Tetralogy of Fallot in the Current Era

Semin Thorac Cardiovasc Surg. 2019 Autumn;31(3):496-504. doi: 10.1053/j.semtcvs.2018.10.015. Epub 2018 Nov 2.

Abstract

Only few studies have reported long-term outcome of the transatrial-transpulmonary approach in the current era of management of tetralogy of Fallot (ToF). We investigated 15-year outcome of correction via a transatrial-transpulmonary approach in a large cohort of successive patients operated in the 21st century. All infant ToF patients undergoing transatrial-transpulmonary ToF correction between 2000 and 2015 were included (N = 177, 106 male, median follow-up 7.1 (interquartile range 3.0-10.9) years. Data regarding postoperative complications, reinterventions, development of atrial and ventricular arrhythmia, cardiac function, and survival were evaluated. Prior shunting was performed in 10 patients (6%). The transatrial-transpulmonary approach resulted in valve-sparing surgery in 57 patients (32%). Postoperative surgical complications included junctional ectopic tachycardia (N = 12, 7%), pericardial (N = 10, 6%) or pleural effusion (N = 7, 3%), chylothorax (N = 7, 4%), bleeding requiring reoperation (N = 4, 3%), and superficial wound infection (N = 1). Fifty-one patients underwent 68 reinterventions, mainly due to pulmonary restenosis (PS) (N = 57). ToF correction at age <2 months and double outlet or double-chambered right ventricle variants of the ToF spectrum were independent predictors for reintervention. Patients undergoing valve-sparing ToF correction had a significant longer PR-free survival than those with a transannular patch (8.5 [95% confidence interval 6.8-10.3] years vs 1.1 [95% confidence interval 0.8-1.5] years; P < 0.001). Overall mortality was 2.8%; mortality rates were higher in premature/dysmature newborns (0.7% vs 9.5%; P < 0.001). Although the 15-year outcome of the transatrial-transpulmonary approach in terms of postoperative complications and mortality rates is excellent, the high incidence of moderate and severe PR is worrisome. Valve-sparing surgery was associated with a substantially lower incidence of PR, yet was surgically not possible in the majority of patients.

Keywords: Congenital heart disease; Pulmonary regurgitation; Pulmonary stenosis; Tetralogy of Fallot; Transatrial-transpulmonary correction.

Publication types

  • Comparative Study

MeSH terms

  • Cardiac Surgical Procedures* / adverse effects
  • Cardiac Surgical Procedures* / mortality
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Postoperative Complications / etiology
  • Postoperative Complications / therapy
  • Pulmonary Valve Insufficiency / diagnostic imaging
  • Pulmonary Valve Insufficiency / mortality
  • Pulmonary Valve Insufficiency / physiopathology
  • Pulmonary Valve Insufficiency / surgery*
  • Pulmonary Valve Stenosis / diagnostic imaging
  • Pulmonary Valve Stenosis / mortality
  • Pulmonary Valve Stenosis / physiopathology
  • Pulmonary Valve Stenosis / surgery*
  • Recovery of Function
  • Retrospective Studies
  • Risk Factors
  • Tetralogy of Fallot / diagnostic imaging
  • Tetralogy of Fallot / mortality
  • Tetralogy of Fallot / physiopathology
  • Tetralogy of Fallot / surgery*
  • Time Factors
  • Treatment Outcome
  • Ventricular Outflow Obstruction / diagnostic imaging
  • Ventricular Outflow Obstruction / mortality
  • Ventricular Outflow Obstruction / physiopathology
  • Ventricular Outflow Obstruction / surgery*