Morphological anomalies in obstructive hypertrophic cardiomyopathy: Insights from four-dimensional computed

Yuki Izumi1, Shuichiro Takanashi2, Mitsunobu Kitamura1

  • 1Hypertrophic Cardiomyopathy Center, Sakakibara Heart Institute, Tokyo, Japan; Department of Cardiology, Sakakibara Heart Institute, Tokyo, Japan.

Journal of Cardiology
|July 13, 2024
PubMed

Insights

Hypertrophic cardiomyopathy (HCM) involves complex anatomy beyond septal hypertrophy. Four-dimensional computed tomography (4D-CT) provides detailed morphological assessment for improved surgical planning in obstructive HCM.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Genetics

Background:

  • Hypertrophic cardiomyopathy (HCM) is a genetic heart disease.
  • Left ventricular outflow tract (LVOT) obstruction significantly impacts HCM symptoms and prognosis.
  • Traditional understanding focused on septal hypertrophy, but other anomalies are now recognized.

Purpose of the Study:

  • To highlight the importance of comprehensive morphological assessment in obstructive HCM.
  • To evaluate the role of four-dimensional computed tomography (4D-CT) in characterizing complex HCM anatomy.
  • To demonstrate how detailed 4D-CT findings can guide septal reduction therapy.

Main Methods:

  • Review of recent evidence on HCM morphology.
  • Comparison of imaging modalities for assessing anomalous structures in HCM.
  • Application of 4D-CT for detailed evaluation of mitral valve, papillary muscles, and muscle bundles.
  • Utilizing 4D-CT for surgical planning in a specialized HCM center.

Main Results:

  • HCM involves complex anatomical variations including mitral valve and papillary muscle anomalies, and apical-basal muscle bundles.
  • Four-dimensional computed tomography offers superior spatial resolution and multiplanar capabilities for detailed morphological assessment.
  • 4D-CT enables precise evaluation of subaortic stenosis and right ventricular outflow tract obstruction.
  • Detailed 4D-CT assessment aids in planning surgical correction for obstructive HCM.

Conclusions:

  • Accurate morphological assessment is crucial for effective septal reduction therapy in obstructive HCM.
  • Four-dimensional computed tomography is a valuable tool for detailed anatomical evaluation in HCM.
  • Comprehensive 4D-CT-guided surgical planning may improve outcomes for patients with obstructive HCM.

Related Concept Videos

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...
565
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...
1.2K
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...
837
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...
812
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...
968
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...
762