The following is the summary of “American Association for Thoracic Surgery (AATS) 2022 Expert Consensus Document: Management of infants and neonates with tetralogy of Fallot” published in the January 2023 issue of Thoracic and cardiovascular surgery by Miller, et al.

The treatment of tetralogy of Fallot (TOF) with pulmonary stenosis is still debated after decades of study. Although there is a shortage of evidence to use as a benchmark, clinicians must nevertheless evaluate novel, ever-evolving therapeutic modalities. As a result, the American Association for Thoracic Surgery tasked the TOF Clinical Practice Standards Committee with developing guidelines for this area of practice, with a particular emphasis on the optimal timing and variety of interventions, the care of high-risk patients, the technical aspects of performing interventions, and the most effective methods for evaluating their results. Each group member was also tasked with writing down a list of questions that needed to be answered in order to advance the field. Researchers acknowledge that the level of clinical experience at different institutions may affect how well this framework may be used in practice. Expert cardiologists and surgeons from around the world make up the TOF Clinical Practice Standards Committee. Using terms related to TOF and its management, a medical librarian searched PubMed, Embase, Scopus, and Web of Science for relevant citations. 

The search was limited to articles written in English and published in 2000 or later. Non Primary sources, such as reviews and articles about pulmonary atresia, an absent pulmonary valve, atrioventricular septal abnormalities, and TOF in adults, were also left out. Over roughly 20,000 hits, investigators narrowed it down to the relevant 163. Newer research, those with higher sample sizes, and those that used randomized or propensity score-matched comparator groups were given more weight. Using a modified Delphi technique, 80% of the members voted, with 75% agreement on each statement, to generate expert consensus statements with class of recommendation and level of evidence. To decrease hospital time, complications, and the likelihood of a transannular patch, it is reasonable to do corrective surgery for asymptomatic newborns between the ages of 3 and 6 months. Both palliative care and main full surgical repair help treat symptomatic newborns. Patients with cerebral hemorrhage, sepsis, or other end-organ damage, as well as those who were born prematurely or with low birth weight, are considered to be at high risk. Palliation may be the best option for these high-risk individuals, and catheter-based techniques may be preferable to surgical palliation in patients with amenable anatomy. The function of catheter-based therapies will be better understood when more research is conducted. 

Both transatrial and transventricular techniques are effective for full surgical repair; however, the smallest ventriculotomy possible should be used. The pulmonary valve should be protected wherever possible, and reconstruction is an option if it’s beyond repair. Confirmation of appropriate relief of the right ventricular outflow obstruction at the conclusion of the surgery is recommended, as is the discovery of a major fixed anatomical obstruction that requires additional intervention. Given their understanding and the identified gaps, the study group proposes several important questions to be answered by future research and possibly by a TOF registry. These include: when to palliate versus proceed with complete surgical correction, and the best type of palliation; the best surgical approach for complete repair to ensure the long-term preservation of right ventricular function; and the utility, efficacy, and durability of different pulmonary valve preservation and reconstruction.