Perils of Imperfect Plumbing

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Presentation transcript:

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

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

TRICUSPID ATRESIA 1.5 : 1

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

Stage I Norwood with Sano Modification:

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

Single Ventricles Fontan Procedure Generally done at 2 - 4 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

Single Ventricles Fontan Procedure

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.

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

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.

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.

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