Volume 114, Nº 4, Supplement, April 2020
DOI: https://doi.org/10.36660/abc.20180130
CASE REPORT
Syncope as a Phenotypic Expression of Hereditary Transthyretin Amyloidosis Val142Ile (Val122Ile)
Nágela S. V. Nunes
João Paulo Moreira Carvalho
Fernanda Salomão Costa
Marcelo Souto Nacif
Joelma Dominato
Claudio Tinoco Mesquita
Evandro Tinoco Mesquita
Figure 1 – A) Electrocardiogram: Sinus rhythm, HR: 88 bpm, indeterminate QRS axis. P-wave in the frontal plane with increased duration (160 ms), with partial Bachmann’s bundle block and tricuspid P wave in D2, D3 and aVF; qR pattern in V1 and Morris index are observed in the horizontal plane, which means right and left atrial enlargement. Pseudo-infarct pattern in precordial leads and presence of low voltage in the frontal plane are also observed. B and C) Resting Cardiac MRI shows diffuse LVH, with areas of diffuse subendocardial late enhancement in the LV (arrows), atria, and interatrial septum. D and E) 99mTc-PYP myocardial scintigraphy showing intense radiotracer uptake in the myocardium (Grade 3)
Keywords: Syncope; Amyloidosis, Familial/genetics; Amyloid Neuropathies, Familial; Cardiomyopathies/diagnosis; Diagnostic, Imaging/methods; Prevalence.
