Journal of Rare Cardiovascular Diseases

The changes in hemodynamics, as well as changes in the aortic media during pregnancy, put the women with bicuspid aortic valve (BAV) and significant aortic stenosis (AS) and/or dilated aortic roots at risk for complications during this period. From 1988–2014,89 pregnant patients with BAV, mean 26.8 ±4.6 years, were observed. There were 52 patients with AS: 22 with mild AS, 30 with moderate to severe AS and 37 patients with aortic insufficiency (AI). Medical history, physical examination, NYHA class assessment, ECG and echocardiography were performed during each trimester (TR) of pregnancy and after delivery. During pregnancy all patients with mild AS remained in NYHA class I. All of them delivered vaginally healthy babies on term. Patients with moderate to severe AS in I TR remained in NYHA class I-II, in 6/30 patients clinical deterioration was observed within III TR. Seventeen women with severe AS delivered by cesarean section, the remaining vaginally. In the group of IA clinical deterioration was observed in III TR in 5/37 patients with severe AI, enlarged left ventricle (LV) and depressed systolic function. Six of them delivered by cesarean section, the remaining vaginally. Conclusions: Patients with mild AS tolerate well the increased cardiovascular demand of pregnancy. In patients with severe AS pregnancy intensifies the hemodynamic disorders. Volume overload of pregnancy is well tolerated in patients with mild and moderate IA. Cardiac complications can be expected in patients with severe IA, LV enlargement and impaired systolic function. In the observed group of BAV patients there was no pregnancy-associated dissection. JRCD 2014; 2 (1): 9–14

bicuspid aortic valve, aortic stenosis, aortic insufficiency, pregnancy, dissection

Fedak PW, Verma S, David TE, et al. Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation 2002; 106: 900–904.

Shi-Min Yuan. Bicuspid aortic valve in pregnancy. Taiwanese Journal of Obstetrics and Gynecology 2014; 53: 476–480.

Keane MG, Wiegers SE, Plappert T, et al. Bicuspid aortic valves are associated with aortic dilatation out of proportion to coexistent valvular lesions. Circulation 2000; 102: III35–III39.

Siu SC, Silversides CK. Bicuspid Aortic Valve Disease. J Am Coll Cardiol. 2010; 55: 2789–2800.

Quinones MA, Waggoner AD, Reduto LA. et al. A new, simplified and accurate method for determining ejection fraction with two-dimensional echocardiography. Circulation 1981; 64: 744–753.

Stamm RB, Carabello BA, Mayers DL, et al. Two-dimensional echocardiographic measurement of left ventricular ejection fraction: prospective analysis of what constitutes an adequate determination. Am Heart J 1982; 104:136–144.

Bolling SF, Iannettoni MD, Dick M.2nd, et al. Shone’s anomaly: operative results and late outcome. Ann Thorac Surg 1990; 49: 887–893.

Sybert VP.Cardiovascular malformations and complications in Turner syndrome. Pediatrics 1998; 101: E11.

Rashid A, Saucedo JF, Hennebry TA. Association of single coronary artery and congenital bicuspid aortic valve with review of literature. J Interv Cardiol 2005; 18: 389–391.

Cripe L, Andelfinger G, Martin LJ, et al. Bicuspid aortic valve is heritable. J Am Coll Cardiol. 2004; 44: 138–143.

Huntington K, Hunter AG, Chan KL. A prospective study to assess the frequency of familial clustering of congenital bicuspid aortic valve. J Am Coll Cardiol. 1997; 30: 1809–1812.

Chan KL, Stinson WA, Veinot JP. Reliability of transthoracic echocardiography in the assessment of aortic valve morphology: pathological correlation in 178 patients. Can J Cardiol. 1999; 15: 48–52.

Cecconi M, Manfrin M, Moraca A. Aortic dimensions in patients with bicuspid aortic valve without significant valve dysfunction. Am J Cardiol. 2005; 95: 292–294.

Michelena HI, Desjardins VA, Avierinos JF, et al. Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community. Circulation 2008; 117: 2776–2784.

Mc Kellar SH, MacDonald RJ, Michelena HI, et al. Frequency of cardiovascular events in women with a congenitally bicuspid aortic valve in a single community and effect of pregnancy on events. Am J Cardiol 2011; 107: 96–99.

Sabet HY, Edwards WD, Tazelaar HD, 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. Mayo Clin Proc 1999; 74: 14–26.

Guntheroth WG. A critical review of the American College of Cardiology/American Heart Association practice guidelines on bicuspid aortic valve with dilated ascending aorta. Am J Cardiol. 2008; 102: 107–110.

Tzemos N, Therrien J, Yip J. Outcomes in adults with bicuspid aortic valves. JAMA 2008; 300: 1317–1325.

Mordi I, Tzemos N. Bicuspid Aortic Valve Disease: A Comprehensive Review. Cardiol Res Pract. 2012; 2012: 196 037.

Child JS. Management of Pregnancy and contraception in Congenital Heart Disease. In: Congenital Heart Disease in Adults, 3rd ed, Perloff, JK, Child, JS, Aboulhosn, J (Eds), Saunders/Elsevier, 2009.

Ayad RF, Grayburn PA, Ko JM, et al. Accuracy of two-dimensional echocardiography in determining aortic valve structure in patients >50 years of age having aortic valve replacement for aortic stenosis. Am J Cardiol. 2011; 108: 1589–1599.

Carro A, Teixido-Tura G, Evangelista A. Aortic Dilatation in Bicuspid Aortic Valve Disease. Rev Esp Cardiol. 2012; 65: 977–981.

Davies RR, Kaple RK, Mandapati D, et al. Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve. Ann Thorac Surg. 2007; 83: 1338–1344.

Waksmonsky CA, Foley MR. Pregnancy in women with a bicuspid aortic valve. www.uptodate.com © 2015 UpToDate® accessed June 12; 2015.

Yap SC, Drenthen W, Pieper PG. Risk of complications during pregnancy in women with congenital aortic stenosis. Int J Cardiol. 2008; 126: 240–246.

Silversides CK, Colman JM, Sermer M, et al. Early and intermediate-term outcomes of pregnancy with congenital aortic stenosis. Am J Cardiol. 2003; 91: 1386–1389

Leśniak-Sobelga A, Tracz W, Kostkiewicz M, et al. Clinical and echocardiographic assessment of pregnant women with valvular heart diseases-maternal and fetal outcome. Int J Cardiol. 2004; 94: 15–23.

Warnes CA, Wiliams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease. A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Circulation 2008; 118: e714 – e833.

Regitz-Zagrosek V, Lundqvist CB, Borghi C, et al. ESC Guidelines on the management of cardiovascular diseases during pregnancy. The Task Force on the Management of Cardiovascular Diseases during Pregnancy. Eur Heart J 2011; 32: 3147–3197.

Tzemos N, Silversides CK, Colman JM. Late cardiac outcomes after pregnancy in women with congenital aortic stenosis. Am Heart J. 2009; 157: 474–480.

Keane MG, Wiegers SE, Plappert T, et al. Bicuspid aortic valves are associated with aortic dilatation out of proportion to coexistent valvular lesions. Circulation 2000; 102: pp. III35–III39.

Braverman A, Güven H, Beardslee MA, et al. The bicuspid aortic valve. Curr Probl Cardiol. 2005; 30: 470–522.

Elkayam U, Bitar F. Valvular Heart Disease and Pregnancy. Part I: Native Valves. J Am Coll Cardiol. 2005; 46: 223–230.

Immer FF, Bansi AG, Immer-Bansi AS, et al. Aortic dissection in pregnancy: analysis of risk factors and outcome. Ann Thorac Surg. 2003; 76: 309–314.

Paternoster DM, Santarossa C, Vettore N, et al. Obstetric complications in Marfan syndrome pregnancy. Minerva Ginecol 1998; 50: 441–443.

Lichtman AD. Acute aortic dissection in pregnancy: cesarean section prior to repair of acute Type A dissection. J Anesthe Clin Res 2011; 2: 8.

Wassmuth R, von Knobelsdorff-Brenkenhoff F, Gruettner H, et al. Cardiac magnetic resonance imaging of congenital bicuspid aortic valves and associated aortic pathologies in adults. European Heart Journal Cardiovascular Imaging 2014; 15: 673–679.

Johnston KW, Rutherford RB, Tilson MD, et al. Suggested standard s for reporting on arterial aneurysms. Subcommittee on Reporting Standards for Arterial Aneurysms, Ad Hoc Committee on Reporting Standards, Society for Vascular Surgery and North American Chapter, International Society for Cardiovascular Surgery. J Vasc Surg. 1991; 13: 452–458.