Double Orifice Mitral Valve in Tricuspid Atresia: A Rare Association

  • Massimiliano Cantinotti
  • Raffaele GiordanoEmail author
  • Chiara Marrone
  • Eliana Franchi
  • Martin Koestenberger
  • Nadia Assanta
Images in Pediatric Cardiology


We here report a neonate with prenatal echocardiographic diagnosis of tricuspid atresia, with normally related great vessels, and large ventricular septal defect. This diagnosis could be confirmed with echocardiography at birth. An additional double mitral orifice was also seen. This is a very rare association.


Pediatric Congenital Tricuspi atresia Mitral valve 




Compliance with Ethical Standards

Conflict of interest

No conflict of interest for all authors.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from all individual participants included in the study.

Supplementary material

Subcostal view, MVO1 = mitral valve orifice 1, MVO2 = mitral valve orifice 2, PA = pulmonary artery, VSD = ventricular septal defect (MOV 3919 kb)

Apical 4-chamber view, MVO1 = mitral valve orifice 1, MVO2 = mitral valve orifice 2. LV = left ventricle, RV = right ventricle (MOV 4100 kb)

Parasternal short axis view, MVO1 = mitral valve orifice 1, MVO2 = mitral valve orifice 2 (MOV 3583 kb) (654 kb)
Depicting an en-face atrial view from a 3D full volume acquisition (apical view) of the two mitral orifices (MOV 653 kb)


  1. 1.
    Zalzstein E, Hamilton R, Zucker N, Levitas A, Gross GJ (2004) Presentation, natural history, and outcome in children and adolescent with double orifice mitral valve. Am J Cardiol 93:1067–1069CrossRefPubMedGoogle Scholar
  2. 2.
    Park IS, Yoo SJ, Kim KS, Hong CY (1991) Tricuspid atresia associated with double-orifice mitral valve and coronary sinus septal defect. Tex Heart Inst J 18:202–205PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Fondazione G. Monasterio CNR-Regione ToscanaMassa and PisaItaly
  2. 2.Department of Advanced Biomedical Sciences, Adult and Pediatric Cardiac SurgeryUniversity of Naples Federico IINaplesItaly
  3. 3.Division of Pediatric Cardiology, Department of PediatricsMedical University GrazGrazAustria

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