82
Specific Conditions
4.4.5.1. HLHS/Norwood Repair
COR LOE
Recommendation
2a C-EO
1. In patients with HLHS, routine 3-dimensional imaging of the
neoaorta to assess for dilation and obstruction is reasonable.
4.4.6. Eisenmenger Syndrome
COR LOE
Recommendations
Diagnostic
1 B-NR
1. In adults with suspected Eisenmenger syndrome, invasive
hemodynamic assessment should be performed to confirm
the diagnosis and to exclude concomitant disease that
contributes to right-to-left shunting or PAH.
1 B-NR
2. Adults with Eisenmenger syndrome should be managed by
specialists with expertise in both congenital cardiolog y and
PAH to improve outcomes.
3. Harm B-NR
3. Adults with Eisenmenger syndrome should be advised against
pregnancy to decrease the risk associated with excess maternal
morbidity and mortality.
erapeutic
1 B-R
4. In adults with Eisenmenger syndrome and an LV ejection
fraction >40% who are symptomatic or have reduced exercise
capacity, initial monotherapy with PAH-directed therapy*
is recommended to improve symptoms, hemodynamics, and
overall survival.
1 B-NR
5. In adults with Eisenmenger syndrome and an LV ejection
fraction >40% who remain symptomatic or have worsening
exercise capacity on a single PAH therapy, dual combination
therapy with an endothelin receptor antagonist and a
phosphodiesterase-5 inhibitor is recommended to improve
symptoms, hemodynamics, and overall survival.
1 C-LD
6. In adults with Eisenmenger syndrome who present with atrial
arrhythmias, prompt restoration and maintenance of sinus
rhythm is recommended to avoid hemodynamic deterioration.
2a
C-LD
7. In adults with Eisenmenger syndrome, a regular exercise
program in combination with directed PAH therapy can be
effective in improving exercise capacity.
