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:
- Heart failure
- Arrhythmias
- Sudden cardiac death
- Infectious endocarditis
- Additionally acquired heart diseases
- Pulmonary hypertension
- Neurological complications
- Hematological impairments
- Rheological impairments
Classification
| Simple ACHD: Simple lesions conferring no functional limitations, or those who have undergone curative surgery | Unrepaired 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 lesion | Single ventricle |
| Biventricular physiology with systemic RV | |
| Physiologic status | Poor exercise tolerance and/or decreased ventricular function (EF < 25% or NYHA 3–4) |
| Cyanosis (SaO2< 80%) | |
| Pulmonary hypertension (systemic or suprasystemic PAP) | |
| Neurodevelopmental delay | |
| Comorbidities | Obesity (BMI > 35) |
| COPD (FEV1< 30% of predicted) | |
| Diabetes mellitus type 1 | |
| Chronic renal insufficiency (GFR< 30%) | |
| Intraprocedural complications | Inotropy or vasopressor need |
| Arrhythmia requiring treatment | |
| Blood product transfusion |
Management
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).
Clinical Update:
Refakis et al. (Current Opinion in Anesthesiology, 2026) highlight that adults with ACHD presenting for noncardiac surgery have independently increased perioperative morbidity and mortality, driven by lesion complexity, residual physiology, ventricular dysfunction, arrhythmias, pulmonary hypertension, and age-related comorbidities, with risk rising markedly in patients >50 years or with renal disease or diabetes. They emphasize the use of composite risk stratification that integrates ACHD complexity, physiologic status (e.g., NYHA class, cyanosis), and surgical risk to determine location of care, monitoring intensity, and need for expert center management, and recommend invasive monitoring and postoperative critical care for intermediate–high-risk patients.
- Read more about this study HERE.
