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AATS publishes new Expert Consensus Document for Management of infants and neonates with tetralogy of Fallot

October 26, 2022 -- AATS announces the publication of the 2022 Expert Consensus Document (ECD): Management of infants and neonates with tetralogy of Fallot. The function of ECDs is to provide recommendations and options about procedures, interventions, case management, pertinent research, best practices, and other aspects of the field of Cardiothoracic surgery. International specialists are brought together by AATS Clinical Practice Standards Committees to conduct rigorous studies of the literature and, through a strict consensus process, produce documents that will guide and direct practitioners throughout the world.

The American Association for Thoracic Surgery (AATS) 2022 Expert Consensus Document: Management of infants and neonates with tetralogy of Fallot

Tetralogy of Fallot with pulmonary stenosis (TOF) is one of the more common congenital heart defects that may present to the pediatric cardiologist and surgeon. Despite decades of experience, aspects of the management of TOF remain controversial. Practitioners must consider newer, evolving treatment strategies with limited data to guide decision-making. The TOF Clinical Practice Standards Committee, a multinational, multidisciplinary group of cardiologists and surgeons with expertise in TOF, was asked to provide a framework on this topic. A group of 16 specialists focused on timing and types of interventions, management of high-risk patients, technical considerations during interventions, and best practices for assessment of outcomes of the interventions. They were also tasked with identifying pertinent research questions for future investigations.

With the assistance of a medical librarian, the group narrowed its search from almost 20,000 documents to 163 for close study and analysis. Greater consideration was given to more recent studies, larger studies, and those using comparison groups with randomization or propensity score matching.

Based on the consensus process, the group reported the following.

In the asymptomatic infant, complete surgical correction between three and six months is reasonable to reduce the length of stay, rate of adverse events and need for a transannular patch. In the majority of symptomatic neonates, both palliation and primary complete surgical correction are useful treatment options. It is reasonable to consider those with low birth weight or prematurity, small or discontinuous pulmonary arteries, chromosomal anomalies, other congenital anomalies, or other comorbidities compromise as high-risk patients. In these high-risk patients, palliation may be preferred; and, in patients with amenable anatomy, catheter-based procedures may prove favorable over surgical palliation. Ongoing research will give further insight into the role of catheter-based interventions. For complete surgical correction, transatrial and transventricular approaches are effective; however, the smallest possible ventriculotomy should be used. When possible, the pulmonary valve should be spared; and if unsalvageable, reconstruction can be considered. At the conclusion of the operation, adequate relief of the right ventricular outflow obstruction should be confirmed, and identification of a significant fixed anatomical obstruction should prompt further intervention.

Given the current knowledge and the gaps identified, the group proposed several key questions to be answered by future research and potentially by a TOF registry:

  • When to palliate or proceed with complete surgical correction, as well as the ideal type of palliation.
  • The optimal surgical approach for complete repair for the best long-term preservation of right ventricular function.
  • The utility, efficacy, and durability of various pulmonary valve preservation and reconstruction techniques.

In conclusion, the group wrote: “Our work provides an expert consensus document for the management of TOF. It should serve as a basic framework about the approach to the majority of patients with TOF. When applied to individual patients, however, due to the heterogeneous nature of the defect and institutional experience and expertise, some treatment strategies may seem inconsistent with this document but exist well within the standard of care. Future large, long-term, multi-institutional, collaborative studies will help guide treatment strategies.”

Read the expert consensus document here.