Adopting a Collaborative Strategy to Address the Complexities of Implanting a Subcutaneous Implantable Cardiac Defibrillator for Secondary Prevention in a Patient With Fabry Disease and Motor Neuron Disease

A man in his mid-40s, diagnosed with motor neuron disease (MND) and Fabry disease (FD), was sent to cardiology after experiencing a cardiac arrest at home secondary to ventricular fibrillation (VF). Fabry disease is a rare X-linked inherited lysosomal storage disorder caused by deficient alpha-galactosidase A (AGAL-A) activity that leads to an accumulation of globotriaosylceramide (Gb3) in affected tissues, including the heart. Motor neuron disease is an uncommon condition that progressively damages parts of the nervous system. He experienced a positive neurological recovery, enabling the cardiology team to investigate the cause of his arrest. Despite his considerable coexisting health issues and reliance on medical and non-medical interventions, he seemed to enjoy a good quality of life. By working together, the multidisciplinary team, alongside the patient and their family, reached an agreement on the treatment offered. In light of his ongoing medical conditions, the patient underwent a subcutaneous implantable cardioverter defibrillator (S-ICD) implantation to reduce the risk of infection while ensuring he got the relevant protection in the event of further life-threatening ventricular arrhythmia. He had a successful recovery, maintaining his quality of life and independence. Here, we describe the challenges involved in ensuring the best interest decision was taken, which required extensive collaboration from various specialties following a cardiac arrest.