Type A aortic dissection in patients with bicuspid aortic valves: clinical and pathological comparison with tricuspid aortic valves

Objective Bicuspid aortic valve (BAV) is associated with a higher risk of type A aortic dissection (AD) compared with tricuspid aortic valve (TAV). We sought to study differences between patients with BAV and TAV with AD. Design and setting Observational descriptive analysis of clinical, imaging and pathological characteristics of all patients with confirmed BAV and AD from 1980–2010, compared with a consecutive TAV group with AD. Results Of 47 patients with BAV (mean age 58±14, 77% male), 31 (66%) had acute AD, 16 (34%) had chronic AD, 40 (85%) had typical BAV, 32 (68%) had hypertension and 11 (23%) had previous aortic coarctation repair. Of 53 patients with TAV (mean age 66±13 (p=0.007), 76% male), 34 (66%) had acute AD (p=1.0) and 46 (87%) had hypertension (p=0.03). More patients with BAV had known aortic dilatation prior to AD (49% versus 17%, p=0.001). Presentation symptoms were identical between groups (p=NS). Maximal ascending aortic diameter at AD was higher in patients with BAV (66±15 mm vs 56±11 mm, p=0.0004). Previous aortic valve replacement (AVR) was more common in BAV (23% vs 6%, p=0.02). Of 11 patients with BAV with previous isolated AVR, 7 had ≥moderate ascending aorta dilatation at the time of surgery. Patients with BAV had increased aortic jet velocity (28% vs 10%) and more severe aortic stenosis (19% vs 0%) at presentation (p=0.04 and 0.002, respectively). In acute AD, aortic medial degeneration affected 75% of BAV specimens and 41% TAV specimens (p=0.01) while aortic atherosclerosis was more frequent in TAV (56% vs 26%, p=0.02). Conclusions Compared with patients with TAV, patients with BAV with type A AD are younger, have less hypertension, more valve stenosis and previous AVR, higher maximal aortic dimension, worse aortic medial degeneration, high prevalence of aortic coarctation, and 1 out of 2 have known aortic dilatation prior to AD. Implementation of current guidelines could have theoretically prevented AD in several patients with BAV.

[1]  Jong-Min Song,et al.  Association between bicuspid aortic valve phenotype and patterns of valvular dysfunction and bicuspid aortopathy: comprehensive evaluation using MDCT and echocardiography. , 2013, JACC. Cardiovascular imaging.

[2]  C. Rapezzi,et al.  The elusive link between aortic wall histology and echocardiographic anatomy in bicuspid aortic valve: implications for prophylactic surgery. , 2012, European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery.

[3]  W. Edwards,et al.  Incidence of aortic complications in patients with bicuspid aortic valves. , 2011, JAMA.

[4]  T. Mihaljevic,et al.  Bicuspid aortic valve surgery with proactive ascending aorta repair. , 2011, The Journal of thoracic and cardiovascular surgery.

[5]  D. Mozaffarian,et al.  Heart disease and stroke statistics--2011 update: a report from the American Heart Association. , 2011, Circulation.

[6]  T. Sundt,et al.  Long-term risk of aortic events following aortic valve replacement in patients with bicuspid aortic valves. , 2010, The American journal of cardiology.

[7]  J. Elefteriades,et al.  Thoracic aortic aneurysm clinically pertinent controversies and uncertainties. , 2010, Journal of the American College of Cardiology.

[8]  J. López-Sendón,et al.  Risk of aortic root or ascending aorta complications in patients with bicuspid aortic valve with and without coarctation of the aorta. , 2009, The American journal of cardiology.

[9]  S. Siu,et al.  Outcomes in adults with bicuspid aortic valves. , 2008, JAMA.

[10]  M. Enriquez-Sarano,et al.  Natural History of Asymptomatic Patients With Normally Functioning or Minimally Dysfunctional Bicuspid Aortic Valve in the Community , 2008, Circulation.

[11]  K. Eagle,et al.  Aortic Diameter ≥5.5 cm Is Not a Good Predictor of Type A Aortic Dissection: Observations From the International Registry of Acute Aortic Dissection (IRAD) , 2007, Circulation.

[12]  J. Elefteriades,et al.  Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve. , 2007, The Annals of thoracic surgery.

[13]  Richard B Devereux,et al.  Recommendations for chamber quantification: a report from the American Society of Echocardiography's Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardio , 2005, Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography.

[14]  S. Colan,et al.  Morphology of bicuspid aortic valve in children and adolescents. , 2004, Journal of the American College of Cardiology.

[15]  Rossella Fattori,et al.  Gender-Related Differences in Acute Aortic Dissection , 2004, Circulation.

[16]  P. Walker,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 , 2003, Circulation.

[17]  B. Strauss,et al.  Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications for aortic dilatation. , 2003, The Journal of thoracic and cardiovascular surgery.

[18]  S. Verma,et al.  Clinical and Pathophysiological Implications of a Bicuspid Aortic Valve , 2002, Circulation.

[19]  A Evangelista,et al.  The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. , 2000, JAMA.

[20]  J. Elefteriades,et al.  Surgical intervention criteria for thoracic aortic aneurysms: a study of growth rates and complications. , 1999, Annals of Thoracic Surgery.

[21]  J. Elefteriades,et al.  What is the appropriate size criterion for resection of thoracic aortic aneurysms? , 1997, The Journal of thoracic and cardiovascular surgery.

[22]  A. Angelini,et al.  Bicuspid aortic valves in hearts with other congenital heart disease. , 1995, The Journal of heart valve disease.

[23]  N C Nanda,et al.  Evaluation of aortic insufficiency by Doppler color flow mapping. , 1987, Journal of the American College of Cardiology.

[24]  J. Seward,et al.  Continuous-wave Doppler echocardiographic assessment of severity of calcific aortic stenosis: a simultaneous Doppler-catheter correlative study in 100 adult patients. , 1985, Circulation.

[25]  W. Edwards,et al.  Risk factors for aortic dissection: a necropsy study of 161 cases. , 1984, The American journal of cardiology.

[26]  S. Rich,et al.  Determination of left ventricular ejection fraction by visual estimation during real-time two-dimensional echocardiography. , 1982, American heart journal.

[27]  R R Miller,et al.  A New, Simplified and Accurate Method for Determining Ejection Fraction with Two‐dimensional Echocardiography , 1981, Circulation.

[28]  W. Edwards,et al.  Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases. , 1999, Mayo Clinic proceedings.