mutation in the TTR(transthyretin)can cause familial amyloid cardiomyopathy
Transthyretin get misfolded, producing an
amyloid protein that infiltrates the myocardium (infiltrative cardiomyopathy).
(*TTN → encodes titin= anchors β-myosin heavy chain to z-disc, contributing to passive myocardial tension) cardiovascularUWorld Mar 28, 2020, 8:10 AM HirotoShishido
pathogenesis/triggers of Prinzmetal(Variant, vasospastic) anginahyperactivity of coronary smooth muscle
due to ☆endothelial dysfunction & autonomic dysfunction
☆dihydroergotamine, triptan(both for migrane), cocaine, amphetamine
triptan ☆blocks calcitonine gene-related peptides(CGRP), decrease neurologic lnflammation and work as vasoconstrictor
sacubitril
mechanisminhibit neprilysin
can be used in combination with valsartan
(Sacubitril is found to decrease mortality and morbidity of patients with chronic heart failure with decreased ejection fraction) pharmacologycardiovascular Dec 31, 2019, 4:28 AM HirotoShishido
neprilysin
mechanism, hypothermia☆metalloprotease that inactivates several peptide hormones including ☆ANP, BNP, glucagon, substance P etc.
hypothermia→
central blood volume increases→ baroreceptors (will register this relative increase as volume overload and) increase the rate of firing→
downregulate ADH ,neprilysin→
↓ADH, ↓neprilysin, ↑ANP, ↑BNP→
increase urinary frequency cardiovascularendocrinology Dec 31, 2019, 3:46 AM HirotoShishido
