Primary Left Atrial Cardiopathy in Transthyretin Amyloidosis Cardiomyopathy by Multimodality Imaging: Implications

Benay Ozbay1, Corey Rearick1, Bharadwaj S Satyavolu1

  • 1Heart and Vascular Institute, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.

PubMed

Insights

Thrombotic event risk in transthyretin amyloid cardiomyopathy (ATTR-CM) is linked to primary left atrial (LA) remodeling, not just atrial fibrillation or LA size. This LA cardiopathy is a key factor in ATTR-CM patients.

Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Cardiac Electrophysiology

Background:

  • Transthyretin amyloid cardiomyopathy (ATTR-CM) is associated with a high risk of thrombotic events (TEs).
  • Understanding the mechanisms driving TE risk in ATTR-CM is crucial for patient management.

Purpose of the Study:

  • To investigate left atrial (LA) remodeling in ATTR-CM.
  • To compare LA remodeling and TE risk in ATTR-CM versus other left ventricular hypertrophy (LVH) phenotypes.
  • To identify independent predictors of TE in ATTR-CM.

Main Methods:

  • Retrospective analysis of 405 subjects undergoing echocardiography, scintigraphy, and electrophoresis.
  • Propensity score matching for age, sex, coronary artery disease, and diabetes.
  • 5-year longitudinal follow-up for thrombotic events (TEs).

Main Results:

  • ATTR-CM patients exhibited reduced left atrial reservoir strain (LASr) and increased LA stiffness compared to LVH controls.
  • LASr in ATTR-CM was depressed independently of diastolic dysfunction and atrial fibrillation (AF).
  • LASr and LA stiffness were independently associated with TEs in ATTR-CM, irrespective of CHA2DS2-VASc score or baseline AF.

Conclusions:

  • Primary LA cardiopathy, characterized by impaired LASr and increased LA stiffness, is associated with TE risk in ATTR-CM.
  • This association is independent of AF, CHA2DS2-VASc score, and LA dilatation.
  • These findings differentiate TE risk factors in ATTR-CM from other LVH phenotypes.
Abstract

Related Concept Videos

Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
39
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
61
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
67
Mitral Stenosis II: Clinical features and Diagnostic Tests01:23

Mitral Stenosis II: Clinical features and Diagnostic Tests

Mitral stenosis is a heart condition in which the mitral valve, which allows blood to flow from the left atrium to the left ventricle, becomes narrowed or stenotic. This narrowing hinders blood flow and leads to clinical symptoms requiring specific medical evaluations and management strategies. The following overview outlines the clinical symptoms, assessments, diagnostic findings, prevention methods, and treatments for mitral stenosis.Clinical ManifestationsDyspnea (shortness of breath): This...
39
Imaging Studies for Cardiovascular System V: CT01:28

Imaging Studies for Cardiovascular System V: CT

Cardiac computed tomography (CT) scanning is an advanced cardiac imaging technique that utilizes CT technology, with or without intravenous (IV) contrast, to produce accurate cross-sectional virtual slices of specific areas of the heart, coronary circulation, and major blood vessels such as the aorta, pulmonary veins, and arteries. The computer processes these slices to generate three-dimensional images. Multidetector CT (MDCT) is a rapid form of CT scanning that captures multiple slices...
71
Acute Coronary Syndrome III: Diagnostic Studies01:30

Acute Coronary Syndrome III: Diagnostic Studies

Diagnosing acute coronary syndrome or ACS begins with a thorough patient history. Notable symptoms include central, crushing chest pain radiating to the left arm, neck, jaw, or back, along with shortness of breath, sweating (diaphoresis), nausea, vomiting, dizziness, and palpitations.It is crucial to note any history of cardiac illnesses and assess risk factors, including age, gender, smoking, hypertension, diabetes, hyperlipidemia, and a sedentary lifestyle.During physical examination, vital...
25