Adult congenital heart disease

Top concerns

1. 1.Identify anatomy + repair status first: 'repaired' rarely means 'normal' — get the most recent cardiology note/echo
2. 2.Any potential right-to-left communication → meticulous de-airing of all IV lines and stopcocks (paradoxical embolism)
3. 3.Eisenmenger physiology: fixed high PVR — drops in SVR worsen right-to-left shunting; avoid abrupt vasodilation, keep them warm, avoid hypoxia/hypercarbia (PVR triggers)
4. 4.Fontan circulation: passive pulmonary flow — preload-dependent, PVR-sensitive; spontaneous or low-pressure ventilation strategies favored
5. 5.Endocarditis prophylaxis per current AHA criteria (cyanotic disease, prosthetic material, prior IE)
6. 6.Cyanotic patients: erythrocytosis, altered hemostasis — hydration status matters

Drug do's and don'ts

• AVOIDAir in any IV line— Treat every bubble as arterial in shunt physiology.
• AVOIDAbrupt SVR reduction in shunt-dependent lesions— Neuraxial and induction plans need vasopressor support ready.
• PREFERRegional techniques (appropriately managed)— Often well tolerated with attention to preload/SVR — not contraindicated by the label 'cardiac'.

Airway & positioning

• Ventilation strategy IS hemodynamics here: high intrathoracic pressure hurts Fontan/PVR-sensitive physiology

Pearls

• Draw the circulation on paper preop; if you can't, call the congenital cardiologist before rooming the patient.
• Bubble discipline is a team sport — brief it out loud.

Suggested reading

• Miller's Anesthesia, 9e (congenital heart disease chapters)
• AHA/ACC 2018 ACHD Guideline

Educational quick-reference only — defer to clinical judgment, institutional protocols, and the primary texts above.