Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon complication of deep vein thrombosis (DVT) and pulmonary embolism (PE), occurring in 3-5% of patients despite therapeutic anticoagulation. Venous thoracic outlet syndrome (VTOS) causing subclavian vein (SCV) thrombosis is also an uncommon condition, not frequently associated with clinically significant PE. In this report, we present two patients with CTEPH and SCV thrombosis caused by VTOS who had successful multidisciplinary management and staged surgical treatment for both conditions. Each patient was young and otherwise healthy before presenting with progressively worsening shortness of breath. Both were found to have multiple peripheral PE and features of respiratory failure characteristic of CTEPH, along with axillary-SCV thrombosis and no other source of DVT. Each patient was treated with anticoagulation and riociguat but had ongoing pulmonary symptoms, and staged surgical treatment was recommended. The first stage was conducted by bilateral pulmonary thromboendarterectomy performed under deep hypothermic circulatory arrest. The second stage was conducted several months later after resolution of pulmonary hypertension, by paraclavicular thoracic outlet decompression that included complete first rib resection with a patent axillary-SCV on subsequent upper extremity imaging. Each patient recovered well with resolution of respiratory and upper extremity symptoms and a return to unrestricted activity by three months after the second operation. These patients are some of the first to have successful staged surgical treatment for CTEPH and SCV thrombosis caused by VTOS, illustrating the value of comprehensive multidisciplinary management for this unusual combination of rare conditions.