15
3.5. Management of Cyanosis
COR LOE
Recommendations
1 B-NR
1. Patients with ACHD and cyanosis should receive annual
screening for and treatment of iron deficiency to improve
exercise capacity and functional status.
1 B-NR
2. In patients with ACHD and cyanosis who present with
persistent, new, or worsening neurologic deficits, urgent
brain imaging to exclude cerebral abscess or stroke should be
performed.
1 B-NR
3. In patients with ACHD and cyanosis who present with
symptoms of hyperviscosity, rehydration with oral or
intravenous fluids should be performed to improve symptoms
and reduce the risk for vascular complications.
1 B-NR
4. Pregnant patients with ACHD and cyanosis should be
closely followed for the entire duration of the pregnancy
by a multidisciplinary cardio-obstetrics team of experts in
maternal-fetal medicine, ACHD, and obstetric anesthesia
to reduce the risk for pregnancy-related cardiovascular and
obstetric complications.
1 C-EO
5. Patients with ACHD and cyanosis who are of childbearing
age should receive comprehensive family planning
consultation, including recommendations on safe and reliable
contraceptive methods, from a multidisciplinary team of
experts in family planning and ACHD.
2a B-NR
6. In patients with ACHD and cyanosis who experience recurrent
unexplained hypertension or tachycardia, evaluation to detect
pheochromocytomas and paragangliomas is reasonable.
2a B-NR
7. In patients with ACHD, cyanosis, and recurrent episodes of
joint pain, it is reasonable to check serum uric acid levels and
evaluate for gout.
2b B-R
8. In patients with ACHD and cyanosis, the use of
supplemental oxygen during exercise may be considered to
improve exercise capacity.
2b C-EO
9. In patients with ACHD, hematocrit >65%, and hyperviscosity
symptoms that persist despite adequate rehydration and
treatment of iron deficiency, phlebotomy may be considered.
3: No
Benefit
B-NR
10. In patients with ACHD and cyanosis, prophylactic
phlebotomy is not recommended.
