摘要
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奴,平均(22.2±13.9)kg。Moil分型Ⅰ型7例,Ⅱ型7例,Ⅲ型2例。主肺间隔缺损大小10.35min,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—120mm Hg(1mmHg=0.133kPa),平均(75.4±25.2)mmHg,肺动脉平均压21~100mmHg,平均(60.3±24.8)mmHg。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)mmHg,术后呼吸机使用(22.6±14.5)h,滞留重症监护室1~70d,总住院14,127d。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。
Objective Aortopulmonary septal defect is rare and accounted for about 0.15 % to 0.60 % of infants and children with congenital heart malformation. Aortoptdmonary septal defect has similar pathophysiologlo findings with patent duetus. Early pulmonary hypertension and congestive heart failure may develop as a resnlt of large left to right shunt from the aseer.ding aorta to the pulmonary artery, with a relatively rapid progression of pathological changes in the pulmonary vessels associated with pulmonary hypertension. This article will describe our surgical experience in the treatment of this disease. Methods From December 1987 to March 2000,16 eases 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 Ⅱand two were type Ⅲ according to Richardson' s classification. The mean scale of εortopulmonary septal defect was (22.6±7.3)mm (range from 10 to 35) . Coexistent ventricular septal defect was found in two cases, atrial septal defect was found in another two cases, patent ductus in one cose and interrupted aortic arch (type B) in two cases. Cardiac catheterization were performed in ten cases, and the systolic and mean pulmonary artery pressure were (75.4 ±25.2) mm Hg (range from 39 to 120) and (55.1 ±25.2 ) mmHg (range from 21 to 81 ) respectively. All patients underwent surgical repair under cardiopulmonary bypass (CBP) at moderate hypothennic temperature. Deep hypothermic circulatory arrest was used in two cases with intermpted aortic arch. The mean duration of aortic damping and CPB were ( 70.4 ±48.1 ) minutes and ( 110.5 ±62.9 ) minutes. Strategies for APW repair were trans-aortic with patch closure in thirteen eases, trans-pulmonary in two cases and trans-window in one case. Other coexistent malformations were corrected simultaneously. Results The post-operative systolic and mean pulmonary artery pressure were decreased to (50.2±18.5) mmHg (range frem 20 to78 mmHg) and (40.0±15.2) mm Hg (range from 18 to65 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). Twe 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. Patients who recovered have gone to work or to school with the resolution of their murmurs and significant improvement in the pulmonary congestion. Their enlarged hearts before operation have reduced by various extents. Conclsion Given the early pathological changes of pulmonry vessels in patients with aortopulmonary septal defect, early diagnosis and surgical treatment, which may decrease the mortality, are of critically important. Coexistent and complicated congenital defects such as interrupted aortic arch may increase the mortality in APW cases.
出处
《中华胸心血管外科杂志》
CSCD
北大核心
2009年第4期245-247,共3页
Chinese Journal of Thoracic and Cardiovascular Surgery