ABSTRACT
Ebstein's anomaly (EA) is associated with poor outcome in symptomatic neonates. Management typically includes prostaglandins or surgical creation of a Blalock-Taussig shunt. Right ventricular afterload reduction may help by improving the forward flow across the pulmonary valve. We report the successful use of oral sildenafil, a pulmonary vasodilator, in a neonate with severe EA.
KEYWORDS
Ebstein's anomaly - sildenafil - cyanosis - neonate
REFERENCES
-
1
Celermajer D S, Bull C, Till J A et al..
Ebstein's anomaly: presentation and outcome from fetus to adult.
J Am Coll Cardiol.
1994;
23
170-176
-
2
Yetman A T, Freedom R M, McCrindle B W.
Outcome in cyanotic neonates with Ebstein's anomaly.
Am J Cardiol.
1998;
81
749-754
-
3
Pavlova M, Fouron J C, Drblik S P et al..
Factors affecting the prognosis of Ebstein's anomaly during fetal life.
Am Heart J.
1998;
135
1081-1085
-
4
Celermajer D S, Cullen S, Sullivan I D et al..
Outcome in neonates with Ebstein's anomaly.
J Am Coll Cardiol.
1992;
19
1041-1046
-
5
Bruckheimer E, Bulbul Z, Pinter E et al..
Inhaled nitric oxide therapy in a critically ill neonate with Ebstein's anomaly.
Pediatr Cardiol.
1998;
19
477-479
-
6
Schulze-Neick I, Hartenstein P, Li J et al..
Intravenous sildenafil is a potent pulmonary vasodilator in children with congenital heart disease.
Circulation.
2003;
108(Suppl 1)
II167-II173
-
7
Atz A M, Lefler A K, Fairbrother D L et al..
Sildenafil augments the effect of inhaled nitric oxide for postoperative pulmonary hypertensive crises.
J Thorac Cardiovasc Surg.
2002;
124
628-629
-
8
Gentles T L, Calder A L, Clarkson P M et al..
Predictors of long-term survival with Ebstein's anomaly of the tricuspid valve.
Am J Cardiol.
1992;
69
377-381
-
9
Ivy D, Loehr J, Schaffer M.
Usefulness of prolonged prostaglandin infusion in neonates with Ebstein's anomaly.
Am J Cardiol.
1993;
72
1327-1329
-
10
Corbin J D, Beasley A, Blount M A et al..
High lung PDE5: a strong basis for treating pulmonary hypertension with PDE5 inhibitors.
Biochem Biophys Res Commun.
2005;
334
930-938
-
11
Humpl T, Reyes J T, Holtby H et al..
Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot study.
Circulation.
2005;
111
3274-3280
-
12
Pham P, Hoyer A, Shaughnessy R et al..
A novel approach incorporating sildenafil in the management of symptomatic neonates with Ebstein's anomaly.
Pediatr Cardiol.
2006;
27
614-617
Sanjeev AggarwalM.D.
Division of Cardiology, The Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University
3901 Beaubien Blvd, Detroit, MI 48201