Functional status after operation for Ebstein anomaly: the Mayo Clinic experience.
OBJECTIVE
The objective of this study was to review the long-term functional outcome of patients with Ebstein anomaly who had cardiac operation at our institution.


BACKGROUND
Ebstein anomaly is a spectrum of tricuspid valvular and right ventricular dysplasia. Many patients will require operation in an attempt to improve quality of life.


METHODS
From April 1, 1972, to January 1, 2006, 539 patients with Ebstein anomaly underwent 604 cardiac operations at the Mayo Clinic in Rochester, Minnesota. Patient records were reviewed, and all patients known to still be alive were mailed a medical questionnaire or contacted by telephone.


RESULTS
At the initial operation at our institution, the mean age of the patients was 24 years (range 8 days to 79 years) and 53% were female patients. Survival at 5, 10, 15, and 20 years was 94%, 90%, 86%, and 76%, respectively. Survival free of late reoperation was 86%, 74%, 62%, and 46% at 5, 10, 15, and 20 years, respectively. Surveys were returned by 285 of 448 (64%) patients known to be alive at the time of this study. Two hundred thirty-seven (83%) patients were in New York Heart Association functional class I or II, and 34% were taking no cardiac medication. One hundred three patients (36%) reported an incident of atrial fibrillation or flutter, 5 patients (2%) reported having had endocarditis, and 1 patient (<1%) reported having a stroke. There were 275 pregnancies among 82 women. The recurrence of congenital heart disease was reported in 9 of 232 (3.9%) liveborn children.


CONCLUSIONS
Patients have good long-term survival and functional outcomes after undergoing surgery for Ebstein anomaly. Atrial arrhythmias are common both before and after surgery. Many patients have had one or more successful pregnancies with a low-recurrence risk of congenital heart disease.
Brown
Morgan L
ML
Division of Cardiovascular Surgery, Mayo Clinic Center Congenital Heart Center, Mayo Clinic College of Medicine, Mayo Clinic and Foundation, Rochester, Minnesota, USA.
Dearani
Joseph A
JA
Danielson
Gordon K
GK
Cetta
Frank
F
Connolly
Heidi M
HM
Warnes
Carole A
CA
Li
Zhuo
Z
Hodge
David O
DO
Driscoll
David J
DJ
D000293:Adolescent; D000328:Adult; D000368:Aged; D006348:Cardiac Surgical Procedures / Q000009:adverse effects*; D002648:Child; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000150:complications / Q000503:physiopathology* / Q000601:surgery*; D017079:Exercise Tolerance; D005260:Female; D006304:Health Status; D006352:Heart Ventricles / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D008910:Minnesota; D011446:Prospective Studies; D011788:Quality of Life; D012307:Risk Factors; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D013997:Time Factors; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000601:surgery*