It is commonly postulated that neurologic complications of atrial myxomas are due to either direct tumor embolization or mycotic aneurysm of cerebral vasculature or rupture of mycotic aneurysms of cerebral arteries. However, the authors report the case of 63-year-old woman with a large left atrial myxoma whose progressive left-sided weakness was due to a different neurologic mechanism, namely, multiple bleeding cavernous malformations, which were visualized by magnetic resonance imaging of the brain. Cerebral cavernous malformations coexist with mesenchymal anomalies of other organs, including the liver, kidneys, and retinas. To the best of the authors’ knowledge, this is only the second reported case of coexistent cerebral cavernous malformations and atrial myxoma.
Case Presentation
A 63-year-old Costa Rican woman with a history of systemic hypertension was hospitalized after developing progressive left-sided weakness, headache, and dizziness over the preceding 2 months. She experienced neither seizures nor loss of consciousness. There was no weight loss, fever, cough, shortness of breath, or chest pain. She used no illicit drugs or over-the-counter products. Her family history was unremarkable. Written informed consent was obtained from the patient for the publication of this case and accompanying images.
On admission, the patient had a regular heart rate of 70 beats/min, blood pressure of 150/70 mm Hg, an oral temperature of 98.5°F, and room-air oxygen saturation of 99%. On physical exam, her lungs were clear to auscultation bilaterally. A cardiac exam revealed normal-intensity first and second heart sounds and a grade 2/6 rumbling diastolic murmur best heard at the apex. A low-intensity sound was heard at the left lower sternal border with each diastole, consistent with tumor plop. There was no peripheral edema. Neurologic examination revealed loss of muscle strength (4 on a scale of 5) and decreased deep tendon reflexes in both the left arm and the left leg.
Complete blood count, coagulation panel, and blood chemistry were unremarkable, including normal serum troponin I and brain natriuretic peptide levels. Liver, kidney, and thyroid function test results were also within normal limits. Electrocardiographic results were normal. Chest x-ray revealed a normal-sized heart with no pulmonary venous congestion.
Computed tomography of the head on presentation revealed lesions in the right parietal and left occipital lobes, with mixed areas of high and low density, consistent with hemorrhage within the lesions and extensive vasogenic edema in the surrounding areas. The patient was started on phenytoin and intravenous steroids. Subsequent magnetic resonance imaging of the head delineated lesions as multiple bleeding cavernous hemangiomas with surrounding edema and mild midline shift ( Figure 1 ).
Transthoracic echocardiography revealed a large heterogeneous multilobulated mass in the left atrium measuring ≥5.6 × 2.7 cm. The mass was adherent to the interatrial septum and glided over the atrial side of the anterior mitral leaflet ( Figure 2 , Video 1 ). It prolapsed into the mitral valve orifice during diastole (tumor plop), causing mild mitral valve stenosis with a mean gradient of 4 mm Hg at a heart rate of 65 beats/min. After intravenous injection of perflutren lipid microsphere contrast (Definity; Lantheus Medical Imaging, North Billerica, MA), there was a heterogenous uptake pattern within the mass. All these findings were pathognomonic for left atrial myxoma ( Video 2 ).
Subsequently, cardiac magnetic resonance imaging was performed using balanced cine gradient-echo sequence (fast imaging employing steady-state acquisition), nonbalanced cine gradient-echo sequence (FASTCINE, GE Healthcare, Milwaukee, WI), and contrast enhancement protocols. The mass exhibited high signal intensity on the T1-weighted and T2-weighted images. No fat was demonstrated within the mass on fat saturation images. On the bright-blood sequences, the mass was not visible on the balanced gradient-echo sequences but could be seen on the nonbalanced bright-blood sequences. After the administration of contrast, enhancement was present at the periphery of the mass. The bright-blood images demonstrated a complete return of the mass into the left atrial cavity in systole and prolapse through the mitral valve into the left ventricle during diastole. There was also suggestion on the bright-blood images that the mass was attached to the interatrial septum via a stalk. On the four-chamber fast imaging employing steady-state acquisition sequences, there was turbulent flow across the mitral valve during diastole indicative of mitral stenosis). All magnetic resonance imaging findings were consistent with left atrial myxoma ( Figure 3 , Video 3 ). Cerebral angiography did not demonstrate aneurysms of any intracerebral vessel.
