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:

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

Table of Contents

Contributors

Adult congenital heart disease (ACHD)

Adult congenital heart disease (ACHD)

Learning objectives

Definition & mechanisms

Common comorbidities:

Classification

Risk factors

Management

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