Abstract

An 8-month-old male reported for cardiac evaluation for a heart murmur. On physical examination, he was non-dysmorphic, pulse oximetry was 98%, and cardiac examination revealed strong upper extremity pulses, impalpable lower extremity pulses, normal S1 and S2, no clicks, a grade 2/6 low pitch, and a vibratory ejection systolic murmur best heard at the lower left sternal border (deemed Stillā€™s murmur). His electrocardiogram demonstrated biventricular hypertrophy. His transthoracic echocardiogram (TTE) demonstrated discrete coarctation of the aorta (COA). Suprasternal imaging showed discrete COA (vertical arrow) formed by the subclavian shelf and dilatated descending thoracic aorta (DA), and a horizontal arrow denotes the left subclavian artery (Panel A). Colour flow mapping demonstrated turbulence across the COA (horizontal arrow) (Panel B, Supplementary data online, Video). Continuous Doppler interrogation demonstrated a peak systolic gradient of 67 mmHg (Panel C) with a typical sawtooth spectral pattern indicating severe COA (vertical arrow shows flow extending into diastole). He underwent elective surgery with resection of the coarctated segment and end-to-end anastomosis of the DA. The excised segment demonstrated a pinhole lumen (Panel D). He continues to do well at follow-up.

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