Cardiac Amyloidosis: Internist and Cardiologist Insight
Cardiac amyloidosis (amyloid cardiomyopathy) is a disease damage to the heart caused by extracellular amyloid deposition. In some cases, there may be local damage to the structures of the heart, for example, the atria; more often, heart damage is part of a systemic (generalized) pathology. Depending...
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| Format: | Article |
| Language: | Russian |
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SINAPS LLC
2020-12-01
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| Series: | Архивъ внутренней медицины |
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| Online Access: | https://www.medarhive.ru/jour/article/view/1106 |
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| author | E. V. Reznik T. L. Nguyen E. A. Stepanova D. V. Ustyuzhanin I. G. Nikitin |
| author_facet | E. V. Reznik T. L. Nguyen E. A. Stepanova D. V. Ustyuzhanin I. G. Nikitin |
| author_sort | E. V. Reznik |
| collection | DOAJ |
| description | Cardiac amyloidosis (amyloid cardiomyopathy) is a disease damage to the heart caused by extracellular amyloid deposition. In some cases, there may be local damage to the structures of the heart, for example, the atria; more often, heart damage is part of a systemic (generalized) pathology. Depending on the amyloid precursor protein, 36 types of amyloidosis are described, among which hereditary and acquired forms are distinguished. Cardiac amyloidosis is diagnosed 1) in the case of the amyloid infiltration in the myocardial bioptates or 2) in the case of non-cardiac amyloid deposition and the left ventricular wall thickening >12 mm without arterial hypertension and other reasons. The heart is most often affected in AL-, ATTR-, AA-, AANF-types of amyloidosis. Cardiac amyloidosis should be considered in patients with a heart failure with an unclear etiology, especially with preserved left ventricular ejection fraction, refractory to treatment, with proteinuria and CKD 4-5, in patients with idiopathic atrial fibrillation and conduction disturbances, in patients with left ventricular wall thickening of unclear etiology, low ECG voltage, unexplained arterial hypotension and pulmonary hypertension. Screening for cardiac amyloidosis should include non-invasive methods such as electrophoresis and immunofixation of blood and urine proteins, the free light lambda and kappa chains of immunoglobulins, 99Tc-DPD scintigraphy, genetic testing (if hereditary variants of amyloidosis are suspected), as well as a histological examination of biopsy samples stained with Congo red and polarizing microscopy. |
| first_indexed | 2024-04-10T03:56:09Z |
| format | Article |
| id | doaj.art-7fc6311d5d7d40dbbc4c006e81e4a731 |
| institution | Directory Open Access Journal |
| issn | 2226-6704 2411-6564 |
| language | Russian |
| last_indexed | 2024-04-10T03:56:09Z |
| publishDate | 2020-12-01 |
| publisher | SINAPS LLC |
| record_format | Article |
| series | Архивъ внутренней медицины |
| spelling | doaj.art-7fc6311d5d7d40dbbc4c006e81e4a7312023-03-13T07:12:10ZrusSINAPS LLCАрхивъ внутренней медицины2226-67042411-65642020-12-0110643045710.20514/2226-6704-2020-10-6-430-457817Cardiac Amyloidosis: Internist and Cardiologist InsightE. V. Reznik0T. L. Nguyen1E. A. Stepanova2D. V. Ustyuzhanin3I. G. Nikitin4ФГАОУ ВО «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» МЗ РФ; ГБУЗ ГКБ им. В.М. Буянова ДЗМФГАОУ ВО «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» МЗ РФГБУЗ ГКБ им. В.М. Буянова ДЗМФГБУ «Национальный медицинский исследовательский центр кардиологии» МЗ РФФГАОУ ВО «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» МЗ РФ; ФГАУ «Лечебно-Реабилитационный Центр» МЗ РФCardiac amyloidosis (amyloid cardiomyopathy) is a disease damage to the heart caused by extracellular amyloid deposition. In some cases, there may be local damage to the structures of the heart, for example, the atria; more often, heart damage is part of a systemic (generalized) pathology. Depending on the amyloid precursor protein, 36 types of amyloidosis are described, among which hereditary and acquired forms are distinguished. Cardiac amyloidosis is diagnosed 1) in the case of the amyloid infiltration in the myocardial bioptates or 2) in the case of non-cardiac amyloid deposition and the left ventricular wall thickening >12 mm without arterial hypertension and other reasons. The heart is most often affected in AL-, ATTR-, AA-, AANF-types of amyloidosis. Cardiac amyloidosis should be considered in patients with a heart failure with an unclear etiology, especially with preserved left ventricular ejection fraction, refractory to treatment, with proteinuria and CKD 4-5, in patients with idiopathic atrial fibrillation and conduction disturbances, in patients with left ventricular wall thickening of unclear etiology, low ECG voltage, unexplained arterial hypotension and pulmonary hypertension. Screening for cardiac amyloidosis should include non-invasive methods such as electrophoresis and immunofixation of blood and urine proteins, the free light lambda and kappa chains of immunoglobulins, 99Tc-DPD scintigraphy, genetic testing (if hereditary variants of amyloidosis are suspected), as well as a histological examination of biopsy samples stained with Congo red and polarizing microscopy.https://www.medarhive.ru/jour/article/view/1106амилоидоз сердцаамилоидная кардиомиопатияхроническая сердечная недостаточность с сохраненной фракцией выброса левого желудочкафибрилляция предсердийгипертрофия левого желудочкахроническая болезнь почекальбуминуриянефротический синдром |
| spellingShingle | E. V. Reznik T. L. Nguyen E. A. Stepanova D. V. Ustyuzhanin I. G. Nikitin Cardiac Amyloidosis: Internist and Cardiologist Insight Архивъ внутренней медицины амилоидоз сердца амилоидная кардиомиопатия хроническая сердечная недостаточность с сохраненной фракцией выброса левого желудочка фибрилляция предсердий гипертрофия левого желудочка хроническая болезнь почек альбуминурия нефротический синдром |
| title | Cardiac Amyloidosis: Internist and Cardiologist Insight |
| title_full | Cardiac Amyloidosis: Internist and Cardiologist Insight |
| title_fullStr | Cardiac Amyloidosis: Internist and Cardiologist Insight |
| title_full_unstemmed | Cardiac Amyloidosis: Internist and Cardiologist Insight |
| title_short | Cardiac Amyloidosis: Internist and Cardiologist Insight |
| title_sort | cardiac amyloidosis internist and cardiologist insight |
| topic | амилоидоз сердца амилоидная кардиомиопатия хроническая сердечная недостаточность с сохраненной фракцией выброса левого желудочка фибрилляция предсердий гипертрофия левого желудочка хроническая болезнь почек альбуминурия нефротический синдром |
| url | https://www.medarhive.ru/jour/article/view/1106 |
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