In diffuse forms of arteriovenous malformation following Fontan procedure, "classical" medical therapy, inhaled nitric oxide and sildenafil, may play a role, until re-direction of hepatic flow to pulmonary circulation cures it. However, in refractory cases, as reported in our 2-year-old patient, unusual medications such as calcium channel blockers can be tried and continued if patients respond adequately.
Keywords: Arteriovenous malformations; Fontan; amlodipine; hypoxemia; intensive care.