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The Gordian knot of thromboembolism in congenital heart disease
  1. George Giannakoulas1,
  2. Maria Boutsikou2
  1. 1Cardiology Department, AHEPA Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
  2. 2Adult Congenital Heart Disease and Pulmonary Hypertension Unit, Royal Brompton Hospital, London, UK
  1. Correspondence to Dr George Giannakoulas, Cardiology Department, AHEPA Hospital, Aristotle University of Thessaloniki, Thessaloniki 54649, Greece; g.giannakoulas{at}

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Patients with cyanotic congenital heart disease (CCHD), with or without the presence of Eisenmenger physiology, are quite susceptible to the development of thrombosis.1 Cerebrovascular events as well as pulmonary thrombosis are two of the most common clinical manifestations in this group of patients with detrimental effects on quality of life and overall prognosis.2–4 Prevention of these episodes is challenging, as a number of different pathogenetical mechanisms of cerebral or pulmonary thrombosis have been previously proposed. Administration of antithrombotic therapy especially in patients with Eisenmenger physiology is controversial due to the increased risk of bleeding complications, which sometimes can be fatal in this population. Thus, the use of antithrombotic therapy in patients with Eisenmenger syndrome is under debate with still no existence of official recommendations.5 Early diagnosis of thrombosis based on the available imaging modalities may lead to a more effective therapeutic management of these patients.

Four possible mechanisms of thrombi formation in the pulmonary arteries of patients with Eisenmenger syndrome have been proposed.6 These include the existence of local vascular injury secondary to pulmonary hypertension, platelet dysfunction or factor deficiencies leading to hypercoagulopathy, cell aggregation due to decreased blood flow in the pulmonary arteries and embolic sources. On the other hand, systemic emboli secondary to atrial …

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  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

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