IJCS | Volume 31, Nº4, July / August 2018

DOI: 10.5935/2359-4802.20180039 457 CASE REPORT International Journal of Cardiovascular Sciences. 2018;31(4)457-460 Mailing Address: Antonio Jose Lagoeiro Jorge Avenida Marques do Paraná, 303. Postal Code: 24033-900, Centro, Niterói, RJ - Brazil. E-mail: lagoeiro@globo.com Cardiac Amyloidosis with Heart Failure and Middle Range Ejection Fraction Antonio Jose Lagoeiro Jorge, Diane Xavier de Ávila, Enoï Guedes Vilar, Mario Luiz Ribeiro, Karima Elias Hallack Bruno, Ana Carolina Pires Universidade Federal Fluminense, Niterói, RJ - Brazil Manuscript received October 30, 2017; revised manuscript February 23, 2018; accepted March 3, 2018. Heart Failure / physiopathology; Amyloidosis; Stroke Volume; Hypertrophy, Left Ventricular; Aged. Keywords Introduction Cardiac amyloidosis (CA) is a disease with a difficult diagnosis, limited management and a reserved prognosis. 1,2 A high level of suspicion is necessary for its identification. There are some clinical clues, such as elderly individuals with unexplained left ventricular hypertrophy (LVH), heart failure with preserved ejection fraction (HFpEF) and restrictive pattern, dissociation between LVH on echocardiography and low voltage on the electrocardiogram, among others. 1,2 CA can occur with several hemodynamic forms and patterns of remodeling, according to the disease evolution stage. It may occur as the restrictive form, with left ventricular ejection fraction > 50%; and dilated form, with reduced ejection fraction. 1,2 Recently, the European Society of Cardiology has established a new classification with the creation of “Heart Failure with Mid-Range Ejection Fraction” (HFmrEF). 3 We report a case of CA with HFmrEF. Case report A female patient, aged 80 years old, was treated at the emergency unit at the first evaluation showing fatigue withmedium exertion, orthopnea, nocturnal paroxysmal dyspnea and lower limb edema. She also had frequent complaints of muscle weakness and asthenia. She was admitted with a diagnosis of acute heart failure (HF). She reported macrocytic anemia 3 years before, with no defined etiological diagnosis. She was submitted to an echocardiogram, which showed LVH and ejection fraction of 64%. She was discharged without an etiological definition of HF. Fifteen days after discharge, she came to the outpatient clinic with pallor (2+/4+) and jugular swelling at 45°; ictus cordis in the sixth intercostal space in the anterior axillary line; presence of third heart sound; pulmonary component of the second heart sound greater than the aortic component, without murmurs. Pulmonary auscultation showed vesicular murmur abolished at both bases; crepitant rales up to the middle third of both hemithoraces. The liver was palpable at 2 cm from the right costal border. She had symmetrical lower- limb swelling, with pitting edema up to the thigh root, cold and painless. The patient was hospitalized for HF compensation and etiological investigation. Laboratory tests showedmacrocytic and hypochromic anemia; vitaminB12deficiency; erythrocyte sedimentation rate of 134 mm; electrophoresis; and proteins with monoclonal lambda chain peak. The electrocardiogram showed junctional rhythm, with lowvoltage. Chest x-ray showed signs of pulmonary congestion and moderate bilateral pleural effusion. The transthoracic echocardiogram showed dilation of the left and right atria, left ventricular ejection fraction (LVEF) of 42% and alterations suggestive of infiltrative heart disease. Global strain with apical sparing pattern (Figure 1A to 1D) was observed. Myocardial resonance (MRI) was suggestive of the presence of subendocardial amyloid deposits and late enhancement of 35%. LVEF was 45% (Figures 1E to 1H). The bone marrow aspirate showed a predominance of plasma cells in > 90%of the slide. Immunohistochemistry confirmed the diagnosis of multiple myeloma. Biopsy of facial lesion and abdominal fat with histopathological analysis showed amyloid deposits (Figure 2).