Jacob KA; Department of Pediatric Cardiac Surgery, Utrecht, the Netherland
Korsuize NA; van Wijk A; Slieker MG; et al

Journal of Thoracic & Cardiovascular Surgery. 172(4):1074-1082.e2, 2026  Vol 1 Oct.

OBJECTIVES: Congenital critical aortic stenosis is frequently accompanied
by endocardial fibroelastosis (EFE), impairing diastolic compliance and
long-term ventricular performance. An early Ross procedure, with or
without concomitant EFE resection may restore left ventricular (LV)
function; however, long-term functional outcomes remain poorly
characterized. We evaluated long-term clinical and functional outcomes
after Ross procedure in neonates and infants with and without EFE.

METHODS: Patients who underwent a Ross operation at <1 year of age
between January 2005 and January 2025 were included. Demographic,
echocardiographic, and long-term clinical data were analyzed. Subgroup
assessment was performed in patients with the most pathologic LV, that is,
with EFE, and included cardiac magnetic resonance imaging and
cardiopulmonary exercise testing. The primary outcome was survival;
secondary outcomes included LV function, EFE recurrence, and exercise
capacity.

RESULTS: Thirty-six patients underwent the Ross procedure (median age, 55
days; interquartile range, 20-136), and 17 patients (47%) had EFE.
Operative mortality was 5.6% (2/36), with no late deaths during follow-up
(10 +/- 4 years). Late echocardiography demonstrated unobstructed LV
outflow and normalized LV function at follow-up. Ten patients with EFE,
after we obtained consent for additional diagnostic evaluation, had mostly
normalized LV function confirmed on cardiac magnetic resonance imaging
with no evidence of recurrent EFE. In these 10 patients, cardiopulmonary
exercise testing demonstrated predominantly normal aerobic capacity.

CONCLUSIONS: Biventricular repair in neonates and infants with critical
aortic stenosis is associated with excellent survival and substantial LV
recovery even in the presence of severe EFE.