An 89-year-old man with progressive effort intolerance, abdominal bloating, and lower extremity edema presents for evaluation of heart failure (HF) in the setting of preserved ejection fraction (EF). He has a history of a stroke. He has several previous admissions for acute decompensated HF. He comes to the appointment in a wheelchair given his limited mobility.

He has blood pressure 94/75 mm Hg, heart rate 81 bpm, respiratory rate 22 breaths/min, and weight 70 kg (body mass index 25 kg/m²). His examination findings are notable for jugular venous distention at 14 cm H₂O, decreased breath sounds at his right base with overlying crackles, an irregular cardiac rhythm with no audible gallop and a short apical systolic murmur, and a palpable liver edge with 2+ lower extremity symmetric edema. His neurologic examination shows left lower limb weakness that is 2/5 and 4/5 in his left upper extremity.

His laboratory test results include hemoglobin level 11.7 g/dL, creatinine level 1.7 mg/dL with estimated glomerular filtration rate 32 mL/min/m², potassium level 4.5 mEq/L, and total bilirubin level 2.1 mg/dL. Additional laboratory test results include N-terminal pro–B-type natriuretic peptide level 4784 pg/mL and high-sensitivity troponin T level 76 ng/L. Test results for monoclonal proteins, including immunofixation of the serum and urine and a kappa/lambda free light chain ratio, were unremarkable.

Initial imaging by cardiac echocardiography shows a nondilated left ventricle (LV) with posterior and septal wall thickness 17 mm, low tissue Doppler velocities, and speckle strain apical sparing with global longitudinal strain -10.4% despite EF 47%. The results of cardiac magnetic resonance imaging raise the suspicion for cardiac amyloidosis with diffuse transmural delayed enhancement in the setting of EF 42% with increased LV wall thickness and elevated spin-lattice relaxation time at 1341 msec. The results of a technetium pyrophosphate scan include a semiquantitative score of 3 on planar imaging with diffuse myocardial uptake on single-photon emission computed tomography/computed tomography.

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