Abstract
Background
Atrial myxomas are rare cardiac tumors, and their association with cerebral aneurysms, although infrequent, presents significant challenges in clinical management.
Case Summary
We present the case of a 54-year-old woman with a history of hypertension and dyslipidemia who experienced persistent headaches. Previously hospitalized for recurrent strokes, she was diagnosed with a left atrial myxoma and multiple cerebral aneurysms. The myxoma was successfully excised, while the aneurysms were managed conservatively.
Discussion
This case is noteworthy for its unusual combination of conditions, and it underscores the critical importance of recognizing and monitoring associated complications. It emphasizes the need for ongoing intensive monitoring in patients with atrial myxomas to effectively manage potential neurological complications.
Take-Home Messages
Early detection of atrial myxoma is vital to prevent vascular and embolic complications. Regular imaging to assess aneurysmal progression is essential and should be conducted under careful supervision.
Key words: cardiac myxoma excision, neurovascular complication
Graphical Abstract
History of Presentation
The patient was a 54-year-old woman with no known history of connective tissue disease or fibromuscular dysplasia; she also denied any family history of cerebral aneurysms. She presented with a longstanding history of lightheadedness, dizziness, and headaches, which began in her teenage years and were relieved with paracetamol. In her late 40s, she started experiencing episodes of chest pain that spontaneously resolved but were associated with near-syncopal attacks. She had been previously admitted for recurrent strokes but was lost to follow-up. Persistence of symptoms, including dizziness, near-syncopal attacks, and headaches, prompted her to seek further consultation. On physical examination, a grade 3 low-pitched systolic murmur was detected at the 3rd intercostal space left parasternal line, along with a grade 2 diastolic murmur at the apex. The neurologic examination revealed a 4+/5 motor deficit in her right upper and lower extremities, with the rest of the neurologic examination being unremarkable.
Take-Home Messages
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•
Early detection of atrial myxoma is critical in preventing severe complications.
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In instances where cerebral aneurysms are present, regular imaging and monitoring are imperative for the management of the associated aneurysm.
Past Medical History
The patient had a known history of hypertension and dyslipidemia, including stroke. She denied any family history of cancer or cardiac tumor.
Differential Diagnosis
Based on transthoracic echocardiography findings, thrombus and infective endocarditis were considered.
Investigations
Transthoracic echocardiography revealed a left atrial mass attached to the interatrial septum, prolapsing into the left ventricle during diastole and back into the left atrium during systole (Figure 1, Video 1). Further characterization with transesophageal echocardiography showed a heterogeneous, echogenic mobile mass attached to the fossa ovalis of the interatrial septum, measuring 31.4 × 9.1 mm, consistent with a left atrial myxoma. (Figure 2, Video 2).
Figure 1.
2-Dimensional Transthoracic Echocardiography in the Apical 4-Chamber View
Transthoracic echocardiography showing (A) left atrial mass (arrow) during systole and (B) left atrial mass (arrow) during diastole.
Figure 2.
Transesophageal Echocardiography in the Midesophageal Short-Axis View at the Level of the Aortic Valve
Transthoracic echocardiography revealing a 9.1 × 31.4 mm left atrial mass (arrow).
Plain cranial magnetic resonance imaging (MRI) showed old lacunar infarcts in the left thalamocapsular area (Figure 3) as well as fusiform aneurysms in the insular segment of the bilateral middle cerebral arteries and the right anterior cerebral artery (Figures 4, 5, and 6).
Figure 3.
Findings on Cranial Magnetic Resonance Imaging
Magnetic resonance imaging with (A) T2 and (B) FLAIR revealing an old lacunar infarct in the left thalamocapsular area (arrow).
Figure 4.
Findings on Cranial Magnetic Resonance Imaging
Magnetic resonance imaging revealing a fusiform aneurysm in the right insular segment of the right middle cerebral artery (arrow).
Figure 5.
Findings on Cranial Magnetic Resonance Imaging
Magnetic resonance imaging revealing a fusiform aneurysm in the proximal insular segment of the left middle cerebral artery with suggestive central thrombus formation (arrow).
Figure 6.
Findings on Cranial Magnetic Resonance Imaging
Magnetic resonance imaging revealing a fusiform aneurysm in the A3 segment of the right anterior cerebral artery (arrow).
Management
The patient underwent preoperative coronary angiography, which revealed angiographically normal coronary arteries. Excision of the left atrial myxoma was performed, revealing a grossly reddish, gelatinous mass with a peduncle attached to the fossa ovalis, measuring 5 × 3 cm (Figure 7). Histopathologic studies confirmed it to be a myxoma (Figure 8). The postoperative course was uneventful.
Figure 7.
Excised Cardiac Mass
Image showing a reddish gelatinous mass with a peduncle (green arrow) attached to the fossa ovalis (yellow arrow), measuring 5 × 3 cm.
Figure 8.
Hematoxylin and Eosin Staining of the Resected Tissue
Slides reveal an abundant loose myxoid stroma (yellow arrow) with scattered round, polygonal, or stellate cells with dense, irregular nuclei (yellow circle), consistent with myxoma.
Outcome and Follow-Up
The operation was uneventful and without complications. However, 9 months postoperatively, a repeat cranial MRI with contrast showed progression of the aneurysm in the proximal insular segment of the left middle cerebral artery, which enlarged from 0.8 × 0.8 cm to 2.3 × 0.7 cm. Additional aneurysms were also identified in the contrast-enhanced images, located in the cortical branches supplying the right occipital lobe and the left inferior parietal lobule, with a total of 6 aneurysms (Table 1). The patient was managed conservatively. Other potential sources of stroke were not fully excluded, as no aortic imaging was performed.
Table 1.
Anatomical Location and Size of Aneurysms on Cranial Magnetic Resonance Imaging
| Location of Aneurysm | Initial MRI | Latest MRI |
|---|---|---|
| Right insular segment of the right middle cerebral artery | 0.6 × 0.38 cm | 0.58 × 0.36 cm |
| Proximal insular segment of the left middle cerebral artery | 0.8 × 0.8 cm | 2.3 × 0.7 cm |
| A3 segment of the right anterior cerebral artery (first fusiform) | 0.5 × 0.4 cm | 0.52 × 0.33 cm |
| A3 segment of the right anterior cerebral artery (second fusiform) | 0.61 × 0.44 cm | 0.5 × 0.5 cm |
| Cortical branches supplying the right occipital lobe | Not detected | 1.3 × 0.4 cm |
| Left inferior parietal lobule | Not detected | 0.7 × 0.3 cm |
MRI = magnetic resonance imaging.
Currently, the patient continues to experience lightheadedness, dizziness, near-syncopal attacks, and headaches, although these symptoms resolve with sleep. She denies any further chest pain, and the physical examination is unremarkable except for residual right-sided weakness. She remains under conservative management.
Discussion
Primary cardiac tumors are rare, accounting for up to 0.29% of cases based on randomized autopsies, in contrast to secondary tumors of the heart.1 Primary cardiac tumors are benign in more than 80% of cases.2 Myxomas, the most common type, are typically located in the left atrium (75%) and less frequently in other chambers of the heart, such as the right atrium, right ventricle, and left ventricle.2 Patients with such tumors may also present with cerebral aneurysms. However, cerebral aneurysms are rare, with only 54 cases reported to date based on a systematic review.3
The signs and symptoms of syncope, dyspnea, and pulmonary edema result from mitral valve obstruction caused by a left atrial myxoma. However, nonspecific signs and symptoms such as fever, fatigue, erythematous rash, weight loss, and cough can also occur.2 Cardiac examinations may reveal a “tumor plop” murmur, characterized by a low-pitched sound during diastole when the mass prolapses into the left ventricle upon auscultation.2 Vascular incidents are the most common complications in patients with multiple cerebral aneurysms caused by left atrial myxoma, followed by headaches and dizziness, which were observed in this patient. Aneurysms are most often localized to the entire area of vascularization, followed by the middle cerebral artery.3 In this patient, aneurysms were found in the entire area of vascularization.
This case is an example of a left atrial myxoma associated with neurologic complications. Cerebral complications caused by atrial myxomas have been documented in 12% of cases and commonly present as ischemic infarcts.4 The connection between cerebral aneurysms and cardiac myxomas is theorized to be due to the penetration and infiltration of cardiac myxomatous tumor cells through the arterial vessel wall, leading to dilatation and pseudoaneurysmal formation.5 In a prospective study, 4 out of 49 patients who underwent resection of a cardiac myxoma died, with 1 of the 4 deaths due to a ruptured cerebral aneurysm leading to cerebral hemorrhage.6
The pathogenesis of cerebral aneurysms in patients with atrial myxoma involves the infiltration and proliferation of myxomatous cells in the cerebral vasculature. This may either result in the remodeling of vessels back to normal without residual effects or, in some cases, lead to incomplete remodeling and aneurysm formation.7 One study reported that overproduction of interleukin-6 (IL-6) by cardiac myxomas contributes to the constitutional symptoms and immunologic abnormalities observed in affected patients, and it may serve as a marker of tumor recurrence.8 Interestingly, elevated IL-6 levels have also been implicated in vascular pathology, as genetic studies suggest that IL-6 promoter polymorphisms are associated with intracranial aneurysmal disease, although this remains unclear.9 These findings indicate that IL-6 may play a common pathogenic role linking cardiac myxoma and cerebral aneurysm formation through its proinflammatory and vascular remodeling effects. There is no clear study demonstrating that the type of myxoma (gelatinous consistency) is correlated with the development of cerebral aneurysms.
Radiation and chemotherapy with doxorubicin and ifosfamide have been proposed to prevent aneurysm growth and myxoma invasion.10 However, there are currently no definitive guidelines for this treatment given its rarity. Chojdak-Łukasiewicz et al3 reviewed 54 studies involving patients who underwent successful surgical removal of cardiac myxomas. In terms of management strategies, 60% of the patients (33 cases) were treated conservatively as the primary approach. Chemotherapy was administered in 5.5% of the cases (3 patients), while 1 patient received radiotherapy and another was treated with stereotactic radiosurgery.3
In this case of left atrial myxoma with multiple cerebral aneurysms, the myxoma was surgically excised as definitive treatment. Although some case reports suggest potential benefits from chemotherapy and low-dose targeted radiation therapy, a conservative approach was opted for managing the aneurysms at this stage.11
Conclusions
This case report highlights the importance of monitoring neurologic signs and symptoms in patients with left atrial myxomas, even after resection. The growth of aneurysms and recurrence of myxoma should also be regularly monitored. As of this writing, there are no clear guidelines on optimal case management.
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Acknowledgments
The authors express their heartfelt gratitude to the Heart and Vascular Institute of Perpetual Help Medical Center, Biñan, where the primary author finished her fellowship, for their support in facilitating this case report. Special thanks are extended to Dr Ray Albert Cabigan, Dr Jo Cris Gutierrez, Dr Lauren Kay Evangelista, Dr Carla Jean Aloc, and Ryan Jorge M. Dela Cruz for their invaluable assistance and encouragement in the preparation of this case report. Finally, the authors express their deepest appreciation to the patient for granting consent and providing full cooperation in sharing the information necessary for this report.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
Appendix
Visual Summary.
| Time | Events |
|---|---|
| Time 0 (initial health visit) | A 54-year-old woman with a history of hypertension and dyslipidemia, who had recurrent strokes, presented with a persistent headache. A work-up revealed a left atrial myxoma on transthoracic echocardiography. |
| 3 Months later | Cranial MRI showed old lacunar infarcts in the left thalamocapsular area, as well as fusiform aneurysms in the insular segment of the bilateral middle cerebral arteries and the right anterior cerebral artery. |
| 4 Months later | The patient was admitted and underwent preoperative coronary angiography, which revealed angiographically normal coronary arteries. She subsequently underwent excision of the left atrial mass under cardiopulmonary bypass. Grossly, the mass had a peduncle attached to the fossa ovalis and was noted to be reddish and gelatinous, measuring 5 × 3 cm. Histopathologic studies confirmed it to be a myxoma. The postoperative course was uneventful. |
| 9 Months later | A repeat cranial MRI with contrast showed progression of an enlarging aneurysm in the proximal insular segment of the left middle cerebral artery, increasing from 0.8 to 2.3 cm. An additional aneurysm was also appreciated in the contrast-enhanced image at the cortical branches supplying the right occipital lobe and the left inferior parietal lobule, bringing the total number of aneurysms to 6. The patient was managed conservatively. |
| Present time | At present, the patient continues to experience dizziness, near-syncopal attacks, and headaches, which resolve with sleep. She denies episodes of chest pain. The physical examination was unremarkable except for the right-sided residual weakness. |
Appendix
Transthoracic Echocardiography Revealing a Left Atrial Mass Attached to the Interatrial Septum, Prolapsing Into the Left Ventricle During Diastole and Back Into the Left Atrium During Systole
Further Characterization With Transesophageal Echocardiography Showing a Heterogeneous, Echogenic Mobile Mass Attached to the Fossa Ovalis of the Interatrial Septum, Consistent With a Left Atrial Myxoma
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Associated Data
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Supplementary Materials
Transthoracic Echocardiography Revealing a Left Atrial Mass Attached to the Interatrial Septum, Prolapsing Into the Left Ventricle During Diastole and Back Into the Left Atrium During Systole
Further Characterization With Transesophageal Echocardiography Showing a Heterogeneous, Echogenic Mobile Mass Attached to the Fossa Ovalis of the Interatrial Septum, Consistent With a Left Atrial Myxoma