马昔腾坦治疗特发性肺动脉高压的临床观察

Clinical Observation on the Treatment of Idiopathic Pulmonary Hypertension with Macitentantan

目的 探讨马昔腾坦治疗特发性肺动脉高压(IPAH)的临床疗效和安全性。方法 回顾性分析2019年9月至2022年10月云南省阜外心血管病医院住院的13例IPAH患者的临床资料。13例患者给予马昔腾坦单药或联合其他靶向药物治疗。用药前收集基线临床指标,治疗1年后复查基线临床指标的变化。结果 与治疗前比较,治疗1年后,13例患者世界卫生组织(WHO)功能分级改善[(2.7±0.9)级比(2.0±0.7)级,P=0.032],6 min步行距离(6MWD)改善[(316.8±136.5) m比(408.3±129.1)m,P=0.002],氨基末端脑利钠肽前体(NT-pro-BNP)水平降低[(1 852.0±1 901.3)ng/L比(724.8±875.8)ng/L,P=0.0327],平均肺动脉压降低[(58.0±19.8)mmHg比(47.3±13.1)mmHg,P=0.014,1 mmHg=0.133 kPa],危险分层改善[(2.1±0.6)比(1.4±0.5),P=0.006]。结论 所有入选的13例IPAH患者,依据危险分层给予马昔腾坦单药治疗或者联用其他种类靶向药物治疗,治疗1年后复查,基线临床指标改善,且无明显药物不良反应。

Objective To explore the horse yesterday teng treatment of idiopathic pulmonary arterial hypertension(IPAH) clinical efficacy and safety. Methods Patients diagnosed with IPAH who were hospitalized in Fuwai Cardiovascular Hospital of Yunnan Province from September 2019 to October 2022 were selected and given marxitentan monotherapy or combined with other targeted drugs. Baseline clinical data were collected before medication, and the changes of baseline clinical indicators were reviewed after 1 year of treatment. Results Among the 13 IPAH patients, there were 2 males(15.4%) and 11 females(84.6%). All of the 13 patients were treated with marictentan targeted drugs for 1 year. The results showed that the WHO cardiac function grade of the patients was improved before and after treatment(grade 2.7±0.9, grade 2.0±0.7, P=0.032). The 6-minute walking distance before and after treatment was significantly improved(6MWD) [(316.8±136.5) m,(408.3±129.1) m, P=0.002]. N-terminal B-type natriuretic peptide was significantly decreased before and after treatment [(1 852.0±1 901.3) ng/L,(724.8±875.8) ng/L, P=0.0327]. The mean pulmonary artery pressure decreased before and after treatment [(58.0±19.8) mmHg,(47.3±13.1) mmHg, P=0.014]. The risk stratification of IPAH patients was improved before and after treatment(2.1±0.6, 1.4±0.5, P=0.006). Conclusion All 13 IPAH cases were treated with marxitentan monotherapy or combined with other targeted drugs according to risk stratification. The baseline clinical indicators were improved with no significant adverse drug reactions after reexamination 1 year later.