Clinical Characteristics of Cryoglobulinemia With Cardiac Involvement in a Single Center

Background: Cryoglobulinemia is a syndrome characterized by the presence of cryoglobulins (CGs) in serum, and cardiac involvement is a rare occurrence that can affect treatment and prognosis. This study aimed to explore the clinical characteristics of cryoglobulinemia with cardiac involvement.Methods: 108 patients diagnosed with cryoglobulinemia who were admitted and treated in Peking Union Medical College Hospital (PUMCH) between June 1985 and June 2019 were enrolled in the present study. Clinical characteristics, therapy, and prognosis of patients with cardiac involvement were retrospectively analyzed.Results: The cryoglobulinemia with cardiac involvement was found in 7 patients, thus reaching the incidence of 6.5%. Heart failure was the main cardiac manifestation found in these patients, all with the involvement of external cardiac organs. Laboratory examinations showed significant elevation of N-terminal brain natriuretic peptide precursor (NT-proBNP) and brain natriuretic peptide (BNP) with negative troponin (cTnI). Electrocardiogram (ECG) was generally normal or only showed low-flat and biphasic multi-lead T waves. Echocardiography was performed in 6 patients, all of whom showed enlargement of heart cavity. Five patients had reduced left ventricular myocardial contractible motion with decreased ejection fraction, 3 patients had pericardial effusion, and 1 patient had left ventricular hypertrophy or severe aortic insufficiency. Cardiac magnetic resonance imaging showed delayed myocardial enhancement in 2 patients. One patient underwent a myocardial biopsy, which showed perivasculitis. Condition in 6 patients who received active treatment targeting improved in the early stage. Three patients (3/7, 42.9%) died due to disease progression during follow-up period.Conclusions: Cryoglobulinemia with cardiac involvement is a rare but serious condition that has relatively high risk of death. When patients with cryoglobulinemia without underlying heart disease experience heart failure, chest pain, or elevation of asymptomatic NT-proBNP and BNP, there is a high possibility of cardiac involvement, even if the electrocardiogram and troponin are negative. Further examinations such as echocardiography, cardiac magnetic resonance imaging, and myocardial biopsy examination could contribute to the diagnosis. Cardiac manifestations could be timely reversed after active targeted treatment. NT-proBNP and echocardiography could be used for the monitoring of disease efficacy.

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Abstract MP31: Left Ventricular Hypertrophy by Electrocardiogram versus Cardiac Magnetic Resonance Imaging as a Predictor for Heart Failure: The MESA Study

Circulation ◽

10.1161/circ.133.suppl_1.mp31 ◽

2016 ◽

Vol 133 (suppl_1) ◽

Author(s):

Abdullahi O Oseni ◽

Waqas T Qureshi ◽

Mohammed F Almahmoud ◽

Alain Bertoni ◽

David A Bluemke ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Heart Failure ◽

Cardiac Magnetic Resonance ◽

Magnetic Resonance ◽

Left Ventricular Hypertrophy ◽

Cardiac Magnetic Resonance Imaging ◽

Ventricular Hypertrophy ◽

Predictive Ability ◽

Left Ventricular ◽

Resonance Imaging

Background: Left ventricular hypertrophy (LVH) is an established risk factor for heart failure (HF). However, it is unknown whether LVH detected by electrocardiogram (ECG-LVH) is equivalent to LVH ascertained by cardiac magnetic resonance imaging (MRI-LVH) in terms of prediction of incident HF using risk prediction models like the Framingham Heart Failure Risk Score (FHFRS). Methods: This analysis included 4745 (mean age 61+10 years, 53.5% women, 61.7% non-whites) from the Multi-Ethnic Study of Atherosclerosis who were free of cardiovascular disease at the time of enrollment. ECG-LVH was defined using Cornell’s criteria while MRI-LVH was derived from left ventricular (LV) mass measured by cardiac MRI. Cox proportional hazard regression was used to examine the association between ECG-LVH and MRI-LVH with incident HF. Harrell’s concordance C-index was used to estimate the predictive ability of the FHFRS when either ECG-LVH or MRI-LVH were included as one of its components. The added predictive ability of ECG-LVH and MRI-LVH were investigated using integrated discrimination improvement (IDI) index and relative IDI. Results: ECG-LVH was present in 291(6.1%) while MRI-LVH was present in 499 (10.5%) of the participants. Over a median follow up of 10.4 years, 140 participants developed HF. Both ECG-LVH [HR (95% CI): 2.25(1.38-3.69)] and MRI-LVH [HR (95% CI): 3.80(1.56-5.63)] were associated with an increased risk of HF in multivariable adjusted models (Table 1). The ability of FHFRS to predict HF was improved with MRI-LVH (C-index 0.871, 95% CI: 0.842-0.899) when compared with ECG-LVH (C-index 0.860, 95% CI: 0.833-0.888) (p < 0.0001). To assess the potential clinical utility of using LVH-MRI instead of ECG-LVH, we calculated several measures of reclassification (Table 1), which were consistent with the statistically significantly improved C-statistic with MRI-LVH. Conclusion: Both ECG-LVH and MRI-LVH are predictive of HF when used in the FHFRS. Substituting MRI-LVH for ECG-LVH improves the predictive ability of the FHFRS.

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