Hanley F L, Sade R M, Blackstone E H, Kirklin J W, Freedom R M, Nanda N C
Department of Cardiac Surgery, Children's Hospital, Harvard Medical School, Boston, MA.
J Thorac Cardiovasc Surg. 1993 Mar;105(3):406-23, 424-7; discussion 423-4.
A total of 171 neonates with pulmonary atresia and intact ventricular septum were entered into a prospective multiinstitutional study between January 1, 1987, and January 1, 1991. Treatment was not assigned randomly but was selected by the responsible physicians. The Z-value (standard deviation units) of the diameter of the tricuspid valve was less than -2 in 52% of patients and less than -4 in 26%; it was highly correlated with right ventricular cavity size (which was small in 90% of patients and was severely reduced in 54%). Coronary artery-right ventricular fistulas were present in 45% of patients, and right ventricular dependency was severe in 9%; diameter (Z-value) of the tricuspid valve was negatively correlated (P < 0.0001) with the prevalence of both. Survival was 81% at 1 month after the first intervention and 64% at 4 years; the hazard function (instantaneous risk of death) declined rapidly after the initial procedure but remained appreciable for 24 months. Multivariable analysis showed small diameter of the tricuspid valve, severe right ventricular coronary dependency, birth weight, and the date and type of initial procedure to be risk factors for time-related death. Subsequent procedures were performed in 51% of patients. Among patients undergoing an initial procedure that did not include a systemic-pulmonary artery shunt, only 49% did not receive a shunt in the subsequent 1 month; small size of the tricuspid valve was the only risk factor for receiving the subsequent shunt. Ninety-eight percent of living patients whose initial procedure did not include a transannular patch were free of a subsequently placed transannular patch within 1 month, but only 45% were free of it 3 years later; no risk factors were identified. Eighteen percent of living patients had received a one-ventricle repair within 3 years, and 32% had received a two-ventricle repair; the remainder (50%) had incompletely separated pulmonary and systemic circulations. The only patient-specific risk factor for not receiving a two-ventricle repair was the Z-value of the tricuspid valve.
1987年1月1日至1991年1月1日期间，共有171例患有肺动脉闭锁且室间隔完整的新生儿进入一项前瞻性多机构研究。治疗并非随机分配，而是由负责的医生选择。三尖瓣直径的Z值（标准差单位）在52%的患者中小于-2，在26%的患者中小于-4；它与右心室腔大小高度相关（90%的患者右心室腔小，54%的患者右心室腔严重缩小）。45%的患者存在冠状动脉-右心室瘘，9%的患者右心室依赖严重；三尖瓣直径（Z值）与两者的发生率呈负相关（P<0.0001）。首次干预后1个月的生存率为81%，4年时为64%；风险函数（即时死亡风险）在初始手术后迅速下降，但在24个月内仍相当可观。多变量分析显示，三尖瓣直径小、严重的右心室冠状动脉依赖、出生体重以及初始手术的日期和类型是与时间相关死亡的危险因素。51%的患者进行了后续手术。在接受的初始手术不包括体肺分流术的患者中，只有49%在随后1个月内未接受分流术；三尖瓣尺寸小是接受后续分流术的唯一危险因素。初始手术不包括跨环补片的存活患者中，98%在1个月内未放置后续的跨环补片，但3年后只有45%未放置；未发现危险因素。18%的存活患者在3年内接受了单心室修复，32%接受了双心室修复；其余（50%）患者的肺循环和体循环未完全分离。未接受双心室修复的唯一患者特异性危险因素是三尖瓣的Z值。
