Patel R G, Freedom R M, Bloom K R, Rowe R D
Am J Cardiol. 1978 Nov;42(5):800-9. doi: 10.1016/0002-9149(78)90100-5.
Stenosis of the semilunar valve in the presence of a functionally single arterial trunk is uncommon. Three patients with truncus arteriosus, two with tetralogy of Fallot and pulmonary atresia and one with pulmonary atresia and intact septum were diagnosed as having stenosis of the truncal or aortic valve on the basis of clinical, echocardiographic, hemodynamic and angiocardiographic findings. Echocardiograms consistently showed multiple diastolic closure lines and abnormal semilunar valves in addition to the aortic override in five patients and hypoplastic right ventricle in the patient with pulmonary atresia and intact septum. Peak systolic gradients between the left ventricle and truncus (or aorta) at cardiac catheterization ranged from 20 to 47 mm Hg. Retrograde aortography confirmed a domed and stenotic semilunar valve. Cyanosis was progressive in the three patients with pulmonary atresia. Two patients had arterial anastomosis, and one had a right ventricle-pulmonary arterial graft in addition to aortic valvotomy. One of the three patients with truncus arteriosus underwent complete repair in addition to truncal valvotomy but he died in the post-operative period. The other two patients with truncus arteriosus died of intractable congestive cardiac failure before surgical intervention. It is suggested that the presence of semilunar valve stenosis in these patients adversely affects the prognosis. The myocardium is already jeopardized as a result of hypoxia in pulmonary atresia and left ventricular diastolic overload in patients with truncus arteriosus. The added burden of semilunar valve stenosis may further compromise the functional status of the myocardium.
功能性单一大动脉干合并半月瓣狭窄并不常见。根据临床、超声心动图、血流动力学及心血管造影检查结果，3例动脉干畸形、2例法洛四联症合并肺动脉闭锁及1例肺动脉闭锁合并完整室间隔的患者被诊断为动脉干或主动脉瓣狭窄。超声心动图在5例患者中持续显示多条舒张期关闭线及异常半月瓣，同时伴有主动脉骑跨，而肺动脉闭锁合并完整室间隔的患者则显示右心室发育不良。心导管检查时左心室与动脉干（或主动脉）之间的收缩期峰值压差为20～47mmHg。逆行主动脉造影证实半月瓣呈圆顶状且狭窄。3例肺动脉闭锁患者的发绀呈进行性加重。2例患者行动脉吻合术，1例除主动脉瓣切开术外还行了右心室-肺动脉移植术。3例动脉干畸形患者中的1例除行动脉干瓣膜切开术外还接受了完全修复，但术后死亡。另外2例动脉干畸形患者在手术干预前死于难治性充血性心力衰竭。提示这些患者中半月瓣狭窄的存在对预后产生不利影响。由于肺动脉闭锁时的缺氧及动脉干畸形患者的左心室舒张期负荷过重，心肌已受到损害。半月瓣狭窄增加的负担可能会进一步损害心肌的功能状态。
