A case of subvalvular pulmonary stenosis differentiated from a double-chambered right ventricle by transesophageal echocardiography: importance of detecting the pulmonary valve

Excerpt

A 57-year-old woman presented with shortness of breath and leg edema. A grade 2/6 systolic ejection murmur was auscultated at the second left sternal border. Transthoracic echocardiography showed a left ventricular ejection fraction of 65%. The parasternal short-axis view showed regional wall thickening around the right ventricular outflow tract (RVOT), indicating a double-chambered right ventricle (DCRV) (Fig. 1a). Cardiac magnetic resonance imaging (MRI) indicated RVOT stenosis (Fig. 1b). The apical three-chamber echocardiographic view revealed a doubly committed subarterial ventricular septal defect (VSD) (Fig. 1c). Transesophageal echocardiography (TEE) detected the pulmonary valve (PV) and revealed subvalvular pulmonary stenosis (SubPS), rather than DCRV (Fig. 1d, e). Color Doppler imaging showed systolic VSD jet and mosaic flow in the SubPS and detected moderate aortic regurgitation due to slight right coronary cusp prolapse. Right heart catheterization demonstrated a pressure gradient of 60 mmHg in the SubPS. Right ventricular abnormal fibromuscular tissue resection, VSD patch closure, and aortic valve replacement were performed. Her postoperative course was uneventful.

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