Aortopathy in Tetralogy of Fallot - Insights from a single-center observational study

The objective of this study was to assess the presence of aortopathy on follow-up in operated and unoperated adolescent and adult patients with Tetralogy of Fallot (TOF) and to identify the various associated factors.

This was a single-center observational study conducted at a tertiary care institute in India. All consecutive patients with a diagnosis of TOF, TOF with pulmonary atresia, or TOF with absent pulmonary valve, either repaired, palliated, or unoperated, >10 years of age who attended our institute between August 2021 and June 2023 were considered for enrolment. Primary outcome (aortopathy) was defined as sinus of Valsalva (SOV)/root diameter of ≥40 mm (for age ≥18 years), SOV/root Z-score ≥+2 (for age <18 years), or ≥ moderate aortic regurgitation by echocardiogram.

The study enrolled 193 patients. Their mean age was 25.0 ± 11.87 years; for the operated patients, the mean duration of follow-up after surgery was 17.42 ± 7.9 years. Mean aortic annulus, SOV, sinotubular junction, and ascending aortic dimension were 22.8 ± 4.26 mm, 33 ± 5.8 mm, 26.9 ± 5.08 mm, and 29.4 ± 5.46 mm, respectively. Aortopathy was noted in 69 (35.8%) out of a total of 193 subjects. TOF with pulmonary atresia, unrepaired TOF, those who had univentricular palliation, and non-confluent pulmonary arteries were associated with the primary outcome. The mean increase in the ascending aortic dimension was 0.68 ± 0.6 mm/year for the cohort.

Aortopathy was noted in 35.8% of TOF patients on follow-up. Pulmonary atresia, absence of definite repair, and non-confluent pulmonary arteries are factors associated with aortopathy in TOF.