小儿结缔组织病并发的肺动脉高压临床分析

目的 研究小儿结缔组织病(CTD)并发的肺动脉高压(PAH)的发病情况、临床特点和预后.方法 回顾性分析我院儿科2000年1月-2007年1月CTD并发PAH住院患儿的临床资料.结果(1)299例有完整心脏彩超资料的CTD患儿共发生PAH 31例(10.4%),女28例,男3例,年龄7～18岁,平均12.5岁.不同CTD中PAH的发病率分别为:重叠综合征(OCTD):62.5%(5/8),抗磷脂综合征(APS)50.0%(2/4),系统性血管炎(SV):28.6%(4/14),皮肌炎(DM):10.7%(3/28),系统性红斑狼疮(SLE)7.6%(17/223).PAH在CTD患儿出现的时间为CTD起病后3周～5年,平均为1.5年.(2)小儿CTD相关PAH起病多隐匿,重者可出现呼吸困难(18/31,58.1%)、心功能衰竭(9/31,29.0%).有雷诺现象和抗磷脂抗体或狼疮抗凝物阳性的PAH患儿易表现为中重度PAH.(3)超声心动图和肺功能尤其肺CO弥散量(DL>CO)检查对早期发现PAH更有意义.(4)治疗后轻中度PAH转归较好,重度PAH可降至轻中度.CTD并发PAH死亡组患儿较存活组患儿动脉氧分压(PaO2)明显降低(P＜0.01),肺动脉收缩压(PASP)明显升高(P＜0.05).结论 CTD患儿并发PAH不少见,一般在CTD发病后平均1.5年,早期症状较隐匿,重者表现为呼吸困难和心功能衰竭.雷诺现象和抗磷脂抗体或狼疮抗凝物阳性者多为中重度PAH.早期定期监测超声心动图和肺功能对早期发现PAH非常必要.严重PAH将影响CTD患儿的预后,PASP分度和PaO2水平,是决定预后的重要因素.

Clinical analysis of pu71monary arterial hypertension secondary to connective tissue disease in children

Objective To study the epidemiology, clinical characteristics and prognosis of childrenwith pulmonary arterial hypertension (PAH) secondary to connective tissue disease(CTD). Methods Theclinical record files of the pediatric inpatient with PAH in the population of CTD in the hospital treatedbetween January 2000 and January 2007 were analyzed retrospectively. Results ( 1 ) In 299 patients withCTD and complete Doppler echoeardiography files, 31 (31/299, 10.4%) patients (28 females and 3males), aged from 7 to 18 years (average: 12.5 years), were found to have PAH, in whom, 5(62.5% )in8 patients with overlapped CTD( OCTD), 2(50. 0% ) in 4 patients with antiphospholipid syndrome ( APS),4(28. 6% )in 14 patients with systematic vasculitis (SV), 3 ( 10. 7% ) in 28 patients with dermatomyesitis(DM), 17 (7. 6% ) in 223 systemic lupus erythematesus (SLE) had PAH. The CTD-associated PAHoccurred in the 3rd week to 5th year after initial CTD manifestations (median onset: 1.5 years). (2) Theonset of CTD-associated PAH was obscure and children with severe CTD-asseciated PAH presented withdyspnea ( 18/31,58. 1% ) and heart failure (9/31,29. 0% ). The children with Raynaud's phenomenon orpositive anticardiophospholipid antibody (ACL) or positive lupus anticoagulant (LA) were prone to havemore severe PAH. (3) Doppler echocardiography and pulmonary function test, especially the test ofpulmonary diffusion function of CO (D<,L>CO) were necessary to detect PAH early. (4) After treatment, thepulmonary arterial pressure in mild and moderate PAH cases could be normalized and in severe PAH easescould be decreased to mild or moderate levels. There was a lower PaO<,2> level( P＜0. 01 ), a higher pulmonaryarterial systolic pressure (PASP) level( P＜0. 05 )in the cases of CTD-PAH who died as compared with thelive patients. Conclusions PAH is a common complication of CTDs, which occurs often 1.5 years afterinitial CTD manifestations. The early associated symptom is obscure and the severe cases manifest withdyspnea and heart failure. Those with Raynaud's phenomenon and positive ACL and LA are prone to developmore severe PAH. Early and regular Doppler echocardiography and pulmonary function test are necessary todetect PAH early. Severe CTD associated PAH in children could lead to poor outcome. PASP classificationand PaO2 level are important factors affecting prognosis.