Pediatric Cardiology

, Volume 40, Issue 1, pp 234–236 | Cite as

Prenatally Diagnosed Ventricular Inversion, Restrictive Ventricular Septal Defect, Pulmonary Stenosis, Hypertensive Left Ventricle and Double Outlet Right Ventricle: Case Report and Literature Review

  • J. M. El-Asmar
  • J. A. Degheili
  • E. Al-Halabi
  • M. SklanskyEmail author
Case Report


We herein describe, for the first time, the fetal presentation of a case of ventricular inversion, restrictive ventricular septal defect, pulmonary stenosis, hypertensive left ventricle and double outlet right ventricle at 34 weeks of gestational age. Postnatal echocardiography confirmed the prenatal diagnosis. The patient was subsequently successfully palliated with a left ventricle to pulmonary artery conduit. This report illustrates the importance of detailed fetal echocardiography to ensure appropriate delivery and neonatal management, and to optimize outcome.


Fetal echocardiography Double-outlet right ventricle Ventricular inversion Complex fetal heart disease 


Compliance with Ethical Standards

Conflict of interest

Drs. El-Asmar, Degheili, Al-Halabi, and Sklansky declare that they have no conflict of interest.

Ethical Approval

This article does not contain any studies with human participants performed by any of the authors.

Informed Consent

Informed consent was not performed on the single patient described in this case report; the patient’s family was not available to obtain informed consent for this case report.


  1. 1.
    Walker SG (2010) Anesthesia for left-to-right shunt lesions. In: Andropoulos D (ed) Anesthesia for congenital heart disease, 2nd edn. Wiley-Blackwell, Oxford, pp 384–386Google Scholar
  2. 2.
    Walters HL, Mavroudis C, Tchervenkov CI et al (2000) Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle. Ann Thorac Surg 69:S249–S263CrossRefGoogle Scholar
  3. 3.
    Lev M, Bharati S, Meng L et al (1972) A concept of double-outlet right ventricle. J Thorac Cardiovasc Surg 64:271–281Google Scholar
  4. 4.
    Serratto M, Arevalo F, Goldman E, Hastreiter A, Miller R (1967) Obstructive ventricular septal defect in double outlet right ventricle. Am J Cardiol 19(3):457–463CrossRefGoogle Scholar
  5. 5.
    Cavalini JF, Aiello VD, Guedes de Souza P, Trevisan IV, Marcial MB, Ebaid M (1998) Double-outlet right ventricle with intact atrial septum and restrictive ventricular septal defect: an analysis of two cases. Pediatr Cardiol 19:490–494CrossRefGoogle Scholar
  6. 6.
    Troise D, Ranieri L, Arciprete P (2001) Surgical repair for double outlet right ventricle and intact ventricular septum. Ann Thorac Surg 71(3):1018–1019CrossRefGoogle Scholar
  7. 7.
    Pace Napoleone C, Formigari R, Chiappini B, Frascaroli G, Gargiulo G (2003) Surgical management of double outlet right ventricle with intact ventricular septum. Ann Thorac Surg 75(2):586–587CrossRefGoogle Scholar
  8. 8.
    Lin C, Huddleston C, Balzer D (2013) Transcatheter ventricular septal defect (VSD) creation for restrictive VSD in double-outlet right ventricle. Pediatr Cardiol 34(3):743–747CrossRefGoogle Scholar
  9. 9.
    Snider AR, Serwer GA, Ritter SB (eds) (1997) Echocardiography in pediatric heart disease, 2nd edn. Mosby-Year Book, St Louis, MO, pp. 297–342Google Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of SurgeryAmerican University of Beirut-Medical CenterBeirutLebanon
  2. 2.Department of AnesthesiologyAmerican University of Beirut-Medical CenterBeirutLebanon
  3. 3.Division of Pediatric Cardiology, Department of PediatricsDavid Geffen School of Medicine at UCLALos AngelesUSA

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