984 ARRHYTHMOGENIC DYSPLASIA: JUST A PROBLEM OF THE YOUNG?


 Arrhythmogenic dysplasia is a disease of genetic aetiology characterised by the progressive replacement of functioning myocardium by fibro-adipose tissue with high arrhythmogenic potential, as well as by morpho-functional impairment. Molecular genetic studies have revealed a deficiency of demosomal proteins such as placoglobin, desmoglein, desmoplazine, placophylline and desmocollin. It usually begins at a young age and in sports subjects. It mainly affects the right ventricle, although over time it can also affect the left sections due to a biventricular alteration of the heart pump. Forms with major left-sided involvement have been described in the literature, but these are rare.
 A 73-year-old man, a sportsman with over 40 marathons, presented with an episode of sustained VT during exercise in 2011. Admitted to our hospital, he was diagnosed with arrhythmogenic cardiomyopathy and was subsequently implanted with an ICD-DR mod.Teligen 100F. After 3 months, however, there was a new episode of VT, so it was decided to subject the patient to transcatheter ablation with radiofrequency, using an endocardial approach, which proved ineffective. Two months later, a new attempt at radioablation with an endocardial approach. Due to the persistence of arrhythmic symptoms, sustained VT episodes, in February 2012 it was decided to proceed with a third ablation, but adopting an endo-epicardial approach, with apparent resolution of the symptomatology. After severals episodes of atrial fibrillation, he underwent a new ablation with pulmonary vein isolation and ablation line at the level of the cavo-tricuspid isthmus in August 2020. On echocardiogram, the left ventricle had normal dimensions with upper limit thicknesses, diffuse parietal hypokinesis with markedly reduced EF (30%), dilated right ventricle (DTD 48 mm) with parietal hypertrabeculature and free wall hypokinesis (TAPSE 1.4) and dilated right atrium. In May 2020 new relapse of atrial fibrillation successfully cardioverted, maintenance therapy set up with sotalol.
 In the literature, mainly forms of arrhythmogenic dysplasia in youth (30 years) have been described, whereas older forms appear to be rare. A search of the Medline database (National Library of Medicine, Bethesda, MD) found 5 cases of ARVD among the elderly; all these patients had died and were diagnosed by histology. The diagnosis is multi-parametric and the main aim of therapy is the prevention of sudden cardiac death. Currently, ICD defibrillator implantation is the only effective therapy for the prevention of sudden death. New drug therapies, radiofrequency ablation and defibrillators are likely to have a favourable impact on the survival of these patients and make it not unlikely that elderly patients with ARVD will be present. The possibility of a late onset of the disease should also be suspected.