The widespread use of Cardiac Implantable Electronic Devices (CIEDs) has transformed the management of cardiac arrhythmias, improving survival and quality of life for millions. However, this progress has introduced a range of device-related complications, which can significantly impact patient outcomes. This review examines “new diseases” linked to CIEDs, categorizing them into physical (e.g., infections, venous obstruction, lead failure, and device recalls) and functional complications (e.g., arrhythmias, pacemaker syndrome, and left ventricular dysfunction). Prevention and management strategies are emphasized. Emerging technologies, such as leadless devices, quadripolar leads, and remote monitoring systems, hold promise in reducing risks and enhancing patient care. Future directions include integrating artificial intelligence for real-time monitoring, improving device durability, and exploring novel materials to minimize infections and mechanical failures. Understanding CIED-related complications is essential for healthcare providers to balance the benefits and risks of these life-saving technologies.

Background: PRKAG2 cardiac syndrome is a rare autosomal dominant glycogen-storage cardiomyopathy that mimics sarcomeric hypertrophic cardiomyopathy (HCM) but features ventricular pre-excitation, progressive conduction disease and concentric hypertrophy due to intracellular glycogen accumulation. The c.905G>A (p.Arg302Gln) variant is one of the most frequently reported pathogenic substitutions. Case summary: We describe a three-generation family carrying the heterozygous PRKAG2 p.Arg302Gln variant. The proband, a 41-year-old man, presented with paroxysmal atrial fibrillation, short PR interval and abnormal intraventricular conduction associated with concentric left ventricular hypertrophy and preserved ejection fraction. Holter monitoring disclosed episodes of high-grade atrioventricular block, prompting implantation of a primary-prevention dual-chamber ICD. Two gene-positive brothers exhibited milder hypertrophy but shared sinus bradycardia, ventricular pre-excitation and supraventricular arrhythmias; one underwent catheter ablation of a posteroseptal accessory pathway. The affected mother displayed a hypertrophic phenotype complicated by sick sinus syndrome and permanent atypical atrial flutter requiring pacemaker implantation. No relevant extracardiac involvement was detected in any family member. Review and novelty: Using this family as a starting point, we provide a concise narrative review of PRKAG2 syndrome with emphasis on the Arg302Gln genotype, molecular mechanisms and emerging treatment strategies. We highlight key multimodality imaging and tissue-characterization features that help distinguish diffuse, concentric glycogen-storage hypertrophy from the often-asymmetric pattern of sarcomeric HCM. Integration of our findings with published Arg302Gln cohorts illustrates the broad phenotypic variability in conduction disease, pre-excitation and atrial arrhythmias. Conclusions: PRKAG2 p.Arg302Gln-related cardiomyopathy should be suspected in patients with otherwise unexplained left ventricular hypertrophy associated with short PR interval, pre-excitation or early brady–tachy arrhythmias. Early recognition of red-flag features, systematic genetic testing, family screening and tailored arrhythmia/device management are crucial, while emerging gene- and pathway-targeted therapies may offer future disease-modifying potential.

Brugada syndrome (BrS) is a cardiac arrhythmic disorder associated with distinctive electrocardiographic (ECG) abnormalities and an increased risk of sudden cardiac death due to ventricular arrhythmias. While the classic BrS ECG pattern is a coved ST-segment elevation in the right precordial leads, a wide spectrum of atypical ECG presentations can mislead the diagnosis. This review discusses rare and under-recognized ECG findings associated with BrS, including its coexistence with right and left bundle branch block, alterations in peripheral leads and in the morphology of the QRS complex, as well as atrioventricular conduction abnormalities. Emphasis is placed on the clinical relevance of these findings, their underlying electrophysiological mechanisms, and their prognostic implications. Recognizing these atypical manifestations is critical to avoid misdiagnosing or failing to recognize the condition in patients with BrS.