4. Drug treatment
The level of pulmonary vascular resistance in SV patients is the key to
determine the long-term postoperative efficacy.24 Nine
drugs have been approved in North America and Europe for the treatment
of pulmonary hypertension. These drugs fall into four categories:
5-phosphodiesterase (PDE-5) inhibitors, endothelin receptor antagonists,
prostacyclin analogues, and soluble guanosine activators. In recent
years, pulmonary vascular targeted drugs such as Sildenafil and Bosentan
have also been used in the perioperative treatment of Fontan patients.
One case report showed that Sildenafil improved the outcomes for
patients with protein-losing bowel disease,25while in
another,Sildenafil was used to treat plastic
bronchitis.26 A new study showed that a single dose of
Sildenafil improved the peak exercise
performancein Fontan
patientscompared with a placebo.27 On the other hand,
there is little evidence to support the use of angiotensin-converting
enzyme (ACE) inhibitors for SV patients.28