PMID- 10670407
OWN - NLM
STAT- MEDLINE
DCOM- 20000224
LR  - 20190607
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 79
IP  - 1
DP  - 2000 Jan
TI  - The adult patient with Ebstein anomaly. Outcome in 72 unoperated patients.
PG  - 27-36
AB  - Knowledge of the long-term outcome in unoperated adult patients with Ebstein 
      anomaly is limited, and the therapeutic approach is still controversial. We 
      studied unoperated adult patients with Ebstein anomaly to define the patterns of 
      presentation, anatomic characteristics, outcome, and predictive factors for 
      survival. Seventy-two unoperated survivors of Ebstein anomaly aged over 25 years 
      attended from 1972 to 1997 were reviewed and followed-up from 1.6 to 22.0 years. 
      Patients were classified in 3 groups of severity according to the 
      echocardiographic appearance of the septal leaflet attachment of tricuspid valve. 
      The mean age at diagnosis was 23.9 +/- 10.4 years, and the most common clinical 
      presentation was an arrhythmic event (51.4%). There were 30 (42%) deaths, 
      including 6 from arrhythmia, 12 related to heart failure, 7 sudden, 2 unrelated, 
      and 3 unascertained. According to Cox regression analysis, predictors of 
      cardiac-related death included age at diagnosis (hazard ratio 0.89 for each year 
      of age, 95% confidence intervals CI[ 0.84-0.94), male sex (3.93, 95% CI, 
      1.50-10.29), degree of echocardiographic severity (3.34, 95% CI, 1.78-6.24), and 
      cardiothoracic ratio > or = 0.65 (3.57, 95% CI, 1.15-11.03). During follow-up, 
      morbidity was mainly related to arrhythmia and refractory late hemodynamic 
      deterioration. The magnitude of tricuspid regurgitation, cyanosis, and the New 
      York Heart Association (NYHA) functional class at time zero were significant risk 
      factors according to the univariate analysis, but not after multivariable 
      confrontation. The results of this study suggest that pattern of presentation, 
      clinical course, and prognosis of unoperated adult patients with Ebstein anomaly 
      are influenced by several factors. Although the initial symptoms are usually mild 
      and commonly related to supraventricular arrhythmias, these are not associated 
      with the long-term outcome. The severity of the morbid anatomy was the main 
      determinant of survival only in extreme cases, but not in those with mild or 
      moderate deformations, which are more common in adults. Other independent risk 
      factors such as cardiothoracic ratio, sex, age at diagnosis, and the 
      echocardiographic evaluation may help to determine the therapeutic approach. 
      Adult patients with Ebstein anomaly should not be considered as a simple low-risk 
      group.
FAU - Attie, F
AU  - Attie F
AD  - Instituto Nacional de Cardiologia Ignacio Chavez, Mexico D.F., Mexico.
FAU - Rosas, M
AU  - Rosas M
FAU - Rijlaarsdam, M
AU  - Rijlaarsdam M
FAU - Buendia, A
AU  - Buendia A
FAU - Zabal, C
AU  - Zabal C
FAU - Kuri, J
AU  - Kuri J
FAU - Granados, N
AU  - Granados N
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
SB  - IM
MH  - Adult
MH  - Age Distribution
MH  - Age of Onset
MH  - Cohort Studies
MH  - Ebstein Anomaly/*diagnostic imaging/mortality/pathology
MH  - Echocardiography/methods/statistics & numerical data
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Male
MH  - Prognosis
MH  - Risk Factors
MH  - Severity of Illness Index
MH  - Time Factors
MH  - Treatment Outcome
EDAT- 2000/02/12 09:00
MHDA- 2000/02/26 09:00
CRDT- 2000/02/12 09:00
PHST- 2000/02/12 09:00 [pubmed]
PHST- 2000/02/26 09:00 [medline]
PHST- 2000/02/12 09:00 [entrez]
AID - 10.1097/00005792-200001000-00003 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2000 Jan;79(1):27-36. doi: 
      10.1097/00005792-200001000-00003.