Chronic thromboembolic pulmonary hypertension secondary to implantable cardioverter defibrillator lead thrombus in a patient with Brugada syndrome: a rare complication requiring a multidisciplinary approach.

We report the case of a 57-year-old male patient with prior syncope associated with sustained ventricular tachycardia in the setting of Brugada syndrome, who was submitted to implantation of a cardioverter defibrillator for secondary prevention. During follow-up, he presented a significant increase in lead impedance, and a transthoracic echocardiogram showed a mass attached to the lead. He was started on oral anticoagulation after infective endocarditis was excluded but nevertheless suffered repeated episodes of pulmonary embolism that led to severe chronic thromboembolic pulmonary hypertension. After heart team discussion, he was referred to pulmonary endarterectomy and replacement of the implantable cardioverter defibrillator with a subcutaneous device. This led to significant improvement of functional class and normalisation of pulmonary haemodynamics. More recently, he suffered syncope in the setting of ventricular fibrillation with appropriate shocks and was started on quinidine without further recurrence of arrhythmic episodes. © BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.