Characteristics and Outcomes of Patients with Cardiac Amyloidosis Requiring Orthotopic Heart Transplantation

Abstract

<h3>Purpose</h3> It has been suggested that patients with cardiac amyloidosis can have similar outcomes post orthotopic heart transplantation (OHT) compared to non-amyloid cardiomyopathies. Our aim is to report characteristics and outcomes after OHT in patients with cardiac amyloidosis in a large single-center quaternary university hospital. <h3>Methods</h3> We performed a retrospective chart review of all patients with AL amyloid who underwent OHT from January 2017 to October 2021. Kaplan-Meier method was used to assess time to death compared to non-amyloid cardiomyopathies. <h3>Results</h3> Analytic sample was 280 patients, with a median follow up of 1.86 years. We had 16 patients with amyloid, aTTR amyloid was the more prevalent (50%), followed by AL (44%) and one case of ApoA1. Our cohort had a male predominance (75%), with a mean age of 61.7 years. Carpal tunnel was the most prevalent extracardiac involvement (56%), followed by renal (31%), peripheral neuropathy (31%), autonomic neuropathy (19%) and gastrointestinal (19%). Mean Vo2 max was of 14ml/min/kg; the median time on the waiting list was 35 days. The pathological light chain was Lambda in 86% of those with AL with a median difference in free light chains of 280 mg/dL. In patients with ATTR, wild type was the most prevalent (63%) compared to 38% with the V122I mutation. We did not observe mortality, graft failure or episode of antibody-mediated rejection. There was no statistical difference in survival at 3-years between amyloid (100%) and non-amyloid (93.4%) patients, Wilcoxon p-value = 0.32. Acute cellular rejection requiring treatment was observed in 2 patients, with a mean time to rejection of 100 days. Multifactorial progression of nephropathy was seen in 31% of patients. Progression of neuropathy was seen in 25% of patients and only one patient reported GI symptom progression. <h3>Conclusion</h3> Patients with cardiac amyloid undergoing OHT with limited extracardiac involvement had comparable survival to patients with non-amyloid cardiomyopathies.