1. Perils of Imperfect Plumbing
SINGLE VENTRICLES
Perils of
Imperfect
Plumbing
R. Dennis Steed, MD
Associate Professor
Department of Pediatrics
Division of Pediatric Cardiology
East Carolina University – Brody School of Medicine
Greenville, NC

2. Single Ventricles Anatomical Substrates
Atresia / hypoplasia of valves
Double inlet ventricles
Severely unbalanced AV canal defects with complex attachments
Atrial isomerism / heterotaxy

4. TRICUSPID ATRESIA
1.5 : 1

7. Single Ventricles Norwood Procedure
Neonatal open procedure
First week of life
Reconstruction of aorta using pulmonary artery and placement of BT shunt

8. Stage I Norwood with Sano Modification:

9. Single Ventricles Glenn Shunt / Hemi - Fontan
6 - 9 months
volume unloads ventricle
addresses any pulmonary artery distortion
perceived decrease in pleural effusions
facilitates completion of Fontan

11. Single Ventricles Fontan Procedure
Generally done at years of age
Intra-atrial baffle of inferior vena caval blood to pulmonary artery
Common to use fenestration
Extracardiac conduit of inferior caval blood to pulmonary artery

12. Single Ventricles Fontan Procedure

13. Key Components of Favorable Post-Fontan Hemodynamics:
1.Normal ventricular function (systolic and diastolic)
2.Lack of important A-V valve incompetence.
3.Low trans-pulmonary gradient.

14. Evaluation of Systolic Ventricular function in Univentricular Hearts:
Use a method that is reproducible to allow for serial comparisons

15. Evaluation of Systolic Ventricular function in Univentricular Hearts:
Left ventricular morphology: routine ejection indices for your lab.
Right ventricular morphology: Mid cavitary two-dimensional area shortening may be most reliable.

16. Evaluation of Diastolic Ventricular function in Univentricular Hearts:
E:A ratios.
May use IVRT with ventricles of left ventricular morphology (sufficient proximity between the aortic valve and a A-V valve.

17. Trans-pulmonary Gradient:
Ideal: 3- 5 mm Hg
Satisfactory: 6-8 mm Hg