Advanced Heart Failure in Friedreich's Ataxia: A Story of Challenges, Opportunities, and Hope
Satoshi Miyashita1, Anthony Zaki2, Kaitlin Schlabach1
1Department of Cardiovascular Medicine, Section of Heart Failure & Cardiac Transplantation, Cleveland Clinic, Cleveland, Ohio, USA.
Background:
Heart failure is one of the leading causes of death in patients with Friedreich's ataxia (FRDA).
Case Summary:
We report a 39-year-old man with late-onset FRDA and advanced heart failure who presented with cardiogenic shock and underwent heart transplantation after being supported with an axillary intra-aortic balloon pump for 24 days. At 2.5 years of follow-up, he remains ambulatory, lives at home, and has had no readmissions. One year after heart transplantation, omaveloxolone treatment was initiated.
Discussion:
Ambulatory diagnosis of stage D heart failure in a patient with FRDA facilitated a timely multi-institutional and multidisciplinary evaluation that made heart transplantation a treatment option. Heart replacement therapy enabled the initiation of omaveloxolone.
Take-Home Messages:
The neurological and cardiovascular trajectories of patients with FRDA are heterogeneous. A multidisciplinary approach and early referral for evaluation of advanced heart failure therapies are essential to optimize outcomes.
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