Heart & Vascular Institute Physician eNewsletter - Fall 2011
Sheila* is a 22-year old mother of two children who was living an otherwise healthy life until one day she experienced a sudden numbness and tingling in her upper extremities. She could not communicate, though she was aware of what was going on around her. The next day, she became feverish and her blood pressure dropped to the 60s.
Sheila went to her local hospital for further evaluation. The following day she became feverish and her blood pressure plummeted to the 60s. She was started on a broad spectrum of antibiotics, vasopressor agents and was electively intubated for airway protection. A carotid ultrasound was normal, but labs showed a remarkable white blood cell count of 25,000, and elevated cardiac biomarkers with CK-MB 51 and Troponin I of 4.8 ng/mL. Physicians performed a CT brain scan, which showed no cerebral hemorrhage. Sheila's MRI showed acute infarction in the left MCA and PICA territories. A transthoracic echocardiogram (OSH) reported reduction in systolic function with an EF of 10 percent. Sheila was transferred to Cleveland Clinic.
Examination and Diagnosis
At Cleveland Clinic, further testing was performed to determine the cause of Sheila's "stroke"—the reason for her sudden numbness and mild expressive aphasia. Sheila's past medical history showed no reported drug or alcohol use, and she was a non-smoker. However, she had suffered migraines with aura for years. Sheila's last pregnancy was two years ago.
Upon transfer to Cleveland Clinic, Sheila's white blood cell count was 21,000, her creatinine was 1.5 and labs showed a CK of 1352, CK-MB of 11 and Troponin T of 0.79 ng/ML. A chest X-ray showed no evidence of infiltrates or effusions. Meanwhile, an ECG showed sinus tachycardia with 1-mm ST depressions and T-wave inversions in the lateral distribution. A repeat echocardiogram confirmed the presence of a severely reduced ejection fraction with multi-territorial wall motion abnormalities, mostly in the apical region. A large atrial mass was discovered in the interatrial septum.
To get a closer look at the heart's valves and chambers, an emergent transesophageal echocardiogram was performed. It revealed a large mass in the left atria (2.5 cm x 2.8 cm x 1.7 cm) with a broad base of attachment to the interatrial septum with multiple front-like projections into the left atrial cavity.
After testing, the differential diagnosis included septic left atrial thrombus, left atrial myxoma, atrial sarcoma or an intra-cardiac foreign body. All of these scenarios could likely result in embolization to her cerebral vasculature, causing the stroke symptoms that prompted Sheila to check in to her local hospital.
The severely reduced ejection fraction was a concern and physicians hypothesized this was the result of multi-vessel thrombotic coronary artery occlusion or a Takatsubo cardiomyopathy. A diagnostic coronary angiogram was performed that revealed completely normal coronary arteries, suggesting the latter diagnosis.
Ultimately, Sheila was diagnosed with left atrial myxoma with embolic complications and Takatsubo cardiomyopathy, which has been linked to stroke as a trigger or complication. This condition was identified because of the characteristic left-ventricle apical ballooning seen on the echocardiogram in conjunction with a normal coronary angiogram. Sheila's case was complex because she presented with two separate, rare diagnoses at the same time.
Within 24 hours of arriving at Cleveland Clinic, Sheila underwent open heart surgery. The atria mass and portion of normal myocardium at the base were completely removed. After a standard, uncomplicated post-operative course, she was discharged after eight days of recovery. Prior to discharge, she received a repeat echocardiogram which showed notable improvement of her left-ventricle function to 45 percent.
The presence of two distinct, rare diagnoses makes Sheila's case interesting. However, cardiac myxoma is the most commonly encountered primary tumor affecting the heart, accounting for half of fall primary cardiac neoplasms. And cardiac myxoma most frequently occurs in the left atrium. Half of cardiac myxoma may produce systemic emboli, and decreased blood flow to the brain is the most common embolic complication. This explains Sheila's initial symptoms of numbness and tingling in the upper extremities and expressive aphasia.
The important lesson to note from Sheila's case is that patients may present with more than one (and rare) diagnoses at the same time. It's critical to make a rapid, accurate diagnosis to facilitate appropriate treatment.
*Not her real name