The use of a preoperative 3D-visualized model of a right atrial hemangioma and coronary-bronchial-pulmonary fistula: a case description
Introduction
Cardiac hemangioma is an uncommon benign tumor (accounting for 5% of all benign cardiac tumors) (1,2). It is most commonly located in the epicardium but has also been reported in the myocardium and endocardium (1,2). Right atrial (RA) hemangiomas are extremely rare.
Typically, hemangiomas present with nonspecific symptoms, and are easily misdiagnosed as cardiac myxoma prior to surgical intervention. Notably, patients with hemangiomas may also have other heart conditions, such as valve or congenital blood vessel disease. Congenital cardiovascular disease and cardiac hemangiomas present with a range of associated symptoms (e.g., chest tightness, chest pain, palpitation, and even no discomfort).
In this article, we report a very rare case of a patient with both a large primary cavernous RA hemangioma and a complex congenital coronary-bronchial-pulmonary fistula (CBPF), and describe the radiological features of both. This case highlights the important role of noninvasive three-dimensional (3D)-visualized model evaluation, based on cardiac computed tomography (CCT), in guiding clinical diagnosis and assessment, and individualized treatment planning preoperatively.
Case presentation
A 46-year-old woman was admitted to Liuzhou People’s Hospital Affiliated to Guangxi Medical University due to chest tightness and chest pain, persisting for 6 days. No obvious association between the chest tightness and movement was observed. The physical examination findings included normal blood pressure, a lack of jugular venous distention, and normal carotid and peripheral pulses. No additional heart sounds were detected during auscultation. Further, the patient had no family history of heart disease.
The electrocardiogram showed a normal sinus rhythm with an abnormal T wave. Common causes of abnormal T waves include arrhythmia, myocardial ischemia, myocardial hypertrophy, and heart valve abnormalities. However, the patient’s cardiac biomarkers were basically normal. Therefore, further examination by echocardiography was necessary.
During her hospitalization, transthoracic echocardiography (Philips IE33), with a 2.5-MHz probe, revealed a large, pedunculated mass (46 mm × 67 mm in size) with uneven echogenicity, attached to the head wall inside the RA (as indicated by the black arrow in Figure 1A). The mass exhibited areas of echo-brightness and uneven visibility. Doppler imaging showed that the mass reached the tricuspid valve ring during diastole with hemodynamic obstruction, with high-velocity peak flow (1.0 cm/s), resulting in increased blood flow to the tricuspid valve hole. The systolic mass then moved back to the right atrium. It was also closely related to the orifice of the inferior vena cava (Figure 1B,1C).
The patient subsequently underwent imaging using the Aquilion ONE ViSSION 640-slice computed tomography (CT) scanner. The scanning parameters were as follows: tube voltage: 100 kVp; intelligent tube current technology enabled. The Ulrich High-pressure Injector was used to automatically inject 0.8 mL/kg of ioversol (Hengrui Medicine) at a rate of approximately 4.0 mL/s. The BolusTracking intelligent triggering method was employed, with the monitoring level set at the descending aorta at the center of the heart, which was designated as the region of interest. When the trigger threshold (300 Hounsfield units) was reached, the arterial phase of the enhanced cardiac scan was triggered and completed. The venous phase and delayed phase of the enhanced scan were imaged 30 and 120 seconds after the arterial phase, respectively.
The CCT examination revealed well-defined, multiple calcified masses with progressive enhancement post-contrast, where the contrast agent remained incompletely filled even at the late phase (Figure 2A-2D). CCT confirmed that the inferior mass locally blocked the orifice of the vena cava, but no invasion of the wall of the inferior vena cava was observed. Unexpectedly, CCT revealed a disorganized, dilated vascular plexus (approximately 2 mm in lumen diameter) on the pulmonary trunk (PT), anastomosed with the left bronchial artery (LBA) and the right coronary artery (RCA) (as indicated by the yellow arrows in Figure 2E-2H), resulting in a complex vascular fistula, which was diagnosed as CBPF. A 3D-visualized model was then created from the raw CT data using the 3D Slicer software (https://www.slicer.org/) (Figure 2I).
The model facilitated surgical planning by providing detailed visualization of the patient‑specific anatomy, thereby improving our understanding of the treatment options. The patient subsequently underwent surgery. During the operation, the right atrium was incised, and a mass was observed, with a maximum diameter of approximately 80 mm. The surface was dark red, the texture was medium, and the tumor capsule was intact (Figure 3A). The pedicle was located at the RA wall at the orifice of the inferior vena cava. The tumor pedicle was located on the superior aspect of the atrial roof near the orifice of the inferior vena cava.
Consistent with the preoperative CCT imaging findings, the tumor had not grown into the inferior vena cava ostium or invaded the inferior vena cava wall. The RA mass and atrial wall were successfully removed and sent for pathological examination. Additionally, a tortuous and disordered vessel with a diameter of approximately 2 mm was observed on the PT. Combined with the preoperative imaging, it was identified as the path of the CBPF. During exploration, the abnormal arterial fistula was sutured intermittently using a sliding suture with a pad. The tricuspid annulus was slightly enlarged, and water injection revealed a small amount of regurgitation of the tricuspid valve. The circulation was opened, the heart restarted spontaneously, and the RA incision carefully sutured. Intraoperative esophageal ultrasound showed massive tricuspid regurgitation, and the surgeon decided to perform tricuspid valvuloplasty under cardiopulmonary bypass on a beating heart. Reexamination by transesophageal echocardiography showed no significant tricuspid regurgitation, confirming that the surgery had been successfully completed.
The surgery lasted for 5 hours, with an estimated blood loss of approximately 300 mL. The diagnosis of cardiac cavernous hemangioma was confirmed postoperatively (Figure 3B), and the CBPF was confirmed and ligated. The patient’s vital signs were closely monitored. The patient recovered well and was discharged smoothly. The patient was reexamined by ultrasound regularly within 1 year of the operation, and the ultrasound results showed that the cardiac structure and function continued to recover well.
All procedures performed in this study were in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Research Ethics Committee of Liuzhou People’s Hospital [approval No. 2025 (KY-E-03)]. Verbal informed consent was obtained from the patient by telephone for the publication of this article and accompanying images.
Discussion
The incidence of primary cardiac tumors is rare, with autopsy series estimating a prevalence of approximately 0.001–0.03% (2,3). Among these, cardiac hemangiomas are benign vascular tumors that account for about 5% of all benign cardiac tumors (1,2). They are often discovered incidentally (3). Histologically, hemangiomas are characterized by vascular endothelial cell proliferation, and can be divided into three types: capillary, cavernous, and arteriovenous (2). The clinical manifestations of hemangiomas vary according to the size and location of the tumors. Symptoms may arise when hemodynamics are affected. Patients may present with one or more symptom, including chest distress and shortness of breath, outflow tract obstruction, congestive heart failure, and even sudden death (4).
In the present case, the patient presented only with chest tightness and chest pain. It is well known that the pressure of the coronary artery is much greater than that of the pulmonary artery, and a side coronary artery fistula connected to the PT causes blood to shunt from left to right. In addition, the RCA supplies blood to the right heart, while the blood supply of the RA tumor mainly depends on the coronary arteries, which might have caused coronary steal syndrome. This could also explain the patient’s symptoms of chest tightness and pain. To identify the cause of the patient’s symptoms of chest tightness and chest pain, the patient underwent transthoracic echocardiography.
Echocardiography has become the most appropriate screening and diagnostic modality (5), as it provides the best information in terms of the size, mobility, and location of cardiac masses. In this case, the cardiac hemangioma was hyperechoic on echocardiography with a pedunculated structure connected to the atrial roof, similar to previous reports (2).
In the CT images of cardiac vascular tumors (especially cavernous vascular tumors), multiple calcifications can be observed. Consistent with previous reports (6), the patient’s contrast-enhanced CCT images showed patchy continuous enhancement in the tumor, and delayed enhancement to the same degree as the blood pool. During the delayed phase, most of the tumor body still showed no contrast agent filling, which might be related to the large size of the tumor, the slow blood flow within the vascular tumor, or the coronary artery blood supply. Sometimes, cardiac hemangiomas need to be distinguished from myxoma in the case of calcification. Cardiac myxomas are cystic-solid in nature. In plain scans, the cystic parts of cardiac myxomas usually display lower density, while the solid parts of angiomas usually display equal density.
Due to the variety of clinical manifestations, there is currently no unified standard for the treatment of cardiac hemangiomas. For young patients with clinically symptomatic or asymptomatic atrial hemangiomas, surgical resection is feasible if there are no obvious contraindications to surgery (6).
Notably, due to the possible co-existence of other cardiovascular diseases, the patient underwent further CCT. CCT revealed an unexpected CBPF, and the images revealed a direct connection of the distal end of the RCA fistula to the main pulmonary artery, but no aneurysmal dilatation was observed. Thus, preoperative CCT examination is essential for thorough preoperative assessment. Cardiac ultrasound can only observe cardiac tumors through a limited number of slices, and its diagnosis often relies on the operator’s experience. When assessing the relationship between the tumor and the surrounding structures, it is prone to interference. Conversely, CCT adopts electrocardiogram gating technology, which has the advantages of a fast acquisition speed, a high spatial resolution, and multi-planar imaging. Through 3D reconstruction technology, it can show the details of RA tumors from different directions, such as the shape, edge, internal calcification morphology, enhancement pattern, and blood supply, as well as the relationship between the RA tumor and RA myocardium, pericardium, coronary arteries, inferior vena cava, and the right lung, and whether the tumor invades the inferior vena cava and has a clear boundary with the right lung. Thus, CCT addresses the limitations of ultrasound.
However, due to the small volume and deep location of fistulas, cardiac ultrasound examination still has difficulties in diagnosing coronary artery fistula, coronary artery-pulmonary artery fistula, CBPF, etc. And it cannot clearly display coronary artery fistulas (4,7), which may be found later as occurred in this case. In the present case, CCT accurately displayed the origin, path and shape of the coronary artery fistula, as well as its anatomical relationship with the surrounding structures. CCT uses maximum density projection, 3D imaging, and multi-directional tracking techniques to repeatedly and carefully track fistulas, find the best surgical suture closure position, measure fistulas, and select the matching suture size in advance. 3D models can help design personalized surgical approaches (8,9). We found the best surgical path through the 3D-visualized model. The model also assists doctors to communicate effectively with patients before surgery, and improves the success rates of surgeries.
The Aquilion ONE ViSION 640 CT scanner has a high number of rows, a fast scan speed, and a 16-cm-wide body detector, enabling imaging in a single cardiac cycle without the need to move the bed board. Even in the presence of complex heart rhythms, such as arrhythmia and atrial fibrillation, the original imaging data of the heart, aorta and coronary artery can be clearly captured in one step with a lower radiation dose, significantly reducing the radiation exposure and discomfort of patients, and the examination time, and improving work efficiency while ensuring image quality.
Some traditional commercial 3D imaging software is expensive, which increases medical costs, and is only limited to a specific system environment. The interface steps are complex, and the reconstruction speed is relatively slow. The reconstruction process often requires more time and manpower for image reconstruction and analysis, and the display of complex and fine tubular structures is not clear. Conversely, 3D Slicer, a free and open-source software, is user-friendly and easy to learn. It can run on a variety of operating systems; thus, users can operate it according to their own system environment. The 3D-visualized models generated using 3D Slicer can realize real-time interactions and visualizations. After importing the original data, the blood vessels and organs around a mass and coronary artery fistula can be visualized and reconstructed. Moreover, it has the advantages of an obvious color difference, high image resolution volume, and high contrast ratio, as well as a better stereoscopic visual effect.
The spatial distribution relationship between the right heart mass and CBPF was presented. We used the free open-source software 3D Slicer to visualize and reconstruct the blood vessels and organs around the mass and coronary artery fistula, and present the spatial relationship between the right heart mass and CBPF through obvious color differences. The surgeons simulated the operation process through a 360-degree rotation model using the transverse midline approach, and through repeated discussions and rehearsals before the surgery, found the best position for the RA incision, and clearly identified the location of the fistula suture, avoiding damage to major blood vessels. In this case, due to the accidental discovery of the coronary artery fistula by CCT, although the initial surgical approach remained unchanged, the closure surgery of the coronary artery fistula was also included in the plan. After the individualized preoperative evaluation of the patient, the operation was successfully completed.
The 3D-visualized model was used to simulate and guide the intraoperative operation, and further improve the surgical plan. Complete resection of the RA tumor relieved the mechanical compression on the heart, improved the cardiovascular blood circulation, relieved the symptoms of chest distress and chest pain, prevented the occurrence of malignant arrhythmia, effectively reduced the risk of thrombosis and embolism, and also prevented the complete blockage of the lesion to the hepatic segment of the inferior vena cava, avoiding venous reflux obstruction and liver dysfunction.
Additionally, the excised tumor was sent for examination to clarify the nature of the cardiac tumors, and cardiogenic cerebral infarction caused by cardiac myxoma. It is well known that prolonged coronary artery fistulas can cause a variety of complications, such as infective endocarditis, myocardial ischemia, arrhythmia, and pulmonary hypertension. In our case, the timely management of the coronary artery fistula with only one surgical anesthesia session effectively prevented the occurrence of these complications, improved the long-term prognosis of the coronary artery fistula, and reduced the risk of reoperation.
In conclusion, our case highlights the critical role of noninvasive 3D-visualized model evaluation, based on enhanced CT, in the preoperative planning for RA hemangioma and CBPF.
Acknowledgments
None.
Footnote
Funding: This study was supported by
Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-2025-944/coif). The authors have no conflicts of interest to declare.
Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Research Ethics Committee of Liuzhou People’s Hospital [approval No. 2025 (KY-E-03)]. Verbal informed consent was obtained from the patient by telephone for the publication of this article and accompanying images.
Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.
References
- Baird C, Blalock S, Bengur R, Ikemba C. Right atrial hemangioma in the newborn: Utility of fetal imaging. Ann Pediatr Cardiol 2012;5:81-4. [Crossref] [PubMed]
- Sánchez-Enrique C, Vivas D, Carnero Alcázar M, Vilacosta I. Giant right atrial hemangioma: Correlation between magnetic resonance imaging and histopathologic evaluation. J Thorac Cardiovasc Surg 2015;150:e51-3. [Crossref] [PubMed]
- Tourmousoglou C, Nikoloudakis N, Kelpis T, Tsotsolis N, Ninios V, Kallifatidis A, Ninios E, Theofilogiannakos EK, Thoma M, Asimaki A, Pitsis A. Left Ventricular Cardiac Hemangioma. Am J Med 2017;130:e337-9. [Crossref] [PubMed]
- Ma X, Yan W, Guo X, Sun Z, Xie M. Right Coronary Artery-Right Atrial Fistula in Right Atrial Myxoma: A Rare Combination. Circ Cardiovasc Imaging 2022;15:e014097. [Crossref] [PubMed]
- Maffei E, Bolognesi M, Cademartiri F. Complex congenital fistula between coronary arteries, bronchial arteries, and pulmonary artery assessed with cardiac computed tomography. Eur Heart J 2017;38:2079. [Crossref] [PubMed]
- Miao H, Yang W, Zhou M, Zhu Q, Jiang Z. Atrial Hemangioma: A Case Report and Review of the Literature. Ann Thorac Cardiovasc Surg 2019;25:71-81. [Crossref] [PubMed]
- Ufuk F, Kılıç O, Kılıç ID. Anomalous origin of the right coronary artery from the pulmonary artery and simultaneous coronary-bronchial artery fistula. Anatol J Cardiol 2020;23:E10-1. [Crossref] [PubMed]
- Huang T, Ma M, Yang Q, Fu X, Xu M. Preoperative 3-dimensional visualization technology-assisted laparoscopic resection of ectopic pheochromocytoma surrounding the renal artery: a case description. Quant Imaging Med Surg 2024;14:3157-61. [Crossref] [PubMed]
- Srnková P, Olejník P. The use of 3D-virtual anatomical model in a child with dysphagia lusoria. Int J Cardiovasc Imaging 2023;39:1343-4. [Crossref] [PubMed]
