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Chronic Thromboembolic Pulmonary Hypertension Due to a Rare Anterior Mediastinal Venous Malformation

Published:November 21, 2022DOI:https://doi.org/10.1053/j.jvca.2022.11.017
      CHRONIC THROMBOEMBOLIC pulmonary hypertension (CTEPH) is a condition characterized by persistent obstructive thrombi that will lead to vascular scarring and, subsequently, progressively elevated pulmonary arterial pressure. These thromboemboli generally originate from deep venous thrombosis (DVT), typically from the lower extremities.
      • Kearon C.
      Natural history of venous thromboembolism.
      The authors present an unusual case of CTEPH due to a rare anterior mediastinal venous malformation (VM) that was misdiagnosed previously in infancy as a cystic hygroma. Mediastinal vascular malformations are uncommon, and embolization to the pulmonary arteries from a VM is a rare event.
      • Nakano TA
      • Zeinati C.
      Venous thromboembolism in pediatric vascular anomalies.
      Early diagnosis and a multidisciplinary approach can enable prompt follow-up and treatment of this condition to mitigate adverse consequences of pulmonary hypertension.
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