The maverick heart sound

摘要

The phonocardiogram and online supplementary audio clip (Figure 1) reveal an early systolic ejection click (EC) with a grade Ⅲ/Ⅵ systolic murmur loudest at the left upper sternal border. The EC decreases with inspiration (Figure 2A; asterisk) consistent with valvular pulmonic stenosis (PS). Transthoracic echocardiogram showed systolic doming of the pulmonary valve (Figure 2B, arrow) and mild PS (peak systolic gradient 19 mm Hg). Cardiac MRI revealed severe pulmonary artery dilatation (60 mm) (Figure 2C).