Cardiac magnetic resonance evaluation of left ventricular remodelling distribution in cardiac amyloidosis

Eduardo Pozo1, Anubhav Kanwar2, Rajiv Deochand3

  • 1The Zena and Michael A. Wiener Cardiovascular Institute/Marie-Josee and Henry R. Kravis Center for Cardiovascular Health, Icahn School of Medicine, New York, New York, USA Servicio de Cardiología, Hospital Universitario de La Princesa, Madrid, Madrid, Spain.

Insights

Cardiac amyloidosis (CA) often shows asymmetric left ventricular hypertrophy (LVH) and unusual remodeling patterns on cardiac magnetic resonance (CMR). Traditional criteria for CA have low sensitivity, highlighting the need for advanced imaging analysis.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Cardiomyopathy Research

Background:

  • Cardiac amyloidosis (CA) presents with characteristic echocardiographic findings like concentric left ventricular hypertrophy (LVH).
  • Cardiac magnetic resonance (CMR) offers detailed anatomical visualization crucial for diagnosing cardiomyopathies.
  • Understanding CA's specific morphological features and remodeling patterns is essential for accurate diagnosis.

Purpose of the Study:

  • To characterize the morphological features and remodeling patterns of CA using CMR.
  • To determine the diagnostic accuracy of CMR for CA.
  • To evaluate the utility of traditional echocardiography and electrocardiography criteria for CA diagnosis.

Main Methods:

  • Retrospective analysis of patients referred for CMR due to suspected CA.
  • CA diagnosis confirmed by endomyocardial biopsy and/or characteristic late gadolinium enhancement patterns.
  • Assessment of left ventricular hypertrophy (LVH), relative wall thickness (RWT), and LV remodeling using cine sequences.

Main Results:

  • In 130 patients, 51 (39.2%) were diagnosed with CA.
  • CA patients exhibited increased LV wall thickness and LV mass index.
  • Asymmetric LVH was present in 68.6%, and atypical remodeling in 42% of CA patients.
  • A model combining RWT, asymmetric LVH, and LVMI achieved 88% diagnostic accuracy for CA.

Conclusions:

  • Asymmetric LVH and non-concentric remodeling are frequent in CA.
  • Increased LV mass index, RWT, and asymmetric LVH are key indicators for CA diagnosis.
  • Traditional diagnostic criteria for CA demonstrate limited sensitivity.
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...
936
Aortic Regurgitation II: Clinical Features and Diagnostic Tests01:22

Aortic Regurgitation II: Clinical Features and Diagnostic Tests

Aortic valve regurgitation (AR) occurs when the aortic valve fails to close properly, allowing blood to flow backward from the aorta into the left ventricle. This backflow can result in two distinct clinical presentations: acute and chronic AR, each characterized by its own set of symptoms and physical findings.Acute Aortic RegurgitationAcute AR presents with a sudden onset of severe symptoms. Patients typically experience profound dyspnea (shortness of breath), chest pain, and signs of left...
1.1K
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...
550
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...
801
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
786