We have located links that may give you full text access.
Predictors of poor outcome among children with heterotaxy syndrome: a retrospective review.
Open Heart 2016
OBJECTIVE: To determine predictors of poor outcome in patients with heterotaxy syndrome.
METHODS: A retrospective review of children with heterotaxy syndrome, in a single tertiary paediatric cardiology centre, was conducted between 1 January 1997 and 1 January 2014 to determine predictors of poor outcome. Poor outcome was defined as death, cardiac transplantation or New York Heart Association (NYHA) functional class III or IV.
RESULTS: There were 35 patients diagnosed with heterotaxy syndrome, 17 of whom were diagnosed antenatally. 22 patients had right atrial isomerism and 13 had left atrial isomerism. The median age of postnatal diagnosis was 2.5 days old (1 day to 19 months). 12 patients had a poor outcome; 6 patients died, 1 underwent cardiac transplantation and 5 had an NYHA functional class of >III. 5 patients had a biventricular repair and the remaining 30 had a univentricular repair. Type of atrial isomerism, univentricular or biventricular anatomy, severity of atrioventricular valve regurgitation or ventricular dysfunction, obstructed pulmonary venous return, occurrence of arrhythmia and presence of pulmonary atresia did not predict poor outcome. Fetal diagnosis also did not confer a survival advantage. The median duration of follow-up in this cohort was 65 months (2 days to 16.8 years).
CONCLUSIONS: Survival for patients with heterotaxy syndrome was 83% over a median follow-up of 65 months. 34% of patients had a poor outcome. None of the variables studied were predictive of death, transplantation or NYHA classification III or IV.
METHODS: A retrospective review of children with heterotaxy syndrome, in a single tertiary paediatric cardiology centre, was conducted between 1 January 1997 and 1 January 2014 to determine predictors of poor outcome. Poor outcome was defined as death, cardiac transplantation or New York Heart Association (NYHA) functional class III or IV.
RESULTS: There were 35 patients diagnosed with heterotaxy syndrome, 17 of whom were diagnosed antenatally. 22 patients had right atrial isomerism and 13 had left atrial isomerism. The median age of postnatal diagnosis was 2.5 days old (1 day to 19 months). 12 patients had a poor outcome; 6 patients died, 1 underwent cardiac transplantation and 5 had an NYHA functional class of >III. 5 patients had a biventricular repair and the remaining 30 had a univentricular repair. Type of atrial isomerism, univentricular or biventricular anatomy, severity of atrioventricular valve regurgitation or ventricular dysfunction, obstructed pulmonary venous return, occurrence of arrhythmia and presence of pulmonary atresia did not predict poor outcome. Fetal diagnosis also did not confer a survival advantage. The median duration of follow-up in this cohort was 65 months (2 days to 16.8 years).
CONCLUSIONS: Survival for patients with heterotaxy syndrome was 83% over a median follow-up of 65 months. 34% of patients had a poor outcome. None of the variables studied were predictive of death, transplantation or NYHA classification III or IV.
Full text links
Related Resources
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app
All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.
By using this service, you agree to our terms of use and privacy policy.
Your Privacy Choices
You can now claim free CME credits for this literature searchClaim now
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app