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  • • A congenital heart lesion that decreases pulmonary arterial blood flow resulting in a right to left shunt

    • Cyanosis and decreased oxygen delivery causes compensatory polycythemia (Hct > 70%) and spontaneous thrombosis

    • Exercise, acidosis, pain worsens cyanosis and can cause hypoxic spells

    • Squatting increases systemic resistance, causing increased pulmonary flow and blood oxygenation

    • β-Blockers (decreases spasm), fluid intake, HCO3 administration, norepinephrine (increases systemic resistance) may help decrease hypoxia

    • Clubbing due to proliferation of capillaries and AV fistulas in extremities

    • Bronchial and mediastinal arteries enlarge

    • Ductus arteriosus maintains flow to lungs during fetal development

    • Alprostadil early can allow time for optimization before definitive treatment

    • Operative options to increase pulmonary flow:

    • -Fontan procedure: Superior vena cava and inferior vena cava rerouted to pulmonary artery

Double-Outlet Right Ventricle (DORV)

  • • Both great arteries (50% of annulus of each valve) arise from right ventricle

    • Uncommon and extremely complex to manage

    • Location of ventricular septal defect (VSD) (always present) determines classification

    • -Subaortic


      -Noncomitted (proximal to valve annuli)

      -Double committed (adjacent to annuli and flow directed at both arteries)

    • 33% of subaortic has subpulmonary obstruction and vice versa

    • Great vessel and coronary artery anatomy can vary

    • Subaortic VSD, rightward, posterior arrangement more common

Single Ventricle (SV)

  • • 1 ventricular chamber receiving blood from both tricuspid and mitral valves

    • Associated with

    • -Abnormal great vessel arrangements, 85%

      -Common AV valve, 30%

      -Stenosis or regurgitation of AV valve, 25%

      -Pulmonary stenosis or atresia, 50%

      -Aortic stenosis, 33%

      -Situs inversus, dextrocardia, and asplenia, 20%

    • Clinical findings and prognosis related to relative pulmonary and systemic blood flow



  • • Extremely rare


  • • 3-5% of congenital heart defects

    • 75% dominant left portion of ventricle

Symptoms and Signs


  • • Symptoms depend on location of VSD and degree of outflow obstruction


  • • Variable based on amount of pulmonary and systemic blood flow

Imaging Findings

  • • Echocardiography

    • Catheterization

  • • Echocardiography and catheterization for diagnosis

  • • Echocardiography and catheterization for diagnosis


  • • Surgical treatment depends on anatomy

    • Creation of intraventricular baffle to direct LV flow to aorta, closing septal defect, avoiding subpulmonary obstruction

    • Arterial switch is option


  • Initial: Shunt/pulmonary artery band, ventricular partitioning, then staged correction toward Fontan procedure



  • • 10-year survival, 60-80%

    • 33% require reintervention for outflow tract reobstruction


  • • 1-year survival, 40-80%

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