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← Section IV · Congenital Heart Disease
IV.J

Principles of Medical and Surgical Management

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Notes

Palliative operations (Increase pulmonary blood flow)

OperationDescriptionComplication
Classic Blalock-Taussig shunt (1944)Subclavian artery to PA anastomosisPA distortion
Modified Blalock-Taussig shuntSubclavian to PA via Gore-Tex interposition graftPA distortion
Central shuntAscending aorta to PA graftLess distortion
Waterston shunt (1960s)Ascending aorta to right PADistortion, PAH from unrestricted flow
Potts shunt (1946)Descending aorta to left PADistortion, PAH
Glenn shunt (1954)SVC to right PA (unidirectional or bidirectional)Pulmonary AV fistulae over time
Pulmonary artery bandConstricting band around MPA to limit pulmonary flowDistortion

Corrective operations

  • PDA ligation (Gross, 1938) - first successful CHD surgery.
  • Coarctation repair (Gross/Crafoord, 1945) - resection with end-to-end anastomosis, patch aortoplasty, or subclavian flap.
  • ASD repair (Gibbon, 1953) - first open-heart on bypass.
  • VSD repair (Lillehei, 1954).
  • Mustard/Senning atrial switch for d-TGA (1954–1959) - historical.
  • TOF repair (Lillehei-Kirklin, 1955) - VSD closure + RVOT reconstruction.
  • Rastelli (1964) - LV baffled through VSD to aorta; RV-to-PA conduit for TGA+VSD+PS.
  • Rashkind balloon septostomy (1967) - catheter-based emergency for TGA.
  • Fontan/Kreutzer (1971) - single-ventricle palliation.
  • Jatene arterial switch for d-TGA (1976) - current standard.

The Fontan concept

Goals:

  1. Separate systemic and pulmonary circulations.
  2. Remove volume load from the single functional pumping chamber.
  3. Direct systemic venous return passively to the PAs.

Total cavopulmonary connection (modern Fontan)

  • SVC anastomosed directly to right PA.
  • IVC directed to PAs through an intra-atrial tunnel or extra-cardiac conduit.
  • Atriopulmonary Fontan (older technique) had high atrial arrhythmia burden.

Fontan candidates

  • HLHS.
  • Hypoplastic right heart / severe pulmonary atresia.
  • Tricuspid or mitral atresia.
  • Double-inlet single ventricle.
  • Complex AVSD with straddling AV valve.

Late Fontan complications

  • Protein-losing enteropathy.
  • Plastic bronchitis.
  • Fontan-associated liver disease (FALD).
  • Atrial arrhythmias (particularly atriopulmonary Fontan).
  • Thromboembolism.
  • Systemic AV valve regurgitation.
  • Cyanosis if fenestration or venous collateral development.

Cards

  • basicIV.J-001
    What is a modified Blalock-Taussig shunt and its purpose?
    → Subclavian artery to pulmonary artery via a Gore-Tex interposition graft. Increases pulmonary blood flow in cyanotic CHD lesions with reduced pulmonary flow (e.g., pulmonary atresia, severe TOF).
  • basicIV.J-002
    What is a Glenn shunt?
    → Superior vena cava anastomosed to the right pulmonary artery (bidirectional Glenn: both PAs). Provides passive pulmonary blood flow to the upper body without a pumping chamber. Later followed by Fontan completion.
  • basicIV.J-003
    What is a Waterston shunt and its major complication?
    → Ascending aorta to right pulmonary artery anastomosis. Historical palliation. Complication: PA distortion and PA hypertension from unrestricted high-pressure flow.
  • basicIV.J-004
    When is a pulmonary artery band used?
    → To limit pulmonary blood flow in lesions with pulmonary overcirculation (large VSD or single-ventricle physiology with high PBF) as a temporizing measure before definitive repair.
  • basicIV.J-005
    What is a Rashkind balloon atrial septostomy used for?
    → Emergent enlargement of an interatrial communication in a newborn with critical d-TGA (or other cyanotic lesions requiring atrial-level mixing) as a temporizing measure until definitive repair.
  • basicIV.J-006
    Which two variants of the Fontan operation exist for single-ventricle palliation?
    → Atriopulmonary Fontan (historical — dilated right atrium anastomosed to PA; high atrial arrhythmia rate). Total cavopulmonary connection (modern — SVC direct to PA and IVC via extracardiac conduit or intra-atrial tunnel).
  • basicIV.J-007
    State four late complications of the Fontan operation.
    → Protein-losing enteropathy, plastic bronchitis, Fontan-associated liver disease, atrial arrhythmias, thromboembolism, systemic AV valve regurgitation.
  • basicIV.J-008
    Why does high pulmonary vascular resistance predict Fontan failure?
    → Because the Fontan circulation drives pulmonary blood flow passively (no pumping chamber in the pulmonary circuit), it relies on a low-resistance pulmonary bed. High PVR raises systemic venous pressure, reducing preload to the systemic ventricle and driving Fontan-related complications.
  • basicIV.J-009
    When was the arterial switch operation (Jatene) first performed and why is it preferred over atrial switch?
    → 1976. The arterial switch relocates the great arteries and coronaries, restoring the morphologic LV as the systemic ventricle. Atrial switch (Mustard/Senning) leaves the morphologic RV as the systemic ventricle → late RV failure and arrhythmias.
  • basicIV.J-010
    What is the Rastelli operation and when is it used?
    → For d-TGA with a large VSD and LVOT obstruction (PS). Baffles LV output through the VSD to the aorta and places an RV-to-PA conduit.