Dilated cardiomyopathy (DCM) is a major cause of advanced heart failure requiring a left ventricular assist device (LVAD) or heart transplantation. Although implantation of an LVAD ameliorates end-organ dysfunction and improves exercise tolerance, right heart failure (RHF) after LVAD implantation remains a major unsolved problem.
1The incidence of RHF in the late phase after LVAD implantation has been reported to be 8-11% and associated with a poor prognosis.
- Teuteberg JJ
- Cleveland Jr, JC
- Cowger J
- Higgins RS
- Goldstein DJ
- Keebler M
- Kirklin JK
- Myers SL
- Salerno CT
- Stehlik J
- et al.
The Society of Thoracic Surgeons Intermacs 2019 Annual Report: The Changing Landscape of Devices and Indications.
Ann Thorac Surg. 2020 Mar; 109: 649-660
2Therefore, it is important to predict the development of late RHF after LVAD implantation. We hypothesized that genetic factors are involved in the development of late RHF after LVAD implantation and examined whether Lamin A/C (LMNA) mutations, which cause DCM with a severe phenotype,
- Rich JD
- Gosev I
- Patel CB
- Joseph S
- Katz JN
- Eckman PM
- Lee S
- Sundareswaran K
- Kilic A
- Bethea B
- et al.
The incidence, risk factors, and outcomes associated with late right-sided heart failure in patients supported with an axial-flow left ventricular assist device.
J Heart Lung Transplant. 2017 Jan; 36: 50-58
3are associated with late RHF and poor prognosis after LVAD implantation.
- Hasselberg NE
- Haland TF
- Saberniak J
- Brekke PH
- Berge KE
- Leren TP
- Edvardsen T
- Haugaa KH.
Lamin A/C cardiomyopathy: young onset, high penetrance, and frequent need for heart transplantation.
Eur Heart J. 2018 Mar 7; 39: 853-860
Abbreviations:DCM (dilated cardiomyopathy), LVAD (left ventricular assist device), RHF (right heart failure), RVSWI (right ventricular stroke work index), PAPi (pulmonary artery pulsatility index)
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- The Society of Thoracic Surgeons Intermacs 2019 Annual Report: The Changing Landscape of Devices and Indications.Ann Thorac Surg. 2020 Mar; 109: 649-660
- The incidence, risk factors, and outcomes associated with late right-sided heart failure in patients supported with an axial-flow left ventricular assist device.J Heart Lung Transplant. 2017 Jan; 36: 50-58
- Lamin A/C cardiomyopathy: young onset, high penetrance, and frequent need for heart transplantation.Eur Heart J. 2018 Mar 7; 39: 853-860
- Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology.Genet Med. 2015 May; 17: 405-424
- Natural history of dilated cardiomyopathy due to lamin A/C gene mutations.J Am Coll Cardiol. 2003 Mar 5; 41: 771-780
Accepted: January 27, 2023
Received in revised form: January 25, 2023
Received: June 25, 2022-LMNA mutations and Right Heart Failure-
Publication stageIn Press Journal Pre-Proof
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