Aortic root dilatation is a well-known complication in patients with congenital aortic valve malformation, tetralogy of Fallot, or a double outlet right ventricle. We report two rare patients who underwent composite graft replacement of the aortic root with a mechanical valve, the so-called Bentall-type operation, after Fontan completion. The pathological examination on the resected aortic wall revealed mucoid degeneration in tunica media and elastic fiber fragmentation. Our report emphasizes the need for close observation of these patients over a long-term period.
FrancoisKZaqoutMBoveT. The fate of the aortic root after early repair of tetralogy of Fallot. Eur J Cardiothorac Surg. 2009;37:1254–1258.
2.
CleuziouJMayrNSchreiberC. Giant aortic aneurysm 18 years after repair of double-outlet right ventricle with pulmonary stenosis. Ann Thorac Surg. 2006;82:e31–e32.
3.
AomiSNakajimaMNonoyamaM. Aortic root replacement using composite valve graft in patients with aortic valve disease and aneurysm of the ascending aorta: twenty years’ experience of late results. Artif Organs. 2002;26:467–473.
4.
NanaumiMAsouTTakedaYLinZBOharaKYoshimuraH. Total cavopulmonary connection via a thoracotomy. Ann Thorac Surg. 2002;74:917–919.
5.
SchmidFXBielenbergKSchneiderAHausslerAKeyserABirnbaumD. Ascending aortic aneurysm associated with bicuspid and tricuspid aortic valve: involvement and clinical relevance of smooth muscle cell apoptosis and expression of cell death-initiating proteins. Eur J Cardiothorac Surg. 2003;23:537–543.
SpadottoVUemuraHUebingA. Successful Bentall procedure in a patient with a Fontan circulation. Interact Cardiovasc Thorac Surg. 2014;19:520–522.
8.
NiwaK. Aortopathy in congenital heart disease in adults: aortic dilatation with decreased aortic elasticity that impacts negatively on left ventricular function. Korean Circ J. 2013;43:215–220.
