Abstract
Introduction: Transthyretin amyloid cardiomyopathy (ATTR) is thought to be a rare cause of HF, but recent studies showed this is the cause of 30% of HFpEF patients older than 75. With new treatments for amyloidosis, timely diagnosis has become critical. Case: A 78-female with HFpEF, HTN, and diabetes followed for progressive functional decline and hospitalizations for HFpEF exacerbations. EKG at baseline showed LBBB with normal QRS voltages. Serial echocardiogram (echo) showed an ejection fraction (EF) of 60-70%, mild LVH, and moderate TR with RVSP >50 mmHg. Latest echo showed progressive LA enlargement, MR, a new small pericardial effusion, and EF 50%. Regadenoson nuclear scan showed normal perfusion with preserved LVEF. Despite adequate control of her HTN, dyspnea continued to worsen. Because of this, the diagnosis of Cardiac amyloidosis was considered. Further review revealed a history of Carpal Tunnel Syndrome (CTS) and speckled pattern within the ventricular wall on echo. A technetium pyrophosphate scintigraphy ( 99m Tc-PYP) confirmed ATTR with grade three uptake, AL amyloid was ruled out with normal SPEP/UPEP, and she was started on Tafamidis. Discussion: Cardiac amyloidosis should be considered in HFpEF patients with functional decline despite medical optimization. It can mimic LVH caused by HTN on echo and easily dismissed in patients with traditional risk factors, but presence of CTS or lumbar spinal stenosis can be supportive. Traditionally ATTR is associated with low-voltage EKG, however this patient presented with normal QRS voltages for years, making the diagnosis more elusive. Conclusion: Advances in noninvasive imaging and treatments allow for early diagnosis of cardiac amyloidosis, reducing morbidity and mortality. Diagnosis is often delayed or missed, as features could be subtle and mimic other more common cardiac disease. Screening  99m Tc-PYP for elderly patients with HFpEF could translate to improved outcomes
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