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
![](images/syncope-as-a-phenotypic-expression-of-hereditary-transthyretin-amyloidosis-val142ile.jpg)
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.