Gaynor J William, Bridges Nancy D, Cohen Mitchell I, Mahle William T, Decampli William M, Steven James M, Nicolson Susan C, Spray Thomas L
Division of Pediatric Cardiothoracic Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, PA, USA.
J Thorac Cardiovasc Surg. 2002 Feb;123(2):237-45. doi: 10.1067/mtc.2002.119337.
This study was undertaken to evaluate factors contributing to a decrease in early mortality and morbidity after the Fontan procedure between January 1, 1992, and December 31, 1999.
Outcomes evaluated were early survival, duration of pleural effusions, and duration of hospitalization. Potential predictors evaluated included anatomic diagnosis, presence of a common atrioventricular valve, preoperative pulmonary artery pressure, type of Fontan operation, type of intentional right-to-left shunt or baffle fenestration, and use of modified ultrafiltration.
The modified Fontan procedure was performed in 332 patients at a median age of 22 months (range, 11-380 months) and a median weight of 11 kg (range, 5.8-120 kg). Prior stage I reconstructive surgery for classic or variant hypoplastic left heart syndrome had been performed in 205 (53%) of 332 patients, and 318 (96%) had undergone an interim superior cavopulmonary connection. A lateral-tunnel Fontan operation was performed in 281 patients, and an extracardiac conduit Fontan operation was performed in 51 patients. An intentional right-to-left shunt was created in 298 (90%) patients. Between 1992 and 1999, the outcome after the modified Fontan operation improved significantly. Overall mortality was 6.6% (22/332), with only 2 deaths since 1994. Morbidity was also reduced, with a decreased duration of pleural effusions and decreased hospital stay. In a multivariable analysis of the entire cohort, only the presence of a common atrioventricular valve (odds ratio, 7.64; 95% confidence limits, 2.07-28.14; P =.0002) and increased preoperative pulmonary artery pressure (odds ratio, 1.46/1 mm Hg increase; 95% confidence limits, 1.2-1.78; P <.001) increased the risk of early death, whereas use of a single-punch fenestration in a lateral-tunnel Fontan (odds ratio, 0.06; 95% confidence limits, 0.01-0.65; P =.02) and use of modified ultrafiltration (odds ratio, 0.14; 95% confidence limits, 0.03-0.72; P =.019) decreased the risk of death. The risk of prolonged pleural effusions (>3 days) was increased in patients with hypoplastic left heart syndrome (odds ratio, 1.73; 95% confidence limits, 1.07-2.81; P =.03) and was decreased by use of a single-punch fenestration in a lateral-tunnel Fontan operation (odds ratio, 0.17; 95% confidence limits, 0.07-0.4; P <.001), as well as by the use of modified ultrafiltration (odds ratio, 0.25; 95% confidence limits, 0.15-0.40; P <.01).
In a contemporary series of Fontan operations performed largely in patients with hypoplastic left heart syndrome or variants, systemic ventricle morphology had no effect on mortality. Some patient characteristics, however, continue to influence outcome. The decrease in mortality and morbidity in the current era is attributed to changes in management strategies, specifically the use of modified ultrafiltration and baffle fenestration.
本研究旨在评估1992年1月1日至1999年12月31日期间，影响Fontan手术早期死亡率和发病率降低的因素。
评估的结果包括早期生存率、胸腔积液持续时间和住院时间。评估的潜在预测因素包括解剖诊断、共同房室瓣的存在、术前肺动脉压、Fontan手术类型、有意的右向左分流或挡板开窗类型以及改良超滤的使用。
332例患者接受了改良Fontan手术，中位年龄22个月（范围11 - 380个月），中位体重11 kg（范围5.8 - 120 kg）。332例患者中有205例（53%）曾接受过经典或变异型左心发育不全综合征的I期重建手术，318例（96%）接受过临时上腔静脉-肺动脉连接术。281例患者接受了侧隧道Fontan手术，51例患者接受了心外管道Fontan手术。298例（90%）患者建立了有意的右向左分流。1992年至1999年期间，改良Fontan手术后的结果有显著改善。总体死亡率为6.6%（22/332），自1994年以来仅有2例死亡。发病率也有所降低，胸腔积液持续时间缩短，住院时间减少。在对整个队列的多变量分析中，只有共同房室瓣的存在（优势比，7.64；95%置信区间，2.07 - 28.14；P = 0.0002）和术前肺动脉压升高（优势比，每升高1 mmHg为1.46；95%置信区间，1.2 - 1.78；P < 0.001）增加了早期死亡风险，而在侧隧道Fontan手术中使用单孔开窗（优势比，0.06；95%置信区间，0.01 - 0.65；P = 0.02）和使用改良超滤（优势比，0.14；95%置信区间，0.03 - 0.72；P = 0.019）降低了死亡风险。左心发育不全综合征患者胸腔积液延长（>3天）的风险增加（优势比，1.73；95%置信区间，1.07 - 2.81；P = 0.03），而在侧隧道Fontan手术中使用单孔开窗（优势比，0.17；95%置信区间，0.07 - 0.4；P < 0.001）以及使用改良超滤（优势比，0.25；95%置信区间，0.15 - 0.40；P < 0.01）可降低该风险。
在当代一系列主要针对左心发育不全综合征或变异型患者进行的Fontan手术中，体循环心室形态对死亡率无影响。然而，一些患者特征继续影响手术结果。当前时代死亡率和发病率的降低归因于管理策略的改变，特别是改良超滤和挡板开窗的使用。
