A 58-year-old woman was admitted to an outside hospital after an episode of syncope. She reported a 4-month history of progressively worsening dyspnea on exertion. There was no other significant medical history. Her blood pressure was 145/76 mm Hg, heart rate was 80 bpm, and oxygen saturation was 97% on room air. Cardiovascular examination revealed a grade III ejection systolic murmur, which could be heard loudest in the second intercostal space at the left sternal border. The ECG was notable for right ventricular hypertrophy. An echocardiogram revealed pulmonary stenosis with marked right ventricular hypertrophy and moderate tricuspid regurgitation. The patient was transferred to our institution for pulmonary valvuloplasty.
On right heart catheterization, a large filling defect was visualized in the right ventricular outflow tract (Figure 1 and Movie I), with a markedly elevated right ventricular pressure of 129/16 mm Hg and a significant pressure gradient across the right ventricular outflow tract (>100 mm Hg). Transthoracic echocardiogram with Doppler imaging confirmed the presence of a mass, which caused a significant obstruction to flow in the right ventricular outflow tract (Movie II). Cardiac magnetic resonance imaging with gadolinium contrast revealed a 4×2-cm sessile vascularized mass originating in the right ventricular outflow tract that involved the pulmonary valve and extended to the pulmonary artery bifurcation (Figure 2).
The mass was excised (Figure 3 and Movie III) and the tricuspid and pulmonary valves were replaced. Histopathology revealed spindle cell pathology characteristic of a leiomyosarcoma (Figure 4). The patient was discharged home on postoperative day 24 in good condition.
The online-only Data Supplement, consisting of movies, can be found at http://circ.ahajournals.org/cgi/content/full/115/4/e47 /DC1.