[A case of familial amyloid polyneuropathy demonstrating marked myocardial uptake of technetium-99m-pyrophosphate].
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Biomedical subjects
Publications and source records attributed to S Okubo.
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To assess the clinical significance of technetium-99m-pyrophosphate (Tc-99m-PYP), -methylene diphosphonate (Tc-99m-MDP) and thallium-201 (Tl-201) myocardial scintigraphy in the diagnosis of cardiac amyloidosis and in the differential diagnosis of cardiac diseases, 12 patients with biopsy-proved systemic amyloidosis (seven with familial amyloid polyneuropathy (FAP) and five with primary amyloidosis) were investigated. The results obtained were as follows: In 10 patients (six with FAP and four with primary amyloidosis) studied by Tc-99m-PYP scintigraphy, two (FAP one, primary amyloidosis one) had diffusely positive myocardial uptake, which was of greater intensity than that of the sternum. Six (four FAP; two primary amyloidosis) also had diffusely positive myocardial uptakes, but the intensity was less than that of the sternum. The remaining two (one FAP; one primary amyloidosis) had only equivocal myocardial uptakes. Two of these patients also had hepatic uptakes and another had both hepatic and thyroid uptakes. The intensity of myocardial uptake of Tc-99m-PYP in patients with echocardiographic left ventricular hypertrophy and/or highly refractile myocardial echoes, so-called granular sparkling appearance (GS) was slightly greater than that in patients with neither myocardial hypertrophy nor GS. FAP had slightly less intensity than primary amyloidosis. In 29 persons with other cardiac diseases and normal subjects examined by Tc-99m-PYP scintigraphy, seven (two dilated cardiomyopathy; two sarcoidosis; three hypertensive heart disease) also had diffusely positive myocardial scans of mild or moderate degree. However, none of them had marked myocardial or other tissue uptakes. Both Tc-99m-PYP and -MDP scintigraphic studies were performed in four patients (three FAP; one primary amyloidosis). In Tc-99m-MDP scintigraphy, diffusely positive myocardial uptakes were observed in two patients with FAP and the remaining two had negative scans. The intensity of Tc-99m-MDP myocardial uptake in each patient was significantly lower than that of Tc-99m-PYP uptake. Tl-201 scintigraphy was carried out in 10 patients (six FAP; four primary amyloidosis). Left ventricular hypertrophy was found in six patients and right ventricular visualization in five. Although electrocardiograms in seven of 10 patients showed QS patterns in the right to mid precordial leads, similar to that seen in antero-septal and extensive anterior myocardial infarctions, neither myocardial perfusion defect nor low uptake on Tl-201 images was detected in nine of them. The scintigrams in another one, which showed low uptake at the apical portion of the left ventricle, were considered normal.(ABSTRACT TRUNCATED AT 400 WORDS)
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The clinical features of 205 cases of rheumatic heart disease in Bangladesh, including unique two-dimensional echocardiographic findings, were reported, and these were compared with those of 387 Japanese cases. The percentage of mitral stenosis (MS), aortic valvular diseases including both aortic stenosis and aortic regurgitation (A), mitral stenosis with aortic valvular diseases (MS + A) were almost the same between the two countries, but that of mitral regurgitation (MR) was higher, mitral stenosis and regurgitation (MSR) was lower in Bangladesh. The mean age of the patients with mitral valvular diseases was very young and the incidence of atrial fibrillation was very low in Bangladesh. Males predominated over females in MS (male/female = 1.2/1) and the incidence of pulmonary hypertension in MS was higher in Bangladesh. A two-dimensional echocardiographic examination showed that the rough zone was heavily involved in the rheumatic process with having a narrow mitral valve orifice while the clear zone was relatively spared and pliable in many patients with MS in Bangladesh. In MR, posterior mitral leaflets were shortened and made immobile by its submitral complex which were also thickened, fused and shortened, and these resulted in a gap or non-coaptation of the two leaflets in many patients.
A 75-year-old man complaining of dyspnea and having sings of postcapillary pulmonary hypertension was diagnosed as pulmonary veno-occlusive disease and confirmed at autopsy. This is the oldest case ever reported. Almost all the small veins 2 mm or less in external diameter were partially or nearly completely occluded by intimal fibrous tissue, and the obstructive changes in the pulmonary arteries were much more limited. Pulmonary veno-occlusive disease is a rare, almost inevitably fatal disease of unknown etiology which has only recently been separated clearly from primary pulmonary hypertension as a distinct entity. Chest roentgenogram finding suggesting postcapillary pulmonary hypertension is a clue to a diagnosis and differentiates this from two other causes of clinical primary pulmonary hypertension, that is, recurrent pulmonary embolism and plexogenic pulmonary arteriopathy.
The current study was done to clarify the recent trend of clinical pulmonary thromboembolism (PTE) in Japan. The time has long passed since thromboembolic diseases of the lung were rare in our country. We have experienced, however, twenty-four clinically manifested patients with PTE during the past two years. Although a number of symptoms and findings have been reported to facilitate the diagnosis, they were for the most part nonspecific and therefore nondiagnostic. Combined perfusion and ventilation scans showing a perfusion defect with normal ventilation were reported to be useful for diagnosis of PTE, but a special attention should be paid to the fact that a massive perfusion defect was accompanied by decreased ventilation of the affected lung. From a pathophysiologic study, an exertional dyspnea associated with increased A-aDO2 and also increased a-ETDCO2 in patients without apparent pulmonary consolidation on their chest roentgenogram was strongly suggestive of PTE. A-aDO2 and a-ETDCO2 were shown to be variables independent to each other. a-ETDCO2 was valuable for clinical diagnosis with direct evidence of high VA/Q units resulting from occluded pulmonary arteries. On the other hand, A-aDO2 which was once elevated in the acute stage of PTE was decreased according to the recovery with a close linear correlation to the actual perfusion defect and was a sensitive indicator suggestive of resolved emboli. A-aDO2 was considered to provide measures of the active severity of PTE unlike a-ETDCO2.
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Computed tomography (CT) was performed for four patients with aneurysms of the sinus of Valsalva (each of the Konno types IVSD and IV, false aneurysm projecting into the interatrial septum, and annulo-aortic ectasia). Aneurysms were detected in three cases, and the sizes closely correlated with those of the resected specimens. However, a relatively small and mobile aneurysm of the IV type was not detected. Apparently, relatively large size (more than 10-20 mm) and immobility were necessary for the detection by CT. Reconstruction of CT images facilitated localization of the aneurysm and determining the extent of annulo-aortic ectasia. Rupture of an aneurysm was identified by observing the time course of CT values in the cardiac chambers using dynamic CT. However, better time resolution of CT is necessary for more precise diagnosis. Two-dimensional echocardiography is more convenient and diagnostically effective than CT. However, CT is a useful and non-invasive examination which overcomes the technical difficulties inherent in echocardiography, such as disturbances by the lung and other artifacts.