Biventricular Arrhythmogenic Cardiomyopathy Associated with a Novel Heterozygous Plakophilin-2 Early Truncating Variant.

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a hereditary condition that can cause sudden cardiac death in young, frequently athletic individuals under the age of 35 due to malignant arrhythmias. Competitive and endurance exercise may hasten the onset and progression of ARVC, leading to right ventricular dysfunction and potentially fatal ventricular arrhythmias earlier in life. In this article, we present a novel, pathogenic, early truncating heterozygous variant in the PKP2 gene that causes biventricular arrhythmogenic cardiomyopathy and affects a family, of which the only member with the positive phenotype is a competitive endurance athlete.

A significant increase in exercise test performance with virtual group motivation: a randomised open-label controlled trial.

AIMS: Exercise stress testing is frequently used for the assessment of coronary artery disease. As the validity of the test result is highly dependent on the patient’s cooperation and motivation, we hypothesised that virtual group motivation would result in a higher exercise capacity and may increase the test’s validity. METHODS: 108 patients at a Swiss teaching hospital with an indication for exercise testing were included in a controlled, open-label trial and randomised 1:1 to treadmill exercise testing whilst either watching a video of a walking group (video group, n = 43), or watching a static image of flowers (image group, n = 43). The video showed a group of five amateur runners, giving the patients the impression of running within the group. As primary outcomes, the performance achieved and the perceived level of comfort during the test were analysed. RESULTS: The video group achieved significantly higher percentages of their age-predicted METs (149 ± 32% vs 135 ± 29%, p = 0.041) and longer exercise durations (11:12 ± 2:54 min vs 08:54 ± 02:39 min, p <0.001). Levels of comfort (8.4 ± 1.4 vs 7.5 ± 1.7 analogue scale, p = 0.011) and closeness to their physical limits (8.9 ± 0.8 vs 8.1 ± 1.5, p = 0.005) were rated significantly higher by patients in the video group. CONCLUSIONS: Patients watching a video of a running group achieved significantly higher maximum exercise levels and longer test durations. This may have implications for the test’s validity. Furthermore, the virtual setting enhanced patient comfort. (This trial was formally registered at clinicaltrials.gov: trial ID NCT03704493.).