PMID- 25149048
OWN - NLM
STAT- MEDLINE
DCOM- 20150127
LR  - 20141202
IS  - 1552-6259 (Electronic)
IS  - 0003-4975 (Linking)
VI  - 98
IP  - 5
DP  - 2014 Nov
TI  - More than 25 years of experience in managing pulmonary atresia with intact 
      ventricular septum.
PG  - 1680-6
LID - S0003-4975(14)01287-9 [pii]
LID - 10.1016/j.athoracsur.2014.05.085 [doi]
AB  - BACKGROUND: Management of pulmonary atresia with intact ventricular septum 
      (PAIVS) is challenging and depends on the individual patient's morphologic 
      characteristics. We evaluated outcomes of 25 years of experience in 
      morphologically driven management of PAIVS. METHODS: Between January 1985 and 
      December 2011, 60 patients were treated for PAIVS. Patients were retrospectively 
      categorized into mild (n=27), moderate (n=18), and severe (n=15) right 
      ventricular (RV) hypoplasia subgroups based on morphologic characteristics at 
      first presentation. Tricuspid valve (TV) annulus z score increases were analyzed 
      in patients who underwent biventricular repair and in patients who underwent 
      1.5-ventricular repair. Median follow-up time was 9.8 years (range, 0-25 years). 
      One patient was lost to follow-up. RESULTS: Fifty-nine (98.3%) patients underwent 
      operation. There were 7 early postprocedure deaths and 1 late death. Estimated 
      overall survival was 86.5% at 10 years after initial admission for the total 
      group, and 96.3%, 77.8%, and 79.4% for the subgroups, respectively. In the 
      respective subgroups, biventricular repair was achieved in 24 (88.9%), 3 (16.7%), 
      and 0 patients. In the patients who underwent biventricular repair, there was a 
      significant increase of the TV annulus z score. At latest follow-up, mean New 
      York Heart Association (NYHA) class was 1.3 for the total group and 1.1, 1.2, and 
      1.5 for the patients who underwent biventricular, 1.5-ventricular, and 
      univentricular repair, respectively. CONCLUSIONS: Our individualized management 
      approach to PAIVS showed good early and long-term results. Early decision on the 
      management goal, determined by individual morphologic characteristics at 
      presentation, is of paramount importance. In specific patients, however, taking 
      into account RV development at a later point in time may improve outcome.
CI  - Copyright (c) 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All 
      rights reserved.
FAU - Schneider, Adriaan W
AU  - Schneider AW
AD  - Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, 
      The Netherlands.
FAU - Blom, Nico A
AU  - Blom NA
AD  - Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, The 
      Netherlands.
FAU - Bruggemans, Eline F
AU  - Bruggemans EF
AD  - Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, 
      The Netherlands.
FAU - Hazekamp, Mark G
AU  - Hazekamp MG
AD  - Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, 
      The Netherlands. Electronic address: m.g.hazekamp@lumc.nl.
LA  - eng
PT  - Journal Article
DEP - 20140819
PL  - Netherlands
TA  - Ann Thorac Surg
JT  - The Annals of thoracic surgery
JID - 15030100R
RN  - Pulmonary Atresia with Intact Ventricular Septum
SB  - IM
MH  - Cardiac Surgical Procedures/*methods
MH  - Female
MH  - Follow-Up Studies
MH  - *Forecasting
MH  - Heart Defects, Congenital/mortality/*surgery
MH  - Humans
MH  - Infant
MH  - Infant, Newborn
MH  - Male
MH  - Netherlands/epidemiology
MH  - Pulmonary Atresia/mortality/*surgery
MH  - Retrospective Studies
MH  - Survival Rate/trends
MH  - Treatment Outcome
EDAT- 2014/08/26 06:00
MHDA- 2015/01/28 06:00
CRDT- 2014/08/24 06:00
PHST- 2013/09/23 00:00 [received]
PHST- 2014/05/20 00:00 [revised]
PHST- 2014/05/27 00:00 [accepted]
PHST- 2014/08/24 06:00 [entrez]
PHST- 2014/08/26 06:00 [pubmed]
PHST- 2015/01/28 06:00 [medline]
AID - S0003-4975(14)01287-9 [pii]
AID - 10.1016/j.athoracsur.2014.05.085 [doi]
PST - ppublish
SO  - Ann Thorac Surg. 2014 Nov;98(5):1680-6. doi: 10.1016/j.athoracsur.2014.05.085. 
      Epub 2014 Aug 19.