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      Catheter Ablation of Left Ventricular Summit Ectopies in Left Ventricular Noncompaction

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          Abstract

          Left ventricular noncompaction (LVNC) is a rare disorder and the true prevalence is largely unknown. Its clinical presentation is highly variable from being asymptomatic to the presence of heart failure, thromboembolic events, arrhythmias, and even risk of sudden cardiac death. A 37-year-old woman presented with frequent and symptomatic premature ventricular complexes (PVCs) and reduced left ventricular systolic function due to LVNC cardiomyopathy. The PVCs were refractory to medical therapy and the patient underwent successful ablation of the left ventricular summit PVCs. There was no recurrence of the PVCs at 6 months follow-up. This case report adds to the growing evidence of the efficacy and safety of performing radiofrequency ablation of ventricular arrhythmias (VAs) refractory to medical therapy in patients with LVNC. The different mechanisms of the VAs and therapeutic options are also reviewed.

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          Most cited references13

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          Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention.

          Classifications of heart muscle diseases have proved to be exceedingly complex and in many respects contradictory. Indeed, the precise language used to describe these diseases is profoundly important. A new contemporary and rigorous classification of cardiomyopathies (with definitions) is proposed here. This reference document affords an important framework and measure of clarity to this heterogeneous group of diseases. Of particular note, the present classification scheme recognizes the rapid evolution of molecular genetics in cardiology, as well as the introduction of several recently described diseases, and is unique in that it incorporates ion channelopathies as a primary cardiomyopathy.
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            2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

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              Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis.

              We sought to describe characteristics and outcome in adults with isolated ventricular noncompaction (IVNC). Isolated ventricular noncompaction is an unclassified cardiomyopathy due to intrauterine arrest of compaction of the loose interwoven meshwork. Knowledge regarding diagnosis, morbidity and prognosis is limited. Echocardiographic criteria for IVNC include-in the absence of significant heart lesions-segmental thickening of the left ventricular myocardial wall consisting of two layers: a thin, compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep recesses. Thirty-four adults (age >16 years, 25 men) fulfilled the diagnostic criteria and were followed prospectively. At diagnosis, mean age was 42 + 17 years, and 12 patients (35%) were in New York Heart Association class III/IV. Left ventricular end-diastolic diameter was 65 + 12 mm and ejection fraction 33 + 13%. Apex and/or midventricular segments of both the inferior and lateral wall were involved in >80% of patients. Follow-up was 44 + 40 months. Major complications were heart failure in 18 patients (53%), thromboembolic events in 8 patients (24%) and ventricular tachycardias in 14 patients (41%). There were 12 deaths: sudden in six, end-stage heart failure in four and other causes in two patients. Four patients underwent heart transplantation. Automated cardioverter/defibrillators were implanted in four patients. Diagnosis of IVNC by echocardiography using strict criteria is feasible. Its mortality and morbidity are high, including heart failure, thrombo-embolic events and ventricular arrhythmias. Risk stratification includes heart failure therapy, oral anticoagulation, heart transplantation and implantation of an automated defibrillator/cardioverter. As IVNC is a distinct entity, its classification as a specific cardiomyopathy seems to be more appropriate.
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                Author and article information

                Journal
                J Med Cases
                J Med Cases
                Elmer Press
                Journal of Medical Cases
                Elmer Press
                1923-4155
                1923-4163
                December 2023
                29 December 2023
                : 14
                : 12
                : 413-418
                Affiliations
                Division of Electrophysiology, Department of Adult Cardiology, Philippine Heart Center, Quezon City 1100, Philippines. Email: efadreguilan@ 123456yahoo.com
                Author information
                https://orcid.org/0000-0003-3944-3469
                Article
                10.14740/jmc4178
                10769650
                2d1955bb-8c9c-4a3f-a6b2-e1f5e85cfc6b
                Copyright 2023, Fadreguilan

                This article is distributed under the terms of the Creative Commons Attribution Non-Commercial 4.0 International License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 28 November 2023
                : 15 December 2023
                Categories
                Case Report

                ventricular arrhythmia,left ventricular noncompaction,cardiomyopathy,radiofrequency ablation,3d electroanatomic mapping

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