<abstract xmlns="http://www.w3.org/1999/xhtml"><p> Left ventricular non-compaction (LVNC) is a cardiomyopathy that can either result from arrested or abnormal myocardial morphogenesis during heart development, or can be acquired later in life. Current practice guidelines recommend different strategies for the management of patients with LVNC. Common clinical features of this disease include cardiac failure, thromboembolism, life-threatening arrhythmia or sudden cardiac death, which could indicate a worse prognosis. The disease may occur alone or in association with other congenital cardiac, neuromuscular, mitochondrial or metabolic disorders. The association of left ventricular non-compaction with other structural cardiac congenital diseases (such as atrial or ventricular septal defect, patent ductus arteriosus, obstruction of ventricular outflow tract) is rare. As clinical manifestations of LVNC are non-specific, particular imaging modalities (echocardiography, cardiovascular magnetic resonance imaging or ECG gated computed tomography) should be used in order to establish the diagnosis of LVNC. Antiarrhythmic drugs and implantable cardioverter defibrillators may be considered for the management of ventricular arrhythmias in patients with ventricular non-compaction. We report the presentation, diagnosis and management of a 46 year-old female with refractory severe cardiac failure, repeated syncope due to LVNC and atrial septal defect, requiring medical therapy and an implantable cardioverter defibrillator as a "life bridge" to heart transplantation.</p></abstract>

Left ventricular non-compaction (LVNC) is a cardiomyopathy that can either result from arrested or abnormal myocardial morphogenesis during heart development, or can be acquired later in life. Current practice guidelines recommend different strategies for the management of patients with LVNC. Common clinical features of this disease include cardiac failure, thromboembolism, life-threatening arrhythmia or sudden cardiac death, which could indicate a worse prognosis. The disease may occur alone or in association with other congenital cardiac, neuromuscular, mitochondrial or metabolic disorders. The association of left ventricular non-compaction with other structural cardiac congenital diseases (such as atrial or ventricular septal defect, patent ductus arteriosus, obstruction of ventricular outflow tract) is rare. As clinical manifestations of LVNC are non-specific, particular imaging modalities (echocardiography, cardiovascular magnetic resonance imaging or ECG gated computed tomography) should be used in order to establish the diagnosis of LVNC. Antiarrhythmic drugs and implantable cardioverter defibrillators may be considered for the management of ventricular arrhythmias in patients with ventricular non-compaction. We report the presentation, diagnosis and management of a 46 year-old female with refractory severe cardiac failure, repeated syncope due to LVNC and atrial septal defect, requiring medical therapy and an implantable cardioverter defibrillator as a "life bridge" to heart transplantation.

Left Ventricular Non-Compaction Associated with Atrial Septal Defect — a Rare Cause of Refractory Severe Cardiac Failure

Family Medicine, Dept M3, Faculty of Medicine, University of Medicine and Pharmacy, Tîrgu Mureș, Romania

Cardiac Rehabilitation Clinic, Tirgu Mures Emergency Clinical County Hospital, Tîrgu Mureș, Romania

Cardiology Clinic, Institute of Cardiovascular Disease and Transplantation, Tîrgu Mureș, Romania

Journal of Cardiovascular Emergencies

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Left ventricular non-compaction (LVNC) is a cardiomyopathy that can either result from arrested or abnormal myocardial morphogenesis during heart development,...

Left Ventricular Non-Compaction Associated with Atrial Septal Defect — a Rare Cause of Refractory Severe Cardiac Failure

Publié en ligne: 30 déc. 2015

Pages: 75 - 81

Reçu: 15 juil. 2015

Accepté: 15 oct. 2015

DOI: https://doi.org/10.1515/jce-2015-0011

Mots clés
isolated left ventricular non-compaction, heart failure, imaging techniques, arrhythmias, implantable cardioverter defibrillator

© 2016

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Left Ventricular Non-Compaction Associated with Atrial Septal Defect — a Rare Cause of Refractory Severe Cardiac Failure

Publié en ligne: 30 déc. 2015

Pages: 75 - 81

Reçu: 15 juil. 2015

Accepté: 15 oct. 2015

DOI: https://doi.org/10.1515/jce-2015-0011

Mots clésisolated left ventricular non-compaction, heart failure, imaging techniques, arrhythmias, implantable cardioverter defibrillator

© 2016

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Mots clés
isolated left ventricular non-compaction, heart failure, imaging techniques, arrhythmias, implantable cardioverter defibrillator