Caneo Luiz Fernando, Turquetto Aida L R, Neirotti Rodolfo A, Binotto Maria A, Miana Leonardo A, Tanamati Carla, Penha Juliano G, Silveira João B D, Alexandre E Silva Thais M, Jatene Fabio B, Jatene Marcelo B
1 Instituto do Coração, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
2 Surgery and Pediatrics, Emeritus Michigan State University, East Lansing, MI, USA.
World J Pediatr Congenit Heart Surg. 2017 May;8(3):376-384. doi: 10.1177/2150135117701405.
The Fontan operation (FO) has evolved and many centers have demonstrated improved results relative to those from earlier eras. We report a single-institution experience over three decades, describing the outcomes and evaluating risk factors.
Successive patients undergoing primary FO were divided into era I (1984-1994), era II (1995-2004), and era III (2005-2014). Clinical and operative notes were reviewed for demographic, anatomic, and procedure details. End points included early and late mortality and a composite of death, heart transplantation (HTX), or Fontan takedown.
A total of 420 patients underwent 18 atriopulmonary connections, 82 lateral tunnels (LT), and 320 extracardiac conduit (EC) Fontan procedures. Forty-six (11%) patients died; early and late mortality were 7.9% and 3.1%, respectively. Eight (1.9%) patients underwent HTX, 11 (2.6%) underwent Fontan conversion to EC, and 1 (0.2%) takedown of EC to bidirectional Glenn shunt. Prevalence of concomitant valve surgery ( P < .001) and pulmonary artery reconstruction ( P < .001) differed over the eras. Preoperative valve regurgitation was associated with likelihood of early mortality (odds ratio [OR] = 3.5, P = .002). Embolic events (OR = 1.9, P = .047), preoperative valve regurgitation (OR = 2.3, P = .029), diagnosis of unbalanced atrioventricular canal defect (OR = 1.14, P = .03), and concomitant valve replacement (OR = 6.9, P = .001) during the FO were associated with increased risk of the composite end point (death, HTX, or takedown).
Technical modifications did not result in improved results across eras, due in part to more liberal indications for surgery in the recent years. Valve regurgitation, unbalanced atrioventricular canal, embolic events, or concomitant valve replacement were associated with FO failure.
Fontan手术（FO）已经发展，许多中心已证明与早期相比结果有所改善。我们报告了一个单一机构三十年的经验，描述结果并评估风险因素。
将接受初次FO的连续患者分为I期（1984 - 1994年）、II期（1995 - 2004年）和III期（2005 - 2014年）。回顾临床和手术记录以获取人口统计学、解剖学和手术细节。终点包括早期和晚期死亡率以及死亡、心脏移植（HTX）或Fontan手术拆除的综合情况。
共有420例患者接受了18例心房肺连接术、82例侧隧道（LT）和320例心外管道（EC）Fontan手术。46例（11%）患者死亡；早期和晚期死亡率分别为7.9%和3.1%。8例（1.9%）患者接受了心脏移植，11例（2.6%）接受了Fontan手术转为EC，1例（0.2%）将EC拆除转为双向Glenn分流术。不同时期同期瓣膜手术（P <.001）和肺动脉重建（P <.001）的发生率有所不同。术前瓣膜反流与早期死亡率相关（比值比[OR] = 3.5，P =.002）。FO期间的栓塞事件（OR = 1.9，P =.047）、术前瓣膜反流（OR = 2.3，P =.029）、诊断为房室管缺损不平衡（OR = 1.14，P =.03）以及同期瓣膜置换（OR = 6.9，P =.001）与综合终点（死亡、HTX或拆除）风险增加相关。
技术改进并未在各时期带来更好的结果，部分原因是近年来手术指征更为宽松。瓣膜反流、房室管不平衡、栓塞事件或同期瓣膜置换与Fontan手术失败相关。
