Adult congenital heart disease (ACHD) - NYSORA

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Adult congenital heart disease (ACHD)

Learning objectives

  • Describe the implications and comorbidities of ACHD
  • Classify the severity and assess the anesthetic risk factors of ACHD
  • Anesthetic management of patients with ACHD

Note: It is important to assess the specific lesion and disease progression of every patient individually and manage accordingly

Definition & mechanisms

  • Congenital heart disease (CHD) is the most common birth defect in humans, affecting nearly 1% of live births
  • Defects range from mild to severe 
  • Only decades ago: High mortality in children with CHD
  • Today, more than 80% of children with CHD survive adolescence and reach adulthood

Common comorbidities:


Simple ACHD: Simple lesions conferring no functional limitations, or those who have undergone curative surgeryUnrepaired lesion:
- Isolated aortic valve disease (13/1000 live births)
- Isolated mitral valve disease (excluding mitral cleft or parachute valve)
- Isolated patent foramen ovale, small atrial septal defect, or ventricular septal defect
Mild pulmonary stenosis
Repaired lesion
- Previously ligated or occluded ductus arteriosus
- Repaired sinus venosus or secundum atrial septal defect
- Without residual defect
- Repaired ventricular septal defect without residual defect
Moderate complexity ACHD: Moderately complex disease that may pose some day-to-day limitations, or who have undergone surgery likely to
require reoperation
Aorta to left ventricular fistula
Partial or total anomalous pulmonary venous drainage
Atrioventricular canal defects (partial or complete)
Coarctation of the aorta
Ebstein’s anomaly
Significant infundibular right ventricular outflow tract obstruction
Ostium primum or sinus venosus atrioseptal defect
Unrepaired ductus arteriosus
Moderate-to-severe pulmonary stenosis or regurgitation
Sinus of Valsalva fistula or aneurysm
Subvalvular or supravalvular aortic stenosis
Tetralogy of Fallot
Ventricular septal defect with associated anomaly, e.g. aortic regurgitation, absent valve, subaortic stenosis, mitral valve disease, right ventricular outflow tract obstruction, straddling atrioventricular valve
Severe complexity ACHD: Adults with severe complexity ACHD with significant functional limitation, they may have had palliative surgery or been
deemed not amenable to intervention
Conduits: valved or non-valved
All types of cyanotic heart disease
Double-outlet ventricle
Eisenmenger syndrome
Fontan procedure or total cavopulmonary connection
Mitral, tricuspid, or pulmonary atresia
Pulmonary hypertension
Any single-ventricle circulation
Transposition of the great vessels
Truncus arteriosus
Very rare complex anomalies, e.g. criss-cross heart, isomerism, ventricular inversion, heterotaxy syndromes

Risk factors

Anatomic lesionSingle ventricle
Biventricular physiology with systemic RV
Physiologic statusPoor exercise tolerance and/or decreased ventricular function (EF < 25% or NYHA 3–4)
Cyanosis (SaO2< 80%)
Pulmonary hypertension (systemic or suprasystemic PAP)
Neurodevelopmental delay
ComorbiditiesObesity (BMI > 35)
COPD (FEV1< 30% of predicted)
Diabetes mellitus type 1
Chronic renal insufficiency (GFR< 30%)
Intraprocedural complicationsInotropy or vasopressor need
Arrhythmia requiring treatment
Blood product transfusion


adult congenital heart disease, achd, echocardiography, ecg, x-ray, catheterization, mri, exercise testing, antithrombotic prophylaxis, normovolemia, fasting, bleeding, preload, afterload, contractility, vascular resistance, arterial line, central venous catheter, intensive care, pain, euvolemia, hypotension, oxygen

Suggested reading

  • Weale J, Kelleher AA. Adult congenital heart disease. Anaesthesia & Intensive Care Medicine. 2021;22(5):290-6.
  • Motta P, Manrique AM, Partington SL, Ullah S, Zabala LM. Congenital heart disease in adults (when kids grow up) pediatric geriatric anesthesia. Curr Opin Anaesthesiol. 2020;33(3):335-342.
  • Baehner T, Ellerkmann RK. Anesthesia in adults with congenital heart disease. Current Opinion in Anesthesiology. 2017;30(3).

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