Key result
Combined medical therapy improves symptoms and exercise tolerance in LVNC.
Why the study?
Left ventricular non-compaction cardiomyopathy is a rare genetic cardiomyopathy with variable presentation requiring comprehensive diagnostic evaluation and management strategies.
Case Report (n=1)
No
May support combined therapy in symptomatic LV non-compaction; hypothesis-generating and requires confirmation in larger studies.
Background : Left non-compaction cardiomyopathy (LVNC) or “spongy myocardium” is a relatively rare primary genetic cardiomyopathy, characterized by prominent wall trabeculations and intertrabecular recesses which communicate with the ventricular cavity. It appears in isolated form or coexists with other congenital heart diseases and/or systemic abnormalities. Material and method: A 28-year-old woman was admitted with exertional dyspnoea, palpitations, non-specific chest pain and progressive fatigue on exertion. In her family history sudden cardiac-related deaths at young age are present. Cardiovascular system examination revealed tachycardia, intermittent extrabeats. The rest EKG showed sinusal tachycardia (105 bpm), negative T-waves in DII, DIII, aVF, V4-V6. Consecutive 24 hours Holter EKG monitoring revealed nonsustained ventricular tachycardia, paroxysmal atrial fibrillation, isolated ventricular extrasystoles. Echocardiography showed left ventricular systolic dysfunction (LVEF:30-35%), slight LV enlargement, normal right ventricle and small left ventricle (LV) trabeculae in the apical area. Cardiac MRI demonstrated dilated LV and the presence of the trabeculations of LV walls suggestive for non-compaction cardiomyopathy. A combined treatment for heart failure and cardiac arrhythmias was initiated with good clinical results. Patient was scheduled for an implantable cardioverter defibrillator “life-saving”. Conclusions: The symptoms of heart failure and cardiac arrhythmias should be considered important in apparently healthy young patients. Besides intensive medical treatment is indicated the implantation of an ICD “life-saving” and in advanced cases heart transplantation. Even if the electrocardiographic findings are non specific for noncompaction, a complete diagnostic evaluation is important, including sophisticated imaging techniques, a screening of first-degree relatives, and an extensive clinical, and genetic appreciation by a multidisciplinary team.
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Timea et al. (2015) conducted a case report in Left Ventricular Non-compaction Cardiomyopathy (n=1). Medical treatment (beta-blockers, diuretic, ACE inhibitor, antiarrhythmics, oral anticoagulation) was evaluated on Clinical response. Combined medical treatment for heart failure and arrhythmias improved clinical symptoms and exercise tolerance in a 28-year-old woman with left ventricular non-compaction cardiomyopathy.
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