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Pulmonary Atresia with Intact Ventricular Septum, a National Comparison Between Interventional and Surgical Approach, in Combination with a Systemic Literature Review

Manhem, Stina ; Odermarsky, Michal LU orcid ; Wåhlander, Håkan and Ekman-Joelsson, Britt-Marie (2024) In Pediatric Cardiology
Abstract
This study aimed to compare long-term morbidity in patients with pulmonary atresia with intact ventricular septum (PA-IVS)
treated with catheter-based intervention (group A) versus those undergoing heart surgery (group B) as initial intervention.
Additionally, we conducted a systematic literature review on PA-IVS treatment. All neonates born in Sweden with PA-IVS
between 2007 and 2019 were screened for inclusion. The inclusion criterion was decompression of the right ventricle for
initial intervention. Medical records were reviewed, as well as the initial preoperative angiogram, and the diagnostic echocar-
diogram. Comparisons between groups were performed with Mann–Whitney U-test and Fisher´s exact test. A... (More)
This study aimed to compare long-term morbidity in patients with pulmonary atresia with intact ventricular septum (PA-IVS)
treated with catheter-based intervention (group A) versus those undergoing heart surgery (group B) as initial intervention.
Additionally, we conducted a systematic literature review on PA-IVS treatment. All neonates born in Sweden with PA-IVS
between 2007 and 2019 were screened for inclusion. The inclusion criterion was decompression of the right ventricle for
initial intervention. Medical records were reviewed, as well as the initial preoperative angiogram, and the diagnostic echocar-
diogram. Comparisons between groups were performed with Mann–Whitney U-test and Fisher´s exact test. A systematic
literature review of original studies regarding treatment of PA-IVS (2002 and onward) was conducted following the Preferred
Reporting Items for Systematic Reviews and Meta-Analyses guidelines, to assess the outcomes of patients with PA-IVS. 34
(11 females) patients were included, 18 in group A and 16 in group B. There was no mortality in either group. Follow-up
time ranged from 2 to 15 years (median 9). All attempted perforations in group A were successful, and 16 out of 18 patients
reached biventricular circulation. In the surgical group 15 out of 16 patients reached biventricular circulation. The literature
review presented heterogeneity in standards for treatment. This retrospective population-based multicenter study demonstrates
that both catheter-based intervention and heart surgery are safe procedures. Our results are comparable to, or exceed, those
in the systematic literature review. The systematic literature review displays a great heterogeneity in study design, with no
definitive golden standard treatment. (Less)
Please use this url to cite or link to this publication:
author
; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Pulmonary atresia with intact ventricular septum, Valve lesions, Catheter-based valve perforation, surgical valvulotomy
in
Pediatric Cardiology
pages
14 pages
publisher
Springer
external identifiers
  • pmid:38967792
ISSN
0172-0643
DOI
10.1007/s00246-024-03566-x
language
English
LU publication?
yes
id
bf5bdfff-eeaf-4298-ac8a-92dd8dd1e6c4
date added to LUP
2024-07-05 15:18:25
date last changed
2024-07-06 03:00:03
@article{bf5bdfff-eeaf-4298-ac8a-92dd8dd1e6c4,
  abstract     = {{This study aimed to compare long-term morbidity in patients with pulmonary atresia with intact ventricular septum (PA-IVS)<br/>treated with catheter-based intervention (group A) versus those undergoing heart surgery (group B) as initial intervention.<br/>Additionally, we conducted a systematic literature review on PA-IVS treatment. All neonates born in Sweden with PA-IVS<br/>between 2007 and 2019 were screened for inclusion. The inclusion criterion was decompression of the right ventricle for<br/>initial intervention. Medical records were reviewed, as well as the initial preoperative angiogram, and the diagnostic echocar-<br/>diogram. Comparisons between groups were performed with Mann–Whitney U-test and Fisher´s exact test. A systematic<br/>literature review of original studies regarding treatment of PA-IVS (2002 and onward) was conducted following the Preferred<br/>Reporting Items for Systematic Reviews and Meta-Analyses guidelines, to assess the outcomes of patients with PA-IVS. 34<br/>(11 females) patients were included, 18 in group A and 16 in group B. There was no mortality in either group. Follow-up<br/>time ranged from 2 to 15 years (median 9). All attempted perforations in group A were successful, and 16 out of 18 patients<br/>reached biventricular circulation. In the surgical group 15 out of 16 patients reached biventricular circulation. The literature<br/>review presented heterogeneity in standards for treatment. This retrospective population-based multicenter study demonstrates<br/>that both catheter-based intervention and heart surgery are safe procedures. Our results are comparable to, or exceed, those<br/>in the systematic literature review. The systematic literature review displays a great heterogeneity in study design, with no<br/>definitive golden standard treatment.}},
  author       = {{Manhem, Stina and Odermarsky, Michal and Wåhlander, Håkan and Ekman-Joelsson, Britt-Marie}},
  issn         = {{0172-0643}},
  keywords     = {{Pulmonary atresia with intact ventricular septum; Valve lesions; Catheter-based valve perforation; surgical valvulotomy}},
  language     = {{eng}},
  month        = {{07}},
  publisher    = {{Springer}},
  series       = {{Pediatric Cardiology}},
  title        = {{Pulmonary Atresia with Intact Ventricular Septum, a National Comparison Between Interventional and Surgical Approach, in Combination with a Systemic Literature Review}},
  url          = {{http://dx.doi.org/10.1007/s00246-024-03566-x}},
  doi          = {{10.1007/s00246-024-03566-x}},
  year         = {{2024}},
}