Details

Autor(en) / Beteiligte

• Narotsky, David L., MD
• Castano, Adam, MD
• Weinsaft, Jonathan W., MD
• Bokhari, Sabahat, MD
• Maurer, Mathew S., MD

Titel

Wild-type Transthyretin Cardiac Amyloidosis: Novel Insights from Advanced Imaging

Ist Teil von

• Canadian journal of cardiology, 2016-09, Vol.32 (9), p.1166.e1-1166.e10

Ort / Verlag

England: Elsevier Inc

Erscheinungsjahr

2016

Link zum Volltext

Quelle

MEDLINE

Beschreibungen/Notizen

• Amyloidosis is caused by extracellular deposition of abnormal protein fibrils, resulting in destruction of tissue architecture and impairment of organ function. The most common forms of systemic amyloidosis are light-chain (AL) and transthyretin-related (ATTR). ATTR can result from an autosomal dominant hereditary transmission of mutated genes in the TTR (ATTRm) or from a wild-type form of disease (ATTRwt), previously known as senile cardiac amyloidosis (SCA). With the aging of the worldwide population, ATTRwt will emerge as the most common type of cardiac amyloidosis that clinicians encounter. Diagnosis of systemic amyloidosis is often delayed, either due to the false assumption that it is a rare disease, or due to misdiagnosis as a result of mistaking it with other conditions. Clinicians must integrate clinical clues from history, physical exam, and common diagnostic tests to raise suspicion for ATTRwt. The historical gold standard for diagnosis of cardiac amyloid is endomyocardial biopsy (EMB) with pathological distinction of precursor protein type, but this method often results in delayed diagnosis given the limited availability of expertise to perform and interpret the EMB. Emerging non-invasive imaging modalities provide easier, accurate screening for ATTRwt. These modalities include: advanced echocardiography, using strain imaging and the myocardial contraction fraction; nuclear scintigraphy, which can differentiate between ATTR and AL cardiac amyloid; and cardiac magnetic resonance, using extracellular volume measurement, late gadolinium enhancement, and distinct T1 mapping. These novel approaches reveal insights into the prevalence, clinical course, morphological effects, and prognosis of ATTRwt.