Korean Circ J.  2013 Nov;43(11):752-760. 10.4070/kcj.2013.43.11.752.

Incidence, Diagnosis and Prognosis of Cardiac Amyloidosis

• Lee MH
• Lee SP
• Kim YJ
• Sohn DW

• KMID: 2224807
• DOI: http://doi.org/10.4070/kcj.2013.43.11.752

Abstract

BACKGROUND AND OBJECTIVES
Cardiac involvement is frequent in systemic amyloidosis and is the most important determinant of the clinical outcome. The aims of this study were to assess the incidence and prognosis of cardiac amyloidosis and discuss the diagnostic issues related to cardiac amyloidosis.
SUBJECTS AND METHODS
We retrospectively studied all patients diagnosed with systemic amyloidosis who presented to our institution from January 1999 to December 2011.
RESULTS
Of the 129 patients with systemic amyloidosis, cardiac amyloidosis was diagnosed in 62 patients. At the 3 years' follow-up of the patients with systemic amyloidosis, there was a statistically significant difference in mortality between patients with cardiac amyloidosis and the rest of the patients (58.1% vs. 37.3%, p=0.008). In the Cox proportional hazard model, old age {hazard ratio (HR) 18.336, p=0.006}, elevation of cardiac troponin I (cTNI) (HR 13.246, p=0.020), left ventricular (LV) systolic dysfunction (HR 5.137, p=0.041) and diastolic dysfunction (HR 64.595, p=0.022) were independently associated with survival in cardiac amyloidosis. In the diagnosis of monoclonal gammopathy, serum or urine protein electrophoresis was not sensitive enough to be used clinically compared to serum free light chain assay (35.8% vs. 96.4%).
CONCLUSION
In systemic amyloidosis, cardiac involvement was the most important determinant of the prognosis, and old age, elevation of cTNI, LV systolic dysfunction and diastolic dysfunction were independently associated with survival in cardiac amyloidosis.

Keyword

Amyloidosis; Monoclonal gammopathy

MeSH Terms

Amyloidosis*
Diagnosis*
Electrophoresis
Follow-Up Studies
Humans
Incidence*
Mortality
Paraproteinemias
Prognosis*
Proportional Hazards Models
Retrospective Studies
Troponin I
Troponin I

Figure

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  Fig. 1 Electrocardiography in a patient with cardiac amyloidosis. Note the low voltage QRS in the limb leads.

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  Fig. 2 Echocardiography in a patient with cardiac amyloidosis. Top left and right, parasternal long-axis and apical four chamber view demonstrates increased left ventricular wall thickening with a small amount of pericardial effusion. Bottom left and right, transmitral doppler flow shows a pseudonormalization pattern with an increased E/E' ratio.

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  Fig. 3 Cardiovascular magnetic resonance imaging in a patient with cardiac amyloidosis. Top row shows diastolic frames from cines (vertical long axis, horizontal long axis, and short axis, respectively) showing a thickened left ventricular wall and presence of pericardial effusions (black arrow). Bottom row shows LGE images in the same planes. The vertical and horizontal long axis image demonstrates global hyperenhancement of both ventricles, both atria and the interatrial septum (white arrows). LGE: late gadolinium enhancement.

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  Fig. 4 Kaplan-Meier survival curves of patients with systemic amyloidosis with cardiac involvement vs. without cardiac involvement.

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  Fig. 5 Kaplan-Meier survival curves of patients with systemic amyloidosis with treatment vs. without treatment.

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  Fig. 6 Kaplan-Meier survival curves of patients with cardiac amyloidosis with treatment vs. without treatment.

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  Fig. 7 Kaplan-Meier survival curves of patients with AL amyloidosis with a good response vs. partial or no response to chemotherapy.

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  Fig. 8 Kaplan-Meier survival curves of patients with AL amyloidosis with cardiac involvement with a good response vs. partial or no response to chemotherapy.

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