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.