Sildenafil in the management of the failing Fontan circulation.
BACKGROUND
Sildenafil is increasingly being used in the management of pulmonary arterial hypertension in the newborn. Its role in patients with congenital cardiac disease is less well defined and as yet has only been reported sporadically.


OBJECTIVE
Present our experience with sildenafil treatment in patients with a failing Fontan circulation.


METHODS
Retrospective review of 13 symptomatic patients after Fontan palliation who received treatment with sildenafil between January, 2006 and July, 2008.


RESULTS
Three patients suffered from protein-losing enteropathy, four patients presented with bronchial casts, two had severe cyanosis after fenestrated Fontan procedure, two had prolonged chylous effusions, one had a previous failure of Fontan and take-down, and one patient had arrhythmias and end-stage cardiac failure requiring conversion to an extra-cardiac Fontan. Sildenafil was used in the dosage of 1-2 milligrams per kilogram 3-4 times per day. Protein-losing enteropathy and alpha-1-antitrypsin levels improved in all three patients on sildenafil treatment. One of these patients had a concomitant catheter creation of a fenestration, as did two patients presenting with bronchial casts and both patients with persistent chylous effusions. All four patients with bronchial casts and two patients with cyanosis improved significantly on sildenafil treatment. Chylous effusions decreased after sildenafil and stent enlargement of a fenestration. There were no significant side effects requiring sildenafil withdrawal over a treatment period ranging from 2 months to 2 years.


CONCLUSIONS
Sildenafil can be used safely and effectively in the treatment of patients with a failing Fontan circulation.
Reinhardt
Zdenka
Z
Birmingham Children's Hospital, NHS Foundation Trust, United Kingdom.
Uzun
Orhan
O
Bhole
Vinay
V
Ofoe
Victor
V
Wilson
Dirk
D
Onuzo
Obed
O
Wright
John G C
JG
Stumper
Oliver
O
D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects*; D006333:Heart Failure / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D010879:Piperazines / Q000008:administration & dosage / Q000627:therapeutic use*; D011183:Postoperative Complications; D011504:Protein-Losing Enteropathies / Q000188:drug therapy* / Q000209:etiology; D011687:Purines / Q000008:administration & dosage / Q000627:therapeutic use; D012189:Retrospective Studies; D012769:Shock / Q000188:drug therapy* / Q000209:etiology; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000008:administration & dosage / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000627:therapeutic use
D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate