Gatzoulis M A, Shore D, Yacoub M, Shinebourne E A
Department of Paediatric Cardiology and Cardiac Surgery, Royal Brompton National Heart and Lung Hospital, London.
Br Heart J. 1994 Jun;71(6):579-83. doi: 10.1136/hrt.71.6.579.
To report recent experience of patients with complete atrioventricular septal defect and tetralogy of Fallot, with emphasis on anatomical features, diagnosis, and management.
Case notes were reviewed and patients were assessed at follow up by clinical examination and cross sectional and Doppler echocardiography.
Tertiary cardiothoracic referral centre.
Between 1987 and 1992 13 patients with atrioventricular septal defect and tetralogy of Fallot (12 with concordant and one with double outlet ventriculoarterial connections) underwent surgery; 10 underwent complete intracardiac repair. 11 patients had Down's syndrome. The complete diagnosis was established preoperatively by cross sectional echocardiography in all but one patient. A tri-leaflet left atrioventricular valve as seen in parasternal short axis views was the diagnostic feature of atrioventricular septal defect, with tetralogy of Fallot diagnosed from the presence of anterocephalad deviation of the outlet septum producing subvalvar pulmonary stenosis as seen in subcostal right anterior oblique views.
Total correction consisted of closure of the atrioventricular septal defect by a combined right atrial and ventricular approach, reconstruction of the atrioventricular valves, and relief of the obstruction within the right ventricular outflow tract. Separate patches were used to close the atrial and ventricular septal defects. Modified Blalock-Taussig shunts were performed in three patients, who await intracardiac repair. Surgical correction was carried out at mean (range) age of 5 (2 to 15) years.
Diagnostic methods, surgical results, and functional state after complete correction.
The presence of an atrioventricular septal defect was missed preoperatively in one patient with tetralogy of Fallot. The characteristic goose neck deformity on the left ventriculogram was not present and the tri-leaflet nature of the left atrioventricular valve was not sought on echocardiography. Of the 10 patients who underwent complete repair, nine are alive and one died 34 days after operation with adult respiratory distress syndrome. Examination at necropsy showed an excellent surgical correction. Mean (range) follow up was 23 (8 to 48) months. All nine patients are alive and well (New York Heart Association Class 1).
Accurate diagnosis and staged management with improved surgical techniques have lowered mortality of this complex combination of cardiac defects. The current policy of this group is to recommend a systemic to pulmonary arterial shunt procedure for symptomatic children younger than 2 years and total correction in older children.
报告完全性房室间隔缺损合并法洛四联症患者的近期经验，重点关注解剖特征、诊断和治疗。
回顾病例记录，并在随访时通过临床检查、横断面及多普勒超声心动图对患者进行评估。
三级心胸转诊中心。
1987年至1992年间，13例房室间隔缺损合并法洛四联症患者（12例心室动脉连接一致，1例为双出口心室动脉连接）接受了手术；10例接受了完全性心内修复。11例患者患有唐氏综合征。除1例患者外，所有患者术前均通过横断面超声心动图明确诊断。胸骨旁短轴视图中可见的三叶式左房室瓣是房室间隔缺损的诊断特征，法洛四联症则根据肋下右前斜视图中可见的流出道间隔向前头侧偏移导致瓣膜下肺动脉狭窄来诊断。
完全矫正包括通过右心房和心室联合入路闭合房室间隔缺损、重建房室瓣以及解除右心室流出道梗阻。分别使用补片闭合心房和心室间隔缺损。3例患者进行了改良Blalock-Taussig分流术，等待心内修复。手术矫正的平均（范围）年龄为5（2至15）岁。
诊断方法、手术结果及完全矫正后的功能状态。
1例法洛四联症患者术前漏诊了房室间隔缺损。左心室造影未出现特征性的鹅颈畸形，超声心动图也未检查左房室瓣的三叶特征。10例接受完全修复的患者中，9例存活，1例术后34天因成人呼吸窘迫综合征死亡。尸检显示手术矫正效果良好。平均（范围）随访时间为23（8至48）个月。所有9例患者均存活且状况良好（纽约心脏协会心功能分级为1级）。
准确的诊断和采用改良手术技术的分期治疗降低了这种复杂心脏缺陷组合的死亡率。该团队目前的策略是，对于2岁以下有症状的儿童推荐体肺分流手术，对于年龄较大的儿童则进行完全矫正。
