Frontera Izquierdo P, Cabezuelo Huerta G
Departamento de Pediatría, Hospital Infantil La Fe, Universidad de Valencia.
Rev Esp Cardiol. 1990 Jun-Jul;43(6):381-4.
We examined the cases of 90 patients found to have univentricular heart at catheterization from January 1971 to January 1988. These patients are the 3.8% of the 2,322 children diagnosed of congenital heart disease by catheterization and angiography in this 17-year period of time. The mean follow-up was 9.5 years. The prognosis is poor: of the 90 patients, 50 died (55.5%) 43 of them in the first year of life. The actuarial survival rate is only the 38.5% at 5 years. Between the 56 children unoperated the mortality rate was the 62.5% and only the 16% (9 patients) survived with good functional results, class I and II of the New York Heart Association. The earlier palliative surgery changes the prognostic and the adverse natural history. The mortality rate in the operated group was the 44.1%. Another 44.1% (15 patients) of the operated group survived, 14 patients in class II and one in class III. The systemic-pulmonary artery shunts are the elective surgical procedure for important pulmonary stenosis, and the Fontan-type procedure is the elective for posterior, definitive repair.
我们研究了1971年1月至1988年1月期间在导管检查中发现患有单心室心脏的90例患者的病例。这些患者占这17年期间通过导管检查和血管造影诊断出先天性心脏病的2322名儿童的3.8%。平均随访时间为9.5年。预后较差：90例患者中，50例死亡（55.5%），其中43例在生命的第一年死亡。5年时的精算生存率仅为38.5%。在56例未接受手术的儿童中，死亡率为62.5%，只有16%（9例患者）存活且功能良好，属于纽约心脏协会的心功能I级和II级。早期姑息性手术改变了预后和不良的自然病程。手术组的死亡率为44.1%。手术组的另外44.1%（15例患者）存活，14例为II级，1例为III级。体肺分流术是治疗重要肺动脉狭窄的选择性手术方法，而Fontan类手术是后期确定性修复的选择性手术。
