83
4.4.6. Eisenmenger Syndrome
COR LOE
Recommendations
erapeutic (cont'd)
2b
C-LD
8. In adults with Eisenmenger syndrome and LV ejection
fraction >40% who are deemed to be at high risk for adverse
outcomes
†
or who have rapidly progressive disease, inhaled
or subcutaneous prostacyclin therapy may be considered as
initial therapy or as an addition to other PAH therapy to
improve symptoms, exercise capacity, and hemodynamics.
2b
C-EO
9. In adults with Eisenmenger syndrome, anticoagulation
therapy may be considered for patients with high-risk
features (atrial arrhythmia, new or enlarging pulmonary
arterial thrombosis, or prior thromboembolic event) and low
bleeding risk to prevent thromboembolic complications.
3: No
Benefit
B-NR
10. Adults with Eisenmenger syndrome should not be routinely
prescribed oral anticoagulation given the high bleeding risk
and lack of long-term survival benefit.
3. Harm B-NR
11. In adults with Eisenmenger syndrome, closure of any
intracardiac or vascular shunt should not be performed given
the increased perioperative risks and risks for short- and long-
term morbidity and mortality.
3. Harm B-NR
12. In adults with Eisenmenger syndrome and intracardiac
shunts who meet indications for permanent pacing or an
ICD, endocardial leads may be potentially harmful given the
increased risk for systemic thromboembolism.
* See Table 43 for evidence and recommendations on specific PAH therapies.
†
Patients at high risk include patients with World Health Organization functional class 3 or
4, a 6-minute walk distance <165 m, tricuspid annular plane systolic excursion <16 mm, and
NT-proBNP level >1,400 pg/mL.
Recommendations on managing chronic cyanosis—including, but not limited to,
management of iron supplementation, hyperviscosity, and risk and management of central
nervous system infections—generally apply to patients with Eisenmenger syndrome and are
provided in Section 3.5, "Management of Cyanosis."
(cont'd)
