Aborted Sudden Death Due to Severe Ventricular Arrhythmia in Timothy Syndrome
Int. j. cardiovasc. sci. (Impr.)
; 34(6): 743-746, Nov.-Dec. 2021. graf
Article
in En
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LILACS-Express
| LILACS
| ID: biblio-1421745
Responsible library:
BR1.1
ABSTRACT
Abstract Timothy Syndrome is a rare autosomal dominant multisystem genetic condition. The CACNA1C gene, codifier of the CaV1.2 calcium channel, is affected, resulting in the loss of voltage-dependent calcium channel inactivation. Relevant clinical characteristics (1) corrected QT interval greater than 480ms; (2) syndactyly. Death often occurs during childhood, and results from ventricular tachyarrhythmias. This study presents the case of a female newborn who suffered a cardiorespiratory arrest, secondary to ventricular arrhythmia. A prolonged QT interval, combined with 21 AV block, was also identified, requiring a definitive cardiac pacemaker implant that, during inpatient care, developed pulmonary sepsis, followed by death.
Full text:
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Index:
LILACS
Type of study:
Prognostic_studies
Language:
En
Journal:
Int. j. cardiovasc. sci. (Impr.)
Journal subject:
CARDIOLOGIA
Year:
2021
Type:
Article