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Isolated noncompaction left ventricular myocardium and polymorphic ventricular tachycardia
Luis Serés1, Jorge Lopez, Eduardo Larrousse
1Department of Cardiology, Hospital Germans Trias i Pujol, Badalona, Barcelona, Spain. Seres@ns.hugtip.scs.es
Clinical Cardiology
|January 24, 2003
Summary
This study details a case of isolated noncompaction of the left ventricular myocardium in a woman experiencing syncope and a family history of sudden death. The condition led to malignant arrhythmias, necessitating a defibrillator implant.
Area of Science:
- Cardiology
- Cardiovascular Medicine
- Genetics
Background:
- Isolated noncompaction of the left ventricular myocardium is a rare congenital cardiomyopathy.
- A family history of sudden death raises suspicion for inherited cardiac conditions.
Observation:
- The patient presented with syncope and a significant family history of premature sudden death.
- Echocardiography and ventriculography revealed prominent left ventricular trabeculations and deep intertrabecular recesses.
- Continuous ECG monitoring detected episodes of polymorphic ventricular tachycardia.
Findings:
- Despite no coronary artery lesions, the patient was diagnosed with isolated noncompaction of the left ventricular myocardium.
- Programmed ventricular stimulation failed to induce arrhythmias, but polymorphic ventricular tachycardia persisted despite beta-blocker therapy.
- A dual-chamber automatic implantable defibrillator was implanted due to persistent malignant arrhythmias.
Implications:
- This case highlights the potential for malignant arrhythmias in isolated noncompaction of the left ventricular myocardium.
- Understanding the physiopathology of arrhythmias in this condition is crucial for patient management.
- Genetic factors may play a significant role in the development and presentation of this cardiomyopathy.