Although rheumatic heart disease (RHD) is the most common cause of valve disease worldwide, few of these patients were included in randomised trials of TAVI for severe aortic stenosis (AS). Transcatheter aortic valve implantation (TAVI) is an established therapy for symptomatic older adults with severe calcific stenosis of a trileaflet aortic valve. They conclude: ‘In young patients with AVB, it is critical to identify underlying heart disease to determine who is at risk for sudden cardiac death and might benefit from implanted cardiac defibrillator therapy, and to define proper treatment in reversible AVB to prevent unnecessary device implantations and unnecessary right ventricular pacing with the risk of dyssynchronous heart failure.’ However, evaluation for acquired causes of AVB also is important, such as ischaemic heart disease, sarcoidosis, giant cell myocarditis, Lyme myocarditis, Chagas disease or a metabolic disorder. In an editorial, Roseboom and Maass 4 agree that genetic testing may be appropriate in younger patients with AVB because some cases may be related to known pathogenic variants for channelopathies or cardiomyopathies which have implications both for patient management and cascade screening of family members. Risk of AVB was inversely proportional to the age of the index case at time of pacemaker implantation with an adjusted risk ratio of 15.8 (4.8–52.3) if a mother had pacemaker implantation before age 50 years ( figure 2) This finding suggests the possibility of pathogenic genetic variants accounting for AVB in some families. 3 In 26 880 consecutive individuals, first degree relatives of a patients with a pacemaker had a relative risk ratio of 2.1 (95% CI 1.8 to 2.5) for development of AVB compared with the general population. Data were merged from a nationwide cohort of parental-linked individuals with a registry of all pacemaker implantations in Denmark from 1982 to 2019. They conclude: ‘Given patients presenting with symptoms of chronic heart failure have a median age >80 years and a combination of life-limiting comorbidities, should future work fail to demonstrate clear superiority of CRT-D, perhaps the default choice should be CRT-P except where an individual assessment of risk suggests otherwise.’Īnother study in this issue of Heart used a large administrative database to address the question of whether the risk of atrioventricular block (AVB) is hereditary. Of course, many other factors affect the decision about device therapy, including differences between countries and healthcare systems. Also, myocardial scar, which serves as an arrhythmia substrate, is unlikely to be affected by medical therapy. On the other hand, previous studies have shown a reduced risk of sudden death with an implanted defibrillator in similar populations. In addition, left ventricular remodelling with CRT may reduce the risk of sudden death. On the one hand, death in patients with HFrEF may be due to pump failure with electromechanical dissociation or asystole, rather than a ventricular tachyarrhythmias. In the accompanying editorial, Straw and colleagues 2 address the question of why a defibrillator might or might not be essential in these patients.
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