Isolated Noncompaction Cardiomyopathy Presenting with Paroxysmal Supraventricular Tachycardia

Abstract

Isolated noncompaction cardiomyopathy is an exceedingly rare congenital cardiomyopathy. A case of isolated noncompaction cardiomyopathy is reported and the literature on the subject collected through a comprehensive literature search is reviewed. Fewer than 100 cases of this condition have been reported. Isolated noncompaction cardiomyopathy is caused by a defect in cardiac morphogenesis resulting in an arrest of compaction of loose interwoven meshwork of myocardial fibers during intrauterine life, which results in severe systolic dysfunction as well as undue hypertrophy of the involved walls of the ventricles. Although the most frequent sites involved are left ventricular apex and inferior wall, involvement of other left ventricular walls and right ventricle has also been reported. Etiology of the isolated noncompaction of myocardium is not clear. Familial cases have been reported and the mode of inheritance is heterogeneous. In X-linked form of the disease, a locus has been found on Xq28, and mutations have been reported in G4.5 gene. The age of onset of symptoms ranges from infancy to the geriatric age. Patients with isolated noncompaction cardiomyopathy have a high incidence of heart failure, arrhythmias, and thromboembolism. The most common presentation is congestive heart failure. Arrhythmias include atrial arrhythmias, ventricular tachycardia, and sudden cardiac death. The patient reported in this article presented with paroxysmal supraventricular tachycardia. Echocardiography is the procedure of choice to establish diagnosis. Due to the lack of associated cardiac anomalies, antenatal detection is difficult. The treatment is that for congestive heart failure, arrhythmias, and thromboembolism. The end-stage congestive heart failure is managed with heart transplantation and potential life-threatening ventricular tachyarrhythmias with an implantable cardioverter defibrillator. Prognosis is poor and the common causes of death are intractable heart failure and sudden cardiac death.

Noncompaction of ventricular myocardium in an elderly woman

“…In this case, its clinical and prognostic implications would remain to be established. For all the above reasons, besides presenting a clinical entity that is rarely observed in the elderly [4], our case report underlines the importance of making echocardiographers more familiar with this condition when examining older patients.…”

“…Although initially described in children, several case series report this entity to also be present in adults, but rarely, in elderly patients [2][3][4]. The prevalence of NVM is estimated to range between 0.05% and 0.24% of all echocardiographic investigations in recent studies, while its incidence in the general population is yet unknown [2,3].…”

Shall a pilot with left ventricular hypertrabeculation/noncompaction fly passengers?

“…Since VTs persisted despite treatment with beta-blockers, a dual-chamber automatic ICD was implanted [9]. In another case with isolated LVHT VTs were detected in addition to atrial arrhythmias, and sudden cardiac death [10].…”

“…Furthermore, few studies that have tested this association applied a sample size big enough from an international cooperative study. The World Health Organization-Cardiovascular Disease and Alimentary Comparison (CARDIAC) Study provides an opportunity with us to examine this association between HR and BP in middle-aged populations [6][7][8][9][10].…”

“…The CARDIAC Study, with a multi-center cross-sectional study design, started in mid 1985, and continues at its phase II stage (e.g., MONALISA Study) [6,10]. The CARDIAC Study was designed to examine the association between various dietary markers and BP ("Core study") and between these factors and cardiovascular disease mortality rates ("Complete study").…”