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Case report
Chronic thromboembolic pulmonary hypertension secondary to implantable cardioverter defibrillator lead thrombus in a patient with Brugada syndrome: a rare complication requiring a multidisciplinary approach
  1. Sofia Alegria,
  2. Filipa Ferreira,
  3. Débora Repolho and
  4. Maria José Loureiro
  1. Pulmonary Hypertension Unit, Department of Cardiology, Hospital Garcia de Orta, Almada, Portugal
  1. Correspondence to Dr Sofia Alegria; asofia.alegria{at}


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.

  • arrhythmias
  • pulmonary embolism
  • pulmonary hypertension
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  • Contributors SA performed substantial contributions to the conception or design of the work, acquisition, analysis; drafting the work and revising it critically for important intellectual content. DR contributed substantially to the conception or design of the work, acquisition, analysis. FF approved the final version published. MJL contributed substantially to the conception or design of the work: revision and final approval. All authors agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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