Why ACHD Patients Are Complex

  • Altered anatomy/physiology: Fontan, Glenn, Mustard, Senning; single ventricle; right heart failure; pulmonary hypertension.

  • Prior surgery/occlusions: adhesions, venous/arterial occlusions.

  • Comorbidities: renal, neurologic, pulmonary, hepatic (Fontan congestion), restrictive lung disease, obstructive sleep apnea, cognitive/psychiatric issues, epilepsy, coagulopathies, iron deficiency, bleeding disorders.

Indications & (Relative) Contraindications

  1. Indications: cardiogenic shock, failure to wean from bypass, bridge to decision/transplant, ECPR, acute decompensation (arrhythmia/infection), peri-procedural stabilization.

  2. Contraindications (relative): multiple organ failure, sepsis with vasoplegia, severe aortic regurgitation, prolonged unwitnessed arrest, no recovery/transplant option.

Cannulation & Monitoring

  • Challenges: limited femoral/neck access, systemic-to-pulmonary shunts, pulmonary hypertension; central vs peripheral must be individualized.

  • Planning: imaging and surgical history are critical; cannula position influences oxygenation/flows.

  • Monitoring: continuous CVP and mixed venous saturation; hemodynamics must account for altered anatomy (e.g., IJ in Glenn = pulmonary artery pressure).

Case Highlights

1) Ebstein Anomaly, Right Heart Failure

  • Strategy: femoral vein–femoral artery VA-ECMO, lower flows, echo guidance.

  • Outcome: stabilized 5 days → surgical tricuspid valve replacement.

  • Learning: tailored cannulation and vigilant hemodynamic monitoring.

2) Fontan Circulation, Cardiogenic Shock

  • Strategy: dual cannulation (right IJ + femoral vein) with femoral arterial return.

  • Course: hemolysis, mild pulmonary hemorrhage, persistent low flows.

  • Outcome: MDT not for transplant → palliative care after 7 days.

  • Learning: limited efficacy of VA-ECMO in Fontan; selection and planning are essential; poor survival around 35%.

ECLS After ACHD Surgery

  • Use rare; mortality high. Risk factors: older age, increased surgical complexity, univentricular physiology, preoperative hospitalization, non-cardiac chronic conditions, renal/neurological/pulmonary complications.

ECPR in Fontan

  • Problems: raised intrathoracic/venous pressures, reduced pulmonary blood flow, difficult cannulation, venous drainage issues, neurological injury.

Bridge Strategies & Management

  • Bridge to VAD/transplant: choose ECLS vs VAD based on lungs, pulmonary hypertension, aortic regurgitation, anticipated wait time.

  • Always define an exit strategy.

  • Tailored circuits/anticoagulation; MDT collaboration is essential.

Take-Home

  • VA-ECMO is lifesaving but variable; outcomes depend on anatomy and comorbidities.

  • Fontan patients: poorest prognosis; best managed at high-volume ACHD/ECMO centers.

  • Future: improved circuits, compact devices, perioperative/bridge use, potential roles with gene/stem cell therapy and xenotransplant.

 

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