High-Risk Anatomic Subsets in Hypoplastic Left Heart Syndrome

World J Pediatr Congenit Heart Surg. 2022 Sep;13(5):593-599. doi: 10.1177/21501351221111390.


Despite overall improvements in outcomes for patients with hypoplastic left heart syndrome, there remain anatomic features that can place these patients at higher risk throughout their treatment course. These include severe preoperative obstruction to pulmonary venous return, restrictive atrial septum, coronary fistulae, severe tricuspid regurgitation, smaller ascending aorta diameter (especially if <2 mm), and poor ventricular function. The risk of traditional staged palliation has led to the development of alternative strategies for such patients. To further improve the outcomes, we must continue to diligently examine and study anatomic details in HLHS patients.

Keywords: Fontan; Norwood procedure; anatomy; cavopulmonary anastomosis; congenital heart disease (CHD); congenital heart surgery; hypoplastic left heart syndrome; outcomes (includes mortality, morbidity).

MeSH terms

  • Heart Defects, Congenital* / surgery
  • Heart Ventricles / surgery
  • Humans
  • Hypoplastic Left Heart Syndrome* / surgery
  • Palliative Care
  • Retrospective Studies
  • Treatment Outcome
  • Tricuspid Valve Insufficiency*