PMID- 18715460
OWN - NLM
STAT- MEDLINE
DCOM- 20081028
LR  - 20110505
IS  - 1747-0803 (Electronic)
IS  - 1747-079X (Linking)
VI  - 3
IP  - 4
DP  - 2008 Jul-Aug
TI  - Protein-losing enteropathy after the Fontan operation: associations and 
      predictors of clinical outcome.
PG  - 262-8
LID - 10.1111/j.1747-0803.2008.00200.x [doi]
AB  - OBJECTIVE: We sought to define what clinical parameters were related to the 
      ultimate outcome in Fontan patients who had developed protein-losing enteropathy 
      (PLE). BACKGROUND: PLE is a serious complication of the Fontan operation. Several 
      preoperative and perioperative findings are associated with later PLE. However, 
      there is limited information regarding postoperative abnormalities contributing 
      to or influencing outcome in PLE. METHODS: We evaluated 44 consecutive Fontan 
      patients with PLE. A matched control group of Fontan patients without PLE was 
      used for comparison (matched-pair analyses). Kaplan-Meier and Cox proportional 
      hazard methods were used for survival analyses. RESULTS: Median age was 18 years 
      (range 4-48 years). Short-axis and apical fractional area change (Delta FA) were 
      less in PLE patients than in controls (50 vs. 57% [P < .0001] and 49 vs. 54% [P = 
      .01]). Five and 10-year actuarial survival rates for the PLE group were 49 +/- 9% 
      and 30 +/- 11%. Deceleration time <120 milliseconds (hazard ratio [HR] = 9.2, P = 
      .04), New York Heart Association classification III or IV (HR = 4.0, P = .01), 
      and lower serum albumin (HR = 0.30, P = .04) were independent predictors of 
      mortality in those with PLE. CONCLUSION: Mild reduction in Delta FA was the only 
      late echocardiographic finding associated with development of PLE. However, short 
      deceleration time, poor New York Heart Association (NYHA) class, and low serum 
      albumin identify a group of patients at the greatest risk for death. Presence of 
      these findings in a PLE patient should lead to aggressive management strategies 
      and may warrant early consideration of transplantation.
FAU - Silvilairat, Suchaya
AU  - Silvilairat S
AD  - Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN 55905, USA.
FAU - Cabalka, Allison K
AU  - Cabalka AK
FAU - Cetta, Frank
AU  - Cetta F
FAU - Grogan, Martha
AU  - Grogan M
FAU - Hagler, Donald J
AU  - Hagler DJ
FAU - O'Leary, Patrick W
AU  - O'Leary PW
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Congenit Heart Dis
JT  - Congenital heart disease
JID - 101256510
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Child, Preschool
MH  - Coronary Circulation
MH  - Echocardiography, Doppler, Pulsed
MH  - Female
MH  - *Fontan Procedure
MH  - Humans
MH  - Kaplan-Meier Estimate
MH  - Male
MH  - Middle Aged
MH  - Postoperative Complications
MH  - Proportional Hazards Models
MH  - Protein-Losing Enteropathies/diagnosis/*etiology/therapy
MH  - Retrospective Studies
MH  - Treatment Outcome
EDAT- 2008/08/22 09:00
MHDA- 2008/10/29 09:00
CRDT- 2008/08/22 09:00
PHST- 2008/08/22 09:00 [pubmed]
PHST- 2008/10/29 09:00 [medline]
PHST- 2008/08/22 09:00 [entrez]
AID - CHD200 [pii]
AID - 10.1111/j.1747-0803.2008.00200.x [doi]
PST - ppublish
SO  - Congenit Heart Dis. 2008 Jul-Aug;3(4):262-8. doi: 
      10.1111/j.1747-0803.2008.00200.x.