Right ventricular myxoma and pulmonary embolism in an adolescent with Carney complex

This case report is approved under Pro00101549 (approved January 3, 2019) with waiver of individual patient consent.A 14-year-old female patient (60.0 kg with body surface area 1.73) with a history of macroprolactinoma status after transsphenoidal resection, presented to the emergency department with 1 day of chest pain and shortness of breath.On arrival, computed tomography imaging demonstrated bilateral pulmonary embolism.A large right ventricular (RV) mass was also visible on computed tomography (Figure 1).The patient was anticoagulated with heparin and transferred to our institution for surgical evaluation.Upon arrival, she was hemodynamically stable and with normal oxygen saturations.Transthoracic echocardiography revealed a large RV mass involving the ventricular free wall, obstructing the RV outflow tract and abutting the pulmonary valve.Echocardiography further revealed mild tricuspid regurgitation with a peak gradient of 40 to 50 mm Hg and trace pulmonary valve regurgitation.RV and left ventricular function were grossly normal.Cardiac magnetic resonance imaging again demonstrated the large intracavitary RV mass measuring 6.2 3 2.9 3 3.2 cm with a stalk attaching to the tricuspid valve apparatus.The mass was heterogeneous in appearance on steady-state free precession, T1-, and T2-weighted sequences.There was no late gadolinium enhancement or fat saturation.Together, preoperative imaging suggested RV myxoma. OPERATIVE DETAILSThe patient was brought to the operating room for mass excision and pulmonary embolectomy.A median sternotomy was performed, systemic heparin was administered, and the ascending aorta, superior vena cava, and inferior vena cava were cannulated for bypass.Cardiopulmonary bypass was initiated, and the patient was cooled to 32 C.An aortic crossclamp was placed and antegrade cardioplegia was administered into the aortic root.An oblique right atriotomy was performed and a small atrial septal defect was created to serve as a left heart vent.The RV was visualized through the tricuspid valve, revealing a large, yellowred mass with smooth margins, attached to the ventricular free wall.Working through the tricuspid valve, the mass was mobilized and the stalk attaching to the RV free wall was excised (Figure 2).The mass was then delivered through the tricuspid valve en bloc.The base of the stalk was further resected until normal-appearing myocardium was reached, and then sent for frozen tissue sections.Tricuspid valve competency was confirmed.Surgical