Raymond C Givens1, Chris Russo, Philip Green, Mathew S Maurer. 1. Clinical Cardiovascular Research Laboratory for the Elderly, Center for Advanced Cardiac Care, Division of Cardiology, Columbia College of Physicians & Surgeons, 622 West 168th Street, PH 12-1291, NY, USA.
Abstract
AIMS: In the USA, transthyretin cardiac amyloidosis usually results from 'wild-type' transthyretin (senile cardiac amyloidosis [SCA]) or the V122I variant. PATIENTS & METHODS: We compared presentations and outcomes among SCA and V122I patients referred to the Center for Advanced Cardiac Care at Columbia University Medical Center (NY, USA) between 2001 and 2012. RESULTS: V122I patients were younger (mean: 71 years, standard deviation [SD]: 7) than SCA patients (mean: 77, SD: 6; p = 0.0002) and 96% were black compared with 3% of SCA patients (p < 0.0001). Average ejection fraction was lower among V122I patients (mean: 25% [SD: 12] vs mean: 47% [SD: 15]; p = 0.0001), as was mean cardiac index. Median time to death or orthotopic heart transplant was 36.4 months for V122I patients and 66.5 for SCA patients (p = 0.09). CONCLUSION: In this study of patients with transthyretin cardiac amyloidosis, V122I patients presented to a tertiary academic medical center at a younger age than SCA patients but had higher levels of cardiac dysfunction, despite genetic screening availability. There was a trend toward shorter time to orthotopic heart transplant or death among V122I patients. Whether this is a result of a different biologic progression or late diagnosis requires further study.
AIMS: In the USA, transthyretincardiac amyloidosis usually results from 'wild-type' transthyretin (senile cardiac amyloidosis [SCA]) or the V122I variant. PATIENTS & METHODS: We compared presentations and outcomes among SCA and V122Ipatients referred to the Center for Advanced Cardiac Care at Columbia University Medical Center (NY, USA) between 2001 and 2012. RESULTS:V122Ipatients were younger (mean: 71 years, standard deviation [SD]: 7) than SCApatients (mean: 77, SD: 6; p = 0.0002) and 96% were black compared with 3% of SCApatients (p < 0.0001). Average ejection fraction was lower among V122Ipatients (mean: 25% [SD: 12] vs mean: 47% [SD: 15]; p = 0.0001), as was mean cardiac index. Median time to death or orthotopic heart transplant was 36.4 months for V122Ipatients and 66.5 for SCApatients (p = 0.09). CONCLUSION: In this study of patients with transthyretincardiac amyloidosis, V122Ipatients presented to a tertiary academic medical center at a younger age than SCApatients but had higher levels of cardiac dysfunction, despite genetic screening availability. There was a trend toward shorter time to orthotopic heart transplant or death among V122Ipatients. Whether this is a result of a different biologic progression or late diagnosis requires further study.
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