Early identification of amyloid heart disease by technetium-99m-pyrophosphate scintigraphy: A study with familial amyloid polyneuropathy

Hongo, Minoru; Hirayama, Junichi; Fujii, Tsuneo; Yamada, Hiroyoshi; Okubo, Shinichi; Kusama, Shozo; Ikeda, Shu‐ichi

doi:10.1016/0002-8703(87)90703-4

Cited by 49 publications

(17 citation statements)

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“…Data for echocardiograms were available in 36 of the 50 patients, and the thickness of the interventricular septum, percent fractional shortening (%FS) and E-wave deceleration time (DT) were 16.2±3.1 mm (normal <12 mm), 28.2±7.6% (normal >28%) and 145.2±38.7 ms (normal 180-250 ms), respectively. Ten patients underwent scintigraphy using 99m technetium-pyrophosphate, which is sensitive for cardiac amyloidosis (17); however, only five showed a positive uptake. The third most frequent manifestation was peripheral neuropathy (n=21, 10.4%), including somatic and autonomic nerve dysfunction and carpal tunnel syndrome.…”

Section: Resultsmentioning

confidence: 99%

Clinical Manifestations at Diagnosis in Japanese Patients with Systemic AL Amyloidosis: A Retrospective Study of 202 Cases with a Special Attention to Uncommon Symptoms

2014

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Objective To retrospectively investigate the clinical manifestations at diagnosis in Japanese patients with systemic AL amyloidosis. Methods We reviewed the medical records of 230 Japanese patients who had visited our hospital and been diagnosed with AL amyloidosis, and abstracted those with the systemic type. The clinical data at diagnosis of systemic AL amyloidosis, including laboratory and imaging findings, were analyzed. Results Two hundred and two patients (mean, 58.7±9.5 years) were enrolled in this study. Immunofixation or immunoelectrophoresis was performed in 173 patients, 144 of whom were positive for M-protein in the serum and/or urine (κ:λ=30:114). The primary clinical manifestations at diagnosis were proteinuria and/or renal dysfunction (54.0%), congestive heart failure (24.8%), peripheral neuropathy (10.4%), hepatomegaly (7.9%) and arrhythmia (5.0%). The remaining patients developed unusual manifestations, such as solitary tumor, lymphadenopathy, gastrointestinal bleeding, intestinal pseudoobstruction, hemorrhagic tendencies and polyarthralgia. Dilatation of the intestine with marked thickening of the gastrointestinal wall on computed tomography and multiple nodular lesions with associated mucosal friability on endoscopy are characteristic findings of systemic AL amyloidosis. Conclusion The clinical pictures of Japanese patients with systemic AL amyloidosis are similar to those previously reported from the US and European nations; however, some patients with this disease develop uncommon symptoms. Conducting laboratory and histological examinations for systemic AL amyloidosis is necessary when making a differential diagnosis of these symptoms.

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Section: Resultsmentioning

confidence: 99%

Clinical Manifestations at Diagnosis in Japanese Patients with Systemic AL Amyloidosis: A Retrospective Study of 202 Cases with a Special Attention to Uncommon Symptoms

2014

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“…2A and B). Myocardial technetium-99m pyrophosphate (Tc-99m-PYP) scintigraphy is also valuable (11,12) (Fig. 2C): this isotope may bind to amyloid fibril-associated calcium molecules, producing a positive shadow in amyloid heart.…”

Section: Diagnosis Of Cardiac Amyloidosismentioning

confidence: 99%

Cardiac Amyloidosis: Heterogenous Pathogenic Backgrounds

Ikeda

2004

Intern. Med.

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Cardiac amyloidosis is a fatal disorder which develops on the basis of the different pathologic conditions in systemic amyloidosis: the most common underlying disease is immunoglobulin light chain-derived primary amyloidosis and the next is transthyretin-related hereditary amyloidosis; the latter disorder, typically represented by familial amyloid polyneuropathy, was long regarded as an endemic disease. However, this disorder has now been shown to involve a highly variable clinical picture due to a large number of transthyretin gene mutations, and many patients with diverse ancestors suffer from severe cardiac amyloidosis. Additionally, senile systemic amyloidosis is now noted as a cause of cardiac dysfunction in elderly individuals. Echocardiogram and myocardial technetium-99m-pyrophosphate scintigraphy can provide characteristic findings. Immunohistochemistry on tissue amyloid, biochemical analysis of serum and urine proteins, and DNA sequencing are usually employed to determine the disease-related amyloid fibril protein.Although systemic amyloidosis has become treatable, the prognosis of each patient who received up-to-date and effective, but nevertheless stressful, therapy depends on the severity of cardiac involvement by amyloid deposition.

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“…Many case reports were published in the early 1980s and later [30,[38][39][40][41][42][43][44][45][46][47][48][49][50][51]. In 1982, Wizenberg et al investigated a group of ten patients with tissue-proven amyloidosis using 99m Tc-PYP (only two had histologically proven cardiac amyloidosis).…”

Section: Amyloid Deposits 99m Tc-aprotininmentioning

confidence: 99%

Nuclear imaging in cardiac amyloidosis

Glaudemans

Slart

Zeebregts

et al. 2009

Eur J Nucl Med Mol Imaging

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Amyloidosis is a disease characterized by depositions of amyloid in organs and tissues. It can be localized (in just one organ) or systemic. Cardiac amyloidosis is a debilitating disease and can lead to arrhythmias, deterioration of heart function and even sudden death. We reviewed PubMed/Medline, without time constraints, on the different nuclear imaging modalities that are used to visualize myocardial amyloid involvement. Several SPECT tracers have been used for this purpose. The results with these tracers in the evaluation of myocardial amyloidosis and their mechanisms of action are described. Most clinical evidence was found for the use of 123 I-MIBG. Myocardial defects in MIBG activity seem to correlate well with impaired cardiac sympathetic nerve endings due to amyloid deposits. 123 I-MIBG is an attractive option for objective evaluation of cardiac sympathetic level and may play an important role in the indirect measurement of the effect of amyloid myocardial infiltration. Other, less sensitive, options are 99m Tc-aprotinin for imaging amyloid deposits and perhaps 99m Tc-labelled phosphate derivatives, especially in the differential diagnosis of the aetiology of cardiac amyloidosis. PET tracers, despite the advantage of absolute quantification and higher resolution, are not yet well evaluated for the study of cardiac amyloidosis. Because of these advantages, there is still the need for further research in this field.

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Early identification of amyloid heart disease by technetium-99m-pyrophosphate scintigraphy: A study with familial amyloid polyneuropathy

Cited by 49 publications

References 30 publications

Clinical Manifestations at Diagnosis in Japanese Patients with Systemic AL Amyloidosis: A Retrospective Study of 202 Cases with a Special Attention to Uncommon Symptoms

Clinical Manifestations at Diagnosis in Japanese Patients with Systemic AL Amyloidosis: A Retrospective Study of 202 Cases with a Special Attention to Uncommon Symptoms

Cardiac Amyloidosis: Heterogenous Pathogenic Backgrounds

Nuclear imaging in cardiac amyloidosis

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