Long-term outcome after neonatal intervention for congenital critical aortic stenosis

Beatrice Bonello; Richard Issitt; Marina Hughes; Michelle Carr; Xavier Iriart; Sachin Khambadkone; Alessandro Giardini; Martin Kostolny; Jan Marek

doi:10.1016/j.ijcard.2024.131932

Long-term outcome after neonatal intervention for congenital critical aortic stenosis

Int J Cardiol. 2024 Jun 15:405:131932. doi: 10.1016/j.ijcard.2024.131932. Epub 2024 Mar 2.

Authors

Beatrice Bonello¹, Richard Issitt², Marina Hughes³, Michelle Carr⁴, Xavier Iriart⁵, Sachin Khambadkone⁶, Alessandro Giardini⁷, Martin Kostolny⁸, Jan Marek⁹

Affiliations

¹ Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK; University College of London, Great Ormond Street Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. Electronic address: Beatrice.bonello@gosh.nhs.uk.
² Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK; University College of London, Great Ormond Street Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. Electronic address: Richard.Issitt@gosh.nhs.uk.
³ Norfolk and Norwich University Hospital NHS Trust, Colney Lane, Norwich NR4 7UY, UK. Electronic address: marina.hughes2@nhs.net.
⁴ Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK. Electronic address: Michelle.Carr@gosh.nhs.uk.
⁵ CHU Bordeaux, Av. du Haut Lévêque, Pessac 33604, France. Electronic address: xavier.iriart@chu-bordeaux.fr.
⁶ Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK; University College of London, Great Ormond Street Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. Electronic address: Sachin.Khambadkone@gosh.nhs.uk.
⁷ Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK. Electronic address: Alessandro.Giardini@gosh.nhs.uk.
⁸ Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK; University College of London, Great Ormond Street Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. Electronic address: martin.kostolny@gosh.nhs.uk.
⁹ Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK; University College of London, Great Ormond Street Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. Electronic address: Jan.Marek@gosh.nhs.uk.

PMID: 38437954
DOI: 10.1016/j.ijcard.2024.131932

Abstract

Background: This study explored long-term outcome and functional status of patients born with critical aortic stenosis (CAS) following neonatal surgical or catheter interventions.

Methods: A 40-year retrospective review of all consecutive patients within a large, single-center referral unit who required neonatal (<30 days) intervention for CAS. Additional detailed evaluation of surviving patients >7 years age was performed, with clinical assessment, objective cardiopulmonary exercise testing and state-of-the-art characterization of myocardial function (advanced echocardiography and cardiac MRI).

Results: Between 1970 and 2010, ninety-six neonates underwent CAS intervention (mean age 9 ± 7.5 days). Early death occurred in 19 (19.8%) and late death in 10 patients. Overall survival at 10 and 30 years was 70.1% and 68.5%, freedom from reintervention was 41.8% and 32.9% respectively. Among the 25 long-term survivors available for detailed assessment (median age 15.7 ± 6.4 years), 55% exhibited impaired peak oxygen uptake. Mean left ventricle (LV) ejection fraction was 65 ± 11.2%, with a mean LV end-diastolic volume z-score of 0.02 ± 1.4. Mean LV outflow tract Vmax was 2.3 ± 1.02 m/s. CAS patients had reduced LV longitudinal and increased radial strain (p = 0.003, p < 0.001 respectively). Five patients had severe LV diastolic dysfunction associated with endocardial fibroelastosis (EFE) (p = 0.0014).

Conclusion: Despite high early mortality rate, long-term survival of patients with CAS is reasonable at the expense of high reintervention rate. With successful intervention, there remained long-term clinical and subclinical LV myocardial impairment, of which EFE was one marker. Long-term follow-up of all CAS patients is crucial, involving detailed myocardial functional assessment to help elucidate physiology and optimise management.

Keywords: (6) critical aortic stenosis; Balloon valvuloplasty; Endocardial fibroelastosis; Left ventricle function; Surgical valvotomy; Survival.

MeSH terms

Adolescent
Adult
Aortic Valve Stenosis* / diagnostic imaging
Aortic Valve Stenosis* / mortality
Aortic Valve Stenosis* / physiopathology
Aortic Valve Stenosis* / surgery
Child
Female
Follow-Up Studies
Humans
Infant, Newborn
Male
Retrospective Studies
Time Factors
Treatment Outcome
Young Adult