Key result
Isolated LV apical hypoplasia can cause RVOT obstruction from exaggerated rightward septal bulging.
Why the study?
Isolated left ventricular apical hypoplasia is a rare and recently recognized congenital cardiac anomaly with limited clinical characterization, especially when combined with right ventricular outflow tract obstruction.
Case Report (n=1)
May prompt RVOT evaluation in LV apical hypoplasia; leaves open prevalence and clinical impact.
Isolated left ventricular (LV) apical hypoplasia is a unusual and recently recognized congenital cardiac anomaly. A 19-year-old man was found to have an abnormal ECG and cardiac murmur identified during a routine health check since joining work. His ECG revealed normal sinus rhythm, right-axis deviation, poor R wave progression, and T wave abnormalities. On physical examination, a 2/6～3/6 systolic murmur was heard at the second intercostal space along the left sternal border. Subsequent echocardiography and cardiac magnetic resonance imaging confirmed the LV apical hypoplasia. Of note, we first found that LV apical hypoplasia was accompanied by RV outflow tract obstruction due to exaggerated rightward bulging of the basal-anterior septum during systole. A close follow-up was performed for the development of heart failure, pulmonary hypertension, and potentially tachyarrhythmia.
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Zhao et al. (2015) conducted a case report in Isolated left ventricular apical hypoplasia with right ventricular outflow tract obstruction (n=1). Diagnostic imaging revealed that isolated left ventricular apical hypoplasia can be accompanied by right ventricular outflow tract obstruction due to exaggerated rightward bulging of the basal-anterior septum.
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