Balloon atrial septostomy is a palliative procedure currently used to bridge medically refractory pulmonary hypertension patients to lung transplantation. In the current report, we present balloon atrial septostomy as an initial therapy for high-risk pediatric pulmonary hypertension patients at our institution. Nineteen patients with median age of 4.3 years (range 0.1-14.3 years) underwent balloon atrial septostomy during initial admission for pulmonary hypertension. There were no procedural complications or deaths within 24 h of balloon atrial septostomy. Patients were followed for a median of 2.6 years (interquartile range 1.0-4.8 years). Three (16%) patients died, 3 (16%) underwent lung transplantation, and 1 (5%) underwent reverse Potts shunt. Transplant-free survival at 30 days, 1 year, and 3 years was 84%, 76%, and 67% respectively. This single-center experience suggests early-BAS in addition to pharmacotherapy is safe and warrants consideration in high-risk pediatric pulmonary hypertension patients

Cash, Michelle

Critser, Paul J.

Evers, Patrick D.

Hirsch, Russel

McGovern, Eimear

English

University of Kentucky

University of Kentucky UKnowledge Pediatrics Faculty Publications Pediatrics 10-1-2020 Balloon Atrial Septostomy as Initial Therapy in Pediatric Pulmonary Hypertension Paul J. Critser Cincinnati Children's Hospital Medical Center Patrick D. Evers Oregon Health and Science University Eimear McGovern University of Kentucky, Eimear.McGovern@uky.edu Michelle Cash Cincinnati Children's Hospital Medical Center Russel Hirsch Cincinnati Children's Hospital Medical Center Follow this and additional works at: https://uknowledge.uky.edu/pediatrics_facpub  Part of the Pediatrics Commons Right click to open a feedback form in a new tab to let us know how this document benefits you. Repository Citation Critser, Paul J.; Evers, Patrick D.; McGovern, Eimear; Cash, Michelle; and Hirsch, Russel, "Balloon Atrial Septostomy as Initial Therapy in Pediatric Pulmonary Hypertension" (2020). Pediatrics Faculty Publications. 308. https://uknowledge.uky.edu/pediatrics_facpub/308 This Article is brought to you for free and open access by the Pediatrics at UKnowledge. It has been accepted for inclusion in Pediatrics Faculty Publications by an authorized administrator of UKnowledge. For more information, please contact UKnowledge@lsv.uky.edu. Balloon Atrial Septostomy as Initial Therapy in Pediatric Pulmonary Hypertension Digital Object Identifier (DOI) https://doi.org/10.1177/2045894020958970 Notes/Citation Information Published in Pulmonary Circulation, v. 10, issue 4. © The Author(s) 2020 This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage). This article is available at UKnowledge: https://uknowledge.uky.edu/pediatrics_facpub/308 Case ReportBalloon atrial septostomy as initial therapy in pediatricpulmonary hypertensionPaul J. Critser1, Patrick D. Evers2, Eimear McGovern3, Michelle Cash1 and Russel Hirsch1,41Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA; 2Division of Pediatric Cardiology, Oregon Health and Science University,Portland, OR, USA; 3Division of Pediatric Cardiology, University of Kentucky College of Medicine, Lexington, KY, USA; 4Department of Pediatrics, University ofCincinnati College of Medicine, Cincinnati, OH, USAAbstractBalloon atrial septostomy is a palliative procedure currently used to bridge medically refractory pulmonary hypertension patientsto lung transplantation. In the current report, we present balloon atrial septostomy as an initial therapy for high-risk pediatricpulmonary hypertension patients at our institution. Nineteen patients with median age of 4.3 years (range 0.1–14.3 years)underwent balloon atrial septostomy during initial admission for pulmonary hypertension. There were no procedural complicationsor deaths within 24 h of balloon atrial septostomy. Patients were followed for a median of 2.6 years (interquartile range 1.0–4.8years). Three (16%) patients died, 3 (16%) underwent lung transplantation, and 1 (5%) underwent reverse Potts shunt. Transplant-free survival at 30 days, 1 year, and 3 years was 84%, 76%, and 67% respectively. This single-center experience suggests early-BAS inaddition to pharmacotherapy is safe and warrants consideration in high-risk pediatric pulmonary hypertension patients.KeywordsPulmonary hypertension, balloon atrial septostomy, pediatricsDate received: 25 June 2020; accepted: 8 August 2020Pulmonary Circulation 2020; 10(4) 1–3DOI: 10.1177/2045894020958970IntroductionSurvival of pediatric pulmonary hypertension (PH) patientshas improved over the last two decades due to new thera-peutic agents. However, pediatric PH remains a progressivedisease with poor prognosis. For patients who fail torespond to medical therapy, current guidelines recommendballoon atrial septostomy (BAS) or reverse Potts shunt as abridge to lung transplantation.1By providing an atrial level shunt, BAS may improve leftventricular preload and cardiac output at the expense ofdecreased systemic saturation. BAS has been associatedwith improved survival in adult patients with PH.2 Tworeports have described BAS in pediatric PH, demonstratingan association with symptomatic improvement.3,4 However,the appropriate timing of BAS remains unclear as severeright ventricular (RV) failure or end stage disease are rela-tive contraindications.1In the current report, we present our institutional experiencewith BAS as an initial therapy for high-risk pediatric PH.CasesBetween 1 January 2011 and 31 December 2019, BAS wasperformed during initial admission for PH in 19 patients.Eleven patients (52%) were female. The median age at pro-cedure was 4.3 years (range 0.1–14.3 years). Eleven patients(58%) had idiopathic pulmonary arterial hypertension,5 (26%) had PH associated with congenital heart disease,2 (10%) had PH after bone marrow transplant, and 1 (5%)had hereditary hemorrhagic telangiectasia. Indications forBAS included history of syncope, PH crisis, or evidence ofRV failure.Preceding the BAS, 18 (95%) patients were admitted toan intensive care unit, 13 (68%) patients were on inotropes,and 6 (32%) patients were treated with inhaled nitric oxide.Corresponding author:Russel Hirsch, Cincinnati Children’s Hospital, 3333 Burnet Avenue, ML 5033,Cincinnati, OH 45229, USA.Email: russel.hirsch@cchmc.orgCreative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the CreativeCommons Attribution-NonCommercial 4.0 License (http://creativecommons.org/licenses/by-nc/4.0/) which per-mits non-commercial use, reproduction and distribution of the work without further permission provided the original work isattributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).! The Author(s) 2020.Article reuse guidelines:sagepub.com/journals-permissionsjournals.sagepub.com/home/pulEleven (58%) patients received pulmonary vasodilator ther-apy with 5 (26%) receiving monotherapy, 4 (21%) receivingdual therapy, and 2 (11%) receiving triple therapy. Eleven(58%) patients received phosphodiesterase-5 inhibitors, 4(21%) patients received an endothelin receptor antagonist,and 4 (21%) patients received prostanoid therapy. Five(24%) had history of syncope, 3 (16%) had experienced aPH crisis, and 11 (58%) had echocardiographic evidence ofRV failure.Fifteen patients underwent BAS at the time of initial car-diac catheterization. The remaining four patients underwentBAS at two, four, six, and eight days after initial cardiaccatheterization. At the time of BAS, the median cardiacindex was 3.1 L/min/m2 (interquartile range (IQR) 2.5–4.3), mean pulmonary artery pressure was 58mmHg (IQR49–72), pulmonary vascular resistance was 16.9 iWU (IQR10.4–22.5), mean right atrial pressure (mRAP) was 8mmHg(IQR 6–13), mean systemic arterial oxygen saturation 96%(IQR 95–96). There were no procedural complications ordeaths within 24 h of BAS. Eleven patients received antic-oagulation after BAS including aspirin in five, enoxaparin inone, clopidogrel in one, and warfarin in five patients.Patients were followed for a median of 2.6 years (IQR1.0–4.8 years). Three patients required a repeat BAS. Atmost recent follow-up, 14 (74%) had patent atrial levelshunt. Of the five patients with intact atrial septum, twohad atrial septal defect closure at time of lung transplant,and three had spontaneous closure at 9, 10, and 15 monthsafter BAS.Three (16%) patients died, 3 (16%) underwent lungtransplantation, and 1 (5%) underwent reverse Pottsshunt. The three patient deaths were within 30 days of theprocedure and were admitted to the intensive care unit priorto BAS. One patient died from sepsis and two patients diedfrom a PH crisis. There were no deaths in patients whosurvived to hospital discharge after BAS.Three (16%) patients underwent lung transplantation at7 months, 1 year, and 2 years after BAS and all are alivewith post-transplant follow-up of 3.5, 4, and 4 years.Transplant-free survival at 30 days, 1 year, and 3 yearswas 84%, 76%, and 67% respectively (Fig. 1).Of the 13 patients with transplant-free survival, at mostrecent follow-up, 3 (23%) were on no pulmonary vasodila-tor therapy, 3 (23%) on monotherapy, 4 (30%) on dualtherapy, and 3 (23) on triple therapy. Ten (77%) patientswere treated with phosphodiesterase-5 inhibitors, 7 (54%)with an endothelin receptor antagonist, and 5 (38%) withprostanoid therapy.DiscussionCurrent pediatric practice guidelines advocate the use ofBAS as a deferred therapy, reserved for those with severedisease—characterized by RV failure, PH crises, functionalclass decline, or syncope—refractory to medical therapy.1,5,6There have been two recent descriptions of thisdeferred-BAS strategy in pediatrics PH, both of which illus-trate improved hemodynamics, functional outcomes, andlow procedural risk in cohorts of significantly ill children.3,4However, there is a conceptual benefit of earlier BAS inthe care of these children, as opposed to those with medic-ally refractory disease. The era prior to the advent ofmodern pharmacotherapy provides insight into the hemo-dynamic and clinical impact of atrial communications,absent the confounding effects of concurrent pulmonaryvasodilators in the modern experience. An initially observedsurvival advantage in adults with idiopathic PAH who had apatent foramen ovale led to several septostomy trials inadults with severe disease illustrating improvements insymptoms, cardiac output, right atrial pressure, oxygendelivery, and survival.7–10 More recently, Sandoval et al.provided a description of the hemodynamic improvementsin 34 adult PH patients who were naı̈ve to pulmonary vaso-dilator therapy.2 In this study, early-BAS led to improve-ments in hemodynamic parameters correlated with survivalafter septostomy as well as further improved survivalamongst those receiving early-BAS and subsequent pharma-cotherapy over those receiving early-BAS alone.In the enclosed single-center case-series, the outcomes ofan early-BAS approach in pediatrics are described. Thisseries demonstrates the safety of early-BAS in pediatrics:we illustrate no procedural complications nor deathswithin 24 h of the procedure. Across the follow-up period,our absolute survival and transplant-free survival are com-parable to the previously reported modern experience fromother centers (Fig. 1). These outcomes are despite severalpatients meeting criteria for relative contraindications forBAS.1,10 Three (16%) had a mRAP> 20mmHg, the highestbeing 33mmHg falling to 13mmHg after BAS. Four (21%)patients had RV cardiac output 2.5Lminm2 with thelowest being 0.85Lminm2. The only death from a PHcrisis was in a patient who met both criteria, 10 days follow-ing BAS. While this complex and high-risk populationdemands individualized care, this single-center experienceFig. 1. Kaplan–Meier survival characteristics illustrating cohort’soverall survival and transplant-free survival.2 | Balloon atrial septostomy in pediatric pulmonary hypertension Critser et al.supports consideration of early-BAS in addition to initiationof aggressive pharmacotherapy in patients with high-riskcharacteristics.Author contributionsPaul Critser, Patrick Evers, and Russel Hirsch contributed to theexperimental design, data analysis, drafting, and revision ofthe work. Eimear McGovern and Michelle Cash contributed tothe experimental design, data analysis, and revision of the work.Conflict of interestThe authors declare that there are no conflicts of interest.Ethical approvalThe institutional review board at Cincinnati Children’s Hospitalapproved this study and waived the requirement of formal consentfor this retrospective study.FundingThis research received no specific grant from any funding agency inthe public, commercial, or not-for-profit sectors.References1. Rosenzweig EB, Abman SH, Adatia I, et al. Paediatric pulmon-ary arterial hypertension: updates on definition, classification,diagnostics and management. Eur Respir J 2019; 53: 1801916.2. Sandoval J, Gaspar J, Pena H, et al. Effect of atrial septostomyon the survival of patients with severe pulmonary arterial hyper-tension. Eur Respir J 2011; 38: 1343–1348.3. Micheletti A, Hislop AA, Lammers A, et al. Role of atrialseptostomy in the treatment of children with pulmonary arter-ial hypertension. Heart 2006; 92: 969–972.4. Chiu JS, Zuckerman WA, Turner ME, et al. Balloon atrialseptostomy in pulmonary arterial hypertension: effect on sur-vival and associated outcomes. J Heart Lung Transplant 2015;34: 376–380.5. Abman SH, Hansmann G, Archer SL, et al. Pediatric pulmon-ary hypertension: guidelines from the American HeartAssociation and American Thoracic Society. Circulation2015; 132: 2037–2099.6. Hansmann G, Koestenberger M, Alastalo TP, et al. 2019updated consensus statement on the diagnosis and treatmentof pediatric pulmonary hypertension: the European PediatricPulmonary Vascular Disease Network (EPPVDN), endorsedby AEPC, ESPR and ISHLT. J Heart Lung Transplant 2019;38: 879–901.7. Rozkovec A, Montanes P and Oakley CM. Factors that influ-ence the outcome of primary pulmonary hypertension. BrHeart J 1986; 55: 449–458.8. Nihill MR, O’Laughlin MP and Mullins CE. Effects of atrialseptostomy in patients with terminal cor pulmonale due topulmonary vascular disease. Cathet Cardiovasc Diagn 1991;24: 166–172.9. Kerstein D, Levy PS, Hsu DT, et al. Blade balloon atrial sep-tostomy in patients with severe primary pulmonary hyperten-sion. Circulation 1995; 91: 2028–2035.10. Rich S, Dodin E and McLaughlin VV. Usefulness of atrialseptostomy as a treatment for primary pulmonary hyperten-sion and guidelines for its application. Am J Cardiol 1997; 80:369–371.Pulmonary Circulation Volume 10 Number 4 | 3

Balloon Atrial Septostomy as Initial Therapy in Pediatric Pulmonary Hypertension

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Balloon Atrial Septostomy as Initial Therapy in Pediatric Pulmonary Hypertension

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