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A low threshold for neonatal intervention yields a high rate of biventricular outcomes in pulmonary atresia with intact ventricular septum

Published online by Cambridge University Press:  23 April 2020

Gareth J. Morgan Open the ORCID record for Gareth J. Morgan [Opens in a new window] ,
Srinivas A. Narayan ,
Sebastian Goreczny ,
Henry Chubb ,
Thomas Krasemann ,
Eric Rosenthal Open the ORCID record for Eric Rosenthal [Opens in a new window]  and
Shakeel A. Qureshi
Gareth J. Morgan*
Affiliation:
The Heart Institute, Children’s Hospital Colorado, University of Colorado, Denver, CO, USA Department of Congenital Cardiology, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
Srinivas A. Narayan
Affiliation:
Department of Congenital Cardiology, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
Sebastian Goreczny
Affiliation:
The Heart Institute, Children’s Hospital Colorado, University of Colorado, Denver, CO, USA Department of Congenital Cardiology, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London, UK Department of Cardiology, Polish Mother’s Memorial Hospital, Research Institute, Lodz, Poland
Henry Chubb
Affiliation:
Department of Congenital Cardiology, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
Thomas Krasemann
Affiliation:
Department of Congenital Cardiology, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London, UK Department of Pediatrics, Division of Pediatric Cardiology, Sophia Kinderziekenhuis, Erasmus Medical Centre, Rotterdam, The Netherlands
Eric Rosenthal
Affiliation:
Department of Congenital Cardiology, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
Shakeel A. Qureshi
Affiliation:
Department of Congenital Cardiology, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
*
Author for correspondence: Gareth J. Morgan, MB, BaO, BCh, MPhil, MRCPCH, FSCAI, Department of Cardiology, Colorado Children’s Hospital, University of Colorado Hospital, 13123 East 16th Avenue, Aurora, CO, 80045, USA. Tel: +1 720 777 3379; Fax: +1 720 777 7177; E-mail: drgarethjmorgan@gmail.com
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Abstract

Aims:

Management strategies for pulmonary atresia with intact ventricular septum are variable and are based on right ventricular morphology and associated abnormalities. Catheter perforation of the pulmonary valve provides an alternative strategy to surgery in the neonatal period. We sought to assess the long-term outcome in terms of survival, re-intervention, and functional ventricular outcome in the setting of a 26-year single-centre experience of low threshold inclusion criteria for percutaneous valvotomy.

Methods and results:

Retrospective analysis of patients diagnosed with pulmonary atresia with intact ventricular septum from 1990 to 2016 at a tertiary referral centre, was performed. Of 71 patients, 48 were brought to the catheterisation laboratory for intervention. Catheter valvotomy was successful in 45 patients (94%). Twenty-three patients (51%) also underwent ductus arteriosus stenting. The length of intensive care and hospital stay was significantly shorter, and early re-interventions were significantly reduced in the catheterisation group. There were eight deaths (17%); all within 35 days of the procedure. Of the survivors, only one has required a Fontan circulation. Twenty-eight patients (74%) have undergone biventricular repair and nine patients (24%) have one-and-a-half ventricle circulation. Following successful valvotomy, 80% of patients required further catheter-based or surgical interventions.

Conclusions:

A low threshold for initial interventional management yielded a high rate of successful biventricular circulations. Although mortality was low in patients who survived the peri-procedural period, the rate of re-intervention remained high in all groups.

Keywords

CHDpercutaneous interventionpulmonary valve perforationcomplicationsoutcomes
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 5 , May 2020 , pp. 649 - 655
Copyright
© The Author(s) 2020. Published by Cambridge University Press

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