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Division of Pediatric Cardiothoracic Surgery, Children's Medical Center, 1935 Medical District Dr, Suite C3211, Dallas, TX 75235
(Email: kristine.guleserian{at}utsouthwestern.edu).
Although it has been well over 50 years since Lillehei's historic first successful intracardiac repair for tetralogy of Fallot (ToF) in a 10-month-old infant using cross circulation [1], surgical techniques continue to evolve and controversy related to surgical strategy persists. The optimal timing of repair and the ideal surgical approach for both the symptomatic and asymptomatic neonate and infant remain areas of ongoing debate.
Restoration of normal cardiovascular anatomy and physiology with early primary repair has been advocated since the earliest reports by such pioneers as Barratt-Boyes and Castaneda [2, 3]. Alleviation of chronic cyanosis, optimization of the developing myocardium, promotion of somatic growth and organ development, particularly with respect to pulmonary vasculature and central nervous system, elimination of the substrate for right ventricular hypertrophy (RVH), and avoidance of the nontrivial risks associated with initial palliative systemic-to-pulmonary artery shunts are all obvious advantages. Despite the increasing trend toward early primary repair, not all centers have adopted this surgical philosophy.
Definitive repair in infants aged older than 12 months has been shown to be associated with increased mortality [4], and improved recovery of RVH has been reported when intracardiac repair is performed before 6 months of age [5]. Primary repair in infants younger than 3 months old has been associated with increased morbidity in terms of prolonged lactate clearance, ventilator days, and length of stay [4], thus the premise for definitive repair between 3 and 6 months of age advocated by most centers appears to be reasonably justified for the asymptomatic infant.
In their retrospective review of 90 consecutive patients aged younger than 4 months with ToF with pulmonary stenosis, and including 25 "high-risk" symptomatic neonates, Tamesberger and colleagues [6] have shown that early primary repair can be performed with excellent early and midterm survival, a finding that is well supported in the literature [4, 7–9]. Their commendable results provide further support that early primary ToF repair in young infants and symptomatic neonates is associated with a higher perceived but not observed operative risk. Although operative survival clearly remains the surgeon's primary concern, it is the longer-term effect of surgical timing as well as the surgical approach that becomes of critical secondary importance in the immediate postoperative period and beyond.
At the time of surgical repair, all patients were deliberately left with a patent foramen ovale. We too support leaving an atrial-level communication as a "pop-off" for right-to-left shunting and routinely place peritoneal drains in all neonates given their nearly certain postoperative right ventricular dysfunction following repair. Unlike the authors, we favor a transatrial-transpulmonary approach, routinely assess the tricuspid valve, perform tricuspid commissuroplasty or annuloplasty when necessary, and perform branch pulmonary arterioplasty with pulmonary homograft as indicated by preoperative pulmonary artery z-score.
In contrast to previous reports [4], younger age at repair was not associated with increased postoperative morbidity, even when considering those with extracardiac anomalies and genetic or metabolic syndromes. The effect of improved myocardial protection, intraoperative perfusion strategies, afterload reduction, and postoperative intensive care cannot be underscored enough when looking at outcomes in this modern era.
Although the reoperation rate was similar between the two groups, it is not surprising that the symptomatic neonatal group required more frequent use of a transannular patch and a greater number of reinterventions for pulmonary stenosis. This observation is indeed more reflective of the severity of right ventricular outflow tract (RVOT) pathology at the time of presentation than age at repair. As such, the 100% reintervention rate inherent with staged repair must be considered when assessing outcomes related to surgical approach.
Early primary ToF repair can certainly be performed with acceptable mortality; however, longer-term follow-up is necessary to ascertain whether early relief of RVOT obstruction will attenuate the untoward effects on myocardial performance, arrhythmia potential, neurologic outcome, and need for reintervention. Until plausible advances in tissue engineering, fetal intervention, and genetic modification for congenital heart disease are realized, further refinement in surgical management of ToF must be targeted at minimizing the need for any type of reintervention.
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