Abstract

BackgroundApproximately 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 presentationA 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.ConclusionsBalloon pulmonary angioplasty, rapidly developing method of treatment of inoperable CTEPH patients, is also extremely useful therapeutic tool in complex PH patients.

Highlights

  • A quarter of patients with advanced sarcoidosis develop pulmonary hypertension (PH), which affects their prognosis

  • Approximately 26% of patients with advanced pulmonary sarcoidosis may develop pulmonary hypertension (PH), which is related with poor prognosis [1, 2]

  • Patients with suspected PH should undergo detailed diagnostic workup and differential diagnosis among others should include chronic lung diseases leading to hypoxia and chronic thromboembolic pulmonary hypertension (CTEPH) [3]

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Summary

Conclusions

Rapidly developing method of treatment of inoperable CTEPH patients, is extremely useful therapeutic tool in complex PH patients.

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