Evidence map›Paper›PMID 40569053›Full record

ArticleEuropean journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery2025

Eliminating left ventricular outlet stenosis lowers the risk for endocardial fibroelastosis recurrence†.

Gregor Gierlinger, Daniel Diaz-Gil, Andreas Tulzer, Roland Mair, Eva Sames-Dolzer, Kerstin Saraci, Steven J Staffa, David Zurakowski, Michaela Kreuzer, Fabian Seeber and 4 more

Abstract read
In one paragraph

Article in European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

14 authors.

Gregor GierlingerDepartment of Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.ORCID 0000-0002-6358-5048
Daniel Diaz-GilDepartment of Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.
Andreas TulzerMedical Faculty, Johannes Kepler University Linz, Linz, Austria.
Roland MairDivision of Pediatric and Congenital Heart Surgery, Kepler University Hospital, Linz, Austria.
Eva Sames-DolzerDivision of Pediatric and Congenital Heart Surgery, Kepler University Hospital, Linz, Austria.
Kerstin SaraciDepartment of Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.
Steven J StaffaDepartment of Anesthesiology, Critical Care, and Pain Medicine, Boston Children's Hospital, Boston, MA, USA.ORCID 0000-0002-7588-7596
David ZurakowskiDepartment of Anesthesiology, Critical Care, and Pain Medicine, Boston Children's Hospital, Boston, MA, USA.ORCID 0000-0003-3610-6942
Michaela KreuzerDivision of Pediatric and Congenital Heart Surgery, Kepler University Hospital, Linz, Austria.ORCID 0000-0002-7499-7646
Fabian SeeberDivision of Pediatric and Congenital Heart Surgery, Kepler University Hospital, Linz, Austria.
Sitaram M EmaniDepartment of Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.
Pedro J Del NidoDepartment of Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.
Rudolf MairDivision of Pediatric and Congenital Heart Surgery, Kepler University Hospital, Linz, Austria.ORCID 0000-0003-0935-1766
Ingeborg FriehsDepartment of Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.

Funding

Ventures-Single Ventricle Research Fund (SVRF) and Single Ventricle Expansion Fund 991414
6 · The paper itself

Abstract

objectivesPatients with endocardial fibroelastosis (EFE) in the setting of congenital critical aortic valve (AoV) stenosis and left ventricular outflow tract obstruction (LVOTO) are at risk for diastolic dysfunction, limiting biventricular circulation. EFE resection is the only available treatment option, but frequently recurs requiring re-resections. We aimed to investigate whether augmentation of a left ventricular outlet stenosis (AoV stenosis ± LVOTO) with a Ross/Ross-Konno procedure prevents EFE recurrence.

methodsPatients born with AoV stenosis ± LVOTO and treated with primary left ventricular (LV) EFE resection at the study centres from January 2010 to December 2021 were included in the study. The inclusion criteria for this retrospective analysis was the presence or absence of a Ross/Ross-Konno procedure for the treatment of a modifiable risk factor of EFE recurrence. Retrospective allocation to either the non-Ross or Ross/Ross-Konno group was carried out accordingly. The primary outcome measure was EFE recurrence.

resultsNinety-three patients were screened, and 60/93 patients (64.5%) met all inclusion criteria. Within those 60 patients, 5/23 (20.7%) in the Ross/Ross-Konno group had EFE recurrence compared to 23/37 (62.2%) in the non-Ross group [difference = 40.5%, 95% confidence interval (CI) 14.6-58.7, P =  0.003] and were less likely to develop EFE recurrence with adjusted hazard ratio of 4.07 (95% CI 1.38-12.0, P =  0.011) and 3.69 (95% CI 1.31-10.42, P =  0.014) when including death as a competing event.

conclusionsThis study found that patients after a Ross/Ross-Konno procedure were less likely to experience EFE recurrence and reinterventions on the LVOT/AoV were significantly reduced. However, patient selection and timing of a Ross/Ross-Konno procedure to prevent EFE recurrence have yet to be identified through prospective trials. IRB PROTOCOL NUMBERS: IRB-P0038762 approved 4/29/2021 (Boston Children's Hospital) and Ek Nr: 1137/2023 approved 10/25/2023 (Medical Faculty of Johannes Kepler University Linz).

Indexed as

Aortic Valve StenosisCardiac Surgical ProceduresEndocardial FibroelastosisVentricular Outflow ObstructionFemaleHeart VentriclesHumansInfantMaleRecurrenceRetrospective StudiesRisk FactorsTreatment OutcomeBorderline left ventricleCritical aortic stenosisEndocardial fibroelastosisHypoplastic left heartRoss/Ross-KonnoStaged ventricular recruitment

Identifiers

PMID40569053
PMCPMC12254124

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.