CAJ | 학술논문

目的提高对儿童肺动脉高压(PAH)的认识和早期诊断与治疗水平.方法回顾性分析上海市儿童医院2006年1月至2012年12月收治的45例非先天性心脏病相关PAH患者的临床资料,对PAH的临床表现、实验室检查、诊断、治疗、预后等进行总结分析.结果 45例患者中,男21例(46.7％),女24例(53.3％);平均年龄2.5岁.包括继发性PAH 21例[间质性肺部疾病4例,上呼吸道梗阻、自身免疫性疾病各3例,肝脏疾病3例中肝血管瘤、肝硬化门脉高压、自身免疫性肝结节病各1例,家族性PAH、心动过速性心肌病、广泛性肺小动脉分支狭窄各2例,人类免疫缺陷病毒(HIV)感染、甲状腺功能减低各1例],特发性PAH 24例(占53.3％).临床表现主要有活动后气促(71.1％),乏力(68.9％),咳嗽(48.9％),胸闷(26.7％),胸痛(33.3％),晕厥(8.9％)等.体征以肺动脉瓣区第2心音亢进(93.3％)最多见,其次是三尖瓣杂音(77.8％)、发绀(53.3％)、肝大(42.2％)、心包积液(28.9％)、双下肢水肿(11.1％)等缺氧和右心功能不全的症状.27例患儿行心导管检查.特发性PAH与继发性PAH的肺动脉收缩压、肺动脉平均压、肺小动脉锲压、肺动脉阻力比较差异有统计学意义(P＜0.05).31例经针对病因治疗及对症治疗后好转,11例无明显好转,3例随访过程中死亡.结论儿童PAH临床少见,症状无特异性,容易被误诊.超声心动图、心导管检查可以诊断.积极寻找病因可改善患儿预后.
목적 제고대인동폐동맥고압(PAH)적인식화조기진단여치료수평.방법 회고성분석상해시인동의원2006년1월지2012년12월수치적45례비선천성심장병상관PAH환자적림상자료,대PAH적림상표현、실험실검사、진단、치료、예후등진행총결분석.결과 45례환자중,남21례(46.7％),녀24례(53.3％);평균년령2.5세.포괄계발성PAH 21례[간질성폐부질병4례,상호흡도경조、자신면역성질병각3례,간장질병3례중간혈관류、간경화문맥고압、자신면역성간결절병각1례,가족성PAH、심동과속성심기병、엄범성폐소동맥분지협착각2례,인류면역결함병독(HIV)감염、갑상선공능감저각1례],특발성PAH 24례(점53.3％).림상표현주요유활동후기촉(71.1％),핍력(68.9％),해수(48.9％),흉민(26.7％),흉통(33.3％),훈궐(8.9％)등.체정이폐동맥판구제2심음항진(93.3％)최다견,기차시삼첨판잡음(77.8％)、발감(53.3％)、간대(42.2％)、심포적액(28.9％)、쌍하지수종(11.1％)등결양화우심공능불전적증상.27례환인행심도관검사.특발성PAH여계발성PAH적폐동맥수축압、폐동맥평균압、폐소동맥계압、폐동맥조력비교차이유통계학의의(P＜0.05).31례경침대병인치료급대증치료후호전,11례무명현호전,3례수방과정중사망.결론 인동PAH림상소견,증상무특이성,용역피오진.초성심동도、심도관검사가이진단.적겁심조병인가개선환인예후.
Objective To improve the knowledge of paediatric pulmonary arterial hypertension(PAH) and to elevate the level of early diagnosis and treatment.Methods The clinical data of 45 PAH patients admitted in Shanghai Children's Hospital from Jan.2006 to Dec.2012 were reviewed,including clinical manifestation,laboratory examination,diagnosis,treatment and prognosis.Results Of the 45 PAH patients,21 cases (46.7％) were male and 24 cases (53.3 ％) were female,with an average age of 2.5 years old.Among them idiopathic PAH was in 24 cases (53.3 ％) and secondary PAH was in 21 cases [including interstitial lung disease in 4 cases,upper airway obstruction in 3 cases,systemic lupus erythematosus in 3 cases,hepatic disease in 3 cases,including hepatic hemangioma 1 case,liver cirrhosis portal hypertension 1 case and autoimmune hepatic sarcoidosis 1 case,tachycardia induced cardiomyopathy in 2 cases,extensive pulmonary small artery stenosis in 2 cases,human immunodeficiency virus (HIV) infection in 1 case,hypothyroidism in 1 case,and familial PAH in 2 cases].Main clinical manifestations were anhelation after exercise (71.1％),fatigue (68.9％),cough (48.9％),chest tightness (26.7％),chest pain (33.3％),syncope (8.9％),et al.The most common physical signs were splitting of the second sound in pulmonary valve area (93.3％),followed by tricuspid murmur (77.8％),cyanosis (53.3％),hepatomegaly (42.2％),hydropericardium (28.9％),and oedema (11.1％),et al.Twenty-seven cases did cardiac catheterization,compared with idiopathic PAH and secondary PAH,pulmonary artery systolic pressure,mean pulmonary artery pressure,pulmonary capillary wedge pressure,pulmonary arteriolar resistance index had significant difference(P ＜ 0.05).Thirty-one cases' condition improved after treatment,11 cases without any improvement and 3 cases died during follow-up visit.Conclusions PAH is a rare disease with no specific symptom and can be easily misdiagnosed in children.Ultrasonic cardiogram and cardiac catheterization are helpful in diagnosis.Actively looking for the cause can improve the prognosis.