Skip to main content
. 2023 Oct 26;18(1):59. doi: 10.5334/gh.1262

Figure 10.

Complete atrial ventricle block of a patient with genetic ATTR amyloidosis

(A) ECG of a 48-year-old female (African ancestry), showing complete atrial-ventricular block on hospital admission after a syncope. She had mild hypertension, a history of paroxysmal atrial fibrillation, and right carpal tunnel syndrome. No known family history of amyloidosis. (B) ECG after pacemaker implantation. Echocardiographic images: (C) Parasternal longitudinal long-axis (D) Apical 3-chamber views, showing only mild and localized (basal septum) LVH (12 mm). Cardiac magnetic resonance (E-H) was also normal: no cardiac hypertrophy, and (F, H) no late gadolinium enhancement; (I) 99 mTc-3,3-diphosphono-1,2 propanodicarboxylic acid (DPD) scintigraphy (chest images) showed no cardiac uptake three hours after radiotracer administration. Genetic testing identified the pathogenic mutation p.Val142Ile in the TTR gene (no other mutations in a large panel of genes studied by next-generation sequencing). Of her three children (adolescents), two have the mutation (no phenotype). (Images courtesy of Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal).