{"title":"野生型转甲状腺素型心脏淀粉样变性的右心房变异。","authors":"Narra Lavanya, Abraham Oomman","doi":"10.15420/usc.2024.30","DOIUrl":null,"url":null,"abstract":"<p><p>Amyloidosis is caused by extracellular deposition of amyloid protein in various organs and tissues. Light-chain amyloidosis is the most common systemic amyloidosis, whereas transthyretin amyloid cardiomyopathy is emerging as the underdiagnosed variant, especially in the elderly. Cardiac MRI and technetium-99m-pyrophosphate scintigraphy are specific non-invasive modalities that have simplified the diagnostic accuracy of cardiac amyloidosis. Identifying the type of amyloidosis is of paramount importance, given the differences in management protocols. Increased left-ventricular wall thickness and diastolic dysfunction are the most easily detectable manifestations of cardiac amyloidosis. Atrial involvement is early in both light-chain and transthyretin amyloidosis and is associated with high risk of arrhythmias and thromboembolic events. We report a case of wild-type transthyretin amyloid cardiomyopathy with predominant involvement of the right atrium and patchy involvement of the right and left ventricles.</p>","PeriodicalId":93393,"journal":{"name":"US cardiology","volume":"19 ","pages":"e08"},"PeriodicalIF":0.0000,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11976736/pdf/","citationCount":"0","resultStr":"{\"title\":\"A Right-atrial Variant of Wild-type Transthyretin Cardiac Amyloidosis.\",\"authors\":\"Narra Lavanya, Abraham Oomman\",\"doi\":\"10.15420/usc.2024.30\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Amyloidosis is caused by extracellular deposition of amyloid protein in various organs and tissues. Light-chain amyloidosis is the most common systemic amyloidosis, whereas transthyretin amyloid cardiomyopathy is emerging as the underdiagnosed variant, especially in the elderly. Cardiac MRI and technetium-99m-pyrophosphate scintigraphy are specific non-invasive modalities that have simplified the diagnostic accuracy of cardiac amyloidosis. Identifying the type of amyloidosis is of paramount importance, given the differences in management protocols. Increased left-ventricular wall thickness and diastolic dysfunction are the most easily detectable manifestations of cardiac amyloidosis. Atrial involvement is early in both light-chain and transthyretin amyloidosis and is associated with high risk of arrhythmias and thromboembolic events. We report a case of wild-type transthyretin amyloid cardiomyopathy with predominant involvement of the right atrium and patchy involvement of the right and left ventricles.</p>\",\"PeriodicalId\":93393,\"journal\":{\"name\":\"US cardiology\",\"volume\":\"19 \",\"pages\":\"e08\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-04-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11976736/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"US cardiology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.15420/usc.2024.30\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"US cardiology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.15420/usc.2024.30","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/1/1 0:00:00","PubModel":"eCollection","JCR":"","JCRName":"","Score":null,"Total":0}
A Right-atrial Variant of Wild-type Transthyretin Cardiac Amyloidosis.
Amyloidosis is caused by extracellular deposition of amyloid protein in various organs and tissues. Light-chain amyloidosis is the most common systemic amyloidosis, whereas transthyretin amyloid cardiomyopathy is emerging as the underdiagnosed variant, especially in the elderly. Cardiac MRI and technetium-99m-pyrophosphate scintigraphy are specific non-invasive modalities that have simplified the diagnostic accuracy of cardiac amyloidosis. Identifying the type of amyloidosis is of paramount importance, given the differences in management protocols. Increased left-ventricular wall thickness and diastolic dysfunction are the most easily detectable manifestations of cardiac amyloidosis. Atrial involvement is early in both light-chain and transthyretin amyloidosis and is associated with high risk of arrhythmias and thromboembolic events. We report a case of wild-type transthyretin amyloid cardiomyopathy with predominant involvement of the right atrium and patchy involvement of the right and left ventricles.