Creation of a restrictive atrial communication in pulmonary arterial hypertension (PAH): effective palliation of syncope and end-stage heart failure

Atrial septostomy (AS) is recommended for pulmonary arterial hypertension (PAH)-associated right ventricular (RV) failure, recurrent syncope, or pulmonary hypertensive crisis (PHC). We aimed to evaluate the feasibility and efficacy of AS to manage PAH from infancy to adulthood. From June 2009 to Dec...

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Main Authors: Anna Bauer, Markus Khalil, Dorle Schmidt, Jürgen Bauer, Anoosh Esmaeili, Christian Apitz, Norbert F. Voelkel, Dietmar Schranz
Format: Article
Language:English
Published: SAGE Publishing 2018-06-01
Series:Pulmonary Circulation
Online Access:https://doi.org/10.1177/2045894018776518
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spelling doaj-d3f9d9001ad34a60ba22b3cae6073bde2020-11-25T03:46:29ZengSAGE PublishingPulmonary Circulation2045-89402018-06-01810.1177/2045894018776518Creation of a restrictive atrial communication in pulmonary arterial hypertension (PAH): effective palliation of syncope and end-stage heart failureAnna Bauer0Markus Khalil1Dorle Schmidt2Jürgen Bauer3Anoosh Esmaeili4Christian Apitz5Norbert F. Voelkel6Dietmar Schranz7Justus Liebig University Clinic Giessen, Hessen Pediatric Heart Center, Giessen, GermanyJustus Liebig University Clinic Giessen, Hessen Pediatric Heart Center, Giessen, GermanyJustus Liebig University Clinic Giessen, Hessen Pediatric Heart Center, Giessen, GermanyJustus Liebig University Clinic Giessen, Hessen Pediatric Heart Center, Giessen, GermanyJohann-Wolfgang Goethe University Clinic, Frankfurt, GermanyDivision of Pediatric Cardiology, University Children’s Hospital, Ulm, GermanyFree University Medical Center (VUMC), Amsterdam, The NetherlandsJohann-Wolfgang Goethe University Clinic, Frankfurt, GermanyAtrial septostomy (AS) is recommended for pulmonary arterial hypertension (PAH)-associated right ventricular (RV) failure, recurrent syncope, or pulmonary hypertensive crisis (PHC). We aimed to evaluate the feasibility and efficacy of AS to manage PAH from infancy to adulthood. From June 2009 to December 2016, transcatheter atrial communications were created in 11 PAH patients (4 girls/women; median age = 4.3 years; range = 33 days–26 years; median body weight = 14 kg; range = 3–71 kg; NYHA-/Ross class IV; n = 11). PAH was classified as idiopathic (n = 6) or secondary (n = 5). History of syncope was dominant (n = 6); two with patent foramen ovale (PFO) admitted with recurrent PHC, three patients required resuscitation before AS. Three patients had PAH-associated low cardiac output. The average pulmonary arterial pressures (PAP systolic/diastolic) were 101/50 (±34/23); the corresponding systemic arterial pressures (SAP) were 99/54 (±23/11); and the mean ratio of PAPd / SAPd was 0.97 (±0.4). Percutaneous trans-septal puncture was uneventfully performed in nine patients; a PFO was dilated in two patients. There was no procedure-related mortality. The median balloon size was 10 mm (range = 6–14 mm); the mean catheter time was 174.6 ± 48 min; fluoroscopy time was 19.8 (±11) min. Syncope and PHC were successfully treated in all patients. The mean arterial oxygen saturation decreased from 97 ± 2 to 89 ± 11.7. One patient died awaiting lung transplantation, one continues to be listed; two patients received a reverse Potts-shunt, one patient died during follow-up; seven patients are stable with PAH-specific treatment. Percutaneous AS is an effective method palliating PAH-associated syncope, PHCs or right (bi-) ventricular heart failure.https://doi.org/10.1177/2045894018776518
collection DOAJ
language English
format Article
sources DOAJ
author Anna Bauer
Markus Khalil
Dorle Schmidt
Jürgen Bauer
Anoosh Esmaeili
Christian Apitz
Norbert F. Voelkel
Dietmar Schranz
spellingShingle Anna Bauer
Markus Khalil
Dorle Schmidt
Jürgen Bauer
Anoosh Esmaeili
Christian Apitz
Norbert F. Voelkel
Dietmar Schranz
Creation of a restrictive atrial communication in pulmonary arterial hypertension (PAH): effective palliation of syncope and end-stage heart failure
Pulmonary Circulation
author_facet Anna Bauer
Markus Khalil
Dorle Schmidt
Jürgen Bauer
Anoosh Esmaeili
Christian Apitz
Norbert F. Voelkel
Dietmar Schranz
author_sort Anna Bauer
title Creation of a restrictive atrial communication in pulmonary arterial hypertension (PAH): effective palliation of syncope and end-stage heart failure
title_short Creation of a restrictive atrial communication in pulmonary arterial hypertension (PAH): effective palliation of syncope and end-stage heart failure
title_full Creation of a restrictive atrial communication in pulmonary arterial hypertension (PAH): effective palliation of syncope and end-stage heart failure
title_fullStr Creation of a restrictive atrial communication in pulmonary arterial hypertension (PAH): effective palliation of syncope and end-stage heart failure
title_full_unstemmed Creation of a restrictive atrial communication in pulmonary arterial hypertension (PAH): effective palliation of syncope and end-stage heart failure
title_sort creation of a restrictive atrial communication in pulmonary arterial hypertension (pah): effective palliation of syncope and end-stage heart failure
publisher SAGE Publishing
series Pulmonary Circulation
issn 2045-8940
publishDate 2018-06-01
description Atrial septostomy (AS) is recommended for pulmonary arterial hypertension (PAH)-associated right ventricular (RV) failure, recurrent syncope, or pulmonary hypertensive crisis (PHC). We aimed to evaluate the feasibility and efficacy of AS to manage PAH from infancy to adulthood. From June 2009 to December 2016, transcatheter atrial communications were created in 11 PAH patients (4 girls/women; median age = 4.3 years; range = 33 days–26 years; median body weight = 14 kg; range = 3–71 kg; NYHA-/Ross class IV; n = 11). PAH was classified as idiopathic (n = 6) or secondary (n = 5). History of syncope was dominant (n = 6); two with patent foramen ovale (PFO) admitted with recurrent PHC, three patients required resuscitation before AS. Three patients had PAH-associated low cardiac output. The average pulmonary arterial pressures (PAP systolic/diastolic) were 101/50 (±34/23); the corresponding systemic arterial pressures (SAP) were 99/54 (±23/11); and the mean ratio of PAPd / SAPd was 0.97 (±0.4). Percutaneous trans-septal puncture was uneventfully performed in nine patients; a PFO was dilated in two patients. There was no procedure-related mortality. The median balloon size was 10 mm (range = 6–14 mm); the mean catheter time was 174.6 ± 48 min; fluoroscopy time was 19.8 (±11) min. Syncope and PHC were successfully treated in all patients. The mean arterial oxygen saturation decreased from 97 ± 2 to 89 ± 11.7. One patient died awaiting lung transplantation, one continues to be listed; two patients received a reverse Potts-shunt, one patient died during follow-up; seven patients are stable with PAH-specific treatment. Percutaneous AS is an effective method palliating PAH-associated syncope, PHCs or right (bi-) ventricular heart failure.
url https://doi.org/10.1177/2045894018776518
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