Background: Approximately a quarter of patients with advanced sarcoidosis develop pulmonary hypertension (PH), which affects their prognosis. We report unusual case of confirmed chronic thromboembolic pulmonary hypertension (CTEPH) in a patient with stage IV sarcoidosis successfully treated with balloon pulmonary angioplasty (BPA).
Case presentation: A 65 years old male with a history of colitis ulcerosa, and pulmonary sarcoidosis diagnosed in 10 years before, on long term oral steroids, with a history of deep vein thrombosis and acute pulmonary embolism chronically anticoagulated was referred to our center due to severe dyspnea. On admission he presented WHO functional class IV, mean pulmonary artery pressure (mPAP) in right heart catheterization (RHC) was elevated to 54 mmHg. Diagnosis of CTEPH was definitely confirmed with typical V/Q scan, and with selective pulmonary angiography (PAG) completes by intravascular imagining (intravascular ultrasound, optical coherent tomography). The patient was deemed inoperable by CTEPH team and two sessions of BPA with multimodal approach resulted in significant clinical and haemodynamical improvement to WHO class II and mPAP decrease to 27 mmHg.
Conclusions: Balloon pulmonary angioplasty, rapidly developing method of treatment of inoperable CTEPH patients, is also extremely useful therapeutic tool in complex PH patients.
Keywords: Balloon pulmonary angioplasty; Chronic thromboembolic pulmonary hypertension; Sarcoidosis.