CDMO Flashcards
Fabry Disease
Severe neuropathic or limb pain, which may be precipitated by stress, extremes of heat or cold, and physical exertion; Telangiectasias and angiokeratomas; proteinuria, polyuria, and polydipsia or otherwise unexplained renal insufficiency are common; Cardiac involvement includes concentric left ventricular hypertrophy, heart failure, coronary artery disease, aortic and mitral valve abnormalities, and conduction abnormalities; Cerebrovascular involvement may lead to transient ischemic attacks and strokes,
HCMP clinical manifestation
Dyspnea, Angina, Arrhythmias (AF, VT/VF SAD)
HCMP treatment
negative inotropes/chronotropes (BB, CCB, disopyramide), second line surgical myectomy (if gradient >50mmHg), alcohol septal ablation, transplant if refractory to drugs/nonobstructive pathophysiology.
Acute HF: precipitated by dehydration or tacky: give fluids or BB, phenylephrine
ICD if SCD.
Amyloidosis ECg findings
Decreased QRS amplitude, pseudo infarction pattern with Q’ws, AVB, hemiblock, BBB
Amyloidosis Echo findings
Biventricular wall thickening, yet with low voltage on ECG, granular sparkling texture, biatrial enlargement, thickened atrial septum, valve thickening, diastolic dysfunction, small effusion
Sarcoidosis ECG findings
AVB, RBBB, VT
Sarcoidosis echo
Regional WMA particularly basal septum, with thinning or mild hyper trophy
Etiology of restrictive cardiomyopathy
Autoimmune, illustrative disease, storage disease, DM, chronic eosinophilic, toxins (radiation, anthracyclines), serotonins, metastatic cancer
Restrictive CMO clinical manifestations
Right sided HF > left sided HF, diuretic refractories, thromboembolic events, poorly tolerated tachyarrhythmias, VT induced syncope and SCD
rCMO treatment
Gentle diuresis, control HR, maintain SR, digoxin may precipitate arrhythmias in amyloid, anti coagulation with AF or low CO, transplant for refractory cases
