Related Experiment Video

Updated: Jun 17, 2026

Cox-Maze IV Procedure Concomitant with Valvular Surgery In Situs Inversus Dextrocardia: A Single-Center Experience in China
08:42

Cox-Maze IV Procedure Concomitant with Valvular Surgery In Situs Inversus Dextrocardia: A Single-Center Experience in China

Published on: February 11, 2022

Isolated left ventricular noncompaction cardiomyopathy accompanied by severe mitral regurgitation

Jong Won Chung1, Sung Jin Lee, Jae Hak Lee

  • 1Division of Cardiology, Department of Internal Medicine, The Catholic University of Korea, St. Mary's Hospital, Seoul, Korea.

Korean Circulation Journal
|December 10, 2009
PubMed

Insights

Isolated left ventricular noncompaction cardiomyopathy (IVNC) is a rare heart muscle condition. This case highlights a young man with IVNC who underwent mitral valve replacement due to chordae rupture.

Area of Science:

  • Cardiology
  • Genetics
  • Developmental Biology

Background:

  • Isolated left ventricular noncompaction cardiomyopathy (IVNC) is a congenital heart defect resulting from abnormal embryonic development of the heart muscle.
  • IVNC often co-occurs with other congenital cardiac abnormalities.
  • The condition is characterized by prominent trabeculations and deep recesses in the ventricular myocardium.

Purpose of the Study:

  • To report a unique case of a 21-year-old male patient with IVNC presenting with severe mitral valve regurgitation and chordae rupture.
  • To document the first case in Korea of mitral valve replacement surgery performed for IVNC-associated mitral valve complications.

Main Methods:

  • Clinical presentation of a 21-year-old male with progressive exertional dyspnea.
  • Diagnostic evaluation using two-dimensional and Doppler echocardiography.
  • Surgical intervention: mitral valve replacement.

Main Results:

  • Echocardiography revealed an enlarged left atrium, dilated left ventricle with preserved systolic function, and severe mitral regurgitation due to chordae rupture.
  • Prominent trabeculations and deep intertrabecular recesses were observed in both the left and right ventricles.
  • The patient successfully underwent mitral valve replacement surgery.

Conclusions:

  • This case underscores the potential for severe mitral valve complications in patients with IVNC.
  • Mitral valve replacement can be a viable treatment option for IVNC patients experiencing significant mitral regurgitation and chordae rupture.
  • Highlights the importance of comprehensive echocardiographic assessment in diagnosing and managing IVNC and its associated complications.

Keywords:
CardiomyopathiesMitral valve regurgitation

More Related Videos

An Image Guided Transapical Mitral Valve Leaflet Puncture Model of Controlled Volume Overload from Mitral Regurgitation in the Rat07:42

An Image Guided Transapical Mitral Valve Leaflet Puncture Model of Controlled Volume Overload from Mitral Regurgitation in the Rat

Published on: May 19, 2020

Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography07:11

Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography

Published on: October 28, 2020

Related Concept Videos

Mitral Regurgitation I: Introduction01:20

Mitral Regurgitation I: Introduction

Mitral regurgitation is characterized by the backward circulation of blood from the left ventricle to the left atrium during systole, a phase of the cardiac cycle when the heart contracts and pumps blood out of the chambers. This abnormal flow occurs primarily due to the dysfunction of the mitral valve or its supporting structures, which include the mitral leaflets, chordae tendineae, annulus, and papillary muscles.Etiology and Mechanisms:Primary Mitral Regurgitation: This type arises from...
Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
Mitral Regurgitation II: Clinical Features and Diagnostic Tests01:23

Mitral Regurgitation II: Clinical Features and Diagnostic Tests

Mitral regurgitation (MR) is a valvular heart disorder in which the mitral valve fails to close tightly, allowing blood to leak backward into the heart. Understanding the clinical manifestations, assessment, diagnostic findings, and medical management of MR is crucial to effectively managing affected patients.Clinical Manifestations of Mitral RegurgitationMitral regurgitation can be acute or chronic, each presenting differently and requiring different approaches:1. Acute Mitral...
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...
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...
Mitral Regurgitation III: Medical Management01:25

Mitral Regurgitation III: Medical Management

Mitral regurgitation (MR) is characterized by retrograde blood circulation from the left ventricle into the left atrium due to inadequate mitral valve closure. The severity of the condition, symptoms, and underlying cause determine treatment strategies.Monitoring and Pharmacological TreatmentPatients with mild to moderate MR typically do not need immediate intervention but regular monitoring to assess progression and guide treatment. Patients with mild MR should have an echocardiogram every 3-5...