Stephens, Elizabeth H

Solitary myofibromas are rare, benign, mesenchymal tumors typically found in the head and neck regions. We report an exceptionally rare case of a solitary cardiac myofibroma and its short-term management and outcome. A two-month-old infant was referred due to a murmur. Echocardiography revealed a 2 × 1 cm solitary mass originating from a mitral papillary muscle, causing severe regurgitation. Magnetic resonance imaging showed the mass to be homogeneous on T1, hyperintense on T2, and hypointense on first-pass perfusion imaging with no enhancement on delayed postcontrast imaging. The mass was surgically excised partially due to its involvement with the mitral valve. Postoperative recovery was uneventful, with echocardiography four months later showing no tumor recurrence and trivial mitral regurgitation. This report provides valuable imaging and pathology examples to aid in the recognition of this rare tumor and demonstrates that successful surgical excision is possible, even with significant cardiac involvement.

Background: Optimal management of cardiac implantable electronic devices (CIEDs) in patients with Ebstein anomaly during tricuspid valve (TV) surgery is unknown. Thus, we aimed to characterize CIED management/outcomes in patients with Ebstein anomaly undergoing TV surgery. Methods: Patients at the Mayo Clinic from 1987 to 2020 with Ebstein anomaly and CIED procedure were reviewed for procedural details, complications, echocardiogram, and lead parameters. Five-year cumulative incidence of CIED complications were estimated using the Kaplan-Meier method. Results: Ninety-three patients were included; 51 were female, and mean age was 40.7±17.5 years. A new CIED was implanted in 45 patients at the time of TV surgery with the majority receiving an epicardial (n=37) CIED. Among 34 patients who had preexisting CIED (11 epicardial, 23 transvenous) at time of TV surgery, 20 had a transvenous right ventricular lead managed by externalizing the lead to the TV (n=15) or extracting the transvenous lead with epicardial lead implantation (n=5). Fourteen patients underwent CIED implantation (4 epicardial, 10 transvenous) without concurrent surgery. Placement of lead across the TV was avoided in 85% of patients. The 5-year cumulative incidence of CIED complications was 24% with no significant difference between epicardial and transvenous CIEDs (26% versus 23%, P =0.96). Performance of lead parameters was similar in epicardial and transvenous leads during median (interquartile range) follow-up of 44.5 (61.1) months. Conclusions: In patients with Ebstein anomaly undergoing TV surgery, the use of epicardial leads and externalization of transvenous leads to the TV can avoid lead placement across the valve leaflets. Lead performance and CIED complications was similar between epicardial and transvenous CIEDs.