PMID- 29288192
OWN - NLM
STAT- MEDLINE
DCOM- 20190212
LR  - 20191210
IS  - 1468-201X (Electronic)
IS  - 1355-6037 (Linking)
VI  - 104
IP  - 17
DP  - 2018 Sep
TI  - Symptomatic partial and transitional atrioventricular septal defect repaired in 
      infancy.
PG  - 1411-1416
LID - 10.1136/heartjnl-2017-312195 [doi]
AB  - OBJECTIVES: Infants with symptomatic partial and transitional atrioventricular 
      septal defect undergoing early surgical repair are thought to be at greater risk. 
      However, the outcome and risk profile of this cohort of patients are poorly 
      defined. The aim of this study was to investigate the outcome of symptomatic 
      infants undergoing early repair and to identify risk factors which may predict 
      mortality and reoperation. METHODS: This multicentre study recruited 51 patients 
      (24 female) in three tertiary centres between 2000 and 2015. The inclusion 
      criteria were as follows: (1) partial and transitional atrioventricular septal 
      defect, (2) heart failure unresponsive to treatment, (3) biventricular repair 
      during the first year of life. RESULTS: Median age at definitive surgery was 179 
      (range 0-357) days. Sixteen patients (31%) had unfavourable anatomy of the left 
      atrioventricular valve: dysplastic (n=7), double orifice (n=3), severely 
      deficient valve leaflets (n=1), hypoplastic left atrioventricular orifice and/or 
      mural leaflet (n=3), short/poorly defined chords (n=2). There were three 
      inhospital deaths (5.9%) after primary repair. Eleven patients (22%) were 
      reoperated at a median interval of 40 days (4 days to 5.1 years) for severe left 
      atrioventricular valve regurgitation and/or stenosis. One patient required 
      mechanical replacement of the left atrioventricular valve. After median follow-up 
      of 3.8 years (0.1-11.4 years), all patients were in New York Heart Association 
      (NYHA) class I. In multivariable analysis, unfavourable anatomy of the left 
      atrioventricular valve was the only risk factor associated with left 
      atrioventricular valve reoperation. CONCLUSIONS: Although surgical repair is 
      successful in the majority of the cases, patients with partial and transitional 
      atrioventricular septal defect undergoing surgical repair during infancy 
      experience significant morbidity and mortality. The reoperation rate is high with 
      unfavourable left atrioventricular valve anatomy.
CI  - (c) Article author(s) (or their employer(s) unless otherwise stated in the text of 
      the article) 2018. All rights reserved. No commercial use is permitted unless 
      otherwise expressly granted.
FAU - Krupickova, Sylvia
AU  - Krupickova S
AD  - Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.
FAU - Morgan, Gareth J
AU  - Morgan GJ
AD  - Department of Paediatric Cardiology, Evelina London Children's Hospital, London, 
      UK.
FAU - Cheang, Mun Hong
AU  - Cheang MH
AD  - UCL Institute of Cardiovascular Science, University College London, London, UK.
FAU - Rigby, Michael L
AU  - Rigby ML
AD  - Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.
FAU - Franklin, Rodney C
AU  - Franklin RC
AD  - Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.
FAU - Battista, Andrea
AU  - Battista A
AD  - Department of Paediatric Cardiology, Great Ormond Street Hospital, London, UK.
FAU - Spanaki, Ariana
AU  - Spanaki A
AD  - Department of Paediatric Cardiology, Evelina London Children's Hospital, London, 
      UK.
FAU - Bonello, Beatrice
AU  - Bonello B
AD  - Department of Paediatric Cardiology, Great Ormond Street Hospital, London, UK.
FAU - Ghez, Olivier
AU  - Ghez O
AD  - Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.
FAU - Anderson, David
AU  - Anderson D
AD  - Department of Paediatric Cardiology, Evelina London Children's Hospital, London, 
      UK.
FAU - Tsang, Victor
AU  - Tsang V
AD  - UCL Institute of Cardiovascular Science, University College London, London, UK.
AD  - Department of Paediatric Cardiology, Great Ormond Street Hospital, London, UK.
FAU - Michielon, Guido
AU  - Michielon G
AD  - Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.
FAU - Marek, Jan
AU  - Marek J
AD  - UCL Institute of Cardiovascular Science, University College London, London, UK.
AD  - Department of Paediatric Cardiology, Great Ormond Street Hospital, London, UK.
FAU - Fraisse, Alain
AU  - Fraisse A
AD  - Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.
LA  - eng
PT  - Journal Article
DEP - 20171228
PL  - England
TA  - Heart
JT  - Heart (British Cardiac Society)
JID - 9602087
RN  - Atrioventricular Septal Defect
SB  - IM
CIN - Heart. 2018 Sep;104(17):1388-1389. PMID: 29472292
CIN - Heart. 2018 Sep;104(17):1470. PMID: 30104396
MH  - *Cardiovascular Surgical Procedures/adverse effects/methods
MH  - Female
MH  - Follow-Up Studies
MH  - Heart Failure/diagnosis/etiology
MH  - Heart Septal Defects/diagnosis/mortality/physiopathology/*surgery
MH  - Humans
MH  - Infant
MH  - Male
MH  - Mitral Valve/abnormalities/physiopathology/*surgery
MH  - Outcome and Process Assessment, Health Care
MH  - *Postoperative Complications/etiology/mortality/surgery
MH  - *Reoperation/methods/mortality
MH  - Risk Factors
MH  - United Kingdom
OTO - NOTNLM
OT  - congenital heart disease
OT  - congenital heart disease surgery
COIS- Competing interests: None declared.
EDAT- 2017/12/31 06:00
MHDA- 2019/02/13 06:00
CRDT- 2017/12/31 06:00
PHST- 2017/07/21 00:00 [received]
PHST- 2017/10/17 00:00 [revised]
PHST- 2017/12/06 00:00 [accepted]
PHST- 2017/12/31 06:00 [pubmed]
PHST- 2019/02/13 06:00 [medline]
PHST- 2017/12/31 06:00 [entrez]
AID - heartjnl-2017-312195 [pii]
AID - 10.1136/heartjnl-2017-312195 [doi]
PST - ppublish
SO  - Heart. 2018 Sep;104(17):1411-1416. doi: 10.1136/heartjnl-2017-312195. Epub 2017 
      Dec 28.