Natural history and disease progression of early cardiac amyloidosis evaluated by echocardiography
| العنوان: | Natural history and disease progression of early cardiac amyloidosis evaluated by echocardiography |
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| المؤلفون: | Alik Sagie, Tamir Bental, Hadas Ofek, Yaron Shapira, M Vaturi, Alon Eisen, Ran Kornowski, Aryeh Abelow, Zaza Iakobishvili, Ashraf Hamdan, S Schwarzenberg, Shirit Kazum, O Itzhaki Ben Zadok, Daniel Monakier |
| المصدر: | European Heart Journal - Cardiovascular Imaging. 22 |
| بيانات النشر: | Oxford University Press (OUP), 2021. |
| سنة النشر: | 2021 |
| مصطلحات موضوعية: | Natural history, medicine.medical_specialty, Cardiac amyloidosis, business.industry, Internal medicine, Disease progression, medicine, Cardiology, Radiology, Nuclear Medicine and imaging, General Medicine, Cardiology and Cardiovascular Medicine, business |
| الوصف: | Funding Acknowledgements Type of funding sources: None. Background Since the diagnosis of cardiac amyloidosis (CA) is often delayed, echocardiographic findings are frequently indicative of already advanced cardiomyopathy. Aims to describe early echocardiographic features in patients subsequently diagnosed with CA and to delineate disease progression. Methods Pre-amyloid diagnosis echocardiographic studies were screened for structural and functional parameters and stratified according to the pathogenetic amyloid subtype (immunoglobulin light-chain (AL) or amyloid transthyretin (ATTR)). Abnormalities were defined based on published guidelines. Results Our cohort included 75 CA patients of whom 42 (56%) were diagnosed with AL and 33 (44%) with ATTR. Forty-two patients had an earlier echocardiography exam available for review. Patients presented with increased wall thickness (1.3 (IQR 1.0, 1.5)cm) ≥3 years before the diagnosis of CA and relative wall thickness (RWT) was increased (0.47 (IQR 0.41, 0.50)) ≥7 years pre-diagnosis. Between 1 to 3 years before CA diagnosis restrictive left ventricular (LV) filling pattern was present in 19% of patients and LV ejection fraction (LVEF)≤50% was present in 21% of patients. Right ventricular dysfunction was detected concomitantly with disease diagnosis. The echocardiographic phenotype of ATTR versus AL-CA showed increased RWT (0.74 (IQR 0.62, 0.92) vs. 0.62 (IQR 0.54, 0.76), p = 0.004) and LV mass index (144 (IQR 129, 191) vs. 115 (IQR 105, 146)g/m2,p = 0.020) and reduced LVEF (50 (IQR 44, 58) vs. (60 (IQR 53, 60)%, p = 0.009) throughout the time course of CA progression, albeit survival time was similar. Conclusions Increased wall thickness and diastolic dysfunction in CA develop over a time course of several years and can be diagnosed in their earlier stages by standard echocardiography Abstract Figure. Schematic proposed timeline of CA |
| تدمد: | 2047-2412 2047-2404 |
| DOI: | 10.1093/ehjci/jeaa356.387 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_________::a3b799b47ba5c24c4464deca685f6336 https://doi.org/10.1093/ehjci/jeaa356.387 |
| Rights: | OPEN |
| رقم الانضمام: | edsair.doi...........a3b799b47ba5c24c4464deca685f6336 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 20472412 20472404 |
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| DOI: | 10.1093/ehjci/jeaa356.387 |