Case Presentation
A 32-year-old woman presented to the emergency room with dyspnea, palpitations and syncope that had arisen approximately in the previous 24 hours. The patient was hypotensive and with progressive worsening orthopnoea. She underwent a transthoracic echocardiogram that showed a massive mobile intracardiac mass of 6 x 6 cm, fluctuating within the left atrium and determining nearly complete mitral valve occlusion, with severe mitral stenosis (Gmed 11mmHg). Fig. 1a and 1b . Associated findings were moderate-to-severe mitral insufficiency (MR),Fig. 1c , with moderate left-ventricular dysfunction, ejection fraction (EF) 40%. Furthermore, right ventricular impairment was present, with severe tricuspid regurgitation (TR) and severe pulmonary hypertension (systolic pulmonary artery pressure (sPAP) 60mmHg).
Due to initial hemodynamic impairment requiring inotropic support and worsening respiratory conditions with the need of high-flow oxygen, the patient underwent emergent surgery. Upon chest opening there was evidence of severe biventricular dilation and dysfunction, prevalently involving the right chambers, with severe right atrial distension (central venous pressure (CVP) 25mmHg). A bi-atrial approach was used, in order to better identify the implant of the pedicle. The root of the pedicle, together with the full thickness of approximately 1cm2 of adjacent interatrial septum were resected. The mass was then completely removed; dimensions were 17 x 7 cm, a solid component together with thrombotic and gelatinous aspects, Fig. 2 . The resulting atrial septal defect was closed with a direct suture. Tricuspid valve repair with Kay technique was performed. Evidence at transesophageal echocardiogram (TEE) of residual severe MR due to multiple jets was seen (Fig. 3a) , requiring a second cardiopulmonary bypass run to perform mitral annuloplasty with a complete semirigid ring (St Jude Medical Saddle n30), Fig. 3b . Inotropic and mechanical circulatory support were necessary for severe biventricular dysfunction. Immediately following intervention, CVP decreased to 5 mmHg and pulmonary hypertension disappeared. At histologic examination, diagnosis of myxoma was confirmed.
The patient had a regular postoperative course, with recovery of good biventricular function and no pulmonary hypertension. She was discharged home on post-operative day 8.
At follow-up after 3 months the patient was asymptomatic, with good functional class (NYHA I), and could undertake all normal daily activities. A transthoracic echocardiogram showed complete excision of the atrial myxoma, with a good result of both mitral and tricuspid valve repair.