Sudden cardiac death (SCD) in an apparently healthy individual is a tragedy. It is important to identify the cause of death and to prevent SCD in potentially at-risk family members. Inherited primary arrhythmia disorders are associated with exercise-related SCD. Despite the well-known benefits of exercise, exercise restriction has been a historical mainstay of therapy for these conditions. However, since familiarity with inherited arrhythmia conditions has increased and patients are often children and young adults, it is necessary to reassess the treatment guidelines regarding exercise constraints. The aim of this review is to analyze the risk of exercise-induced SCD in patients with inherited cardiac conditions and explore the challenges faced when advising patients about exercise limitations. We searched for publications on cardiac channelopathies in PubMed with the following medical subject headings (MeSH): "long QT syndrome"; "short QT syndrome"; "Brugada syndrome"; and "catecholaminergic polymorphic ventricular tachycardia". The abstracts of these articles were scanned, and articles of relevance, along with pertinent references, were read in full. The analysis was restricted to reports published in English. The findings of this analysis suggest that exercise with low-to-moderate cardiovascular demand may be possible under regular clinical follow-up in inherited primary arrhythmia disorders. Recent data show that patients with inherited primary arrhythmia disorders are at low risk for events once a comprehensive treatment program has been established. Recreational activity is likely safe for these individuals, with personalized management based on individual patient preferences and priorities.
Sudden cardiac death (SCD) is the most devastating complication of hypertrophic cardiomyopathy (HCM). The greatest challenge in the management of HCM is identifying those at increased risk, since an implantable cardioverter-defibrillator (ICD) is a potentially life-saving therapy. We sought to summarize the available data on SCD in HCM and provide a clinical perspective on the current differing and somewhat conflicting data on risk stratification, with balanced guidance regarding rational clinical decision-making. Additionally, we sought to determine the status of the current implementation of guidelines compiled by HCM experts worldwide. The HCM Risk-SCD model helps improve the risk stratification of HCM patients for primary prevention of SCD by calculating an individual risk estimate that contributes to the clinical decision-making process. Improved risk stratification is important for decision-making before ICD implantation for the primary prevention of SCD.
Left ventricular aneurysms (LVA) are mainly a late consequence of transmural myocardial infarction. Approximately 80% of LVA are located in the anterior and/or apical walls, most commonly associated with left anterior descending artery occlusion but any region may be engaged. Basal inferior wall aneurysms are rare and constitute nearly 3% of all LVA. A calcified LVA is seldom observed in modern clinical practice. And a calcified basal inferior LVA is an even rarer coincidence. We report a case of an 82-year-old women with life threatening arrhythmia revealing a giant calcified aneurysm of the basal inferior wall, medically treated with good outcomes. The exact incidence of left ventricular aneurysms (LVA) following myocardial infarctions is hard to precise but it is clearly decreasing. Eighty percent (80%) of LVA are located in the anterior or apical walls, but any region may be engaged. Basal inferior wall aneurysms constitute 3% of all LVA. Echocardiography is the first diagnostic tool and there is still no clear guidelines on how to treat LVAs. Surgery is preferred but medical treatment may help improve the quality of life.
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