DeCure for Tetralogy of Fallot

One hundred consecutive patients with tetralogy of Fallot operated on between 1995 and 2003 were reviewed. Thirty-one patients received initial palliation, indicated by age under 3 months, unfavourable anatomy, major associated defects, or genetic disorders with poor life expectancy. There were no hospital deaths after palliation; 28 of these patients later underwent complete repair with one hospital death (3.5%). Two patients with severe chromosomal abnormalities died at home. Primary repair was carried out in 69 patients with one hospital death (1.4%). A transannular patch was used in 80% of patients and was not an incremental risk factor for death. Eight reoperations occurred, all in the primary repair group, for residual right ventricular outflow tract obstruction with or without residual ventricular septal defect, or isolated residual ventricular septal defect. The authors concluded that an initial palliative procedure does not influence the outcome of later repair in terms of mortality or morbidity.

A separate retrospective study of 19 patients undergoing transatrial-transpulmonary correction between 2009 and 2010 reported a mean age of 7.4 years. The immediate postoperative pressure gradient between the pulmonary artery and right ventricle was 18.77 mm Hg. There were 2 deaths (10.5%). Complications included conduction disorders in 100% of patients, dominated by right bundle branch block, and pleuropulmonary complications in 41.20%. After a mean follow-up of 11.43 years, no patient had died and all were asymptomatic without significant residual lesion. The authors concluded that the transatrial-transpulmonary approach is associated with low morbidity and mortality in their experience.

A 2004 study stated that long-term results of corrective surgery for tetralogy of Fallot are excellent, with most patients reaching adulthood, but noted that reoperations late after correction may complicate results. A 2020 case report described tetralogy of Fallot in a child with VACTERL association, noting that TOF is associated with many syndromes, most commonly Down syndrome, velocardiofacial syndrome, Holt Oram syndrome, and Poland's syndrome. A 2024 report described a patient 30 years after surgical correction who remains in good condition with only poorer exercise tolerance, and provided a historical overview of treatment methods without discussing contemporary treatments.

What remains missing is prospective data comparing different surgical strategies in randomised or adequately controlled settings, particularly for the transatrial-transpulmonary approach versus standard transventricular repair. Long-term follow-up studies with systematic assessment of right ventricular function, arrhythmia burden, and reoperation rates are scarce, and no trial has stratified patients by genetic syndrome or pulmonary artery anatomy to guide the choice between primary repair and staged palliation. Funding for multicentre registries and randomised trials of surgical technique, especially in developing-country populations, is lacking.