Our mission is to become a worldwide reference for education in the field for all professionals involved in the process to disseminate knowledge & skills of Acute Cardiovascular Care.
Our mission is to promote excellence in clinical diagnosis, research, technical development, and education in cardiovascular imaging in Europe.
Our mission is to promote excellence in research, practice, education and policy in cardiovascular health, primary and secondary prevention.
Our mission is to reduce the burden of cardiovascular disease in Europe through percutaneous cardiovascular interventions.
Our mission is to improve the quality of life of the population by reducing the impact of cardiac rhythm disturbances and reduce sudden cardiac death.
Our mission is to improve quality of life and longevity, through better prevention, diagnosis and treatment of heart failure, including the establishment of networks for its management, education and research.
The ESC Working Groups' goal is to stimulate and disseminate scientific knowledge in different fields of cardiology.
The ESC Councils' goal is to share knowledge among medical professionals practising in specific cardiology domains.
OUR MISSION: TO REDUCE THE BURDEN OF CARDIOVASCULAR DISEASE
Dr. Adel Shabana
Bicuspid aortic valve may be complicated with aortic valve stenosis and/or regurge in addition to risk of endocarditis. Furthermore, it is associated with coarctation and aortopathy that may result in aortic aneurysm, dissection or rupture.The definitive treatment is surgery for the valve and/or aortic root depending on severity of valve lesion, aortic diameter and rate of progression of aortopathy.
Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality, affecting approximately 1-2% of the general population (1,2). Adverse cardiac outcomes related to the valve and/or root (3) put a large burden on cardiovascular in addition to the many undergoing aortic valve replacement who will also need aortic root surgery. Although inheritance is variable, it is a familial disease (4,5) with an estimated 10% chance of a first degree relative being affected (6,7) and 20-30% when including aortopathy into the calculation (8,9). Additionally, certain gene mutations (NOTCH1) have been associated. The BAV is composed of not three aortic normal leaflets- as in the normal tricuspid valve- but two abnormal leaflets, usually unequal in size, possibly resulting from inadequate fibrillin-1 production during valvulogenesis (10). The most common form has the two commissures located in an anteroposterior direction giving left and right cusps. Less common is having the commissures located on the right and left sides of the annulus leading to anterior and posterior cusps. Type 1 BAVs are more likely to stenose as adults while type 2 valves will have complications earlier. Rarely there is fusion of the left and non-coronary cusps. A raphe is present on the right and anterior cusps respectively, and this can make the valve appear tricuspid on echocardiography. (11) (Figure 1).
Figure 1: Classification and incidence of BAV.
Complications are 1) aortic stenosis, 2) aortic regurgitation (or incompetence), 3) endocarditis. Calcification usually also occurs.Due to abnormal stresses, even a normally functioning BAV can progress and damage gradually to reveal abnormal folding and creasing throughout the cardiac cycle, with more turbulent flow, as well as restricted motion (12)Aortopathy and coarctation have implications on the prognosis of BAV. In several cohort studies, the long-term survival of patients was similar to the normal population but with more cardiac events and need for surgical interventions on the valve and/or aorta (13,14). Natural history if BAV ranges from severe aortic stenosis in childhood to longstanding asymptomatic disease into the elderly years.
Aortopathy refers to “dilated aortic root”. It is usually associated with BAV and may lead to aortic aneurysm, dissection and rupture (19,20). The prevalence of ascending aorta dilatation in patients with BAV ranged from 7.5% to 79% according to the study population and region of aorta examined. (21-22). Incidence is higher in patients with fusion of the right and non-coronary cusps than the more common phenotype of fusion of the right and left coronary cusps. To explain the mechanism of aortopathy are the genetic theory (aortic root fragility and elastic fragmentation due to inherited developmental defect) and the hemodynamic theory (abnormal stress on aortic root due to turbulent flow through BAV) (23-27). Three different shapes of proximal aorta in BAV were described, and were correlated to the type of BAV cusp configuration (28). Serial echocardiographic examinations have found higher rates of growth at all levels of the proximal aorta above the annulus in patients with BAV in comparison to those with tricuspid aortic valves (29-31). Aortic dissection is a lethal sequel in patients with BAV and occurs at a younger age. Recent data suggest that BAV increases the risk of aortic dissection 9-fold over that in the general population (32). Its incidence is less than 1% up in some studies yet reaches 4% in others (33). It is worth noting that aortic root dilatation is an important predictor of dissection and rupture in BAV-associated aneurysms, even if the aortic valve is normally functioning. (34)
Coarctation of aorta is another major abnormality that can be associated with 20% of BAV or even more. In the presurgical era, this association was found to increase the risk of death from aortic dissection more than that if coarctation exists without BAV (35). Coarctation is sometimes regarded as a part of the diffuse aortopathy associated with BAV.Less common associated congenital anomalies with BAV include: ventricular septal defects, ebstein’s anomaly, hypoplastic left heart syndrome, abnormal coronary anatomy, patent ductus arteriosus, atrial septal defects and bicuspid pulmonary valve.
Echocardiography: The mainstay of diagnosis is echocardiography (transthoracic or transoesophageal) which can provide a definitive diagnosis in the majority of patients with 92% sensitivity and 96% specificity when images are adequate (36). The echocardiographic report should adequately evaluate aortic valve morphology and measure the severity of any stenosis or regurge. It should also include aortic measurements at the aortic annulus, sinuses, sinotubular junction, and mid-ascending aorta. Doppler: In addition, evaluation for aortic coarctation using Doppler interrogation of the proximal descending aorta should be performed. Aortic magnetic resonance angiography or CT angiography is indicated if morphology of the aortic root and/or ascending aorta cannot be assessed accurately or fully by echocardiography. As recommended by the recent north American and European guidelines, patients with BAV who have documented dilation of aorta should have serial assessment of aortic morphology to detect progression of aortopathy early enough before complications occur (37). Recent tools for diagnosis and risk stratification have been suggested using metalloproteinase plasma assays and magnetic resonance imaging (38).
Aortic valve replacement is the only definitive treatment for BAV associated with severe stenosis and/or regurgitation. The indications for surgery are similar to those with tricuspid aortic valve. New techniques of repair such as transcatheter aortic valve implantation have also been reported in BAV (39) but are still considered as a relative contraindication in recent guidelines. In patients who refuse surgery or are not candidates for surgical intervention, medical therapy is limited and is mainly used to treat existent cardiac conditions such as heart failure, coronary artery disease or hypertension. However, there is a possible role of beta blockers and ARBs in delaying progression of aortic dilatation, extrapolating from patients with aortopathy associated with Marfan’s syndrome, but have not been confirmed in clinical studies.
The recent ESC guidelines for valvular heart disease management recommend that sugery should be considered for any patient with aortic root diameter ≥55 mm (Table 1). However, in patients with BAV, surgery in aortic diameters ≥ 50 mm should be based on patient age, body size, comorbidities, type of surgery, and the presence of additional risk factors (family history, systemic hypertension, coarctation of the aorta, or increase in aorticdiameter ≥ 2 mm/year in repeated examinations, using the same technique and confirmed by another technique). The ACC/AHA new guidelines recommend surgical repair of aortic root if aortic root diameter is ≥5.5 cm (Class I). This recommendation has changed in 2014 from previous 2006 recommendation cutoff value of 5 cm. It should be noted also that the ACC/AHA guidelines considered the evidence of rapid progression of A maximum aortic cross-section: body height ratio of 10 cm2/m has been proposed as additional measurement to decide for aortic root surgery and has been found to be associated with an increased risk for aortic dissection in several studies. (40-42)
Table 1: Indications of aortic root surgery in patients with BAV according to measured aortic root diameter.
Bicuspid aortic valve may be complicated with aortic valve stenosis and/or regurge in addition to risk of endocarditis. Furthermore, it is associated with coarctation and aortopathy that may result in aortic aneurysm, dissection or rupture. The definitive treatment is surgery for the valve and/or aortic root depending on severity of valve lesion, aortic diameter and rate of progression of aortopathy.
1 - Bicuspid aortic valve. Williams DS. J Insur Med. 2006;38(1):72-42 - Clinical significance of the bicuspid aortic valve. Ward C. Heart. 2000;83(1):81-53 - Outcomes in adults with bicuspid aortic valves. Tzemos N, Therrien J, Yip J, Thanassoulis G, Tremblay S, Jamorski MT, Webb GD. JAMA. 2008;300(11):1317–25.4 - Mutations in NOTCH1 cause aortic valve disease. Garg V, Muth AN, Ransom JF, Schluterman MK, Barnes R, King IN, Grossfeld PD, Srivastava D. Nature. 2005;437(7056):270–4.5 - Genetic insights into bicuspid aortic valve formation.Laforest B, Nemer M. Cardiol Res Pract. 2012;2012:180297. doi: 10.1155/2012/1802976 - Bicuspid aortic valve disease: A comprehensive review.Mordi I and Tzemos N. Cardiol Res Pract. 2012; 2012:196037. doi: 10.1155/2012/1960377 - Bicuspid aortic valve is heritable. Cripe L, Andelfinger G, Martin LJ, Shooner K, Benson DW. J Am Coll Cardiol. 2004 Jul 7;44(1):138-43.8 - Aortopathy is Prevalent in Relatives of Bicuspid Aortic Valve Patients. Biner S, Rafique AR, Ray I, Cuk O, Siegel RJ, and Tolstrup K. J Am Coll Cardiol. 2009; 53(24): 2288–959 - 2014 AHA/ACC Guideline for the Management of Patients with Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP 3rd, Guyton RA, O'Gara PT, Ruiz CE, Skubas NJ,Sorajja P, Sundt TM 3rd, Thomas JD, Anderson JL, Halperin JL, Albert NM, Bozkurt B, Brindis RG, Creager MA,Curtis LH, DeMets D, Guyton RA, Hochman JS, Kovacs RJ, Ohman EM, Pressler SJ, Sellke FW, Shen WK,Stevenson WG, Yancy CW. J Thorac Cardiovasc Surg. 2014 Jul;148(1):e1-e132. 10 - Clinical and pathophysiological implications of a bicuspid aortic valve. Fedak PW, Verma S, David TE, Leask RL, Weisel RD, Butany J. Circulation. 2002;106(8):900-4.11 - The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shap.Schaefer BM, Lewin MB, Stout KK, Gill E, Prueitt A, Byers PH, Otto CM. Heart 2008;94(12):1634-38. 12 - The congenitally bicuspid aortic valve: how does it function? Why does it fail? Robicsek F, Thubrikar MJ, Cook JW, Fowler B. Ann Thorac Surg 2004;77:177-85.13 - Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community. Michelena HI, Desjardins VA, Avierinos JF, Russo A, Nkomo VT, Sundt TM, Pellikka PA, Tajik AJ, Enriquez-Sarano M. Circulation 2008;117:2776-84.14 - Incidence of aortic complications in patients with bicuspid aortic valves. Michelena HI1, Khanna AD, Mahoney D, Margaryan E, Topilsky Y, Suri RM, Eidem B, Edwards WD, Sundt TM 3rd,Enriquez-Sarano M. JAMA 2011;306(10):1104-12.15 - Left Ventricular Outflow Obstruction: Subaortic Stenosis, Bicuspid Aortic Valve, Supravalvar Aortic Stenosis, and Coarctation of the Aorta. Aboulhosn J, Child JS. Circulation. 2006;114:2412–2216 - Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation. Roberts WC & Ko JM. Circulation 2005;111:920–5.17 - Bicuspid aortic valves are associated with aortic dilatation out of proportion to coexistent valvular lesions.Keane MG, Wiegers SE, Plappert T, Pochettino A, Bavaria JE, Sutton MG. Circulation. 2000;102:III35-III39. 18 - Congenital bicuspid aortic valve: analysis of 63 surgical cases. Yotsumoto G, Moriyama Y, Toyohira H, Shimokawa S, Iguro Y, Watanabe S, Masuda H, Hisatomi K, Taira A. J Heart Valve Dis. 1998;7(5):500-3. 19 - Aortic root dilatation in young men with normally functioning bicuspid aortic valves. Nistri S, Sorbo MD, Marin M, Palisi M, Scognamiglio R, Thiene G. Heart. 1999;82(1):19-2220 - Ascending aortic dilatation associated with bicuspid aortic valve: pathophysiology, molecular biology, and clinical implications. Tadros TM, Klein MD, Shapira OM. Circulation 2009;119(6):880-90.21 - Frequency of aortic root dilation in children with a bicuspid aortic valve. Gurvitz M, Chang RK, Drant S, Allada V. Am J Cardiol. 2004;94:1337–1340.22 - Changes in size of ascending aorta and aortic valve function with time in patients with congenitally bicuspid aortic valves. Ferencik M, Pape LA. Am J Cardiol. 2003;92:43– 46. 23 - Bicuspid aortic valve disease: the role of oxidative stress in Lrp5 bone formation. Rajamannan NM. Cardiovasc Pathol. 2011;20(3):168-76.24 - Is aortopathy in bicuspid aortic valve disease a congenital defect or a result of abnormal hemodynamics? A critical reappraisal of a one-sided argument. Girdauskas E, Borger MA, Secknus MA, Girdauskas G, Kuntze T Eur J Cardiothorac Surg. 2011;39(6):809-14.25 - Relation of bicuspid aortic valve morphology to the dilatation pattern of the proximal aorta: focus on the transvalvular flow. Girdauskas E, Disha K, Borger MA, Kuntze T. Cardiol Res Pract. 2012;2012:478259. doi: 10.1155/2012/478259.26 - Pathophysiological implications of different bicuspid aortic valve configurations. Kari FA, Beyersdorf F, Siepe M. Cardiol Res Pract. 2012;2012:735829. doi: 10.1155/2012/735829. 27 - Increased ascending aortic wall stress in patients with bicuspid aortic valves. Nathan DP, Xu C, Plappert T, Desjardins B, Gorman JH 3rd, Bavaria JE, Gorman RC, Chandran KB, Jackson BM. Ann Thorac Surg. 2011;92(4):1384-928 - Bicuspid aortic valve disease. Siu SC and Silversides CK. J Am Coll Cardiol. 2010;55(25):2789-800. 29 - The bicuspid aortic valve and its relation to aortic dilation.Yuan SM, Jing H, Lavee J. Clinics (Sao Paulo). 2010;65(5):497-50530 - Failure to prevent progressive dilation of ascending aorta by aortic valve replacement in patients with bicuspid aortic valve: comparison with tricuspid aortic valve. Yasuda H1, Nakatani S, Stugaard M, Tsujita-Kuroda Y, Bando K, Kobayashi J, Yamagishi M, Kitakaze M, Kitamura S, Miyatake K. Circulation. 2003;108:II291–II294. 31 - Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve. Davies RR1, Kaple RK, Mandapati D, Gallo A, Botta DM Jr, Elefteriades JA, Coady MA. Ann Thorac Surg. 2007;83:1338 –1344. 32 - Replacement of the Proximal Arch. Should It Be Routine in Patients with Bicuspid Aortic Valve and Ascending Aneurysms? Bolman RM III. Tex Heart Inst J. 2011;38(6):672-3.33 - A critical review of the ACC/AHA practice guidelines on bicuspid aortic valve with dilated ascending aorta. Guntheroth WG. Am J Cardiol 2008;102:107-10.34 - Aortic aneurysm in patients with functionally normal or minimally stenotic bicuspid aortic valve. Pachulski RT, Weinberg AL, Chan KL. Am J Cardiol. 1991;67(8):781-2.35 - Bicuspid aortic valve and coarctation: two villains part of a diffuse problem. Warnes CA. Heart. 2003;89(9):965-6. 36 - Diagnostic value of cardiac CT in the evaluation of bicuspid aortic stenosis: comparison with echocardiography and operative findings. Tanaka R, Yoshioka K, Niinuma H, Ohsawa S, Okabayashi H, and Ehara S. American Journal of Roentgenology 2010;195(4):895–899.37 - ESC Committee for Practice Guidelines (CPG); Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology (ESC); European Association for Cardio-Thoracic Surgery (EACTS). Guidelines on the management of valvular heart disease (version 2012): the Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS). Vahanian A, Alfieri O, Andreotti F, Antunes MJ, Barón-Esquivias G, Baumgartner H, Borger MA, Carrel TP, De Bonis M, Evangelista A, Falk V, Lung B, Lancellotti P, Pierard L, Price S, Schäfers HJ, Schuler G, Stepinska J, Swedberg K, Takkenberg J, Von Oppell UO, Windecker S, Zamorano JL, Zembala M. Eur J Cardiothorac Surg. 2012;42(4):S1-44. doi: 10.1093/ejcts/ezs45538 - Hemodynamic predictors of aortic dilatation in bicuspid aortic valve by velocity-encoded cardiovascular magnetic resonance. den Reijer PM, Sallee D 3rd, van der Velden P, Zaaijer ER, Parks WJ, Ramamurthy S, Robbie TQ, Donati G,Lamphier C, Beekman RP, Brummer ME. J Cardiovasc Magn Reson. 2010;12:4. doi: 10.1186/1532-429X-12-4 39 - Transcatheter implantation of an aortic valve prosthesis in a female patient with severe bicuspid aortic stenosis.Kochman J, Huczek Z, Koltowski L, and Michalak M. Eur Heart J. 2012;33(1):112-11840 - Current indications for surgical repair in patients with bicuspid aortic valve and ascending aortic ectasia. Etz CD, Misfeld M, Borger MA, Luehr M, Strotdrees E, Mohr FW. Cardiol Res Pract. 2012;2012:313879. doi: 10.1155/2012/31387941 - Bicuspid aortic valve surgery with proactive ascending aorta repair. Svensson LG1, Kim KH, Blackstone EH, Rajeswaran J, Gillinov AM, Mihaljevic T, Griffin BP, Grimm R, Stewart WJ,Hammer DF, Lytle BW. J Thorac Cardiovasc Surg. 2011;142(3):622-9.42 - Relationship of aortic cross-sectional area to height ratio and the risk of aortic dissection in patients with bicuspid aortic valves. Svensson LG, Kim KH, Lytle BW, and Cosgrove DM. J Thorac Cardiovasc Surg. 2003;126(3):892-3.
Dr Adel Shabana, Heart Hospital, Hamad Medical Corporation, Doha, Qatar.Author's disclosures: None declared.
© 2017 European Society of Cardiology. All rights reserved