Neo-aortic root dilation and valve regurgitation up to 21 years after staged reconstruction for hypoplastic left heart syndrome

Primary and secondary aortopathy associated with adult congenital heart disease - retrospective study 

“…Evidence of aortic dilatation has been reported in patients after the Fontan procedure. In a study with a median follow-up of 9 years neo-aortic root dilatation was observed in 98% of the patients (26). Histological analysis demonstrated ndings seen in other forms of CHD-associated aortopathies, such as fragmentation of elastic bers and deposition of myxoid material (27).…”

Aneurysm formation after the Norwood procedure: Case report and review of the literature

“…3,4 Neoaortic root dilation has been found to be a common occurrence after staged reconstruction for hypoplastic left heart syndrome, with 98% of patients demonstrating a neoaortic dilation with a z score greater than 2 at a median of 9.2 years of follow-up. 5 The etiology of neoaortic aneurysm formation may involve myxoid degeneration as opposed to atherosclerosis 3 ; however, it is unclear whether any contributing factors exist (eg, hypertension, hyperlipidemia, smoking status, pregnancy) and whether medical therapy can mitigate them.…”

Valve-sparing neo-aortic root replacement after Fontan completion for hypoplastic left heart syndrome

“…Neoaortic root dilatation is a common and progressive disorder affecting survivors of staged palliation for HLHS. 3 Valve-sparing neo-aortic root replacement can provide an effective and durable solution, particularly if valve incompetence or extrinsic compression of the adjacent pulmonary arteries exists. Although technically more demanding than replacing the aorta above the sinotubular junction, 4,5 the valve-sparing inclusion technique is necessary to eliminate all the affected tissue and to provide circumferential support below the valve annulus, thus eliminating the potential for recurrent root dilatation and neoaortic valve regurgitation associated with it.…”