Cause of Ascending Aortic Dilatation in Patients with Bicuspid Aortic Valves: The Final Link
14 August 2018
08 October 2018
29 November 2018 (online)
Background This article discusses about the cause of dilatation of the ascending aorta in patients with congenitally bicuspid aortic valves (CBAVs). Some members of the profession believe that it is genetic, while others attribute it to turbulence. The author previously presented in vitro data proving that CBAVs are inherently morphological stenotic, even in the absence of measurable gradient and clinical symptoms. This article reinforces the view that the dilatation of the ascending aorta in patients with CBAV represents a “poststenotic dilatation” by demonstrating that the structure of the aortic wall is normal in infants who have CBAV as a sole cardiac anomaly.
Methods The aortic wall was studied in newborns who had CBAV as the sole cardiovascular anomaly and in those where CABV was associated with other inborn cardiovascular defects.
Results We found that in patients where CBAV is the sole cardiovascular anomaly, aortic structure is normal at birth, but abnormal if additional cardiovascular anomalies are present.
Conclusions Dilatation of the ascending aorta in patients, where CBAV is the only cardiovascular anomaly, is caused by turbulence (poststenotic dilatation). If additional cardiovascular anomalies are present, this process may be exaggerated by genetic abnormalities of the aortic wall.
- 1 Conti CA, Della Corte A, Votta E. , et al. Biomechanical implications of the congenital bicuspid aortic valve: a finite element study of aortic root function from in vivo data. J Thorac Cardiovasc Surg 2010; 140 (04) 890-896 , 896.e1–896.e2
- 2 Verma S, Yanagawa B, Kalra S. , et al. Knowledge, attitudes, and practice patterns in surgical management of bicuspid aortopathy: a survey of 100 cardiac surgeons. J Thorac Cardiovasc Surg 2013; 146 (05) 1033-1040.e4
- 3 Elefteriades JA. Editorial comment: should aortas in patients with bicuspid aortic valve really be resected at an earlier stage than those in patients with tricuspid valve?. Cardiol Clin 2010; 28 (02) 315-316
- 4 Morgagni JB. The Seats and Causes of Diseases Investigated by Anatomy. Boston, MA: Wells and Lilly; 1824
- 5 Halsted WS. An experimental study of circumscribed dilatation of an artery immediately distal to a partially occluding band, and its bearing on the dilatation of the subclavian artery observed in certain cases of cervical rib. J Exp Med 1916; 24 (03) 271-286
- 6 Holman E. The obscure physiology of poststenotic dilatation; its relation to the development of aneurysms. J Thorac Surg 1954; 28 (02) 109-133
- 7 Robicsek F. The Post-Stenotic Dilatation of Great Vessels. Acta Morphol; 1954
- 8 Robicsek F. Post-stenotic dilatation of the great vessels. Acta Med Scand 1955; 151 (06) 481-485
- 9 Robicsek F, Sanger PW, Taylor FH, Magistro R, Foti E. Pathogenesis and significance of post-stenotic dilatation in great vessels. Ann Surg 1958; 147 (06) 835-844
- 10 Robicsek F, Stamou S. The bicuspid aortic valve. How does it function. Why does it fail?. Acta Chir Belg 2007; ;(3, Supp 107): 91
- 11 Nataatmadja M, West M, West J. , et al. Abnormal extracellular matrix protein transport associated with increased apoptosis of vascular smooth muscle cells in Marfan syndrome and bicuspid aortic valve thoracic aortic aneurysm. Circulation 2003; 108 (Suppl. 01) II329-II334
- 12 Fedak PW, Verma S, David TE, Leask RL, Weisel RD, Butany J. Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation 2002; 106 (08) 900-904
- 13 Erbel R, Aboyans V, Boileau C. , et al; ESC Committee for Practice Guidelines; The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. Eur Heart J 2014; 35 (41) 2873-2926
- 14 Robicsek F, Padera RF, Thubrikar MJ. Dilatation of the ascending aorta in patients with congenitally bicuspid aortic valves. HRS Proc Intensive Care and Cardiovasc Anesth 2012; 4 (02) 109-118
- 15 Robicsek F, Cook JW, Reames Sr MK, Skipper ER. Size reduction ascending aortoplasty: is it dead or alive?. J Thorac Cardiovasc Surg 2004; 128 (04) 562-570