single ventricles perilsofimperfectplumbing r. dennis steed, md associate professor department of...
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SINGLE VENTRICLESSINGLE VENTRICLES
Perils Perils of of
Imperfect Imperfect PlumbingPlumbing
R. Dennis Steed, MDAssociate ProfessorDepartment of PediatricsDivision of Pediatric CardiologyEast Carolina University – Brody School of MedicineGreenville, NC
Single VentriclesSingle VentriclesAnatomical SubstratesAnatomical Substrates
• Atresia / hypoplasia of valvesAtresia / hypoplasia of valves
• Double inlet ventriclesDouble inlet ventricles
• Severely unbalanced AV canal defects Severely unbalanced AV canal defects with complex attachmentswith complex attachments
• Atrial isomerism / heterotaxyAtrial isomerism / heterotaxy
TRICUSPID ATRESIA
1.5 : 1
Single VentriclesSingle VentriclesNorwood ProcedureNorwood Procedure
• Neonatal open procedureNeonatal open procedure
• First week of lifeFirst week of life
• Reconstruction of aorta using pulmonary Reconstruction of aorta using pulmonary artery and placement of BT shuntartery and placement of BT shunt
Stage I Norwood with Sano Stage I Norwood with Sano ModificationModification::
Single VentriclesSingle VentriclesGlenn Shunt / Hemi - Glenn Shunt / Hemi - FontanFontan
• 6 - 9 months6 - 9 months• • volume unloads ventriclevolume unloads ventricle
• addresses any pulmonary artery distortionaddresses any pulmonary artery distortion
• perceived decrease in pleural effusionsperceived decrease in pleural effusions
• facilitates completion of Fontanfacilitates completion of Fontan
Single VentriclesSingle VentriclesFontan ProcedureFontan Procedure
• Generally done at 2 - 4 years of ageGenerally done at 2 - 4 years of age
• Intra-atrial baffle of inferior vena caval blood to Intra-atrial baffle of inferior vena caval blood to pulmonary arterypulmonary artery
• Common to use fenestrationCommon to use fenestration
• Extracardiac conduit of inferior caval blood to Extracardiac conduit of inferior caval blood to pulmonary arterypulmonary artery
Single VentriclesSingle VentriclesFontan ProcedureFontan 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:
1.E:A ratios.
2.May use IVRT with ventricles of left ventricular morphology (sufficient proximity between the aortic valve and a A-V valve.
Trans-pulmonary Gradient:
1.Ideal: 3- 5 mm Hg
2.Satisfactory: 6-8 mm Hg