Rychik J, Levy H, Gaynor J W, DeCampli W M, Spray T L
Divisions of Cardiology and Cardiothoracic Surgery, Children's Hospital of Philadelphia, PA 19104, USA.
J Thorac Cardiovasc Surg. 1998 Dec;116(6):924-31. doi: 10.1016/S0022-5223(98)70042-X.
Pulmonary atresia with intact ventricular septum is an anatomically heterogeneous anomaly with a variety of surgical strategies possible. We sought to compare the outcome of patients with a single ventricle approach to those with a biventricular repair and to compare outcome of patients with coronary abnormalities to those with normal coronary arteries.
A retrospective review of our surgical database revealed 67 patients with pulmonary atresia with intact ventricular septum operated on between 1981 and 1998. Patients were categorized on the basis of initial surgical strategy: strategy A, aortopulmonary shunt alone (n = 31); strategy B, right ventricular recruitment (n = 32); strategy C, heart transplantation (n = 4). Tricuspid valve size (Z-score) and coronary anatomy were determined. Right ventricular-coronary artery dependency was noted in 8 patients.
Overall actuarial survivals at 1, 5, and 8 years were 82%, 76%, and 76%. Mortality was highest in infancy (10 of 16 deaths). Outcome was equivalent for all 3 strategies. There was no difference in tricuspid valve size between survivors and nonsurvivors (mean Z-score -2.0 (2.5) vs -2.0 (1.9), P =.83). There was no difference in survival based on severity of coronary abnormality. Only one third of patients had a successful biventricular repair, and the tricuspid valve was significantly larger in these than in patients who had Fontan operation (mean Z-score -0.53 [1.6], range -3.5 to 1, versus mean Z-score -3.03 [2.7], range -5.5 to 0, P =.002).
Surgical outcome for patients born with pulmonary atresia with intact ventricular septum is satisfactory. The strategies of biventricular repair, single ventricle palliation, and heart transplantation allow for equal outcome among all anatomic subtypes.
室间隔完整的肺动脉闭锁是一种解剖结构异质性异常，有多种可能的手术策略。我们试图比较采用单心室手术方法的患者与采用双心室修复手术的患者的治疗结果，并比较有冠状动脉异常的患者与冠状动脉正常的患者的治疗结果。
对我们的手术数据库进行回顾性研究，发现1981年至1998年间有67例室间隔完整的肺动脉闭锁患者接受了手术。患者根据初始手术策略进行分类：策略A，仅行体肺分流术（n = 31）；策略B，右心室扩大术（n = 32）；策略C，心脏移植（n = 4）。确定三尖瓣大小（Z值）和冠状动脉解剖结构。8例患者存在右心室-冠状动脉依赖。
1年、5年和8年的总体精算生存率分别为82%、76%和76%。婴儿期死亡率最高（16例死亡中有10例）。三种策略的治疗结果相当。存活者与非存活者的三尖瓣大小无差异（平均Z值-2.0 [2.5] 对-2.0 [1.9]，P = 0.83）。基于冠状动脉异常严重程度的生存率无差异。只有三分之一的患者双心室修复成功，这些患者的三尖瓣明显大于接受Fontan手术的患者（平均Z值-0.53 [1.6]，范围-3.5至1，而平均Z值-3.03 [2.7]，范围-5.5至0，P = 0.002）。
室间隔完整的肺动脉闭锁患者的手术治疗结果令人满意。双心室修复、单心室姑息治疗和心脏移植策略在所有解剖亚型中都能取得相同的治疗结果。
