Interventional therapy in sarcoidosis-associated pulmonary arterial stenosis and pulmonary hypertension.

BACKGROUNDS: Pulmonary sarcoidosis is often complicated by pulmonary hypertension, a complication that is associated with increased disability and mortality. To this point, however, little progress has been made in the treatment of sarcoidosis associated with pulmonary hypertension (SAPH). METHODS: A prospective study was performed on 72 consecutive Chinese sarcoidosis patients followed at an outpatient clinic. The patients were evaluated by Doppler echocardiography and computed tomography pulmonary angiography. SAPH was confirmed by right heart catheterisation. The clinical parameters were compared before and 2 months after treatment with oral glucocorticoids. Eight stage III and IV patients with moderate to severe proximal pulmonary arterial stenosis (PAS) and SAPH underwent interventional therapy (IT) after prednisone treatment and were followed up at 3-month intervals. RESULTS: After 2 months of prednisone treatment, 32 stage III and IV patients continued to display varying degrees of PAS and SAPH. Eight patients underwent IT without severe complications and made improvements in pulmonary arterial pressure, pulmonary vascular resistance, arterial oxygen saturation and WHO functional classification, with the improvements lasting more than 3 months. CONCLUSIONS: PAS caused by external compression in sarcoidosis is a significant reason for SAPH. IT is effective and safe in the treatment of PAS and SAPH.

Novel Angiographic Classification of Each Vascular Lesion in Chronic Thromboembolic Pulmonary Hypertension Based on Selective Angiogram and Results of Balloon Pulmonary Angioplasty.

BACKGROUND: Balloon pulmonary angioplasty (BPA) is an alternative therapy for patients with chronic thromboembolic pulmonary hypertension who are ineligible for standard therapy, pulmonary endarterectomy. Although there are several classifications of vascular lesions, these classifications are based on the features of the specimen removed during pulmonary endarterectomy. Because organized thrombi are not removed during balloon pulmonary angioplasty, we attempted to establish a new classification of vascular lesions based on pulmonary angiographic images. We evaluated the success and complication rate of BPA in accordance with the location and morphology of thromboembolic lesions. METHODS AND RESULTS: We reviewed 500 consecutive procedures (1936 lesions) of BPA in 97 patients with chronic thromboembolic pulmonary hypertension and investigated the outcomes of BPA based on the lesion distribution and the angiographic characteristics of the thromboembolic lesions, as follows: type A, ring-like stenosis lesion; type B, web lesion; type C, subtotal lesion; type D, total occlusion lesion, and type E, tortuous lesion. The success rate was higher, and the complication rate was lower in ring-like stenosis and web lesions. The total occlusion lesions had the lowest success rate. Tortuous lesions were associated with a high complication rate and should be treated only by operators with extensive experience with BPA. CONCLUSIONS: We modified the previous angiographic classification and established a new classification for each vascular lesion. We clarified that the outcome and complication rate of the BPA are highly dependent on the lesion characteristics.