Implantable Cardioverter-Defibrillator Implantation in a Patient with Atrial Standstill

Abstract

We report a 55-year-old female patient who presented with no P waves but with a wide QRS complex escape rhythm at 44 beats/min and prolonged QTc of 0.55 seconds on ECG. The patient had recurrence of ventricular fibrillations and loss of consciousness, and underwent defibrillation and cardiopulmonary resuscitation (CPR) several times because of cardiac arrest. The transthoracic echocardiography showed dilated cardiomyopathy and enlargement of both atria. The Doppler echocardiography documented the absence of A wave in the tricuspid and mitral valve flow. An electrophysiologic study demonstrated electrical inactivity in the right and left atria. Atrial pacing with maximum output did not capture the atria. These findings together with her electrocardiographic finding indicated atrial standstill. Sudden cardiac death was her first clinical manifestation of ventricular arrhythmia. The patient remained asymptomatic after receiving a single chamber implantable cardioverter-defibrillator (ICD) with VVI pacemaker function.

Fever-Induced QTc Prolongation and Ventricular Fibrillation in a Healthy Young Man

“… 10 , 11 Fever-related arrhythmias is commonly reported in Brugada syndrome, which is caused by sodium channel mutation. 12 - 14 However, this patient showed no evidence of Brugada syndrome in repeated provocation test with sodium channel blocker and continuous ECG monitoring for 30 days.…”

“…In contrast to the controversial genetic study, however, a combination of quantified ECG repolarization parameters can identify genotype with sensitivity/specificity of 85%/70% for type 1, 83%/94% for type 2, and 47%/63% for type 3 long QT syndrome 10,11. Fever-related arrhythmias is commonly reported in Brugada syndrome, which is caused by sodium channel mutation 12-14. However, this patient showed no evidence of Brugada syndrome in repeated provocation test with sodium channel blocker and continuous ECG monitoring for 30 days.…”

Wszczepiono CRT-P i co dalej…

“…6). Można się zastanowić, czy pacjentowi nie należało pierwotnie wszczepić CRT-D [8]. Omówienie Dystrofia Emery'ego-Dreifussa jest rzadko spotykaną chorobą genetyczną.…”

A homozygous SCN5A mutation associated with atrial standstill and sudden death

“…Atrial standstill can be persistent or transient, diffuse, or partial. Most reported cases are secondary to Ebstein's anomaly, myocarditis, muscular dystrophies, amyloidosis, or ischemic heart disease . Genetic studies of atrial standstill are rare.…”

“…1 The channel consists of a transmembrane pore-forming -subunit (Na V 1.5), myocarditis, muscular dystrophies, amyloidosis, or ischemic heart disease. 7,8 Genetic studies of atrial standstill are rare. A few familial cases have reported atrial standstill to be associated with novel SCN5A variants, but these cosegregate with genetic variation or deletions in other genes, such as GJA5 (connexin 40) or RYR2.…”