Stroke in a Young Individual with Left Ventricular Noncompaction and Left Atrium Standstill

Isolated left ventricular noncompaction (LVNC) is a rare cardiomyopathy with morphologic characteristics of two distinct myocardial layers i.e., thin compacted epicardial and thick noncompacted endocardial layers. The noncompacted myocardium consists of prominent ventricular trabeculae and deep intertrabecular recesses. It can lead to arrhythmias, heart failure or systemic embolisms. Electrocardiographic patterns of patients with LVNC are various and non-specific; however, the most common findings are intraventricular conduction delay, left ventricular hypertrophy, and repolarization abnormalities. We reported the first case, to the best of our knowledge, of a 29-year-old man who had recent cerebral infarction and incidental LVNC with spontaneous left atrial standstill.

Atrial standstill treated with implantation of permanent pacemaker / 대한내과학회지

Persistent atrial standstill is an extremely rare arrhythmia that was first described by Chavez et al. Electrocardiographically, atrial standstill is characterized by bradycardia, the absence of a P wave, and a junctional narrow complex escape rhythm. Atrial standstill is usually classified into two types. The transient type is observed in drug intoxication, such as with digitalis or quinidine, and hyperkalemia. The persistent type is uncommon, often accompanied by syncopal attacks or brain embolism. We report a case of persistent atrial standstill in an 83-year-old man who was treated with implantation of a permanent pacemaker.

Implantable Cardioverter-Defibrillator Implantation in a Patient with Atrial Standstill

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.

Emery-Dreifuss Muscular Dystrophy with Cardiac Involvement

Emery-Dreifuss muscular dystrophy has become recognized as a distinct neuromuscular disorder with features including X-linked inheritance, insidious onset in childhood of a distinct pattern of muscle contractures and weakness, slow progression without loss of ambulation, and occurrence by mid-childhood of atrial conduction defects, which, if untreated, cause sudden death. We report a case of Emery-Dreifuss dystrophy with cardiac involvement of atrial standstill. The patient was 24 year-old man, who had suffered from dyspnea and bradycardia and was inserted by VVI type permanent pacemaker. Cardiac involvement usually becomes evident as muscle weakness progress and provided that the diagnosis is made sufficiently early, the insertion of a cardiac pacemaker can be life saving.