Tetralogy of Fallot: morphological variations and implications for surgical repair

Research output: Contribution to journalArticle

Authors

  • Saad Khan
  • John Stickley
  • David Barron
  • William Brawn
  • Timothy Jones
  • Robert H Anderson
  • Adrian Crucean

Colleges, School and Institutes

External organisations

  • Birmingham Children’s Hospital, Birmingham, UK
  • Birmingham Children's Hospital
  • Newcastle University

Abstract

Objectives: Tetralogy of Fallot is characterized by anterocephalad deviation of the outlet septum, along with abnormal septoparietal trabeculations, which lead to subpulmonary infundibular stenosis. Archives of retained hearts are an important resource for improving our understanding of congenital heart defects and their morphological variability. This study aims to define variations in aortic override, coronary arterial patterns and ventricular septal defects in tetralogy of Fallot as observed in a morphological archive, highlighting implications for surgical management. Methods: The Birmingham Children's Hospital archive contains 211 hearts with tetralogy of Fallot, of which 164 were analysed [69 (42.1%) unrepaired and 95 (57.9%) operated specimens]. A detailed morphological and geometric analysis was performed using a rigorous 5-layer review process. Results: Anomalies were observed in the orifices, origins and course of the coronary arteries: 20 hearts (13.0%) had more than 2 orifices and 3 hearts (1.9%) had a single orifice. In 7 hearts (4.3%), a coronary artery crossed the right ventricular outflow tract. The extent of aortic override ranged from 31.0% to 100% (median of 59.5%). The ventricular septal defect was most often perimembranous (139, 84.8%), but we also found muscular (14, 8.5%), atrioventricular (7, 4.3%) and doubly committed juxta-arterial (2, 1.2%) variants. Conclusions: Anatomical variations are common and can impact surgical management. Anomalous coronary arteries may require a conduit rather than a transannular patch. Variability in aortic override determines the size of patch used to baffle blood to the aorta. The type of ventricular septal defect affects patch closure and the risk of postoperative conduction defects.

Details

Original languageEnglish
Article numberezy474
Pages (from-to)101-109
Number of pages9
JournalEuropean Journal of Cardio-Thoracic Surgery
Volume56
Issue number1
Early online date16 Jan 2019
Publication statusPublished - 1 Jul 2019

Keywords

  • tetralogy of Fallot, morphology, coronary arteries, aortic override, cardiac surgery