CAJ | 학술논문

目的探讨先天性心脏病修复术后肺动脉高压(pulmonary arterial hypertension,PAH)患者的基线临床特征.方法回顾性分析上海市肺科医院心肺血管中心2006年9月至2011年8月连续确诊的122例先天性心脏病修复术后PAH患者的基线临床资料.结果 122例先天性心脏病修复术后PAH患者中,女性82例(67.2％),修补/封堵术时年龄中位数14.8(7.1,24.9)岁,术后诊断PAH年龄中位数24.8(16.6,32.2)岁,从修复术至诊断PAH时间间隔中位数7.3 (2.4,12.6)年.临床症状以劳力性呼吸困难(109/122,89.3％)为主,体征以肺动脉瓣区第二心音亢进(113/122,92.6％)为主.确诊时符合世界健康组织肺高压功能Ⅰ/Ⅱ级的患者稍多(65/122,53.3％).6 min步行距离为(408.4±103.3) m.Borg呼吸困难评分为2.0(1.0,3.0).肺动脉平均压(71.6±24.2)mmHg(1 mmHg =0.133 kPa),肺血管阻力指数(29.1±16.0) Wood U· m2,心指数(2.7±0.9) L·min-1·m-2.靶向PAH药物治疗率90.2％(110/122),以5型磷酸二酯酶抑制剂为主.结论先天性心脏病修复术后,部分患者发生PAH.与欧美发达国家相比,本研究中先天性心脏病修复术后PAH患者手术年龄大,术后发生PAH时间短,诊断PAH年龄轻,靶向PAH药物治疗率相当,以5型磷酸二酯酶抑制剂为主.
목적 탐토선천성심장병수복술후폐동맥고압(pulmonary arterial hypertension,PAH)환자적기선림상특정.방법 회고성분석상해시폐과의원심폐혈관중심2006년9월지2011년8월련속학진적122례선천성심장병수복술후PAH환자적기선림상자료.결과 122례선천성심장병수복술후PAH환자중,녀성82례(67.2％),수보/봉도술시년령중위수14.8(7.1,24.9)세,술후진단PAH년령중위수24.8(16.6,32.2)세,종수복술지진단PAH시간간격중위수7.3 (2.4,12.6)년.림상증상이로력성호흡곤난(109/122,89.3％)위주,체정이폐동맥판구제이심음항진(113/122,92.6％)위주.학진시부합세계건강조직폐고압공능Ⅰ/Ⅱ급적환자초다(65/122,53.3％).6 min보행거리위(408.4±103.3) m.Borg호흡곤난평분위2.0(1.0,3.0).폐동맥평균압(71.6±24.2)mmHg(1 mmHg =0.133 kPa),폐혈관조력지수(29.1±16.0) Wood U· m2,심지수(2.7±0.9) L·min-1·m-2.파향PAH약물치료솔90.2％(110/122),이5형린산이지매억제제위주.결론 선천성심장병수복술후,부분환자발생PAH.여구미발체국가상비,본연구중선천성심장병수복술후PAH환자수술년령대,술후발생PAH시간단,진단PAH년령경,파향PAH약물치료솔상당,이5형린산이지매억제제위주.
Objective To explore the baseline clinical characteristics of patients with pulmonary arterial hypertension after operation for congenital heart disease (AO-CHD-PAH) in China.Methods A total of 122 consecutive patients diagnosed as AO-CHD-PAH in Shanghai Pulmonary Hospital from September 1,2006 to August 31,2011 were retrospective analyzed.Results Sixty-seven percent patients were female.The median age at procedure and diagnosis were 14.8 and 24.8 years old,respectively.The median duration from procedure of CHD to diagnosis of PAH was 7.3 years.The most frequent initial symptom (109/122,89.3％) attributed to PAH was dyspnea on exertion.The frequent sign was accentuation of second heart sound on pulmonary valve area (113/122,92.6％).Incidence of WHO functional PAH classes Ⅰ / Ⅱ was 53.3％ (65/122) and mean 6 minutes walk distance was limited to (408.4 ± 103.3) m.Borg dyspnea score was 2.0 (1.0,3.0).Right heart catheterization demonstrated severe elevated mean pulmonary arterial pressure[(71.6 ±24.2) mmHg,1 mmHg =0.133 kPa] and pulmonary vascular resistance index [(29.1 ±16.0) Wood U·m2]in this patient cohort.Cardiac index was (2.7 ±0.9) L·min-1·m-2,90.2％ (110/122) patients received PAH-specific therapy.The majority of PAH-specific therapy was phosphodiesterase type 5 inhibitor,which is somehow different from PAH-specific therapy regimen of American-European developed countries.Conclusions PAH-specific therapy rate is satisfactory for pulmonary arterial hypertension after operation for congenital heart disease in China and phosphodiesterase type 5 inhibitors are predominant PAH-specific medication in China.