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Overview
Top Take-Home Messages
1. Adults with congenital heart disease benefit from routine care
at ACHD centers and in collaboration with ACHD cardiologists.
Multidisciplinary teams are useful for complex-care decision-making.
2. Patients with anatomic or physiologically moderate or complex
ACHD who undergo cardiac or noncardiac procedures are
recommended to have an ACHD cardiologist involved in their care to
offer expert guidance on procedures, anesthesia, and postprocedural
management.
3. The possibility of endocarditis is important to evaluate in acute
or subacute malfunction of bioprosthetic pulmonary valves, and
endocarditis has been added to the physiological classification
system (subacute bacterial endocarditis in the past year is stage D).
4. Most pregnant patients with ACHD can undergo vaginal delivery
safely, with appropriate risk stratification and monitoring.
5. Updated recommendations for tetralogy of Fallot include (a) referral
for pulmonary valve replacement in patients with repaired tetralogy
of Fallot, according to right ventricular end-systolic volume criteria
(>80 mL/m
2
) and other metrics rather than end-diastolic volume;
and (b) new approaches to arrhythmia management, including
ablation of ventricular tachycardia.
6. Strategies for patients with secundum atrial septal defect and
pulmonary arterial hypertension now include recommendations for
closure for many patients with a significant left-to-right shunt and
pulmonary vascular resistance ≤2 Wood units or >2 to <5 Wood
units.
7. Rhythm control is typically preferred over rate control for atrial
arrhythmias in complex patients, such as those with a systemic right
ventricle or Fontan circulation.
8. New recommendations on guideline-directed medical therapy for
heart failure in patients with ACHD include people with a systemic
right or left ventricle and discussions of pacing strategies for a
systemic right ventricle and Fontan circulation.
9. Patients with Eisenmenger syndrome can be treated with pulmonary
vasodilators, using either phosphodiesterase-5 inhibitors or
endothelin receptor antagonists as initial therapy.
10. Fontan screening for liver disease includes recommendations for at
least annual imaging and laboratory evaluation, including alpha-
fetoprotein, and at least 1 consultation with a hepatologist.
