Guidelines Summary
The European Society of Cardiology (ESC) updated their 2010 guidelines on the management of adult congenital heart disease (ACHD) in 2020. [9, 10] Select class I and III recommendations are outlined.
RV Outflow Tract Obstruction (OTO)
In valvular pulmonary stenosis (PS), balloon valvuloplasty is the intervention of choice, if anatomically suitable.
As long as no valve replacement is required, RVOTO intervention at any level is recommended regardless of symptoms when the stenosis is severe (Doppler peak gradient >64 mmHg).
If surgical valve replacement is the only option, it is indicated in (1) symptomatic patients with severe stenosis; or (2) asymptomatic patients with severe stenosis in the presence of ≥1 of the following:
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Objective decrease in exercise capacity
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Falling RV function and/or progression of tricuspid regurgitation (TR) to at least moderate
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RV systolic pressure (SP) >80 mmHg
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Right-to-left (RL) shunting via an ASD or VSD
CHD-Associated Pulmonary Arterial Hypertension (PAH)
Counsel patients with congenital heart disease (CHD) and confirmed precapillary pulmonary hypertension (PH) against pregnancy.
All patients with PAH-CHD should undergo risk assessment.
In low- and intermediate-risk patients with repaired simple lesions and precapillary PH, initial oral combination therapy or sequential combination therapy is recommended; treat high-risk patients with initial combination therapy including parenteral prostanoids.
After Repair of Tetralogy of Fallot
Pulmonary valve replacement (PVRep) is recommended in symptomatic patients with severe pulmonary regurgitation (PR) and/or at least moderate RVOTO.
In those without a native outflow tract, catheter intervention (transcatheter pulmonary valve implantation [TPVI]) is preferred if anatomically feasible.
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Pulmonic Stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows a parasternal short-axis view of the thickened pulmonary valve.
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Pulmonic Stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows a Doppler scan of the peak velocity (5.2 m/s) and gradients (peak 109 mmHg, mean 65 mmHg) across the valve.
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Pulmonic Stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows moderately severe pulmonary insufficiency (orange color flow) is also present.
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Pulmonic Stenosis. This is an echocardiogram of a patient with severe pulmonic stenosis. The first segment shows the parasternal short-axis view of the thickened pulmonary valve. The second segment shows the presence of moderate pulmonary insufficiency (orange color flow). AV = aortic valve; PA = pulmonary artery; PI = pulmonary insufficiency; PV = pulmonary valve.
