Key result
A 53-year-old man with hyper-immunoglobulin E-emia and HFpEF was diagnosed with primary amyloid light-chain amyloidosis, highlighting the need to consider infiltrative cardiomyopathies in HFpEF.
Case Report (n=1)
No
Infiltrative cardiomyopathies such as cardiac amyloidosis should be considered in patients presenting with HFpEF and hypertrophic cardiomyopathy features, even with atypical presentations like hyper-IgE-emia.
Single-case report warrants no practice change in HFpEF; leaves open infiltrative cardiomyopathy screening in hyper-IgE subsets.
BACKGROUND: Considering the increased prevalence of heart failure with preserved ejection fraction (HFpEF) as a result of the aging population, the pathophysiology of HFpEF needs to be examined. Furthermore, many comorbidity profiles in patients with HFpEF have been reported. Hypertrophic cardiomyopathy is a well-known specific etiology of HFpEF. Cardiac amyloidosis, which mimics infiltrative and hypertrophic cardiomyopathy, resulting from intensive amyloid deposition, is easily overlooked. CASE REPORT: A 53-year-old man with a 2-week history of persistent breathlessness was referred to our hospital. Upon admission, transthoracic echocardiography showed concentric mild left ventricular (LV) hypertrophy without a characteristic granular sparkling appearance or pericardial effusion, preserved ejection fraction, and bi-atrial enlargement with normal ventricular chambers. Doppler-derived LV diastolic filling demonstrated a prominent restrictive pattern indicating LV stiffness and elevated LV filling pressure. Blood tests revealed severe elevation of B-type natriuretic peptide and marked elevation of immunoglobulin E without eosinophilia. He was diagnosed with primary amyloid light-chain (AL) amyloidosis via skin and endomyocardial biopsy. CONCLUSIONS: We encountered a rare case of hypertrophic cardiomyopathy with HFpEF and identified a Doppler-derived restrictive filling pattern suggestive of early-stage heart failure in infiltrative cardiomyopathies. We suggest that infiltrative cardiomyopathies, such as cardiac amyloidosis, should be considered if hypertrophic cardiomyopathy is observed in a patient with HFpEF.
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Nojima et al. (2016) conducted a case report in Amyloid Light-Chain Amyloidosis with Heart Failure with Preserved Ejection Fraction (n=1). A 53-year-old man with hyper-immunoglobulin E-emia and HFpEF was diagnosed with primary amyloid light-chain amyloidosis, highlighting the need to consider infiltrative cardiomyopathies in HFpEF.
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