A Comparative Study of Histopathological Changes in the Ascending Aorta and the Risk Factors Related to Histopathological Conditions and Aortic Dilatation in Patients With Tetralogy of Fallot and a Functionally Univentricular Heart

Bentall’s Procedure for Annuloaortic Ectasia and Severe Aortic Regurgitation in a Patient With Repaired Tetralogy of Fallot and Aortic Valvular Reconstruction

“…2 The histopathological changes observed include elastic fiber fragmentation and/or loss, MEMA, smooth muscle nuclear loss, smooth muscle cell disorganization, and translamellar fibrosis and resemble those observed in patients with Marfan syndrome and aortic dilatation. 2 We further demonstrated the existence of "exonic DNA variants" involving the fibrillin-1 gene in one or more exons (exon 24-28). The "DNA sequence variants" were more pronounced in patients with TOF and dilated aorta in the presence of abnormal aortic histopathology.…”

“…As far as we are aware, there have been only limited studies in the English literature addressing specifically the existence of intrinsic aortopathy in TOF (autopsy series n ¼ 2, surgical series n ¼ 2). 1,2 A substantial cohort of patients with repaired TOF continue to exhibit a dilated aortic root or develop progressive aortic root dilatation and AR necessitating reoperation on the aortic root. [1][2][3][4][5][6][7] Early repair of TOF is advocated to abrogate this complication.…”

“…1,2 A substantial cohort of patients with repaired TOF continue to exhibit a dilated aortic root or develop progressive aortic root dilatation and AR necessitating reoperation on the aortic root. [1][2][3][4][5][6][7] Early repair of TOF is advocated to abrogate this complication. [1][2][3][4][5][6][7] In our previous investigations, we demonstrated that the majority of TOF patients have significant lamellar loss and intrinsic aortopathy of the aortic media.…”

“…[1][2][3][4][5][6][7] Early repair of TOF is advocated to abrogate this complication. [1][2][3][4][5][6][7] In our previous investigations, we demonstrated that the majority of TOF patients have significant lamellar loss and intrinsic aortopathy of the aortic media. 2 The histopathological changes observed include elastic fiber fragmentation and/or loss, MEMA, smooth muscle nuclear loss, smooth muscle cell disorganization, and translamellar fibrosis and resemble those observed in patients with Marfan syndrome and aortic dilatation.…”

“…[1][2][3] The aortic root dilatation is often progressive with development of aortic regurgitation (AR) demanding aortic valve replacement or repair and has also been reported to occur in patients not treated surgically as well. [1][2][3] Literature search till date revealed only three isolated reports of aortic dissection and one report of annuloaortic ectasia without dissection in patients with TOF necessitating Bentall's procedure. [3][4][5][6] Case Report…”