2:1 electronic AV block due to inappropriate automatic post­ventricular atrial refractory period extension.

A 16-year-old female with dual-chamber pacemaker (Medtronic Azure XT DR), due to symptomatic third-degree congenital atrioventricular (AV) block, presented to our ambulatory with dizziness and presyncopal episodes preceded by prodromes, occurring over the last few months. The device was programmed in DDD mode with an upper rate of 150 bpm. A head-up Tilt Test (HUTT) revealed the unexpected emergence of 2:1 electronic AV block at a sinus rate of 130 bpm.

Common ECG interpretation software mistakes Part III: Computer errors that should never be missed.

Electrocardiogram interpretation software mistakes can lead to incorrect diagnoses and inappropriate treatments. Occasionally, the consequences of not recognizing such mistakes are disastrous. This final chapter on software mistakes describes three relatively common computer errors that should never be missed because not recognizing them can result in stroke, cardiac arrest, and even death. In each of the scenarios covered, we describe the clinical background, and provide simple recommendations on how such mistakes can be easily identified and corrected.

Novel Phenotypic Effects of a Rare SCN5A (c.2482C>T) Mutation.

In a familial cohort with 8 heterozygous carriers of a rare pathogenic SCN5A mutation (c.2482C>T), 4 female mutation carriers manifested with fetal ventricular tachycardia and 2:1 atrioventricular block. One presented with multifocal ectopic premature Purkinje-related complexes-like phenotype and atrial fibrillation later in life. These novel findings inform the need for robust fetal monitoring of mutation carriers.

Pseudo 2:1 AV Block From Acquired Long QT Syndrome From Eating Clay.

Age-related degeneration of the cardiac conduction system is an important cause of heart block and a leading indication for permanent pacemaker implantations. However, variations in the myocardial refractory periods from QT interval prolongation or concealed atrioventricular node penetration from premature ventricular complexes can lead to unusual forms of conduction block not requiring permanent pacemaker implantation. (Level of Difficulty: Advanced.).

Second degree heart block associated with QT prolongation.

Pseudo 2:1 AV block when sinus intervals are shorter than the ventricular refractory period has been reported with long QT syndrome (LQTS). We report the characteristics and treatment of a patient suffering from congenital LQTS with episodes of true 2:1 AV block. The pseudo 2:1 AV block relates to the extreme prolongation of ventricular refractoriness. Several histologic studies have documented abnormalities within the conduction system, including apoptosis. Because of the rare occurrence and poor prognosis of the LQTS with impaired AV conduction, international guidelines for diagnosis and treatment are needed.