Key result
Isolated LV apical hypoplasia features a spherical LV, fat replacement, and variable heart failure presentation.
Why the study?
Isolated left ventricular apical hypoplasia is a rare inborn cardiomyopathy with limited clinical case descriptions in the literature.
Isolated left ventricular apical hypoplasia is a rare inborn cardiomyopathy with distinct morphological features that can lead to biventricular heart failure and arrhythmias.
Alerts clinicians to rare LVAH in unexplained LV dysfunction; review of 42 cases leaves natural history and management open.
The review of data of literature concerning the description of clinical cases of isolated left ventricular apical hypoplasia (LVAH) in 42 patients are presented. left ventricular apical hypoplasia is characterized by 1) a spherical left ventricular with a cut-off tip and a swelling of the interventricular septum to the right in combination with systolic dysfunction of left ventricular; 2) replacement of the myocardium of the upper limb with fat tissue; 3) localization of papillary muscles on the flattened apex of the left ventricular; 4) elongated right ventricle (RV), flowing around the top of the upper left vertex. The course of this condition may be asymptomatic, or manifest as a clinic of biventricular heart failure with high pulmonary hypertension and heart rhythm disturbances.
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Скидан et al. (2018) conducted a review in Isolated left ventricular apical hypoplasia (n=42). Isolated left ventricular apical hypoplasia was evaluated. Isolated left ventricular apical hypoplasia in 42 reviewed cases was characterized by a spherical left ventricle, fat tissue replacement, and variable presentation from asymptomatic to heart failure.
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