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  • br Inherited Arrhythmia Clinics br

    2019-04-25


    10. Inherited Arrhythmia Clinics
    The evaluation and treatment of rho kinase inhibitor suspected of having inherited arrhythmias requires a multidisciplinary team and approach. The presentation is often that of a proband or family member who has experienced a life-threatening arrhythmia, sudden cardiac arrest, or SCD. In the usual circumstances, there are profound and far-reaching medical and psychosocial implications of both presentation of the inherited arrhythmia and genetic testing on patients and families. Recent evidence suggests that a structured inherited arrhythmia (or inherited cardiovascular disease) clinic improves rho kinase inhibitor the likelihood of making a diagnosis in suspected cases of inherited arrhythmias and SCD. The promise of an appropriately resourced, structured clinic is that of a comprehensive evaluation of patients and families, more efficient use of diagnostic testing and therapy and ready access to a broad range of medical, genetics, and psychosocial expertise in managing families afflicted by inherited arrhythmias. An inherited cardiovascular disease clinic is an invaluable resource to patients and families not only at the time of the initial evaluation but also in an ongoing fashion as medical, genetic, and social questions relevant to the inherited heart disease arise. There are different operational models for inherited arrhythmia clinics; the choice may be determined by the health system or the regulations that exist in a given country. The personnel and workflow in an ideal inherited arrhythmia clinic is illustrated schematically in Figure 4. The key personnel include a clinic coordinator who is responsible for patient intake, collection and collation of medical records, scheduling appointments for patients and family members, and assisting with questions relating to insurance coverage. The physicians are typically a clinical cardiologist/electrophysiologist with expertise in inherited arrhythmias and medical genetics or a medical geneticist with an interest in cardiac arrhythmias partnering with a clinical electrophysiologist. If the inherited arrhythmia clinic is part of a larger program in inherited heart disease, experts in cardiomyopathy will likely be available; otherwise access to such experts is essential. The increasing complexity and demands of the proper diagnosis and management of patients with inherited cardiovascular disease creates an opportunity for the development of specialized training for clinical electrophysiologists interested in the care of patients with inherited arrhythmias. Such a specialty track would consolidate aspects of care involving indications and interpretation of genetic test results and pharmacological and device therapy. The management of patients with inherited arrhythmias includes expert judgment regarding the indications, type, and interpretation of genetic testing. In collaboration with a genetic counselor, patients and families should be properly prepared regarding expectations and outcomes of genetic testing. Arguably the most important part of the testing procedure is reviewing the test results and implications with patient and family, with counselor being prepared to discuss the implications for other family members, the meaning of variants of uncertain significance, mosaicism, and issues related to paternity and consanguinity. The genetic counselor is an essential and, in some countries, legally mandated provider in this aspect of the care of patients and families with suspected inherited arrhythmias. Patients in an inherited arrhythmia clinic may be survivors of sudden cardiac arrest. The management of the recovery of these patients from their index event may require the expertise of psychologists and psychiatrists, and the intervention of physical and occupational therapists. Moreover, the diagnosis of an inherited disease of any kind, particularly one that carries with it the risk of significant morbidity and premature mortality, is often associated with significant emotional distress that at times will require referral of patients and families.