J Cardiothorac Vasc Anesth
June 2023
Unlabelled: To characterize patient preferences for medical surrogate decision-makers in the ICU to capture the complexity of decision-making preferences and highlight potential conflicts between patients' preferences and clinicians' surrogate decision-maker identification in usual clinical practice.
Design: Prospective qualitative cross-sectional study.
Setting: Two ICUs in a quaternary referral center in the eastern United States.
Familial hypertrophic cardiomyopathy (HCM), linked to mutations in myosin, myosin-binding proteins and other sarcolemmal proteins, is associated with increased risk of life threatening ventricular arrhythmias, and a number of animal models have been developed to facilitate analysis of disease progression and mechanisms. In the experiments here, we use the αMHC mouse line in which one αMHC allele harbors a common HCM mutation (in βMHC, Arg403 Gln). Here, we demonstrate marked prolongation of QT intervals in young adult (10-12week) male αMHC mice, well in advance of the onset of measurable left ventricular hypertrophy.
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