主肺间隔缺损的外科治疗

目的 探讨主肺间隔缺损的外科治疗经验.方法 1987年12月至2007年3月,16例主肺间隔缺损病儿(人),其中男11例,女5例,年龄5个月～22岁,平均(8.2±7.1)岁;体重5.8～50.0 kg,平均(22.2±13.9)kg.Mori分型Ⅰ型7例,Ⅱ型7例,Ⅲ型2例.主肺间隔缺损大小10～35mm,平均(22.6±7.3)mm.其中合并室间隔缺损、房间隔缺损和肺主动脉弓离断畸形(B型)各2例,动脉导管未闭1例.术前10例行心导管检查,肺动脉收缩压50～120 mm Hg(1 mm Hg=0.133 kPa),平均(75.4±25.2)mm Hg,肺动脉平均压21～100 mm Hg,平均(60.3±24.8)m Hg.14例在中低温体外循环下完成手术,2例在深低温停循环完成手术.结果 主动脉阻断(70.6±48.1)min,体外循环(110.5±62.9)min.术后10例肺动脉收缩压(50.2±18.5)mm Hg,平均肺动脉压(40.0±15.2)mm Hg,术后呼吸机使用(22.6±14.5)h,滞留蕈症监护室1～70 d,总住院14～127 d.2例合并主动脉弓离断者围术期分别死于急性肾衰和术后低心排出量综合征.14例痊愈,平均随访(8.8±7.5)年,1例主肺动脉间隔小残余漏,余心功能明显改善.结论 主肺间隔缺损者肺血管病理改变早,早期诊断、及时手术,能降低病死率.

Surgical experience for the treatment of aortopulmonary septal defect

Objective Aortopulmonary septal defect is rare and accounted for abont 0.15% to0.60% of infants and children with congenital heart malformation. Aortopulmonary septal defect has similar pathophysiologic findings with patent ductus. Early pul-monary hypertension and congestive heart failure may develop as a result of large left to right shunt from the ascending aorta to the pul-monary artery, with a relatively rapid progression of pathological changes in the pulmonary vessels associated with pulmonary hyperten-sion. This article will describe our surgical experience in the treatment of this disease. Methods From December 1987 to March 2000,16 cases with aortopulmonary septal defect underwent surgical treatment, 11 were males and 5 were females. The mean age and weight were (8.2 ± 7.1) years (range from 5 months to 22 years) and (22.2± 13.9) kg (range from 5.8 to 50.0) respectively. Seven cases were type Ⅰ, seven were type Ⅱ and two were type Ⅲ according to Richardson's classification. The mean scale of aortop-ulmonary septal defect was (22.6± 7.3)mm (range from 10 to 35) . Coexistent ventriculor septal defect was found in two cases, atri-al septal defect was found in another two cases, patent ductus in one case and interrupted aortic arch (type B) in two cases. Cardiac catheterization were perforomed in ten cases, and the systolic and mean pulmonary artery pressure were (75.4± 25.2) mmHg (range from 39 to 120) and (55.1± 25.2) mmHg (range from 21 to81) respectively. All patients underrwent surgical repair under cardiop-ulmonary bypass (CBP) at moderate hypothermic temperature. Deep hypothermic circulatory arrest was used in two cases with inter-rupted aortic arch. The mean duration of aortic clamping and CPB were (70.4± 48.1) minutes and (110.5± 62.9)minutes. Strate-gies for APW repair were trans-aortic with patch closure in thirteen cases, trans-pulmonary in two cases and trans-window in one case.Other coexistent malformations were corrected simultaneously. Results The poet-operative systolic and mean pulmonary artery pres-sure were decreased to (50.2±18.5) mmHg (range from 20 to 78 mmHg) and (40.0± 15.2) mmHg (range from 18 to 65 mmHg) respectively. The mean duration of postoperative mechanical ventilation was (22.6 ±14.5) hours (range 12-70), that for ICU-staying was (45.8 ± 55.5) days (1-70), and that for hospitalization was (49.1 ±32.2) days (14-127). Two APW cases with interrupted aortic arch died of lethal acute renal failure and low cardiac output syndrome perioperatively. During the mean follow-up period of (8.8±7.5) years, there was no re-operation case or late death in 14 survived patients. All of them showed improvement in the heart function except for one patient who had small residual shunt. Pationts who recovered have gone to work or to school with the resolution of their murmus and significant improvement in the pulmonary congestion. Their enlarged hearts before operation have reduced by var-ious extents. Condusion Given the early pathological changes of pulmonary vessels in patients with aortopulmonary septal defect,early diagnosis and surgical treatment, which may decrease the mortality, are of critically important. Coexistent and complicated con-genital defects such as interrupted aortic arch may increase the mortality in APW cases.