中文摘要：

目的探讨对单纯性动脉导管未闭合并重度肺动脉高压伴有差异性发绀的青少年患者在联合靶向降肺压治疗下实施封堵术后的疗效。方法回顾性分析3例动脉导管未闭合并重度肺动脉高压和差异性发绀、接受经导管动脉导管未闭封堵术，并联合靶向降肺压药物治疗患者封堵动脉导管未闭前、后的肺动脉压力、肺循环阻力情况及术后随访情况。结果封堵术后3例患者肺动脉收缩压[（102±21）mmHg／）S．（124＋11）mmHg，P=0．125]、肺总阻力[（17．35±4．79）Wood US．（21．31±3．41）Wood，P=0．125]、肺小动脉阻力[（15．61±4．77）Wood US．（19．39±3．18）Wood，P=0．125]及超声估测肺动脉收缩压[（102＋37）mmHgm（111±34）mmHg，P=0．750]与封堵前比较，差异无统计学意义。3例患者中1例术前行肺活检，提示为肺动脉高压Heath-EdwardsI-Ⅱ级病理改变。3例封堵术后血氧饱和度均上升至正常，治疗后短期内生活质量提高，术后1年后起则频繁出现右心功能衰竭或因肺血管梗阻性病变引起频繁咯血，2例在术后3年余死亡。结论当动脉导管未闭合并重度肺动脉高压患者出现差异性发绀时，在联合靶向降肺压治疗的前提下，实施动脉导管未闭封堵术短期内能提高患者生活质量，但并不能改善生存时间及预后。进行肺活检了解肺血管病变程度时．建议多点取材，结合右心导管检查结果进行综合评价，对合并重度肺动脉高压的患者治疗后应该长期随访，客观评价其治疗效果。

英文摘要：

Objectives To evaluate the therapeutic effect of transcatheter closure combined with disease-specific targeted pulmonary hypertension （PH） therapy for patent ductus arteriosus （PDA） with severe PH and differential cyanosis in youth patients. Methods Information of 3 youth patients who were diagnosed as PDA with severe PH and differential cyanosis were studied retrospectively.All of them received transcatheter closure combined with disease-specific targeted PH therapy. Comparisons of systolic pulmonary arterial pressure （SPAP）,pulmonary vascular resitance（PVR） before and after transcatheter PDA closure and follow-up evaluation were presented. Results There were no statistical differences in SPAP[ （ 102±21 ）mm Hg vs. （ 124±11 ）mm Hg,P=0.125 ], puhnonary arteria resistance [ （ 17.35±4.79）Wood vs. （21.31±3.41 ）Wood,P=0.125 ], pulmonary arteriolar resistance [ （ 15.61±4.77）Wood vs. （ 19.39±3.18）Wood,P=0.125 ], and SPAP by echocardiography [ （102±37）mm Hg vs. （111±34）mm Hg,P=0.750] before and after transcatheter PDA closure. Lung biopsy was presented before surgery in 1 of the 3 patients and showed a pathologic change of PH with Heath-Edwards Grading Grading I - lI . However, the therapeutic effect was not as good as the early stage of PH patients, which implied a reasonable obstructed vasculopathy of PH. Although the arterial oxygen saturation of the 3 cyanotic patients rose to a normal level and had a better quality of life short after transcatheter PDA closure, they frequently suffered from right heart failure and recurrent hemoptysis induced by obstructed vasculopathy. Two of them passed away in 3 years post-operation. Conclusions For PDA patients with severe PH and differential cyanosis, transcatheter PDA closure combined with disease-specific targeted PH therapy can improve their quality of life or prolong survival timecannot be prolonged and the prognosis cannot be improved. An objective assessment for the therapy on PDA patients with severe PH and differen