Saxena K K, Joseph G, Thomas C S, John S, Krishnaswami S
Department of Cardiology & Cardiothoracic Surgery, Christian Medical College & Hospital, Vellore.
Indian Heart J. 1991 Nov-Dec;43(6):449-53.
Tetralogy of Fallot with absent pulmonary valve is a rare congenital malformation. The diagnostic features and surgical management in ten patients with this malformation have been described. There were five male and five female patients, (age 3 1/2 to 26 years). The common symptoms were recurrent respiratory tract infection and cyanosis. Examination revealed hyperdynamic precordial pulsations, single second heart sound and an early diastolic murmur along the left sternal border in all. Electrocardiograms were indistinguishable from typical tetralogy of Fallot. Chest X-ray revealed cardiomegaly, prominent pulmonary conus and dilated pulmonary arteries in all cases, lung vascularity being variable. Echocardiographic findings were diagnostic and are discussed in detail. Cardiac catheterization and angiocardiography revealed annular stenosis in all, along with aneurysmal dilatation of main and branch pulmonary arteries. Eight patients underwent intracardiac repair. There was no perioperative mortality. All patients have been followed up for 350 months (mean 29 months).
法洛四联症合并肺动脉瓣缺如为一种罕见的先天性畸形。本文描述了10例患有该畸形患者的诊断特征及外科治疗情况。患者中男性5例，女性5例，年龄为3岁半至26岁。常见症状为反复呼吸道感染及发绀。体格检查发现所有患者心前区搏动增强、第二心音单一以及沿胸骨左缘有舒张早期杂音。心电图表现与典型法洛四联症难以区分。胸部X线显示所有病例均有心脏增大、肺动脉圆锥突出及肺动脉扩张，肺血管纹理情况各异。超声心动图检查结果具有诊断价值，将详细讨论。心导管检查及心血管造影显示所有患者均有瓣环狭窄，同时伴有主肺动脉及分支肺动脉瘤样扩张。8例患者接受了心内修复手术。围手术期无死亡病例。所有患者均已随访350个月（平均29个月）。
