Homozygous Familial Hypercholesterolemia: Anesthetic Challenges and Review of Literature.
Autor: Ladha, Suruchi; Makhija, Neeti; Kiran, Usha; Aarav, Sudheer K.
Publication year: 2020
World journal for pediatric & congenital heart surgery
issn:2150-136X 2150-1351
doi: 10.1177/2150135117702127
Abstract:
Homozygous familial hypercholesterolemia (HoFH; Fredrickson IIa) is a rare autosomal dominant hereditary disease associated with increased low-density lipoprotein cholesterol. We hereby report anesthetic challenges in a rare case of HoFH having severe progressive aortic stenosis, mitral regurgitation, diffuse aortic and carotid vessel involvement, and severe left ventricular dysfunction. The patient underwent a Konno aortoventriculoplasty with mechanical prosthesis along with mitral valve repair.
Language: eng
Rights:
Pmid: 28952401
Tags: Humans; Male; Child; Computed Tomography Angiography; Echocardiography; Cardiac Surgical Procedures/*methods; Echocardiography, Transesophageal; Aortic Valve/diagnostic imaging/*surgery; Anesthesia/*methods; anesthetic challenges; Aortic Valve Stenosis/diagnosis/etiology/*surgery; calcification; Calcinosis/diagnosis/etiology/*surgery; cardiovascular; homozygous familial hypercholesterolemia; Hyperlipoproteinemia Type II/*complications/diagnosis
Link: https://pubmed.ncbi.nlm.nih.gov/28952401/