Document Type : Case Report

Authors

1 Associate Professor, Heart Valve Disease Research Center, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran

2 Cardiologist, Heart Valve Disease Research Center, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran

3 Heart Valve Disease Research Center, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran

4 Professor, Heart Valve Disease Research Center, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran

Abstract

BACKGROUND: Double-orifice mitral valve (DOMV) is an extremely rare cardiac malformation. It has been found to be accompanied by congenital anomalies (CAs), however, it can be detected as an isolated anomaly. The clinical findings of a DOMV are variable and depend predominantly on the associated cardiac abnormalities, particularly atrioventricular septal defects or mitral valve (MV) regurgitation and/or stenosis. CASE REPORT: In this regard, we describe an isolated DOMV in an 18-year-old young girl who complained of a short-term nonspecific chest pain. She underwent transthoracic and transesophageal echocardiographic examinations. The examinations revealed a DOMV without any valvular or structural malformations. Other examinations were unremarkable. The patient did need neither medical nor surgical interventions. CONCLUSION: The isolated cases of DOMV do not need therapy and might be only followed up using echocardiographic examinations. However, a careful echocardiographic examination for detection of concomitant structural malformations would be of great importance in the management of such cases. 

Keywords

  1. Erdemli O, Ayik I, Karadeniz U, Yamak B, Birincioglu CL, Caglar K. A double-orifice atrioventricular valve case: Intraoperative transesophageal echocardiography in diagnosis and treatment. Anesth Analg 2003; 97(3): 650-3.
  2. Han J, He Y, Li Z, Zhang Y, Chen J, Wang L, et al. Isolated double-orifice mitral valve anomaly on 3-dimensional transesophageal echocardiography. J Ultrasound Med 2009; 28(11): 1589-92.
  3. Khani M, Rohani A. Double-orifice mitral valve associated with bicuspid aortic valve. Asian Cardiovasc Thorac Ann 2017; 25(5): 386-7.
  4. Wojcik A, Klisiewicz A, Szymanski P, Rozanski J, Hoffman P. Double-orifice mitral valve-echocardiographic findings. Kardiol Pol 2011; 69(2): 139-43.
  5. Ender J, Sgouropoulou S. Value of transesophageal echocardiography (TEE) guidance in minimally invasive mitral valve surgery. Ann Cardiothorac Surg 2013; 2(6): 796-802.
  6. Bano-Rodrigo A, Van PS, Trowitzsch E, Van Praagh R. Double-orifice mitral valve: A study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol 1988; 61(1): 152-60.
  7. Zalzstein E, Hamilton R, Zucker N, Levitas A, Gross GJ. Presentation, natural history, and outcome in children and adolescents with double orifice mitral valve. Am J Cardiol 2004; 93(8): 1067-9.
  8. Formigari R, Di Donato RM, Gargiulo G, Di Carlo D, Feltri C, Picchio FM, et al. Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome. Ann Thorac Surg 2004; 78(2): 666-72.
  9. Marino B, Vairo U, Corno A, Nava S, Guccione P, Calabro R, et al. Atrioventricular canal in Down syndrome. Prevalence of associated cardiac malformations compared with patients without Down syndrome. Am J Dis Child 1990; 144(10): 1120-2.
  10. Digilio MC, Marino B, Toscano A, Giannotti A, Dallapiccola B. Congenital heart defects in Kabuki syndrome. Am J Med Genet 2001; 100(4): 269-74.
  11. Chatfield KC, Schrier SA, Li J, Clark D, Kaur M, Kline AD, et al. Congenital heart disease in Cornelia de Lange syndrome: Phenotype and genotype analysis. Am J Med Genet A 2012; 158A(10): 2499-505.
  12. Das BB, Pauliks LB, Knudson OA, Kirby S, Chan KC, Valdes-Cruz L, et al. Double-orifice mitral valve with intact atrioventricular septum: Aan echocardiographic study with anatomic and functional considerations. J Am Soc Echocardiogr 2005; 18(3): 231-6.
  13. Anwar AM, McGhie JS, Meijboom FJ, Ten Cate FJ. Double orifice mitral valve by real-time three-dimensional echocardiography. Eur J Echocardiogr 2008; 9(5): 731-2.
  14. Trowitzsch E, Bano-Rodrigo A, Burger BM, Colan SD, Sanders SP. Two-dimensional echocardiographic findings in double orifice mitral valve. J Am Coll Cardiol 1985; 6(2): 383-7.
  15. van Buuren F, Faber L, Bogunovic N. Double orifice mitral valve with normal function: An echocardiography and MRI study of a rare finding. Eur Heart J 2011; 32(2): 137.