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A 37-year-old man with chest pain, ECG changes, and elevated cardiac enzymes

Cleveland Clinic Journal of Medicine. 2009 March;76(3):199-205 | 10.3949/ccjm.75a.07008
March 1, 2009|Cleveland Clinic Journal of Medicine
Arash Aghel, MD;Richard A. Krasuski, MD
Author and Disclosure Information

Arash Aghel, MD
Department of Internal Medicine, Cleveland Clinic

Richard A. Krasuski, MD
Department of Cardiovascular Medicine, Section of Clinical Cardiology, Cleveland Clinic

Address: Richard Krasuski, MD, Department of Cardiovascular Medicine, J2-4, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195; e-mail krasusr@ccf.org

Dr. Krasuski has disclosed that he has received honoraria from Pfizer for teaching and speaking and honoraria and consulting fees from Actelion for teaching, speaking, and consultation.

TREATING ACUTE PERICARDITIS

4. Which is the most appropriate treatment for acute pericarditis?

  • Steroids
  • A nonsteroidal anti-inflammatory drug (NSAID) or aspirin
  • Opioids
  • Colchicine
  • Colchicine plus an NSAID or aspirin

An NSAID or aspirin is the basis of treatment for acute pericarditis and is very effective in relieving symptoms. Aspirin 2–4 g daily, indomethacin (Indocin) 75–225 mg daily, or ibuprofen (Motrin) 1,600–3,200 mg daily are prescribed most often; ibuprofen is preferred because it has a lower incidence of adverse effects than the others.9

Colchicine is recommended in addition to aspirin or NSAIDs for the treatment of acute pericarditis. Although in the past colchicine was reserved for recurrent pericarditis, the Colchicine for Acute Pericarditis (COPE) trial10 found it to be beneficial for first episodes of pericarditis as well.10 In this study, patients were randomized to receive conventional treatment with aspirin 800 mg every 6 or 8 hours or aspirin at the same dose combined with colchicine 0.5 to 1.0 mg daily. Colchicine showed significant benefit over conventional therapy, resulting in reduced rates of recurrence.

CASE CONTINUES: HEPATIC LESIONS ON MRI

Although aspirin and colchicine were started at the time of admission, our patient’s symptoms fail to improve. A suspicion remains that a neoplastic disorder could be the underlying cause of the presentation and could explain his chronic malaise, pericardial disease, and fever. In view of the liver hemangiomas reported previously on CT, we decide to evaluate the liver further with magnetic resonance imaging (MRI).

Figure 2. Magnetic resonance imaging shows multiple lesions in the liver.
To our surprise, the MRI reveals innumerable hepatic lesions, some of which show radiographic features consistent with hemangiomas, while the remainder are atypical and appear to warrant a biopsy (Figure 2). An oncology consultation is obtained and the need for biopsy is confirmed.

Since our patient’s symptoms have improved significantly during the past few days and his fever has resolved, biopsy is scheduled on an outpatient basis. Biopsy with ultrasonographic guidance is performed a week later and yields a pathologic diagnosis of hemangioma. The improvement, however, is short-lived, and his pain and dyspnea recur after 2 months. A follow-up echocardiogram is ordered.

A remarkable echocardiographic finding

Figure 3. Echocardiogram, four-chamber view, showing the tumor (crosshairs) in the right atrium (RA). LA = left atrium, LV = left ventricle, RV = right ventricle.
To our astonishment, the echocardiogram reveals a mass in the right atrial free wall and right ventricle that appears to be invading the myocardial tissue (Figure 3).

The original echocardiogram that was performed a little over 2-1/2 months ago is re-reviewed. It very subtly suggests a complexity to the pericardial effusion in the area of the current mass, apparent only when the two studies are directly compared. Clearly, there has been interval development of a mass easily detectable by echocardiography. Although a small mass may have been obscured by the pericardial effusion in the original echocardiogram, the development of a mass of this size in such a short time suggests a rapidly growing tumor.

Figure 4. Cardiac MRI; arrow points to the tumor. RA = right atrium, RV = right ventricle, LA = left atrium, LV = left ventricle.
Cardiac MRI is performed, which confirms the finding and characterizes the mass as measuring 5.1 by 4.8 cm within the pericardial space adjacent to the right atrium and atrioventricular groove and adherent to the right atrium. There are small excrescences of soft tissue through the midportion of the right atrial wall, suggesting tissue invasion (Figure  4).

CARDIAC TUMORS

5. Which is the most common primary cardiac tumor?

  • Myxoma
  • Papillary fibroelastoma
  • Sarcoma
  • Lymphoma

Primary cardiac tumors are rare, with an incidence on autopsy series ranging between 0.0017% and 0.33%,11,12 making them far less common than metastases to the heart.

Myxomas are benign cardiac tumors and are the most common primary cardiac neoplasm. Approximately 80% of myxomas originate in the left atrium, typically presenting with one or more of the triad of intracardiac obstruction, systemic embolization, and constitutional symptoms.14

Cardiac papillary fibroelastomas, the second most common cardiac tumors, are benign and predominantly affect the cardiac valves.15

Only one-fourth of all cardiac tumors are malignant. Nearly all of these malignant tumors are sarcomas, with angiosarcoma being the most common morphologic type, accounting for 30% of primary cardiac sarcomas.13

Primary cardiac lymphomas are extremely rare and account for only 1.3% of all primary cardiac tumors.16

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