Quantitative Approach to Fragmented QRS in Arrhythmogenic Cardiomyopathy: From Disease towards Asymptomatic Carriers of Pathogenic Variants.
Fragmented QRS complexes (fQRS) are common in patients with arrhythmogenic cardiomyopathy (ACM). A new method of fQRS quantification may aid early disease detection in pathogenic variant carriers and assessment of prognosis in patients with early stage ACM. Patients with definite ACM (n = 221, 66%), carriers of a pathogenic ACM-associated variant without a definite ACM diagnosis (n = 57, 17%) and control subjects (n = 58, 17%) were included. Quantitative fQRS (Q-fQRS) was defined as the total amount of deflections in the QRS complex in all 12 electrocardiography (ECG) leads. Q-fQRS was scored by a single observer and reproducibility was determined by three independent observers. Q-fQRS count was feasible with acceptable intra- and inter-observer agreement. Q-fQRS count is significantly higher in patients with definite ACM (54 ± 15) and pathogenic variant carriers (55 ± 10) compared to controls (35 ± 5) (p < 0.001). In patients with ACM, Q-fQRS was not associated with sustained ventricular arrhythmia (p = 0.701) at baseline or during follow-up (p = 0.335). Both definite ACM patients and pathogenic variant carriers not fulfilling ACM diagnosis have a higher Q-fQRS than controls. This may indicate that increased Q-fQRS is an early sign of disease penetrance. In concealed and early stages of ACM the role of Q-fQRS for risk stratification is limited.
['Roudijk|Rob W|RW|', 'Bosman|Laurens P|LP|', 'van der Heijden|Jeroen F|JF|', 'de Bakker|Jacques M T|JMT|', 'Hauer|Richard N W|RNW|', 'van Tintelen|J Peter|JP|0000-0003-3854-6749', 'Asselbergs|Folkert W|FW|0000-0002-1692-8669', 'Te Riele|Anneline S J M|ASJM|', 'Loh|Peter|P|']
fragmented QRS arrhythmogenic cardiomyopathy fQRS electrocardiography ventricular arrhythmia genetics inherited cardiomyopathies sudden cardiac death