IMAGES OF OSLER

Cases from the Osler Medical Service atJohns Hopkins University

Prepared by Dung Le, MD Aimee Zaas, MD

PRESENTING FEATURES: A 59-year-old man with a pastmedical history of blindness due to optic neuritis presentedwith severe lateral chest pain. While walking at home 2 daysbefore admission, he tripped over a couch and hit his left sideand back. He denied having presyncopal symptoms, such asdizziness, lightheadedness, nausea, dyspnea, or palpitations.He had no chest pain before the fall and was certain that hedid not lose consciousness. He had no prior history of car-diac disease, seizure disorder, melena, hematemesis, or or-thostatic symptoms. His past medical history was also nota-ble for an axonal sensory neuropathy of undetermined ori-gin, gastroesophageal reflux disease, and mild non-insulin-dependent diabetes mellitus. His medications includedglyburide and ranitidine. He did not smoke cigarettes, con-sume alcohol, or use illicit drugs.

On physical examination, he was in moderate distress due topain. His vital signs were normal, as were his lung and heartexaminations. He had decreased visual acuity and disconjugategaze. There was tenderness to palpation along the lower leftlateral and posterior ribs. Abdominal examination was normal.There were no hematomas or lacerations. Stool was guaiac neg-ative. Neurologic examination revealed a previously describedsensory neuropathy, as well as distal weakness of the upper andlower extremities. Chest radiograph, electrocardiogram, andlaboratory studies were normal. Due to persistent left-sidedpain, a computed tomographic scan of the abdomen was per-formed. The scan revealed no intraabdominal pathology; how-ever, because there was suggestion of a mass or clot in the leftatrium, additional images were obtained (Figure 1).

What is the diagnosis?

Figure 1. Computed tomographic scan revealing a left atrial mass (M).

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DIAGNOSIS: Left atrial myxoma

DISCUSSION: A transthoracic echocardiogram (ECG)(Figure 2) revealed mild left ventricular hypertrophy,with an ejection fraction of 45% and a 7.5- � 3.5-cm massin the left atrium. The mass, which was adherent to theintraatrial septum and prolapsed into the left ventricleduring atrial systole, was consistent with a left atrial myx-oma. Mitral valve flow was not impaired. Preoperativecardiac catheterization revealed normal coronary arter-ies, with neovascularization of the myxoma by the rightcoronary artery (Figure 3).

Cardiac myxomas are nonmalignant intracardiacmasses that are found in the left atrium in 75% of patients(1). Before angiocardiography and ECG were available,diagnosis was made most frequently at autopsy. The first

surgical removal of a myxoma was reported in 1954, andthe first diagnosis by ECG was made in 1959 (2). Cardiacimaging now allows earlier diagnosis and subsequent sur-gical resection.

Left atrial myxomas can also be diagnosed as incidentalfindings on imaging studies. However, myxomas can of-ten cause systemic symptoms, particularly fever, malaise,weight loss, anemia, and embolic phenomenon. The car-diac symptoms of myxomas can mimic mitral stenosis,with dyspnea and a diastolic murmur, particularly whenthe patient is recumbent (1– 4). Left ventricular outflowtract obstruction may also occur. The “tumor plop” of aleft atrial myxoma, which is a low-pitched early to mid-diastolic sound resulting from abrupt contact of the myx-

Figure 2. Transthoracic echocardiogram demonstrating a left atrial myxoma (M) prolapsing into left ventricle (LV). The myxomameasured 7.5 � 3.5 cm.

Figure 3. Cardiac catheterization with right coronary artery (arrow), and collateral vessels (arrowheads) feeding the myxoma(M).

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oma with the left ventricular wall, is similar to an S3 gal-lop but is rarely heard. In the largest reported series of 112patients with left atrial myxomata (1), 67% of patientshad some cardiac finding at presentation, with heart fail-ure being the most common; 34% had systemic symp-toms such as fever or malaise; 26% had neurologic signs,particularly stroke; and 10% were diagnosed incidentallyby imaging. Myxomas that were more than 5 cm in diam-eter were more likely to be associated with cardiac symp-toms, auscultatory findings, and abnormalities on theECG and chest radiograph (1).

Myxomas are sporadic, present most commonly in thethird to sixth decade, and are slightly more common inwomen. Familial syndromes involving atrial myxoma in-clude the Carney complex, the LAMB (lentigines, atrialmyxoma, mucocutaneous myxomas, and blue nevi) syn-drome, and the NAME (nevi, atrial myxoma, myxoidneurofibromas, and ephilides) syndrome (4). Left atrialmyxomas usually occur in the intraatrial septum near thefossa ovalis. They arise from cardiac stromal cells thatdifferentiate along endothelial lines and produce vascular

endothelial growth factor (3–5). The differential diagno-sis includes lipoma, sarcoma, rhabdomyoma, clot, andmetastatic tumor.

The patient underwent a thoracotomy and resection ofthe left atrial myxoma (Figure 4). He was discharged afteran uneventful perioperative course. Long-term prognosisis excellent following surgical excision, although recur-rences are reported.

REFERENCES1. Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial car-

diac myxoma. A series of 112 consecutive cases. Medicine (Balti-more). 2001;80:159 –172.

2. Schweizer P, Meyer J, Messmer BJ, et al. Special diagnostic proce-dures in atrial tumours of the heart [in German]. Dtsch Med Wochen-schr. 1978;103:1133–1135.

3. Burke AP, Virmani R. Cardiac myxoma. A clinicopathologic study.Am J Clin Pathol. 1993;100:671–680.

4. Reynen K. Cardiac myxomas. N Engl J Med. 1995;333:1610 –1617.5. Kono T, Koide N, Hama Y, et al. Expression of vascular endothelial

growth factor and angiogenesis in cardiac myxoma: a study of fifteenpatients. J Thorac Cardiovasc Surg. 2000;119:101–107.

Figure 4. Pathology specimen of the left atrial myxoma demonstrating myxoid stroma.

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696 December 1, 2002 THE AMERICAN JOURNAL OF MEDICINE� Volume 113