Luber J M, Castaneda A R, Lang P, Norwood W I
Circulation. 1983 Sep;68(3 Pt 2):II144-7.
Fifty-seven patients underwent reparative surgery between 1973 and 1981 for various forms of double-outlet right ventricle. Pulmonic stenosis was present in 35 (61%), 15 (43%) of whom required a valved conduit for repair. Subaortic stenosis was present in six patients (11%). Hospital mortality was 12% (7/57), with three of the deaths among five patients with associated defects of the atrioventricular valves. During a mean follow-up of 3.6 years (range 2 months to 9 years), there have been no late deaths. Repeat cardiac catheterization in 28 of 50 survivors (56%) showed a residual ventricular septal defect (Qp/Qs greater than 1.5) in six patients, residual pulmonic stenosis (gradient greater than 35 mm Hg) in six patients, and subaortic obstruction (gradient greater than 50 mm Hg) in two patients: The indications for reoperation in 12 of 50 survivors was a residual ventricular septal defect in five patients, atrioventricular valve regurgitation in three patients, subaortic or subpulmonic obstruction in three patients, and aortic valve regurgitation in one patient. None of the patients died after repeat surgery. Our results indicate that surgical risk is related to the anatomic complexity of the malformation. Although a good hemodynamic outcome was achieved at initial surgery in the majority of patients, a residual abnormality was present in 24% needing surgery. When present, significant defects can be repaired with low mortality and morbidity.
1973年至1981年间，57例患者因各种类型的右心室双出口接受了修复手术。35例（61%）存在肺动脉狭窄，其中15例（43%）需要使用带瓣管道进行修复。6例患者（11%）存在主动脉下狭窄。住院死亡率为12%（7/57），5例伴有房室瓣相关缺陷的患者中有3例死亡。在平均3.6年（范围2个月至9年）的随访期间，无晚期死亡病例。50例幸存者中的28例（56%）接受了重复心导管检查，结果显示6例患者存在残余室间隔缺损（肺循环血流量/体循环血流量大于1.5），6例患者存在残余肺动脉狭窄（压差大于35 mmHg），2例患者存在主动脉下梗阻（压差大于50 mmHg）：50例幸存者中有12例再次手术的指征为5例患者存在残余室间隔缺损，3例患者存在房室瓣反流，3例患者存在主动脉下或肺动脉下梗阻，1例患者存在主动脉瓣反流。再次手术后无患者死亡。我们的结果表明，手术风险与畸形的解剖复杂性相关。尽管大多数患者在初次手术时取得了良好的血流动力学结果，但24%的患者存在需要手术治疗的残余异常。当存在明显缺陷时，可以以低死亡率和低发病率进行修复。
