CAJ | 학술논문

目的：观察波生坦治疗先天性心脏病相关肺动脉高压的临床疗效及安全性。方法临床诊断先天性心脏病相关肺动脉高压患者24例，其中4例为外科手术后患者，20例为临床诊断重度的肺动脉高压存在手术禁忌的患者。所有患者均按照设定的目标剂量口服波生坦治疗，服药2、4、6月时测定6MWD、UCG估测肺动脉压，评定心功能等级，复查肝肾功能。结果24例失访1例，死亡1例，症状有效缓解率达84.2%。肺动脉收缩压服药后2、4月与服药前比较无显著性差异（P=0.096），服药后6月时有显著性差异[（97.8±14.9）mmHg vs（80.9±25.0）mmHg，P=0.029]。服药后2、4、6月的6MWD较服药前显著提高[（317.0±134.1）m vs（488±98.8）m vs（496.3±89.0）m vs（491.3±114.2）m，P=0.004、0.003、0.004）。服药后2、4、6月心功能分级较服药前提高（2.9±0.5 vs 2.0±0.5，1.8±0.4，1.7±0.5，P均<0.001）。服药后2、4、6月复查肾功能正常，复查谷草转氨酶及谷丙转氨酶结果与服药前相比均无显著性差异（P>0.05）。结论口服波生坦可有效减轻先天性心脏病相关肺动脉高压患者的临床症状，降低肺动脉压，改善运动耐量及心功能分级，患者耐受性好。
목적：관찰파생탄치료선천성심장병상관폐동맥고압적림상료효급안전성。방법림상진단선천성심장병상관폐동맥고압환자24례，기중4례위외과수술후환자，20례위림상진단중도적폐동맥고압존재수술금기적환자。소유환자균안조설정적목표제량구복파생탄치료，복약2、4、6월시측정6MWD、UCG고측폐동맥압，평정심공능등급，복사간신공능。결과24례실방1례，사망1례，증상유효완해솔체84.2%。폐동맥수축압복약후2、4월여복약전비교무현저성차이（P=0.096），복약후6월시유현저성차이[（97.8±14.9）mmHg vs（80.9±25.0）mmHg，P=0.029]。복약후2、4、6월적6MWD교복약전현저제고[（317.0±134.1）m vs（488±98.8）m vs（496.3±89.0）m vs（491.3±114.2）m，P=0.004、0.003、0.004）。복약후2、4、6월심공능분급교복약전제고（2.9±0.5 vs 2.0±0.5，1.8±0.4，1.7±0.5，P균<0.001）。복약후2、4、6월복사신공능정상，복사곡초전안매급곡병전안매결과여복약전상비균무현저성차이（P>0.05）。결론구복파생탄가유효감경선천성심장병상관폐동맥고압환자적림상증상，강저폐동맥압，개선운동내량급심공능분급，환자내수성호。
Objective To investigate the effect of oral bosentan in the treatment of congenital heart disease-associated pulmonary arterial hypertension. Methods 24 patients with congenital heart disease- associated pulmonary arterial hypertension, including 4 receiving heart surgery and 20 with surgical contraindications, were enrolled in this study. All the patients were given oral bosentan and followed up regularly for analyzing the outcomes and side effects. Results One patient was lost to follow up and one patient died. Systolic pulmonary artery pressure showed no significant changes at 2 (93.6 ± 17.2 mmHg) and 4 months (85.7±25.5 mmHg) of bosentan treatment compared to that before the medication (97.8±14.9 mmHg) (P=0.096), but decreased significantly after a 6-month therapy (80.9 ± 25.0 mmHg, P=0.029). The 6-minute walking distance increased significantly after a 2, 4, and 6-month therapy [(488±98.8, 496.3±89.0, and 491.3±114.2 m, respectively;P=0.004, 0.003, and 0.004 vs the distance before medication (317.0 ± 134.1)]. The New York heart functional classification was improved significantly after a 2, 4, and 6-month therapy [(2.0±0.5, 1.8±0.4, and 1.7±0.5, respectively;P<0.001 vs pre-medication score (2.9± 0.5)). Hepatic and renal function remained normal, and ALT and AST showed no significant variations during the medication (P>0.05). Conclusion Oral bosentan can effectively relieve the symptoms, decrease pulmonary artery hypertension, and improve exercise tolerance and cardiac function classification in patients with pulmonary artery hypertension associated with congenital heart disease with good safety and mild side effects.