Abstract
Ebstein anomaly is a myopathy of the right ventricle that results in variable degrees of failure of delamination of the tricuspid valve leaflets from the underlying endocardium, leading to severe tricuspid valve regurgitation and some degree of right ventricular dysfunction. In neonates or infants who remain in congestive heart failure or profoundly cyanotic while receiving appropriate medical therapy, operation is required. Current strategies include biventricular or single-ventricle repair. In children and adults, medical management may be used, but most patients eventually require surgery. Tricuspid valve repair is preferred; we believe the cone repair is the most anatomic repair and is the operation of choice. Tricuspid valve replacement may be necessary in cases in which the valve is not repairable. A bidirectional cavopulmonary shunt is useful in patients with severe right ventricular dilatation and/or dysfunction. Transplantation rarely is necessary.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 396-402 |
| Number of pages | 7 |
| Journal | Current Treatment Options in Cardiovascular Medicine |
| Volume | 11 |
| Issue number | 5 |
| DOIs | |
| State | Published - 2009 |
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ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
Cite this
Ebstein malformation of the tricuspid valve : Current concepts in management and outcomes. / Brown, Morgan L.; Dearani, Joseph A.
In: Current Treatment Options in Cardiovascular Medicine, Vol. 11, No. 5, 2009, p. 396-402.Research output: Contribution to journal › Review article
}
TY - JOUR
T1 - Ebstein malformation of the tricuspid valve
T2 - Current concepts in management and outcomes
AU - Brown, Morgan L.
AU - Dearani, Joseph A.
PY - 2009
Y1 - 2009
N2 - Ebstein anomaly is a myopathy of the right ventricle that results in variable degrees of failure of delamination of the tricuspid valve leaflets from the underlying endocardium, leading to severe tricuspid valve regurgitation and some degree of right ventricular dysfunction. In neonates or infants who remain in congestive heart failure or profoundly cyanotic while receiving appropriate medical therapy, operation is required. Current strategies include biventricular or single-ventricle repair. In children and adults, medical management may be used, but most patients eventually require surgery. Tricuspid valve repair is preferred; we believe the cone repair is the most anatomic repair and is the operation of choice. Tricuspid valve replacement may be necessary in cases in which the valve is not repairable. A bidirectional cavopulmonary shunt is useful in patients with severe right ventricular dilatation and/or dysfunction. Transplantation rarely is necessary.
AB - Ebstein anomaly is a myopathy of the right ventricle that results in variable degrees of failure of delamination of the tricuspid valve leaflets from the underlying endocardium, leading to severe tricuspid valve regurgitation and some degree of right ventricular dysfunction. In neonates or infants who remain in congestive heart failure or profoundly cyanotic while receiving appropriate medical therapy, operation is required. Current strategies include biventricular or single-ventricle repair. In children and adults, medical management may be used, but most patients eventually require surgery. Tricuspid valve repair is preferred; we believe the cone repair is the most anatomic repair and is the operation of choice. Tricuspid valve replacement may be necessary in cases in which the valve is not repairable. A bidirectional cavopulmonary shunt is useful in patients with severe right ventricular dilatation and/or dysfunction. Transplantation rarely is necessary.
UR - http://www.scopus.com/inward/record.url?scp=70349240464&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=70349240464&partnerID=8YFLogxK
U2 - 10.1007/s11936-009-0041-5
DO - 10.1007/s11936-009-0041-5
M3 - Review article
C2 - 19846038
AN - SCOPUS:70349240464
VL - 11
SP - 396
EP - 402
JO - Current Treatment Options in Cardiovascular Medicine
JF - Current Treatment Options in Cardiovascular Medicine
SN - 1092-8464
IS - 5
ER -