Ross operation in a neuro-cardio-facial-cutaneous syndrome patient

Congenital heart diseases are a major part of Costello and cardio-facio-cutaneous syndromes. Subaortic stenosis was reported rarely and Ross operation never in these syndromes.We reported a girl patient whose manifestations were consistent with these syndromes. Distinction between these syndromes was not possible as genetic testing was not carried out. She developed severe neoaortic regurgitation 2.5 years after the Ross operation and died due to the complications of aortic valve replacement.Ross operation may be an unsuitable option in these syndromes due to the possibility of subtle pulmonic valve pathology.