Systemic sclerosis complicated by chronic thromboembolic pulmonary hypertension treated with balloon pulmonary angioplasty: A case report

Abstract

Background: Systemic sclerosis (SSc) is known to induce pulmonary hypertension (PH), resulting in poor prognosis. Pulmonary hypertension secondary to connective tissue disease is usually classified as Group 1. In patients with SSc, PH owing to left heart failure (Group 2) and interstitial pneumonia (Group 3) are also known; however, there have been limited reports on chronic thromboembolic PH (CTEPH), which corresponds to Group 4. Case summary: A 39-year-old female with SSc was admitted with shortness of breath on exertion for the past 4 months. Right heart catheterization revealed severe PH. Group 1 PH secondary to collagen tissue disease was suspected; however, thoracic computed tomography and lung perfusion scan led to the diagnosis of CTEPH of Group 4. We treated the PH with several medications and balloon pulmonary angioplasty (BPA), which improved the PH and right heart failure. Consequently, her overall condition also improved. Discussion: This is a rare case report of SSc complicated by CTEPH, which was treated with BPA. Patients with SSc are prone to complications of various type of PH. Thus, it is important to distinguish CTEPH in terms of treatment choice and prognosis.