Beyond convention: non-compacted myocardium, ventricular tachycardia, and systolic dysfunction in dextrocardia patients: a case series.

Abstract

Background: Noncompaction of the left ventricle (LVNC) is linked to a higher risk of sudden cardiac death and stroke. Its prevalence ranges from 0.014% to 1.3% in the general population, rising to 7.5% in patients with dextrocardia.

Case summary: A male in his late 70s presented with worsening dyspnea and leg swelling, with dextrocardia and frequent extrasystoles. Imaging revealed right-sided pleural effusion, severely reduced ejection fraction, and ventricular and atrial dilatation. He developed sustained monomorphic VT, was treated with amiodarone, had successful coronary stenting, and received an ICD with no further hospital readmissions or ICD events. A second case involved a male in his late 50s who presented with dyspnea. He had dextrocardia with situs inversus, subsegmental pulmonary embolism, and LVNC. He was treated with enoxaparin, medical therapy, and Holter monitoring, which showed mild arrhythmias. He declined ICD placement but remained event-free during the first year of follow-up.

Discussion: LVNC is a rare condition resulting from abnormal myocardial development during embryogenesis, leading to a two-layered myocardial structure. Diagnosis is based on imaging criteria. LVNC is linked to arrhythmias, heart failure, and conduction abnormalities, requiring interventions such as ICD placement, arrhythmia monitoring, and genetic testing. Further research is needed on genetic associations and long-term outcomes in dextrocardia patients.