Korean J Med.  2016 Apr;90(4):334-340. 10.3904/kjm.2016.90.4.334.

Paraganglioma Presenting as Dilated Cardiomyopathy with Cardiogenic Shock and Sepsis

• Lee CW
• Hong SP
• Lee JH
• Kim JW
• Lee HC
• Kwon HH

• KMID: 2268848
• DOI: http://doi.org/10.3904/kjm.2016.90.4.334

Abstract

Rarely, a paraganglioma can lead to disastrous cardiac complications such as heart failure, cardiomyopathy, or myocardial infarction and inflammatory complications such as sepsis. We describe a 41-year-old man who developed severe dilated cardiomyopathy with cardiogenic shock and sepsis who had a paraganglioma in the retroperitoneum. Echocardiography showed extensive global hypokinesia with severe left ventricular systolic dysfunction. In addition, leukocytosis with elevated inflammatory markers and positive blood cultures indicated sepsis. Abdominal enhanced computed tomography and magnetic resonance imaging showed a large heterogeneous mass with cystic changes in the retroperitoneum. Serum and urine samples indicated elevated levels of catecholamine and its metabolites, and a 131I-meta-iodobenzylguanidine (MIBG) scan indicated uptake of MIBG. After intensive medical treatment and surgical removal of the paraganglioma, the dilated cardiomyopathy and sepsis resolved. The diagnosis of paraganglioma should be considered in patients presenting with acute heart failure or sepsis of nonspecific origin.

Keyword

Paraganglioma; Pheochromocytoma; Cardiomyopathy, Dilated; Shock, Cardiogenic; Sepsis

MeSH Terms

3-Iodobenzylguanidine
Adult
Cardiomyopathies
Cardiomyopathy, Dilated*
Diagnosis
Echocardiography
Heart Failure
Humans
Hypokinesia
Leukocytosis
Magnetic Resonance Imaging
Myocardial Infarction
Paraganglioma*
Pheochromocytoma
Sepsis*
Shock, Cardiogenic*
3-Iodobenzylguanidine