Giant schwannoma of right ventricle: a complex resection guided by TEE.
Central Message.
Giant right ventricular cardiac schwannomas of the base of the heart pose major surgical challenges. Perioperative TEE can be a vital adjunct to guide precise intervention.
Cardiac schwannomas are among the rarest of primary neurogenic tumors.1 Giant cardiac schwannomas can present as a diagnostic and surgical challenge. This case report was exempt from institutional review board approval per institutional guidelines for retrospective single-patient reports. Informed written consent was obtained from the patient for this publication.
Case Report
A 30-year-old woman presented with a 3-month history of exertional dyspnea and chest pain. Imaging (contrast-enhanced computed tomography of the chest, cardiac magnetic resonance imaging, and whole-body fluorodeoxyglucose positron emission tomography-computed tomography scan) showed a large (18.9 × 13.8 × 12.7 cm) intrapericardial mass arising from the ventricular wall, engulfing the right coronary artery (Figure 1). An ultrasound-guided biopsy confirmed a benign, S100-positive schwannoma with a mitotic index of less than 5%.
Figure 1.
Preprocedure contrast-enhanced computed tomography of the chest in (A) axial and (B) coronal views demonstrated the mass (white arrows) with the RCA (right coronary artery) and its branches embedded and stretched out in the mass (yellow arrows) with pericardial effusion (white asterisk). C, Coronary angiography of the embedded and stretched-out RCA.
General anesthesia was induced with etomidate (0.2 mg/kg), fentanyl (5 μg/kg), and vecuronium (0.1 mg/kg). Intraoperative monitoring included 5-lead electrocardiogram, radial arterial line, quadruple-lumen central venous catheter, cerebral oximetry (near-infrared spectroscopy), and continuous 3-dimensional transesophageal echocardiography (3D-TEE). Pre-excision tumor capsule delineation using 3D-TEE with multiplanar reconstruction (MPR) identified the thin but intact capsular plane along the inferior right ventricular (RV) wall (Figure 2, A, Video 1).
Figure 2.
Intraoperative 3-dimensional TEE, MPR mode. A, RV-focused display demonstrating the thin adherent capsule (white arrowheads) along the inferoposterior basal RV wall and corroboration with intraoperative findings. B, LV-focused display demonstrating unchanged wall motion and chamber size after coronary clamp with corresponding intraoperative surgical image (blue arrow). C, Intraoperative 2-dimensional TEE showing intracardiac air confined to the right heart during beating-heat on-pump repair of the RV wall injury with corresponding intraoperative surgical image (yellow arrow). TEE, Transesophageal echocardiography; MPR, multiplanar reconstruction; RV, right ventricle; LV, left ventricle; TV, tricuspid valve; RVOT, right ventricular outflow tract; RCA, right coronary artery; LA, left atrium.
An on-pump beating-heart resection was performed via median sternotomy with a left sixth intercostal extension. The frozen section was deferred because the preoperative biopsy had confirmed benign pathology and the mass was macroscopically encapsulated. The posterior descending artery (PDA), which was engulfed by the tumor, was test-clamped; 3D-TEE with MPR provided real-time ischemia surveillance, confirming unchanged ventricular function and stable wall motion (Figure 2, B, Video 1). Saphenous vein bypass grafting, which was planned in the event of new-onset myocardial ischemia, was thus avoided.
Release of the tumor capsule from the inferior RV wall caused a defect in the free wall adjacent to the tricuspid valve. This was repaired using a 0.4-mm-thick expanded polytetrafluoroethylene patch (Bard). The patch was secured with interrupted sutures, followed by a 2-layer reinforcement of the overlying RV myocardium, using 4-0 polypropylene (PROLENE; Ethicon) sutures. The team used continuous carbon dioxide field flooding and Trendelenburg positioning during the repair. In the event of significant left-heart air entrainment, demonstrated by TEE identification of “echo-bright” bubbles (air) in the left atrium or left ventricle, the surgical team was prepared for immediate aortic crossclamping and active aortic root venting to isolate the systemic circulation and evacuate air (Figure 2, C, Video 1).
The 1329-g tumor was resected with R0 (microscopically negative) margins. Postbypass TEE and a 22-month follow-up (transthoracic echocardiogram/cardiac magnetic resonance) confirmed complete excision with preserved biventricular and tricuspid valve function (Figure 3, Video 1).
Figure 3.
A, Gross pathologic imaging of the intact tumor specimen. B, The microscopic finding showed a typical biphasic architecture pattern of Antoni A and B area. C, Follow-up 2-dimensional transesophageal echocardiography. D, Follow-up cardiac magnetic resonance imaging at 10 months. TV, Tricuspid valve; RVOT, right ventricular outflow tract; RA, right atrium; LA, left atrium; RV, right ventricle; LV, left ventricle.
Discussion
Primary cardiac tumors are exceedingly rare (incidence: 0.0017%-0.19%), with schwannomas representing <2% of cases.1 We report a unique 1.3-kg benign, S100-positive schwannoma localized to the basal inferior RV wall. This anatomical site is reported in only 11.1% (3/27) of previous cases.2 Most presentations are sporadic (90%) and arise from the atrium (66%).2,3 Genetic screening was deferred in our patient because she did not exhibit clinical stigmata of familial neurofibromatosis or schwannomatosis.4 Complete resection with R0 margins is key to favorable outcomes, because there have been reports of late recurrence in the cellular variant.3,4
Alternative cardioplegic arrest with planned coronary bypass was deferred; active coronary perfusion allowed safe microdissection of embedded vessels, avoiding injury. Backup plans for potential RV failure after wall resection included immediate conversion to cardioplegic arrest for larger patch reconstruction, a volume-unloading bidirectional Glenn shunt, or temporary venoarterial extracorporeal membrane oxygenation mechanical circulatory support.
Unlike routine myxoma resections performed under cardioplegic arrest, this mass was excised using a pump-supported beating-heart technique. This approach preserved coronary perfusion and avoided cardioplegic arrest in a young, structurally normal heart. To mitigate risks, the team used continuous carbon dioxide field flooding and Trendelenburg positioning. Immediate aortic crossclamping and venting were prepared as contingencies for potential left-heart air entrainment. The application of TEE in this case significantly diverged from its routine practice as a standard monitor in the resection of common cardiac masses, such as atrial myxomas. Standard myxoma resections are typically performed under cardioplegic arrest, where TEE serves a primarily episodic role—confirming the diagnosis prebypass and assessing valvular competence postbypass. In contrast, the resection of this giant ventricular schwannoma required a continuous, real-time navigational approach. Live 3D MPR mode enhanced the detection of potential PDA-territory ischemia by providing simultaneous, real-time visualization of the inferior wall and posterior septum in multiple orthogonal planes, offering a level of early diagnostic precision that far exceeds the capabilities of traditional 12-lead electrocardiogram or conventional 2-dimensional TEE monitoring.5
3D TEE with MPR transitioned from a monitoring adjunct to a live surgical guide through: (1) diagnostic refinement and surgical road-mapping by identifying the thin, intact plane along the inferior RV wall not visible to the surgeon; (2) ischemia surveillance by providing real-time monitoring of regional wall motion abnormalities during test clamping of the tumor-embedded PDA, ultimately avoiding bypass grafting; (3) air embolism prevention by differentiating right-sided from left-sided intracardiac air during the repair of a surgical RV wall rent, guiding carbon dioxide flooding and venting strategies; and (4) functional verification by facilitating immediate postexcision verification of biventricular function, tricuspid valve competence, and completeness of resection.
Conclusions
Real-time imaging with perioperative TEE can be a vital current adjunct to multimodality imaging in transforming complex cardiac tumor surgery into a precise and successful intervention with good outcomes.
Declaration of Generative AI and AI-Assisted Technologies in the Writing Process
During the preparation of this work, the authors used Gemini 3.1 Pro (Google LLC) in order to organize the point-by-point response to reviewers and to help with language polishing, structural reorganization, and clarity refinement. After using this tool, the authors reviewed and edited the content as needed and take full responsibility for the content of the publication.
Conflict of Interest Statement
The authors reported no conflicts of interest.
The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.
Footnotes
Drs Nair and Pillai are co-first authors and contributed equally to this article.
Supplementary Data
Key intraoperative TEE-guided surgical steps in cardiac schwannoma excision. Video available at: https://www.jtcvs.org/article/S2666-2507(26)00315-9/fulltext.
References
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Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Key intraoperative TEE-guided surgical steps in cardiac schwannoma excision. Video available at: https://www.jtcvs.org/article/S2666-2507(26)00315-9/fulltext.