arrhythmias following MI was shown to be superior to that of propranolol.
Beta-blockers are known to be effective against post-infarction ventricular arrhythmias and amiodarone has recently been shown to have this property. The purpose of this prospective randomized study was to compare the effects of beta-blockers and amiodarone during the first 6 months following infarction. Nine days after the onset of myocardial infarction, 97 patients were put on either amiodarone (48) or propranolol (49). Holter monitoring was performed on four occasions: on the 7th post-infarction day (baseline), then on the 21st, 90th and 180th days (under treatment). On D7 the two groups were similar in age, sex, risk factors, medical history, characteristics of the infarction and type of arrhythmia. For result analysis purposes the patients were divided into two categories depending on whether their arrhythmia was "moderate" (less than 10 monomorphous and isolated ventricular extrasystoles per hour) or "severe" (at least 10 ventricular extrasystoles per hour, or polymorphous or repetitive ventricular extrasystoles). Concerning the frequency of "severe" arrhythmia, there was no statistical difference between the two treatment groups on D7 (p = 0.53), but differences in favour of amiodarone became increasingly important during the study (p = 0.08 on D21; p = 0.07 on D90; p = 0.04 on D180).(ABSTRACT TRUNCATED AT 250 WORDS)
A 67 year-old patient receives 5-fluorouracil for vocal chord cancer. During the perfusion, atypical angina pain occurs, accompanied with offset of ST above the baseline in standard leads and in V4 through V6. The pain subsides spontaneously in 45 minutes. These ECG alterations are followed 48 hours later by diffuse inverted T waves with lengthened QT. Cardiac ultrasonography and isotopic angiography do not show any abnormality of the left ventricular function, but myocardial tomoscintigraphy with labelled thallium show a lower hypofixation on exertion. The cardiac toxicity of 5-fluorouracil is in frequent. It is usually believed that it involves a coronary spasm, as suggested by the ECG tracing in the reported cases. The incident, which may be painful or painless, may result in a myocardial infarction or even sudden death during the perfusion. Therefore, it is advisable to discontinue the treatment as soon as an angina-type pain occurs.
Unlike amiodarone, beta-blockers have proved ineffective in the treatment of severe ventricular rhythm disorders encountered in hypertrophic myocardiopathy. We tried to evaluate the efficacy of sotalol, a betablocker with anti-arhythmic properties, like amiodarone, on the supraventricular and ventricular rhythm disorders of this disease. 13 patients (6 men and 7 women, mean age 53.5 +/- 26.5 years) were evaluated with a 48 hours electrocardiographic recording, before and 8 and 120 days after a sotalol treatment at a mean dose of 290 mg/day (160 to 640 mg). The diagnosis of hypertrophic myocardiopathy was established on standard clinical, phonomecanographic (13 cases), sonocardiographic (13 cases) and haemodynamic (9 cases) criteria. Five patients presented bursts of supraventricular tachycardia on DO. Their number decreased to 4 on D8 and to 3 to D120. Six patients presented Lown's class III or IV ventricular rhythm disorders on DO. Their number went from 2 on D8 to 4 on D120. Therefore, in this short series, sotalol has shown a non-negligible efficacy on the rhythm disorders encountered in hypertrophic myocardiopathy.
It has long been known that the morphology of ventricular repolarization is extremely sensitive to acute or chronic myocardial anoxia. In patients with coronary disease, we are used to observe a wide variety of ST segment, T wave and sometimes U wave abnormalities. The classical description of "lesions" or "ischaemia", as well as their anatomical locations called, perhaps rather arbitrarily, "subendocardial" or "subepicardial" account for ST segment displacements and T wave inversions the patterns of which are well known to all of us. It may happen, however, that repolarization takes a frankly unusual appearance very different from the conventional images. On several occasions we were surprised to find, in a situation of acute or subacute coronary obstruction, a highly atypical and characteristically very transient repolarization pattern with considerable lengthening of QT and broad and deep inversion of T wave which, to our knowledge, have never been described in the literature. A systematic search for such unusual ECG patterns, conducted over the last 4 years in the Cardiology department of the Bicêtre Hospital on patients with ischaemic heart disease, has yielded 16 cases which are reported in the present study.
An elderly patient, receiving long-term oral diltiazem at the usual dosage, presented a sudden attack of junctional bradycardia at 35 b X min-1; this was badly tolerated by the patient. The diltiazem blood level was normal. After recovery, nodal investigations were also normal. The treatment of this accident due to a calcium-blocker is stressed: the intravenous injection of a calcium salt only was sufficient, with a return to near-normal sinus function, so avoiding the necessity of pacing.
Serum selenium concentration was prospectively measured using the method of thermoelectric atomic absorption spectrophotometry in 129 study participants distributed as follows in three groups: control group (n = 48) volunteers without known cardiac or lung disease; group I: patients with congestive cardiomyopathy (n = 48), group II: patients with myocardial infarction (n = 31). Serum selenium level was 84.73 +/- 1.79 micrograms.l-1 (m +/- sem) in the control group, 68.53 +/- 2.26 micrograms.l-1 in group I and 73.55 +/- 2.33 micrograms.l-1 in group II. The difference between group I and group II versus the control group was significant (p less than 0.01). There was no significant difference between group I and II.
Serum selenium concentrations were measured by electrothermal atomic absorption spectrophotometry in 31 controls and 101 patients with the following results (mean +/- s.e.m; microgram . l-1: controls: 86.27 +/- 2.26; 24 chronic alcoholics without denutrition: 63.29 +/- 3.19; 38 patients with denutrition (64.75 +/- 3.68) divided into chronic alcoholics (n = 27); 65.24 +/- 3.49 and non-alcoholics (n = 11): 63.55 +/- 4.15; 39 patients with cardiomyopathy (68.89 +/- 3.43) divided into chronic alcoholics (n = 21): 67.61 +/- 3.10 and non-alcoholics (n = 18): 72.56 +/- 3.82. Compared with values in the control group, the fall in mean serum selenium concentrations was statistically significant (P less than 0.001) in all groups of patients. In contrast, there was no significant difference in mean serum selenium concentrations between patients with cardiomyopathy and those with denutrition, alcoholic or not. Chronic alcoholism and denutrition are accompanied by a fall in blood selenium level. The decrease of serum selenium concentrations in alcoholic and non-alcoholic patients of the third group suggests that congestive cardiomyopathy is associated with selenium deficiency. These findings indicate that patients with congestive cardiomyopathy should be evaluated not only for alcoholism, but also for their nutritional status.
The electrocardiogrammes of 71 patients (39 men and 32 women) with transient or intermittent complete left bundle branch block (LBBB) were studied. Two tracings, one with and the other without LBBB were analysed in each case. The interval between the two recordings was less than 90 days in all cases (average 10 days). The diagnosis of left ventricular hypertrophy (LVH) was established from the ECG without LBBB. The sensitivity and specificity of the classical criteria or indices of LVA and of different associations of indices of LVH were assessed on the ECGs with LBBB. The best criteria of LVH in the presence of LBBB were the SV2 + RV6 greater than or equal to 32 mm (sensitivity 80%; specificity 81%), Sokolow's index greater than or equal to 33 mm (sensitivity 78%, specificity 81%); followed by SV1 greater than or equal to 23 mm (sensitivity 73%, specificity 86%), SV1 + SV2 + RV6 + RV7 greater than or equal to 65 mm (sensitivity 88%, specificity 63%), SV1 + SV2 greater than or equal to 54 mm (sensitivity 73%, specificity 74%). These six parameters allow correct diagnosis of LVH in 81%, 79%, 78%, 79% and 73% of cases, respectively. The SV1 + SV2 + RV5 + RV7 and the SV1 + SV2 + RV6 + RV7 greater than or equal to 65 mm indices are the most stable (same sensitivity and specificity for several consecutive threshold values, i.e. 62 to 67 mm and 64 to 66 mm respectively); the results obtained with these two indices are therefore more likely to be reproducible than those of the other indices as they seem less dependent on the sampling. The indices of LVH based on the QRS amplitude in the precordial leads remain valid in the presence of LBBB and are sufficiently reliable for the diagnosis of LVH to be clinically useful.
The authors report one case of biventricular hypertrophic cardiomyopathy in a 23 year old female patient with severe localized obstruction of the right ventricle (105 mm Hg gradient, almost complete angiographic disappearance of the middle and apical portions of the right ventricle during systole). Echocardiography, hemodynamic findings, and angiography demonstrated nonobstructive involvement of the left ventricle. Among other family members, only her father was noted to have minimal hypertrophic cardiomyopathy. A favorable response to therapy was obtained over 14 months with 320 mg of propranolol given daily (disappearance of palpitations and ventricular tachycardia which had previously been noted on a continuous 24 hour recording before treatment). Other very rare similar cases in the literature are analyzed. It is not possible to distinguish these cases from those with predominantly left ventricular involvement by clinical and radiological findings alone. However, the ECG can often demonstrate right ventricular involvement (right ventricular hypertrophy and/or right bundle branch block). Lastly, it appears that biventricular hypertrophic cardiomyopathies in young patients can initially be localized to only the right ventricle.
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Two cases of tetraparesis due to cervico-occipital spinal cord compression in infants with achondroplasia are reported. Neurological disorders in achondroplasia are produced by structural anomalies of the cranium and spinal canal. They can be divided into two categories: hydrocephalus and radicular and spinal cord compression syndromes. Cervico-occipital compression is more frequent in childhood and may occur very early, in the first months of life. Early diagnosis of this complication and anatomic evaluation can now be obtained by computed tomography, which shows the exact dimensions of the foramen magnum, and myelography. Achondroplasia requires very close neurologic monitoring and, when findings are abnormal, radiologic investigations of the cervico-occipital zone. This allows for early surgical management which is the only means of obtaining significant therapeutic results.
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