Left ventricular endocardial calcification in a patient with myeloproliferative disease.

Abstract: The case of a 57-year-old male with a history significant for myeloproliferative disease, chronic renal failure, hypertension, and prostate cancer is described. His complete blood count was remarkable for neutrophilia and, notably, eosinophilia. Subsequent to two syncopal episodes, a transthoracic echocardiogram was performed as part of the workup, which showed an unusual calcified mass in the left ventricular apical region but separate from the apical myocardium, with normal left ventricular systolic function. A transesophageal echocardiogram and computed tomography of the chest confirmed the presence of extensive calcification in the left ventricle of unusual location and shape. This patient probably had Loeffler endocarditis related to myeloproliferative disorder, complicated by calcification of the endocardial sclerotic lesions.

Key Words: endocardial calcification, echocardiography, Loeffler endocarditis

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Loeffler endocarditis causing calcification can be related to myeloproliferative disorder. Echocardiographic detection of a conspicuous left ventricular endocardial calcification is an unusual mode of presentation.

Case Report

A 57-year-old male presented with two syncopal episodes. His medical history was significant for hypertension and chronic renal failure, but he had never been on dialysis. He also had a 3-year history of myeloproliferative disease and a 2-year history of prostate cancer. His laboratory workup was remarkable for the following; white blood cell count of 51,400 K/L (n: 3,000 to 9,900); eosinophils, 3%, at 1,542 (n: 0 to 10% or 0 to 990); considered to be a relative eosinophilia, red blood cell count, 2.5 M/uL; hemoglobin, 5.5 g/dL; hematocrit, 16.4%; platelet count, 1,298 K/L; Cr, 4.3 mg/dL; Ca, 8.7 mg/dL; and P[O.sub.4]; 4.8 mg/dL.

On cardiac examination, a diastolic murmur was heard along the left sternal border. Electrocardiography showed voltage criteria for left ventricular hypertrophy with a strain pattern. Transthoracic echocardiography showed an unusual calcified mass about 3 X 2 cm in the left ventricular apical region but separate from the apical myocardium (Fig. 1), as well as trace mitral regurgitation, trace tricuspid regurgitation, and mild to moderate aortic regurgitation. Transesophageal echocardiography showed calcification of the endocardial surface of both papillary muscles and of the adjacent left ventricular wall as well as calcification of some mitral chordal structures (Fig. 2). Left ventricular wall motion was normal, with an ejection fraction (EF) of 65%. Computed tomography of the chest without contrast confirmed those calcifications in the region of the left ventricle (Fig. 3). Neither physical nor echocardiographic signs of pericardial constriction were detected.

We speculated that our patient had Loeffler endocarditis related to myeloproliferative disorder, complicated by calcification of the endocardial sclerotic lesions.

Discussion

Loeffler endocarditis is a rare form of progressive endocarditis associated with a highly increased number of eosinophilic granulocytes in the blood. The acute form is marked by eosinophilic involvement of various organs including the heart with myocarditis, endocarditis, and arteritis, leading to cardiac enlargement of all chambers, marked by leathery, grayish-white lesions that extend into the myocardium, papillary muscles, and chordae tendineae. The insidious form is characterized by a gradual decrease of tolerance to physical effort and congestive failure. Mitral and tricuspid incompetence as well as mitral stenosis and electrocardiographic ST-segment and T-wave abnormalities are the most common manifestations. (1)

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Apart from Loeffler endocarditis, myocardial calcification is most commonly dystrophic, resulting from ischemic heart disease (2) with myocardial infarction and scar formation.

The most common site of calcification is in the anterior wall of the left ventricle. Such myocardial calcification appears as a thin, curvilinear mass, usually found within the periphery of the infarct, in the distribution of the interven-tricular septum and cardiac apex. (3)

Metastatic cardiac calcification is associated with elevated levels of serum calcium. In our patient, serial serum calcium estimations were normal. Other causes of ventricular calcification include chronic kidney disease after cardiac trauma and calcified granulomas. (4) In the literature, extensive arterial calcification has been described in a patient with clas-sical POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin defects) associated with a myeloproliferative disorder. (5)

Conclusion

Cardiac calcification is not uncommon in patients with end-stage renal disease, particularly in the aortic valve, mitral valve, and mitral annulus region. Our patient did not have a history of myocardial infarction or end-stage renal disease. We speculate that he had Loeffler endocarditis related to myeloproliferative disorder, complicated by calcification of the endocardial sclerotic lesions. To our knowledge, such extensive calcification related to this disorder has not been previously described in the literature.

References

1. Loffler W. Endocarditisparietalis fibroplastica mit Bluteosinophilie: Ein eigenartiges Krankheitsbild. Schweizerischemedizinische Wochenschrift. Basel 1936;66:817-820.

2. Lasser A. Calcification of the myocardium. Hum Pathol 1983;14:824.

3. Freundlich IM, Lind TA. Calcification of the heart and great vessels. CRC Crit Rev Clin Radiol Nucl Med 1975;6:171-216.

4. Jing J. Kawashima A. Sickler A, et al. Metastatic cardiac calcification in a patient with chronic renal failure undergoing hemodialysis: radiographic and CT findings. AJR Am J Roentgenol 1998;170:903-905.

5. Jackson A, Burton IE. Premature vascular calcification in a case of PO-EMS syndrome. Eur J Radiol 1991 Nov-Dec;13:203-206.

The world is wide, and I will not waste my life in friction when it
could be turned into momentum.
--Frances Willard

Rami N. Khouzam, MD, Khawar Shaikh, MD, and Ivan A. D'Cruz, MD, FRCP

From the University of Tennessee Health Science Center and VA Medical Center, Division of Cardiovascular Diseases, Memphis, TN.

The authors have no commercial interest in any drug, device, or equipment mentioned in the submitted article that they would like to disclose.

Reprint requests to Rami N. Khouzam, MD, 699 Hotchkiss Lane, Memphis, TN 38104. Email: [e-mail]

Accepted May 4, 2005.

RELATED ARTICLE: Key Points

* Loeffler endocarditis causing calcification is known to occur in patients with chronic prolonged eosinophilia.

* Cardiac calcification of an unusual configuration and location was seen on echocardiography and computed tomography.

* Other causes of cardiac calcification were considered and thought very unlikely; ventricular systolic function and wall motion were normal on echocardiography.