Poll: Tafamidis For Transthyretin Cardiac Amyloidosis: Part 1

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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Clinical Topics: Geriatric Cardiology, Heart Failure and Cardiomyopathies, Cardiovascular Care Team, Noninvasive Imaging

Keywords: Amyloidosis, Geriatrics

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