Korean Circ J.  2010 Nov;40(11):604-608. 10.4070/kcj.2010.40.11.604.

A Case of Chloroquine-Induced Cardiomyopathy That Presented as Sick Sinus Syndrome

• Lee JH
• Chung WB
• Kang JH
• Kim HW
• Kim JJ
• Kim JH
• Hwang HJ
• Lee JB
• Chung JW
• Kim HL
• Choi YS
• Park CS
• Youn HJ
• Lee MY

• KMID: 1826182
• DOI: http://doi.org/10.4070/kcj.2010.40.11.604

Abstract

A 52-year-old woman with rheumatoid arthritis who had been treated with prednisone and hydroxychloroquine for >12 years presented with chest discomfort and a seizure. She was diagnosed with restrictive cardiomyopathy combined with sick sinus syndrome. A myocardial muscle biopsy was performed to identify the underlying cardiomyopathy, which showed marked muscle fiber hypertrophy, fiber dropout, slightly increased interstitial fibrous connective tissue, and extensive cytoplasmic vacuolization of the myocytes under light microscopy. Electron microscopy of the myocytes demonstrated dense, myeloid, and curvilinear bodies. The diagnosis of hydroxychloroquine-induced cardiomyopathy was made based on the clinical, hemodynamic, and pathologic findings. This is the first case report describing chloroquine-induced cardiomyopathy involving the heart conduction system.

Keyword

Hydroxychloroquine; Cardiomyopathy, restrictive; Sick sinus syndrome

MeSH Terms

Arthritis, Rheumatoid
Biopsy
Cardiomyopathies
Cardiomyopathy, Restrictive
Connective Tissue
Cytoplasm
Female
Heart Conduction System
Hemodynamics
Humans
Hydroxychloroquine
Hypertrophy
Light
Microscopy
Microscopy, Electron
Middle Aged
Muscle Cells
Muscles
Patient Dropouts
Prednisone
Seizures
Sick Sinus Syndrome
Thorax
Hydroxychloroquine
Prednisone

Figure

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  Fig. 1 Initial electrocardiogram findings. A: the rhythm strip shows sinus pause. B: the 12-lead electrocardiogram shows a junctional rhythm with sick sinus syndrome.

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  Fig. 2 Chest radiography demonstrated marked cardiomegaly without pulmonary congestion.

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  Fig. 3 Two-dimensional echocardiography reveals severe concentric LV hypertrophy, mild LV systolic dysfunction, a small pericardial effusion, and increased RV free wall thickness with mild RV systolic dysfunction. A: parasternal long axis view. B: parasternal short axis view at the mid-ventricular level. C: magnified apical 4-chamber view. D: mitral inflow view (E velocity, 90.28 cm/s; A velocity, 36.77 cm/s; deceleration time, 216.64 ms; E/E': 33.4). LV: left ventricular, RV: right ventricular.

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  Fig. 4 Cardiac MRI findings. A: the black-blood coronal axis view on cardiac MRI demonstrates marked concentric left ventricle hypertrophy, suggesting hypertrophic cardiomyopathy. Right ventricle wall thickening is also noted and there is a small pericardial effusion. On delayed enhancement, signal hyperenhancement after gadolinium administration is observed within the septal wall on the coronal axis view (B) and lateral wall on the short axis view (C).

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  Fig. 5 Myocardial biopsy findings. A and B: light microscopy of the myocardial biopsy specimen showing diffusely enlarged myocytes with extensive cytoplasmic vacuolization (hematoxylineosin staining; A: original magnification ×100, B: ×200 scale bar, 200 µm). C: curvilinear bodies. This appearance is typical and characteristic of chloroquine and hydroxychloroquine toxicity. D: the transmission electron microscopy of the skeletal muscle biopsy specimen displays central replacement and displacement of the sarcomeres by marked accumulation of secondary lysosomes, including myeloid bodies (scale bar 1 µm).

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