Advanced

Total Anomalous Pulmonary Venous Connection Morphology and Outcome From an International Population-Based Study

Seale, Anna N.; Uemura, Hideki; Webber, Steven A.; Partridge, John; Roughton, Michael; Ho, Siew Y.; McCarthy, Karen P.; Jones, Sheila; Shaughnessy, Lynda and Sunnegardh, Jan, et al. (2010) In Circulation 122(25). p.237-2718
Abstract
Background-Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results-We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422... (More)
Background-Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results-We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions-Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival. (Circulation. 2010;122:2718-2726.) (Less)
Please use this url to cite or link to this publication:
author
, et al. (More)
(Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
pulmonary vein stenosis, congenital, defects, heart, pulmonary veins, follow-up studies
in
Circulation
volume
122
issue
25
pages
237 - 2718
publisher
Lippincott Williams and Wilkins
external identifiers
  • wos:000285454500017
  • scopus:78650682143
ISSN
1524-4539
DOI
10.1161/CIRCULATIONAHA.110.940825
language
English
LU publication?
yes
id
8a76c404-f8e3-44bc-876a-b61e1863b91d (old id 1814812)
date added to LUP
2011-03-02 13:31:27
date last changed
2018-07-15 03:46:16
@article{8a76c404-f8e3-44bc-876a-b61e1863b91d,
  abstract     = {Background-Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results-We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions-Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival. (Circulation. 2010;122:2718-2726.)},
  author       = {Seale, Anna N. and Uemura, Hideki and Webber, Steven A. and Partridge, John and Roughton, Michael and Ho, Siew Y. and McCarthy, Karen P. and Jones, Sheila and Shaughnessy, Lynda and Sunnegardh, Jan and Hanséus, Katarina and Berggren, Hakan and Johansson, Sune and Rigby, Michael L. and Keeton, Barry R. and Daubeney, Piers E. F.},
  issn         = {1524-4539},
  keyword      = {pulmonary vein stenosis,congenital,defects,heart,pulmonary veins,follow-up studies},
  language     = {eng},
  number       = {25},
  pages        = {237--2718},
  publisher    = {Lippincott Williams and Wilkins},
  series       = {Circulation},
  title        = {Total Anomalous Pulmonary Venous Connection Morphology and Outcome From an International Population-Based Study},
  url          = {http://dx.doi.org/10.1161/CIRCULATIONAHA.110.940825},
  volume       = {122},
  year         = {2010},
}