Aborted Sudden Death Due to Severe Ventricular Arrhythmia in Timothy Syndrome
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 2:1 AV block, was also identified, requiring a definitive cardiac pacemaker implant that, during inpatient care, developed pulmonary sepsis, followed by death.
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Format: | Digital revista |
Language: | English |
Published: |
Sociedade Brasileira de Cardiologia
2021
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Online Access: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2359-56472021005004202 |
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