Advances in the Treatment of Congenital Cardiovascular Malformations in Children

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Cardiology".

Deadline for manuscript submissions: closed (25 November 2022) | Viewed by 16851

Special Issue Editors


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Guest Editor
McGovern Medical School, University of Texas Health, Houston, TX 77030, USA
Interests: clinical and experimental research; congenital heart defects; education; Fontan circulation; hypoxemia; mathematical and computational models; mechanical assistance

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Guest Editor
Division of Cardiology, Department of Pediatrics, University of Texas Health, McGovern Medical School, Houston, TX 77030, USA
Interests: peri-operative management of complex congenital heart defects; cardio-pulmonary interactions

Special Issue Information

Dear Colleagues,

We would like to inform you about our decision to organize a Special Issue on the “Advances in the Treatment of Congenital Cardiovascular Malformations in Children”.

The management of complex congenital heart defects involves many aspects, including the diagnostic procedures, the indications for treatment, the interventional and surgical procedures, and the post-operative management, starting from the fetal life through the pediatric age, with long-term effects.

Therefore, we invited worldwide known colleagues, involved in all these steps of the management of complex congenital heart defects, to provide us with the state-of-the-heart advanced treatment, based on their research projects as well as on the translation in their direct clinical experience.

The list of colleagues who have already accepted our invitation, includes people working in some of the most well-known hospitals in the world, from North America, to Europe, East Asia and Australia, with reputations of being the referral point for all researchers and clinicians involved in the treatment of complex congenital heart defects.

The collection of all the scientific contributions, provided by these Authors and their collaborators, will certainly constitute the timely gold standard, to follow as guidelines in the years to follow.

The format proposed by the Editors, the open access journal Children, will facilitate the free consultation of all the published contributions, which will be made available to all the readers involved in the management of the children with complex congenital heart defects.

Prof. Antonio Francesco Corno
Prof. Jon Nicolaus Meliones
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • cardiopulmonary bypass
  • complex congenital heart defect
  • congenital heart surgery
  • mechanical assistance
  • pharmacological treatment
  • prenatal diagnosis and interventions
  • single ventricle

Published Papers (6 papers)

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Review

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14 pages, 599 KiB  
Review
22q11.2 Deletion Syndrome: Impact of Genetics in the Treatment of Conotruncal Heart Defects
by Carolina Putotto, Flaminia Pugnaloni, Marta Unolt, Stella Maiolo, Matteo Trezzi, Maria Cristina Digilio, Annapaola Cirillo, Giuseppe Limongelli, Bruno Marino, Giulio Calcagni and Paolo Versacci
Children 2022, 9(6), 772; https://0-doi-org.brum.beds.ac.uk/10.3390/children9060772 - 25 May 2022
Cited by 9 | Viewed by 3073
Abstract
Congenital heart diseases represent one of the hallmarks of 22q11.2 deletion syndrome. In particular, conotruncal heart defects are the most frequent cardiac malformations and are often associated with other specific additional cardiovascular anomalies. These findings, together with extracardiac manifestations, may affect perioperative management [...] Read more.
Congenital heart diseases represent one of the hallmarks of 22q11.2 deletion syndrome. In particular, conotruncal heart defects are the most frequent cardiac malformations and are often associated with other specific additional cardiovascular anomalies. These findings, together with extracardiac manifestations, may affect perioperative management and influence clinical and surgical outcome. Over the past decades, advances in genetic and clinical diagnosis and surgical treatment have led to increased survival of these patients and to progressive improvements in postoperative outcome. Several studies have investigated long-term follow-up and results of cardiac surgery in this syndrome. The aim of our review is to examine the current literature data regarding cardiac outcome and surgical prognosis of patients with 22q11.2 deletion syndrome. We thoroughly evaluate the most frequent conotruncal heart defects associated with this syndrome, such as tetralogy of Fallot, pulmonary atresia with major aortopulmonary collateral arteries, aortic arch interruption, and truncus arteriosus, highlighting the impact of genetic aspects, comorbidities, and anatomical features on cardiac surgical treatment. Full article
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9 pages, 1315 KiB  
Review
Biventricular Conversion for Hypoplastic Left Heart Variants: An Update
by Christopher E. Greenleaf and Jorge D. Salazar
Children 2022, 9(5), 690; https://0-doi-org.brum.beds.ac.uk/10.3390/children9050690 - 10 May 2022
Cited by 3 | Viewed by 1770
Abstract
Ongoing concerns with single-ventricle palliation morbidity and poor outcomes from primary biventricular strategies for neonates with borderline left heart structures have led some centers to attempt alternative strategies to obviate the need for ultimate Fontan palliation and limit the risk to the child [...] Read more.
Ongoing concerns with single-ventricle palliation morbidity and poor outcomes from primary biventricular strategies for neonates with borderline left heart structures have led some centers to attempt alternative strategies to obviate the need for ultimate Fontan palliation and limit the risk to the child during the vulnerable neonatal period. In certain patients who are traditionally palliated toward single-ventricle circulation, biventricular circulation is possible. This review aims to delineate the current knowledge regarding converting certain patients with borderline left heart structures from single-ventricle palliation toward biventricular circulation. Full article
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10 pages, 1536 KiB  
Review
The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries
by Matteo Trezzi, Enrico Cetrano, Sonia B. Albanese, Luca Borro, Aurelio Secinaro and Adriano Carotti
Children 2022, 9(4), 515; https://0-doi-org.brum.beds.ac.uk/10.3390/children9040515 - 05 Apr 2022
Cited by 2 | Viewed by 4135
Abstract
Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals is a complex congenital heart defect that includes a heterogeneous subgroup of patients. Variation in the sources of pulmonary blood flow contributes to the complexity of the lesion and the diversity of approaches [...] Read more.
Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals is a complex congenital heart defect that includes a heterogeneous subgroup of patients. Variation in the sources of pulmonary blood flow contributes to the complexity of the lesion and the diversity of approaches to its management. Unifocalization and rehabilitation focus on mobilization of collateral arteries and growth of native pulmonary arteries, respectively, with the ultimate surgical goal of achieving separated systemic and pulmonary circulations with the lowest possible right ventricular pressure. Regardless of the strategy, outcomes have altered the natural history of the disease, with a complete repair rate of approximately 80% and low early and late mortality rates. Given this heterogeneity of pulmonary vasculature, a tailored approach should be adopted for each patient, using all diagnostic methods currently offered by technical developments. Full article
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15 pages, 294 KiB  
Review
Extracorporeal Membrane Oxygenation in Congenital Heart Disease
by Tanya Perry, Tyler Brown, Andrew Misfeldt, David Lehenbauer and David S. Cooper
Children 2022, 9(3), 380; https://0-doi-org.brum.beds.ac.uk/10.3390/children9030380 - 09 Mar 2022
Cited by 6 | Viewed by 2933
Abstract
Mechanical circulatory support (MCS) is a key therapy in the management of patients with severe cardiac disease or respiratory failure. There are two major forms of MCS commonly employed in the pediatric population—extracorporeal membrane oxygenation (ECMO) and ventricular assist device (VAD). These modalities [...] Read more.
Mechanical circulatory support (MCS) is a key therapy in the management of patients with severe cardiac disease or respiratory failure. There are two major forms of MCS commonly employed in the pediatric population—extracorporeal membrane oxygenation (ECMO) and ventricular assist device (VAD). These modalities have overlapping but distinct roles in the management of pediatric patients with severe cardiopulmonary compromise. The use of ECMO to provide circulatory support arose from the development of the first membrane oxygenator by George Clowes in 1957, and subsequent incorporation into pediatric cardiopulmonary bypass (CPB) by Dorson and colleagues. The first successful application of ECMO in children with congenital heart disease undergoing cardiac surgery was reported by Baffes et al. in 1970. For the ensuing nearly two decades, ECMO was performed sparingly and only in specialized centers with varying degrees of success. The formation of the Extracorporeal Life Support Organization (ELSO) in 1989 allowed for the collation of ECMO-related data across multiple centers for the first time. This facilitated development of consensus guidelines for the use of ECMO in various populations. Coupled with improving ECMO technology, these advances resulted in significant improvements in ECMO utilization, morbidity, and mortality. This article will review the use of ECMO in children with congenital heart disease. Full article
19 pages, 1579 KiB  
Review
The Current Status of Neuroprotection in Congenital Heart Disease
by Kei Kobayashi, Christopher Liu, Richard A. Jonas and Nobuyuki Ishibashi
Children 2021, 8(12), 1116; https://0-doi-org.brum.beds.ac.uk/10.3390/children8121116 - 02 Dec 2021
Cited by 3 | Viewed by 2802
Abstract
Neurological deficits are a serious and common sequelae of congenital heart disease (CHD). While their underlying mechanisms have not been fully characterized, their manifestations are well-known and understood to persist through adulthood. Development of therapies to address or prevent these deficits are critical [...] Read more.
Neurological deficits are a serious and common sequelae of congenital heart disease (CHD). While their underlying mechanisms have not been fully characterized, their manifestations are well-known and understood to persist through adulthood. Development of therapies to address or prevent these deficits are critical to attenuate future morbidity and improve quality of life. In this review, we aim to summarize the current status of neuroprotective therapy in CHD. Through an exploration of present research in the pre-operative, intra-operative, and post-operative phases of patient management, we will describe existing clinical and bench efforts as well as current endeavors underway within this research area. Full article
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Other

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12 pages, 1571 KiB  
Systematic Review
Feeding Neonates and Infants Prior to Surgery for Congenital Heart Defects: Systematic Review and Meta-Analysis
by Douglas Bell, Jessica Suna, Supreet P. Marathe, Gopinath Perumal, Kim S. Betts, Prem Venugopal, Nelson Alphonso and QPCR Group
Children 2022, 9(12), 1856; https://doi.org/10.3390/children9121856 - 29 Nov 2022
Cited by 2 | Viewed by 1294
Abstract
Background: Necrotising enterocolitis (NEC) is a significant cause of mortality and morbidity in neonates requiring cardiac surgery. Feeding practices vary significantly across institutions and remain controversial. We conducted a systematic review of the literature and a meta-analysis to identify associations between feeding practices [...] Read more.
Background: Necrotising enterocolitis (NEC) is a significant cause of mortality and morbidity in neonates requiring cardiac surgery. Feeding practices vary significantly across institutions and remain controversial. We conducted a systematic review of the literature and a meta-analysis to identify associations between feeding practices and necrotising enterocolitis. Methods: This study was carried out in accordance with the PRISMA guidelines. A literature search was performed in November 2022 using the Cochrane Central Register, Embase, and Pubmed. Two investigators then independently retrieved eligible manuscripts considered suitable for inclusion. Data extracted included gestational age, birth weight, sex, nature of congenital heart lesion, type of operation performed, time on ventilator, ICU stay, hospital stay, post-operative feeding strategy, and complications. The methodological quality was assessed using the Downs and Black score for all randomised control trials and observational studies. Results: The initial search yielded 92 studies. After removing duplicates, there were 85 abstracts remaining. After excluding ineligible studies, 8 studies were included for the meta-analysis. There was no significant risk of NEC associated with pre-operative feeding [OR = 1.22 (95% CI 0.77,1.92)] or umbilical artery catheter placement [OR = 0.91 (95% CI 0.44, 1.89)] and neither outcome exhibited heterogeneity [I2 = 8% and 0%, respectively]. There was a significant association between HLHS and NEC [OR = 2.56 (95% CI 1.56, 4.19)] as well as prematurity and NEC [OR 3.34 (95% CI 1.94, 5.75)] and neither outcome exhibited heterogeneity [I2 = 0% and 0%, respectively]. Conclusions: There was no association between NEC and pre-operative feeding status in neonates awaiting cardiac surgery. Pre-operative feeding status was not associated with prolonged hospital stay or need for tube assisted feeding at discharge. HLHS and prematurity were associated with increased incidence of NEC. Full article
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