Utilization rates and determinants of PYP and CMR among patients with unexplained left ventricular hypertrophy on

Nathaniel A Fessehaie1, Hilary Bediako2, Michael J Kallan3

  • 1Department of Internal Medicine, Brigham and Women's Hospital, Boston, MA, United States of America.

Insights

Cardiac amyloidosis (CA) diagnosis rates are low, with only 8.2% of eligible patients undergoing further testing. Advanced disease indicators like severe diastolic dysfunction and wall thickness increased the likelihood of follow-up testing for CA.

Area of Science:

  • Cardiology
  • Medical Diagnostics
  • Cardiac Imaging

Background:

  • Cardiac amyloidosis (CA) is an underdiagnosed cause of heart failure with significant morbidity and mortality.
  • Echocardiography is a key screening tool for CA, but subsequent diagnostic test utilization is not well understood.

Purpose of the Study:

  • To investigate the utilization rates of cardiac MRI (CMR) and PYP scans in patients with echocardiographic features suggestive of CA.
  • To identify clinical and echocardiographic factors associated with the use of CMR and PYP scans for CA diagnosis.

Main Methods:

  • Retrospective cohort study of patients (age ≥ 18) with moderate-to-severe left ventricular hypertrophy and diastolic dysfunction on echocardiography from December 2018 to September 2020.
  • Logistic regression models were used to determine factors associated with undergoing CMR or PYP scan.

Main Results:

  • Only 8.2% (83/1015) of eligible patients underwent further diagnostic testing for CA.
  • Factors associated with increased testing included older age (65+), Black race, HFrEF diagnosis, severe diastolic dysfunction, severe left ventricular wall thickness, and echocardiogram ordered by a cardiologist.

Conclusions:

  • Follow-up diagnostic testing for CA remains low even in patients with suggestive echocardiographic findings.
  • Characteristics indicative of more advanced disease were associated with higher rates of further testing, highlighting a need for earlier diagnostic strategies.
Abstract

Related Concept Videos

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...
64
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...
42
Mitral Valve Prolapse II: Assessment and Management01:22

Mitral Valve Prolapse II: Assessment and Management

IntroductionA range of clinical features characterizes Mitral Valve Prolapse (MVP), but it is important to note that many individuals with MVP are asymptomatic and may remain so throughout their lives. For those who do exhibit symptoms, the following are the key clinical features:Palpitations: This is a common symptom where individuals feel an irregular or rapid heartbeat. Palpitations in MVP are often due to arrhythmias such as premature ventricular contractions or supraventricular...
45
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
71
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
Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
40