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