title: Amyloid Light-Chain Amyloidosis Manifesting as Heart Failure with Preserved Ejection Fraction in a Patient with Hyper-Immunoglobulin E-emia.
abstract: 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.
  
  
  METHODS
  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.
authors.affiliation: Department of Cardiology, Nishinomiya Municipal Central Hospital, Nishinomiya, Japan.
authors.forename: Yuhei
authors.initials: Y
authors.lastname: Nojima
authors.affiliation: Department of Cardiology, Nishinomiya Municipal Central Hospital, Nishinomiya, Japan.
authors.forename: Madoka
authors.initials: M
authors.lastname: Ihara
authors.affiliation: Department of Cardiology, Nishinomiya Municipal Central Hospital, Nishinomiya, Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Kurimoto
authors.affiliation: Department of Cardiology, Nishinomiya Municipal Central Hospital, Nishinomiya, Japan.
authors.forename: Shinsuke
authors.initials: S
authors.lastname: Nanto
mesh_terms: D000686:Amyloidosis / Q000175:diagnosis*; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis; D003937:Diagnosis, Differential; D004417:Dyspnea / Q000209:etiology; D006331:Heart Diseases / Q000175:diagnosis*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000209:etiology*; D007073:Immunoglobulin E / Q000097:blood*; D008297:Male; D008875:Middle Aged; D013318:Stroke Volume
chemical_list: D007073:Immunoglobulin E