Abstract
Background
The left atrial myxoma is the most common type in clinical practice, whereas biatrial myxomas are extremely rare (less than 3%). Over the past five decades, approximately 50 cases of biatrial myxoma have been reported in the literature. Here, we report a rare, large, asymptomatic biatrial myxoma, involving the inferior vena cava (IVC) and part of the posterior wall of the left atrium (LAPW).
Case presentation
In this case, a 75-year-old elderly woman was incidentally found to have a biatrial myxoma with atrial flutter after chest trauma. Further evaluation with cardiac computed tomography angiography (CTA) was performed on patient for delineating the tumor’s size, morphology, attachment site, extension, internal enhancement characteristics, and to rule out concomitant coronary artery disease. Intraoperative findings showed the tumor extended into the IVC and the LAPW. Finally, the patient underwent surgical resection of the tumor under cardiopulmonary bypass (CPB), along with excision of the left atrial appendage, and we reconstructed LAPW and IVC. The patient recovered uneventfully and was discharged on postoperative day 10. At a 5-month follow-up, no recurrence of atrial myxoma was observed.
Conclusions
Biatrial myxoma is extremely rare and sometimes can be challenging to diagnose. Currently, there are no guidelines or consensus statements regarding the best diagnostic methods. Based on our case, we suggest that computed tomography (CT) and cardiac CTA can provide sufficient details about diagnostic value in cases where transthoracic echocardiography (TTE) fails to provide a clear diagnosis. Surgery is mandatory once cardiac myxoma is diagnosed. In cases of giant cardiac myxomas involving the IVC and the LA, careful preoperative planning is required, including venous cannulation for CPB and reconstruction of cardiac structures after complete tumor excision.
Keywords: Heart tumor, Cardiac benign tumor, cardiac myxoma, biatrial myxoma, cardiac CTA, cardiac surgery
The incidence of primary cardiac tumors is very low, with autopsy studies reporting a prevalence of 0.17%–0.19% [1, 2]. Among primary benign cardiac tumors, approximately 50% are cardiac myxomas [3]. Cardiac myxomas presents with a variety of clinical signs, like obstructive symptoms (67%) or embolic events (29%), etc [4]. Sometimes, TEE and magnetic resonance imaging (MRI) can be used for diagnosing cardiac myxomas, but these examinations are relatively complicated and time-consuming [5]. We describe a 75-year-old female with a huge biatrial myxoma, which was discovered incidentally during a chest CT scan. We introduced cardiac CTA for supplementary diagnosis.
Case presentation
A 75-year-old elderly woman underwent a chest CT scan following chest trauma, which revealed a space-occupying lesion in the atrium. TTE showed the mass had uneven internal echoes and was approximately 66.3 mm × 52.3 mm which located on the midportion of the atrial spectrum, protruding into both atria (Fig. 1). The temperature was normal on admission, the pulse was 80/min, the respiration rate was 16/min and the blood pressure was 128/68 mmHg. No obvious abnormalities were found in laboratory examinations. Electrocardiogram demonstrated the presence of atrial flutter. No obvious coronary artery stenosis was found in the cardiac CTA. According to CT images, the tumor occupied most of the atrium and was low-attenuation (approximately 0–40 HU) with well-demarcated and calcifications. Cardiac CTA revealed no significant enhancement. The tumor appeared circumscribed with clear edge and extended to the orifice of the IVC (Fig. 2). Based on these findings, we highly suspected it was a cardiac myxoma.
Fig. 1.
Echocardiogram showed the location and spatial relationship of the tumor to the mitral and tricuspid valves. Red star: the tumor. LA: left atrium; RA: right atrium; LV: left ventricle; RV: right ventricle; MV: mitral valve; TV: tricuspid valve
Fig. 2.
Images of tumor in CT and CTA. (A) CT showed a giant mass (triangle) occupying the atrium, The mass contained calcification (arrow). (B and C) Cardiac CTA showed the tumor protruded into the left atrium (a) and right atrium (b) and had clear edge. Red arrow: inferior vena cava
Considering the patient’s age and the presence of atrial flutter, we performed simultaneous resection of the tumor and left atrial appendage (LAA) during surgery. A median sternotomy and pericardial incision were performed after general anesthesia. Femoral vein cannulation was adopted instead of IVC cannulation due to the excessively large size of the tumor. CPB was established via cannulation of the femoral vein, superior vena cava (SVC), and ascending aorta (AO), followed by administration of cardioplegic solution through the aortic root. After cardiac arrest, the right atrium (RA) was opened. A grayish-white, firm, encapsulated mass was found occupying the RA (Fig. 3). The tumor originated from the fossa ovalis near the orifice of the coronary sinus (CS), extending inferiorly along the fossa ovalis.
Fig. 3.
(A and B) Images showed the anatomical location of the tumor within the right atrium after intraoperative incision of the right atrium. (C) displayed the structure of the tumor. Ao: aorta; SVC: superior vena cava; IVC: inferior vena cava; CS: coronary sinus; TV: tricuspid valve
involving part of the LAPW and the posterior wall of the IVC. The tumor, which was measured approximately 8 × 6 × 4 cm (Fig. 4), was carefully excised from interatrial septum to a portion of the LAPW and IVC. Subsequently, the LAA was sutured closed, and the reconstruction of IVC, LA was performed using a bovine pericardial patch (Fig. 5).
Fig. 4.
The actual size of the tumor after resection from the atrial septum (A and B)
Fig. 5.
Patches in the atrium: a: patches of the posterior wall of the left atrium and the posterior wall of the inferior vena cava; b: atrial septal patch
The procedure was performed smoothly, and postoperative recovery was satisfactory. Histopathological examination confirmed the diagnosis of cardiac myxoma (Fig. 6). The patient was discharged in good condition on postoperative day 10 and continued to do well at 5-month follow-up. Follow-up echocardiography showed no evidence of recurrence.
Fig. 6.
The tumor contained numerous proliferated small blood vessels and was composed of stellate and spindle-shaped myxoma cells and a small number of lymphocytes and plasma cells
Discussion
Biatrial or multiple cardiac myxomas are more commonly seen in patients with Carney syndrome, which is characterized by familial inheritance [6]. Patients are sometimes much younger and are more likely to recurrence after surgery [7, 8]. In our case, the patient was 75 years old and had no family history of cardiac myxoma, making the possibility of Carney syndrome lower.
Patients with larger atrial myxomas (> 5 cm) usually present with clinic symptoms: a triad of embolization, intracardiac obstruction and constitutional signs [9, 10]. Echocardiography was diagnostic in more than 95% of cases and continues to be the diagnostic procedure of choice [9]. In cases that TTE failed to establish a clear diagnosis, additional imaging modalities such as transesophageal echocardiography (TEE), CT, or MRI are often required [11]. TTE, CT, and MRI constitute complementary modalities that, when integrated, offer invaluable insights for cardiac imaging [12]. Nevertheless, TEE is an invasive procedure, CT scanning cannot achieve true real-time imaging, and the imaging planes are relatively fixed, high-quality MR imaging requires a relatively stable cardiac rhythm [13]. In our case, we preferred to prioritize cardiac CTA for further diagnosis, especially for elderly people. Cardiac CTA can provide both diagnostic information of the tumor, clarify the condition of coronary artery and more friendly to patients. After obtaining the diagnosis, biatrial myxomas should be surgically resected after careful preoperative planning. The intracardiac defect following myxoma resection can be reconstructed using patches.
Conclusion
Biatrial myxoma is extremely rare in clinical practice, and giant biatrial myxoma involving the IVC and the LAPW is even rarer. Meanwhile, the diagnosis of cardiac myxoma can be challenging in certain cases. For patients in whom TTE is inconclusive, we suggest that preoperative CT and cardiac CTA may facilitate diagnosis and are relatively easy to perform. Although we have achieved favorable outcomes in the only case of biatrial myxoma we have encountered so far, our experience should be interpreted with caution due to the extremely small sample size and the inherent limitations of CT imaging.
Acknowledgements
Not applicable.
Abbreviations
- CTA
computed tomography angiography
- LAPW
posterior wall of the left atrium
- IVC
inferior vena cava
- LA
left atrium
- CPB
cardiopulmonary bypass
- TEE
transesophageal echocardiography
- MRI
magnetic resonance imaging
- TTE
transthoracic echocardiography
- CT
computed tomography
- SVC
superior vena cava
- AO
ascending aorta
- RA
right atrium
- CS
coronary sinus
- LAA
left atrial appendage
Author contributions
Y. L. designed the study, collected the date and wrote the article, Y. F., J. C. and J. G. performed the operation, and revised the manuscript critically. D. W. collected figures. All authors read and approved the final manuscript.
Funding
No funding sources.
Data availability
No datasets were generated or analysed during the current study.
Declarations
Ethics approval and consent to participate
Not applicable.
Consent for publication
Not applicable.
Competing interests
The authors declare no competing interests.
Footnotes
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Jing Cao, Dechao Wang and Jianjun Ge contributed equally to this work.
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Data Availability Statement
No datasets were generated or analysed during the current study.