Cargando…

Long-term outcomes of transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension

BACKGROUND: Transcatheter Potts shunt (TPS) is a palliation alternative for children with severe pulmonary arterial hypertension (PAH). Debates on the long-term outcomes remain unsolved. OBJECTIVES: To evaluate long-term clinical and procedural outcomes of TPS intervention. METHODS: Single-center re...

Descripción completa

Detalles Bibliográficos
Autores principales: Haddad, Raymond N., Levy, Maryline, Szezepanski, Isabelle, Malekzadeh-Milani, Sophie, Bonnet, Damien
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9645092/
https://www.ncbi.nlm.nih.gov/pubmed/36386337
http://dx.doi.org/10.3389/fcvm.2022.1028304
_version_ 1784826891964776448
author Haddad, Raymond N.
Levy, Maryline
Szezepanski, Isabelle
Malekzadeh-Milani, Sophie
Bonnet, Damien
author_facet Haddad, Raymond N.
Levy, Maryline
Szezepanski, Isabelle
Malekzadeh-Milani, Sophie
Bonnet, Damien
author_sort Haddad, Raymond N.
collection PubMed
description BACKGROUND: Transcatheter Potts shunt (TPS) is a palliation alternative for children with severe pulmonary arterial hypertension (PAH). Debates on the long-term outcomes remain unsolved. OBJECTIVES: To evaluate long-term clinical and procedural outcomes of TPS intervention. METHODS: Single-center retrospective data review of children with severe PAH who had TPS between 2009 and 2018. Patients who died per-operatively and early post-procedure were excluded. Long-term outcomes of survivors were evaluated. RESULTS: Out of 13 identified patients (53.8% males), 7 had endovascular stenting of probe/patent arterial ducts, while 6 individuals had aorta-to-pulmonary radiofrequency perforation and covered stent placement. Compared to baseline, the overall clinical condition significantly improved at discharge (p < 0.001) and stayed better at the last visit (p < 0.05) despite frequent clinical worsening events across follow-up. Improvement in functional class across follow-up was significant (p < 0.001). There was, however, no significant improvement in other disease markers (TPASE, 6MWD z-scores, and NT-proBNP levels) or reduction in PAH medications. The median follow-up was 77.4 months (IQR, 70.7–113.4). Survival was 100% at 1 year and 92.3% at 6 years. Freedom from reinterventions was 77% at 1 year and 21% at 6 years. Nine (69.2%) patients had stent reinterventions at a median of 25 months (IQR, 9.5–56) postoperative. Balloon dilatation and restenting were performed in 53.8% and 46.2% of patients, respectively. High-pressure post-dilatation of implanted stents was performed in 53.8% of patients during TPS intervention for incomplete stent expansion and/or residual pressure gradient and was associated with higher rates of reinterventions (p = 0.021). Stent malfunctioning was present in 46.2% of patients at last follow-up. Two patients are listed for heart-lung transplantation. CONCLUSION: Survivors of TPS procedures experience significant improvement in functional class that can be durable. Clinical worsening and stent malfunctioning are frequent morbid events indicating recurrent transcatheter reinterventions throughout follow-up. Six-year survival is, however, satisfactory.
format Online
Article
Text
id pubmed-9645092
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Frontiers Media S.A.
record_format MEDLINE/PubMed
spelling pubmed-96450922022-11-15 Long-term outcomes of transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension Haddad, Raymond N. Levy, Maryline Szezepanski, Isabelle Malekzadeh-Milani, Sophie Bonnet, Damien Front Cardiovasc Med Cardiovascular Medicine BACKGROUND: Transcatheter Potts shunt (TPS) is a palliation alternative for children with severe pulmonary arterial hypertension (PAH). Debates on the long-term outcomes remain unsolved. OBJECTIVES: To evaluate long-term clinical and procedural outcomes of TPS intervention. METHODS: Single-center retrospective data review of children with severe PAH who had TPS between 2009 and 2018. Patients who died per-operatively and early post-procedure were excluded. Long-term outcomes of survivors were evaluated. RESULTS: Out of 13 identified patients (53.8% males), 7 had endovascular stenting of probe/patent arterial ducts, while 6 individuals had aorta-to-pulmonary radiofrequency perforation and covered stent placement. Compared to baseline, the overall clinical condition significantly improved at discharge (p < 0.001) and stayed better at the last visit (p < 0.05) despite frequent clinical worsening events across follow-up. Improvement in functional class across follow-up was significant (p < 0.001). There was, however, no significant improvement in other disease markers (TPASE, 6MWD z-scores, and NT-proBNP levels) or reduction in PAH medications. The median follow-up was 77.4 months (IQR, 70.7–113.4). Survival was 100% at 1 year and 92.3% at 6 years. Freedom from reinterventions was 77% at 1 year and 21% at 6 years. Nine (69.2%) patients had stent reinterventions at a median of 25 months (IQR, 9.5–56) postoperative. Balloon dilatation and restenting were performed in 53.8% and 46.2% of patients, respectively. High-pressure post-dilatation of implanted stents was performed in 53.8% of patients during TPS intervention for incomplete stent expansion and/or residual pressure gradient and was associated with higher rates of reinterventions (p = 0.021). Stent malfunctioning was present in 46.2% of patients at last follow-up. Two patients are listed for heart-lung transplantation. CONCLUSION: Survivors of TPS procedures experience significant improvement in functional class that can be durable. Clinical worsening and stent malfunctioning are frequent morbid events indicating recurrent transcatheter reinterventions throughout follow-up. Six-year survival is, however, satisfactory. Frontiers Media S.A. 2022-10-26 /pmc/articles/PMC9645092/ /pubmed/36386337 http://dx.doi.org/10.3389/fcvm.2022.1028304 Text en Copyright © 2022 Haddad, Levy, Szezepanski, Malekzadeh-Milani and Bonnet. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Cardiovascular Medicine
Haddad, Raymond N.
Levy, Maryline
Szezepanski, Isabelle
Malekzadeh-Milani, Sophie
Bonnet, Damien
Long-term outcomes of transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension
title Long-term outcomes of transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension
title_full Long-term outcomes of transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension
title_fullStr Long-term outcomes of transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension
title_full_unstemmed Long-term outcomes of transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension
title_short Long-term outcomes of transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension
title_sort long-term outcomes of transcatheter potts shunt in children with suprasystemic pulmonary arterial hypertension
topic Cardiovascular Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9645092/
https://www.ncbi.nlm.nih.gov/pubmed/36386337
http://dx.doi.org/10.3389/fcvm.2022.1028304
work_keys_str_mv AT haddadraymondn longtermoutcomesoftranscatheterpottsshuntinchildrenwithsuprasystemicpulmonaryarterialhypertension
AT levymaryline longtermoutcomesoftranscatheterpottsshuntinchildrenwithsuprasystemicpulmonaryarterialhypertension
AT szezepanskiisabelle longtermoutcomesoftranscatheterpottsshuntinchildrenwithsuprasystemicpulmonaryarterialhypertension
AT malekzadehmilanisophie longtermoutcomesoftranscatheterpottsshuntinchildrenwithsuprasystemicpulmonaryarterialhypertension
AT bonnetdamien longtermoutcomesoftranscatheterpottsshuntinchildrenwithsuprasystemicpulmonaryarterialhypertension