Yanru He1,2, Naqiang Lv1,2, Aimin Dang3,4, Nan Cheng1,2. 1. From the Fuwai Hospital, National Clinical Research Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College, Beijing, China. 2. Y. He, MD, Fuwai Hospital, National Clinical Research Center for Cardiovascular Diseases, CAMS and Peking Union Medical College; N. Lv, MD, PhD, Fuwai Hospital, National Clinical Research Center for Cardiovascular Diseases, CAMS and Peking Union Medical College; A. Dang, MD, PhD, Fuwai Hospital, National Clinical Research Center for Cardiovascular Diseases, CAMS and Peking Union Medical College; N. Cheng, MD, Fuwai Hospital, National Clinical Research Center for Cardiovascular Diseases, CAMS and Peking Union Medical College. 3. From the Fuwai Hospital, National Clinical Research Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College, Beijing, China. amdangfw@163.com amdang@fuwaihospital.org. 4. Y. He, MD, Fuwai Hospital, National Clinical Research Center for Cardiovascular Diseases, CAMS and Peking Union Medical College; N. Lv, MD, PhD, Fuwai Hospital, National Clinical Research Center for Cardiovascular Diseases, CAMS and Peking Union Medical College; A. Dang, MD, PhD, Fuwai Hospital, National Clinical Research Center for Cardiovascular Diseases, CAMS and Peking Union Medical College; N. Cheng, MD, Fuwai Hospital, National Clinical Research Center for Cardiovascular Diseases, CAMS and Peking Union Medical College. amdangfw@163.com amdang@fuwaihospital.org.
Abstract
OBJECTIVE: This study was performed to explore the clinical manifestations and longterm prognosis in patients with Takayasu arteritis (TA) with pulmonary artery involvement (PAI). METHODS: The medical records of 194 patients with TA who underwent traditional catheter angiography or computed tomography of pulmonary artery from 2009 to 2016 were retrospectively reviewed. The clinical manifestations, angiographic features, and mortality of 128 patients with TA with PAI were further analyzed. RESULTS: Patients with TA with PAI had a higher risk of pulmonary hypertension (PH) than patients with TA alone (61.7% vs 7.6%, p < 0.001). Patients with PAI and PH more frequently developed dyspnea, hemoptysis, and lower limbs edema (all p < 0.05) than those without PH. Patients with PH also had a higher incidence of bilateral PAI (84.8% vs 34.7%, p < 0.001) and a higher pulmonary artery obstruction index [23 (interquartile range 20-27) vs 10 (6-15), p < 0.001]. Left heart disease was presented in 39 (30.5%) patients with TA with PAI. During the median followup of 38 (21-58) months, 19 and 2 deaths occurred among patients with and without PH, respectively. Among patients with PAI, the mortality rate was 7 times higher in patients with than without PH (p = 0.009). Independent predictors of mortality were the disease duration (p = 0.047), New York Heart Association class III/IV (p = 0.019), right ventricular systolic dysfunction (p = 0.019), and respiratory failure (p = 0.007). CONCLUSION: Patients with TA with PAI have a higher risk of developing PH than patients with TA alone. The presence of PH in patients with PAI increases the risk of early mortality.
OBJECTIVE: This study was performed to explore the clinical manifestations and longterm prognosis in patients with Takayasu arteritis (TA) with pulmonary artery involvement (PAI). METHODS: The medical records of 194 patients with TA who underwent traditional catheter angiography or computed tomography of pulmonary artery from 2009 to 2016 were retrospectively reviewed. The clinical manifestations, angiographic features, and mortality of 128 patients with TA with PAI were further analyzed. RESULTS:Patients with TA with PAI had a higher risk of pulmonary hypertension (PH) than patients with TA alone (61.7% vs 7.6%, p < 0.001). Patients with PAI and PH more frequently developed dyspnea, hemoptysis, and lower limbs edema (all p < 0.05) than those without PH. Patients with PH also had a higher incidence of bilateral PAI (84.8% vs 34.7%, p < 0.001) and a higher pulmonary artery obstruction index [23 (interquartile range 20-27) vs 10 (6-15), p < 0.001]. Left heart disease was presented in 39 (30.5%) patients with TA with PAI. During the median followup of 38 (21-58) months, 19 and 2 deaths occurred among patients with and without PH, respectively. Among patients with PAI, the mortality rate was 7 times higher in patients with than without PH (p = 0.009). Independent predictors of mortality were the disease duration (p = 0.047), New York Heart Association class III/IV (p = 0.019), right ventricular systolic dysfunction (p = 0.019), and respiratory failure (p = 0.007). CONCLUSION:Patients with TA with PAI have a higher risk of developing PH than patients with TA alone. The presence of PH in patients with PAI increases the risk of early mortality.