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The patient was diagnosed as a case of CTEPH due to secondary APS. He underwent PEA and was discharged on lifelong anticoagulation. Clinical follow-ups thereafter showed improvement of functional capacity and pulmonary artery pressures. In conclusion, management of such cases was combination of standard treatment of CTEPH, in addition to specific management of secondary APS to avoid recurrence of the disease.Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the leading causes of severe pulmonary hypertension (PH). The dis

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