Comprehensive cardiac evaluation was performed on a 75-year-old African American woman referred to our hypertrophic cardiomyopathy (HCM) centre for refractory diastolic heart failure. The patient carried a diagnosis of non-obstructive HCM for several years and had a daughter with a diagnosis of HCM. A 12-lead electrocardiogram (Panel A) showed sinus bradycardia without left ventricular (LV) hypertrophy. Transthoracic echocardiogram (Panels B–D, see Supplementary data online, Videos S1Supplementary data online, Videos S1–S4S4) showed thickened LV (anterior septum 23 mm, posterior septum 30 mm) without any resting or provocable LV outflow obstruction and abnormal global longitudinal strain (−13%) with relative apical sparing. Additional imaging with cardiac magnetic resonance imaging (Panels E–G) demonstrated substantial, patchy, and mid-myocardial delayed enhancement (25% of the myocardium, arrows, Panel G). The myocardium was notably difficult to null. Given the available information, infiltrative cardiomyopathy was suspected (amyloid), and a subsequent 99mTc-pyrophosphate scan suggested cardiac amyloidosis [heart/contralateral [CL] counts >1.5; Panels H (1 h) and I (3 h)]. Endomyocardial biopsy and Congo red stain (Panels J and K) confirmed amyloid fibrils infiltrating the myocardium. Genetics for HCM panel subsequently demonstrated a typical mutation for amyloidosis in African Americans [heterozygous for pathogenic Val122Ile mutation in transthyretin (TTR) gene].
from #AlexandrosSfakianakis via Alexandros G.Sfakianakis on Inoreader http://ift.tt/2ofX8lJ
via IFTTT
Εγγραφή σε:
Σχόλια ανάρτησης (Atom)
Δημοφιλείς αναρτήσεις
-
Publication date: Available online 4 January 2018 Source: European Journal of Radiology Author(s): Peiyao Zhang, Jing Wang, Qin Xu, Zhen...
-
Medicine by Alexandros G. Sfakianakis,Anapafseos 5 Agios Nikolaos 72100 Crete Greece,00302841026182, Butyric Acid from Probiotic Staphyloco...
-
Medicine by Alexandros G. Sfakianakis,Αλέξανδρος Γ. Σφακιανάκης A Novel Technique for Endoscopic Repair of Large Anterior Skull Base Defect...
-
2016-11-22T07-55-59Z Source: International Journal of Medical Science and Public Health Banothu Srinivas, Madhu Mohan Reddy B. Backgrou...
-
2016-10-15T06-30-01Z Source: The Southeast Asian Journal of Case Report and Review Sangita Deepak Kamath, Neeraj Jain, Saurabh Pathak, Ba...
-
BACKGROUND AND PURPOSE: Lesion load is a common biomarker in multiple sclerosis, yet it has historically shown modest association with cl...
-
Abstract The development of focused ion beam-scanning electron microscopy (FIB-SEM) techniques has allowed high-resolution 3D imaging of n...
-
Vol.48 No.2 from #AlexandrosSfakianakis via Alexandros G.Sfakianakis on Inoreader http://ift.tt/1S2Z7n2 via IFTTT
-
Does CBD Oil Lower Blood Pressure? This article was originally published at SundayScaries." Madeline Taylor POSTED ON January 13, 20...
Δεν υπάρχουν σχόλια:
Δημοσίευση σχολίου