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