Andrews R, Tulloh R, Sharland G, Simpson J, Rollings S, Baker E, Qureshi S, Rosenthal E, Austin C, Anderson D
Department of Congenital Heart Disease, Guy's Hospital, Guy's and St Thomas' NHS Trust, London, UK.
Arch Dis Child. 2001 Dec;85(6):474-7. doi: 10.1136/adc.85.6.474.
Staged reconstructive surgery has radically altered the prognosis of hypoplastic left heart syndrome (HLHS). Antenatal diagnosis allows for appropriate counselling, and time to consider treatment options. We report outcome from a centre where most cases are antenatally diagnosed and delivered on site.
Information was collated on 188 consecutive cases of HLHS between 1995 and 2000, including timing of diagnosis, outcome of pregnancy, and age and outcome at each stage of surgery. At Guy's Hospital, 174 cases were diagnosed antenatally, of whom 50 underwent surgery. Fourteen others (five diagnosed antenatally at other centres, and nine diagnosed postnatally) also underwent surgery.
Survival after stage I (the Norwood operation) was 52% (33/64). Postoperative survival after stage II (the hemi-Fontan operation, performed in 29), and stage III (the Fontan operation, performed in 10), was 100%. Two late deaths occurred 3 and 10 months after stage II, giving overall survival of 48% (31/64). At follow up, three children have neurological impairment, and one had poor right ventricular function necessitating cardiac transplantation.
Antenatal diagnosis allows informed decisions about treatment options, and facilitates preoperative care. Mortality following stage I is high, irrespective of timing of diagnosis, but medium term outcome for survivors is good.
分期重建手术已彻底改变了左心发育不全综合征（HLHS）的预后。产前诊断有助于进行适当的咨询，并留出时间考虑治疗方案。我们报告了一个中心的治疗结果，该中心大多数病例为产前诊断并在当地分娩。
整理了1995年至2000年间连续188例HLHS病例的信息，包括诊断时间、妊娠结局以及手术各阶段的年龄和结局。在盖伊医院，174例病例为产前诊断，其中50例接受了手术。另外14例（5例在其他中心产前诊断，9例产后诊断）也接受了手术。
I期（诺伍德手术）后的生存率为52%（33/64）。II期（半Fontan手术，29例接受此手术）和III期（Fontan手术，10例接受此手术）后的术后生存率为100%。II期后3个月和10个月发生了2例晚期死亡，总体生存率为48%（31/64）。随访时，3名儿童有神经功能障碍，1名右心室功能差需要进行心脏移植。
产前诊断有助于就治疗方案做出明智的决定，并便于术前护理。I期后的死亡率很高，与诊断时间无关，但幸存者的中期结局良好。
