Congenital Long QT Syndrome (CLQTS) is the most common inherited arrhythmia. The QT interval, which marks the duration of ventricular depolarization and repolarization in the myocardium, can be prolonged due to mutations in genes coding for the ion channel proteins that govern the cardiac action potential. The lengthening of the QT interval can lead to a wide range of clinical symptoms, including seizures, torsades de pointes, and fatal arrhythmias. There is a growing body of evidence that has revealed the genetic mutations responsible for the pathophysiology of CLQTS, and this has led to hypotheses regarding unique triggers and clinical features associated with specific gene mutations. Epidemiologic evidence has revealed a 1-year mortality rate of approximately 20% in untreated CLQTS patients, and a <1% of 1-year mortality rate in treated patients, underscoring the importance of timely diagnosis and effective clinical management. There are many phenotypic syndromes that constitute CLQTS, including but not limited to, Jervell and Lange-Nielsen syndrome, Romano and Ward syndrome, Andersen-Tawil syndrome, and Timothy syndrome. In this review, we aim to (1) summarize the genetic, epidemiologic, and pathophysiological basis of CLQTS and (2) outline the unique features of the phenotypic subtypes and their clinical management.

IntroductionThe presence of chronic obstructive pulmonary disease (COPD) can impact the management of acute myocardial infarction (AMI) and is associated with higher mortality. Few studies addressed COPD impact on heart failure hospitalisations (HFHs) in AMI survivors.Material and methodsAdult survivors of an AMI between January and June 2014 were identified from the US Nationwide Readmissions Database. The impact of COPD on HFH within 6 months, fatal HFH and the composite of in-hospital HF or 6-month HFH was studied.ResultsOf 237,549 AMI survivors, patients with COPD (17.5%) were older, more likely female, had a higher prevalence of cardiac comorbidities and a lower coronary revascularization rate. In-hospital HF was more frequent in patients with COPD (47.0% vs. 25.4%; p < 0.001). HFH within 6 months occured in 12,934 (5.4%) patients, at a 114% higher rate in patients with COPD (9.4% vs. 4.6%, OR = 2.14, 95% CI : 2.01–2.29; p < 0.001), which was attenuated to a 39% higher adjusted risk (OR = 1.39, 95% CI: 1.30–1.49). Findings were consistent across subgroups of age, AMI type, and major HF risk factors. Mortality during a HFH (5.7% vs. 4.2%, p < 0.001) and the rate of the composite HF outcome (49.0% vs. 26.9%, p < 0.001) were significantly higher in patients with COPD.ConclusionsCOPD was present in 1 of 6 AMI survivors and was associated with worse HF related outcomes. The increased HFH rate in COPD patients was consistent across several clinically relevant subgroups and these findings highlight the need for optimal in-hospital and post-discharge management of these higher-risk patients.

With increases in life expectancy and the size of the aging population, cognitive decline and neurodegenerative pathologies are expected to increase in the next few decades. Age-related increases in risk for dementia and cardiovascular disease have been researched widely. Epidemiology trends reveal a predicted increase of neurodegenerative disease to more than 65 million by 2030 in the United States. There are several risk factors for the development of cardiovascular disease that have been widely studied for their impact on dementia; such as: diabetes, hypertension, and hyperlipidemia. Several pathophysiologic mechanisms exist by which cardiovascular disease could impact dementia including cerebral hypoperfusion, reactive oxidative species, and increased cleavage of amyloid precursor protein into amyloid beta plaques and accumulation of neurofibrillary tangles. Emerging evidence also suggests that treatment of cardiovascular disease risk factors could reduce the risk of dementia development. In this review, we seek to examine the relationship between cardiovascular disease and dementia by examining epidemiologic trends, common risk factors, pathophysiologic mechanisms and implications for clinical management.

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