Key result
Isolated LV apical hypoplasia carries substantial morbidity, with ~70% of cases developing overt symptoms.
Why the study?
Isolated left ventricular apical hypoplasia is a recently described congenital abnormality with distinctive morphological features that require further characterization.
Systematic Review (n=59)
May warrant surveillance for heart failure and arrhythmias in isolated left ventricular apical hypoplasia; leaves open need for prospective cohorts to define prognosis.
Isolated left ventricular (LV) apical hypoplasia is a recently described congenital abnormality characterized by: (1) a truncated and spherical LV configuration with rightward bulging of the interventricular septum, (2) deficiency of the myocardium within the LV apex with adipose tissue infiltrating the apex, (3) origin of the papillary muscle in the flattened anterior apex, and (4) elongation of the right ventricle wrapping around the deficient LV apex. In this report, we demonstrate these characteristic features with cardiac magnetic resonance imaging and summarize the existing information on isolated LV apical hypoplasia.
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Vanhecke et al. (2011) conducted a systematic review in Isolated Left Ventricular Apical Hypoplasia (n=59). Isolated Left Ventricular Apical Hypoplasia was evaluated on Development of overt clinical symptoms (heart failure or arrhythmogenic events). Isolated Left Ventricular Apical Hypoplasia was associated with substantial long-term morbidity, with 69.5% of reported cases developing overt clinical symptoms driven by heart failure or arrhythmogenic events.
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