PMID- 27843561
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220408
IS  - 2053-3624 (Print)
IS  - 2053-3624 (Electronic)
IS  - 2053-3624 (Linking)
VI  - 3
IP  - 2
DP  - 2016
TI  - Predictors of poor outcome among children with heterotaxy syndrome: a 
      retrospective review.
PG  - e000328
LID - e000328
AB  - 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.
FAU - McGovern, Eimear
AU  - McGovern E
AD  - Department of Paediatric Cardiology , Our Lady's Children's Hospital , Crumlin, 
      Dublin , Ireland.
FAU - Kelleher, Eoin
AU  - Kelleher E
AD  - Department of Paediatric Cardiology , Our Lady's Children's Hospital , Crumlin, 
      Dublin , Ireland.
FAU - Potts, James E
AU  - Potts JE
AD  - Department of Cardiology , British Columbia Children's Hospital , Vancouver, 
      British Columbia , Canada.
FAU - O'Brien, John
AU  - O'Brien J
AD  - StatisticaMedica Ltd , Dublin , Ireland.
FAU - Walsh, Kevin
AU  - Walsh K
AD  - Department of Paediatric Cardiology , Our Lady's Children's Hospital , Crumlin, 
      Dublin , Ireland.
FAU - Nolke, Lars
AU  - Nolke L
AD  - Department of Paediatric Cardiology , Our Lady's Children's Hospital , Crumlin, 
      Dublin , Ireland.
FAU - McMahon, Colin J
AU  - McMahon CJ
AD  - Department of Paediatric Cardiology , Our Lady's Children's Hospital , Crumlin, 
      Dublin , Ireland.
LA  - eng
PT  - Journal Article
DEP - 20161011
PL  - England
TA  - Open Heart
JT  - Open heart
JID - 101631219
PMC - PMC5073560
OTO - NOTNLM
OT  - CONGENITAL HEART DISEASE
OT  - Heterotaxy
OT  - Left atrial isomerism
OT  - Right atrial isomerism
OT  - Survival
COIS- Conflicts of Interest: None declared.
EDAT- 2016/11/16 06:00
MHDA- 2016/11/16 06:01
PMCR- 2016/10/11
CRDT- 2016/11/16 06:00
PHST- 2015/08/15 00:00 [received]
PHST- 2016/02/23 00:00 [revised]
PHST- 2016/03/29 00:00 [accepted]
PHST- 2016/11/16 06:00 [entrez]
PHST- 2016/11/16 06:00 [pubmed]
PHST- 2016/11/16 06:01 [medline]
PHST- 2016/10/11 00:00 [pmc-release]
AID - openhrt-2015-000328 [pii]
AID - 10.1136/openhrt-2015-000328 [doi]
PST - epublish
SO  - Open Heart. 2016 Oct 11;3(2):e000328. doi: 10.1136/openhrt-2015-000328. 
      eCollection 2016.