Isolated Ventricular Noncompaction Cardiomyopathy Presenting as Recurrent Syncope

Abstract

Isolated ventricular noncompaction (IVNC) occurs because of interruption of trabecular morphogenesis in the myocardium leading to ventricular noncompaction. Patients present with heart failure or with systemic complications secondary to thromboembolism or arrhythmias. High index of suspicion is necessary for early diagnosis. We present a case of 48-year-old male with unexplained recurrent syncope who was eventually diagnosed with IVNC.

Isolated Non-Compacted Myocardium: Should We Consider the Presence of Extrasystoles as the Initial Manifestation?

“…1,4 The clinical status is variable, and it may be asymptomatic or present as heart failure, arrhythmias, thromboembolic events and sudden death. [2][3][4]6 In the present report, the patient had ventricular arrhythmia and systolic heart failure as initial manifestations -found in more than 50% of the cases. [2][3][4]6 Ventricular arrhythmias were reported in 47% of adults.…”

“…[2][3][4]6 In the present report, the patient had ventricular arrhythmia and systolic heart failure as initial manifestations -found in more than 50% of the cases. [2][3][4]6 Ventricular arrhythmias were reported in 47% of adults. 2 Diastolic dysfunction occurs due to abnormal ventricular relaxation and restrictive filling caused by hypertrabeculation, whereas systolic dysfunction may result from subendocardial hypoperfusion and microcirculatory dysfunction.…”

“…2 This trabeculation process is of utmost importance, since it allows a greater surface-to-volume ratio and an increase in muscle mass before coronary arteries are established. 5,6 In the LV non-compacted myocardium, there is a persistence of the trabeculation and deep recesses, Case Report which communicate with the ventricular cavity due to non-compaction. 2 Without the compaction completion, myocardial dysfunction occurs secondary to the failure of the efficient rotational ventricular system to develop contractile performance.…”

“…2 Diastolic dysfunction occurs due to abnormal ventricular relaxation and restrictive filling caused by hypertrabeculation, whereas systolic dysfunction may result from subendocardial hypoperfusion and microcirculatory dysfunction. 2,6 Mechanical dyssynchrony between the compacted and non-compacted myocardium may cause global LV dysfunction. 2 Other rhythm abnormalities were also reported on the electrocardiogram, such as ST-segment depression and T-wave inversion, along with right-bundle branch block.…”

“…[1][2][3][4][5] It is currently classified as a primary genetic cardiomyopathy by the American Heart Association (AHA) and characterized as an unclassified cardiomyopathy by the World Health Organization (WHO) and the European Society of Cardiology (ESC). 1,4 The prevalence of isolated non-compacted myocardium in adults remains unclear, 1,3,4,6 although observational studies have found a prevalence of 0.01 to 0.26% in specialized services. 1,2 However, the estimated prevalence may be higher, as asymptomatic individuals rarely undergo imaging studies.…”