Advertisement

Functionally Univentricular Heart with Right Heart Hypoplasia (Tricuspid Atresia with Normally Related Great Arteries and Pulmonary Atresia with Intact Ventricular Septum)

  • James M. Galas
  • Deemah R. Mahadin
  • Ralph E. Delius
Chapter

Abstract

Univentricular hearts can present with a hypoplastic left-sided ventricle, a hypoplastic right-sided ventricle, or a ventricle with ambiguous morphology. This chapter will focus on the various forms of hypoplastic right ventricle. The most common forms of this defect are tricuspid atresia and pulmonary valvar atresia with an intact ventricular septum. Almost all forms of hypoplastic right heart syndrome are treated with a series of three operations. The first operation typically either augments or restricts pulmonary blood flow, depending on the specific anatomy. The second stage consists of redirecting superior vena cava flow directly to the lungs. The final operation (Fontan procedure) consists of redirecting the remainder of systemic venous return directly to the lungs. The end result is an in series circulation, with separate pulmonary and systemic circulations.

Keywords

Functionally univentricular heart with right heart hypoplasia Tricuspid atresia Pulmonary atresia with intact ventricular septum Hypoplastic right heart syndrome Univentricular heart Fontan procedure Modified Blalock-Taussig shunt 

References

  1. 1.
    Fyler DC, Buckley LP, Hellenbrand WE, et al. Report of the New England Regional Infant Cardiac Program. Pediatrics. 1980;65:388–461.Google Scholar
  2. 2.
    Gewillig M. Congenital heart disease: the Fontan circulation. Heart. 2005;91:839–46.CrossRefGoogle Scholar
  3. 3.
    Ferencz C, Rubin JD, McCarter RJ, et al. Congenital heart disease: prevalence at live birth, the Baltimore-Washington infant study. Am J Epidemiol. 1985;121:31–6.CrossRefGoogle Scholar
  4. 4.
    Calder AI, Co EE, Sage MD. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol. 1987;59:436–42.CrossRefGoogle Scholar
  5. 5.
    Guleserian KJ, Armsby LB, Thiagarajan RR, et al. Natural history of pulmonary atresia with intact ventricular septum and right-ventricle–dependent coronary circulation managed by the single-ventricle approach. Ann Thorac Surg. 2006;81:2250–8.CrossRefGoogle Scholar
  6. 6.
    Weber HS, Hellenbrand WE, Kleinman CS, et al. Predictors of rhythm disturbances and subsequent morbidity after the Fontan operation. Am J Cardiol. 1989;64:762–7.CrossRefGoogle Scholar
  7. 7.
    Song MK, Bae EJ, Kwon BS, et al. Intra-atrial reentrant tachycardia in adult patients after Fontan operation. Int J Cardiol. 2015;187:157–63.CrossRefGoogle Scholar
  8. 8.
    Guzzetta NA, Foster GS, Mruthinti N, et al. In-hospital shunt occlusion in infants undergoing a modified Blalock-Taussig shunt. Ann Thorac Surg. 2013;96(1):176–82.CrossRefGoogle Scholar
  9. 9.
    Kogon BE, Villari CR, Shah N, et al. Occlusion of the modified Blalock–Taussig shunt: unique methods of treatment and review of catheter-based intervention. Congenit Heart Dis. 2007;2:185–90.CrossRefGoogle Scholar
  10. 10.
    Allen HD, Driscoll DJ, Shaddy RE, et al. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adults. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. Chapter 20.Google Scholar
  11. 11.
    Heinemann M, Breuer J, Steger V, et al. Incidence and impact of systemic venous collateral development after Glenn and Fontan procedures. Thorac Cardiovasc Surg. 2001;49(3):172–8.CrossRefGoogle Scholar
  12. 12.
    Griffiths ER, Kaza AK, Wyler von Ballmoos MC, et al. Evaluating failing Fontans for heart transplantation: predictors of mortality. Ann Thorac Surg. 2009;88(2):558–64.CrossRefGoogle Scholar
  13. 13.
    Allen HD, Driscoll DJ, Shaddy RE, et al. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adults. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. Chapter 72.Google Scholar
  14. 14.
    Egbe AC, Connolly HM, Khan AR, et al. Outcomes in adult Fontan patients with atrial tachyarrhythmias. Am Heart J. 2017;186:12–20.CrossRefGoogle Scholar
  15. 15.
    Deal BJ, Mavroudis C, Backer CL. Arrhythmia management in the Fontan patient. Pediatr Cardiol. 2007;28(6):448–56.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • James M. Galas
    • 1
  • Deemah R. Mahadin
    • 1
  • Ralph E. Delius
    • 2
  1. 1.Division of CardiologyChildren’s Hospital of Michigan, Wayne State University School of MedicineDetroitUSA
  2. 2.Division of Pediatric Cardiovascular SurgeryChildren’s Hospital of Michigan, Wayne State University School of MedicineDetroitUSA

Personalised recommendations