一侧肺动脉缺如伴其他先天性心脏病的诊断与治疗

DIAGNOSIS AND SURGERY OF CONGENITAL HEART DISEASES WITH UNILATERAL ABSENCE OF PULMONARY ARTERY

１９７７年３月至１９９４年６月间，北京阜外心血管病医院共收住院１７例一侧肺动脉缺如（ＵＡＰＡ）伴其他先天性心脏病的患者。１２例左侧ＵＡＰＡ患者均有紫绀，其中伴法乐四联症９例，右室双出口２例，单心室１例。５例右侧ＵＡＰＡ中伴法乐四联症１例，动脉导管未闭４例。另外，还合并一侧肺不发育、永存左上腔静脉等。手术１１例，占同期先天性心脏病手术的０．０７％。左侧ＵＡＰＡ的紫绀患者（５例伴发法乐四联症，２例伴发右室双出口），均在体外循环下做了根治术。其中应用带瓣外通道３例，跨环带单瓣补片３例。右侧ＵＡＰＡ伴动脉导管未闭４例中，在体外循环下直视缝合３例，左侧开胸结扎１例，合并畸形做相应处理。手术死亡２例均为法乐四联症患者，死因与手术技术和肺灌注损伤有关。术后出现并发症３例，经相应处理均痊愈。存活患者平均住院时间１４天。随诊８例，随诊时间２个月至８９个月，平均３５．７个月。除１例曾有咯血外，其他患者均能胜任日常工作。据此，我们可以认为ＵＡＰＡ伴其他先天性心脏病主要靠心血管造影电影诊断，一旦确诊，应尽早手术。紫绀患者根治术应用带瓣外通道或跨环带单瓣补片，尤其是应用同种材料时，可以有效地阻止肺动脉瓣反流，ＵＡＰＡ一般无须重建，当?

From March 1977 to June 1994, 17 cases (mean age 12± 7.9 yrs.)of congenital heart diseases with unilateral absence of pulmonary artery (UAPA)were admitted to Fu Wai Hospital. The entities of CHD consisted of TOF with left UAPA in 9 cases and right UAPA in 1,double outlet of right ventricle (DORV)and single ventricle (SV)with left UAPA in 2 and 1, respectively and PDA with right UAPA in 4. There were other associated anomalies including agenesis of lung, persistent left 2superior vena cava (PLSVC), etc.Surgical corrections of the anomalies were performed on 11 cases that amounted to 0.07% of all the CHD operated on during the same period of time. 5 cases of TOF and 2 of DORV with left UAPA were repaired by using valved conduits in 3,transannular patches with monocusp in 3.4 cases of PDA with UAPA were repaired through the pulmonary artery under CPB in 3 and by ligation through left thoracotomy in 1.2 cases of TOF died due to technical problems of sewing and pulmonary perfusion injuries.All of the 9 survivals had an average of 14 days in the hospital.8 patients had been followed up from 2 to 89 months with a mean of 35.7 months. They were all alive and well except one had once suffered from hemoptysis.UAPA associated with CHD can be diagnosed by cineangiography. Operation should be done as soon as the diagnosis is established. The use of valved conduit,transvalvular patch with monocusp and especially homograft of the aorta or pulmonary artery with valves intact can reduce or avoid pulmonary regurgitation in TOF and DORV. There is no need to reconstruct the UAPA.Large collaterals between the systemic and pulmonary circulation in the involved lung should be treated either before or during the operation. It is safer to close the PDA with pulmonary hypertension through median incision and under CPB.