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Advances in the Diagnosis and Management of Congenital Heart Disease...
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Advances in the Diagnosis and Management of Congenital Heart Disease in Children

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

Deadline for manuscript submissions: closed (5 March 2023) | Viewed by 17345

Special Issue Editors

Prof. Dr. P. Syamasundar Rao

E-Mail Website
Guest Editor
UT-Houston McGovern Medical School, 6410 Fannin Street, UTPB Suite #425, Houston, TX 77030, USA
Interests: congenital heart disease; pediatric cardiology; tricuspid atresia; physiologically advantageous ventricular septal defect; interventional pediatric cardiology; neonatal cardioloy; echocardiography
Special Issues, Collections and Topics in MDPI journals
Dr. Arpit Agarwal

E-Mail Website
Guest Editor
Baylor College of Medicine, The Children's Hospital of San Antonio, Pediatric Cardiology, San Antonio, TX 78207, USA
Interests: pediatrics; cardiology; CTA; MRI; clinical informatics; echocardiography; 3D printing; fetal echocardiography; artificial intelligence
Prof. Dr. Harinder R. Singh

E-Mail Website
Guest Editor
Pediatric Cardiology, Baylor College of Medicine, The Children’s Hospital of San Antonio, San Antonio, TX 78207, USA
Interests: pediatric cardiology; pediatric electrophysiology; cardiac devices; adult congenital heart defects; autonomic nervous system

Special Issue Information

Dear Colleagues,

Over the last 2–3 decades, we have made huge strides in the management of congenital heart diseases. Advancements in non-invasive imaging, cardiac CTA, cardiac MRI, and echocardiography have transformed the field of imaging. Evolving technologies such as 3D printing, virtual reality, and mixed reality have greatly enhanced our ability to experience three-dimensional images and changed the paradigm of presurgical planning. The introduction of electromapping in the electrophysiology map has significantly reduced the need for radiation. Three-dimensional rotational angiography has provided new dimensions in the cardiac path for the management of complex congenital heart disease. The surgical techniques have matured, and mortality has significantly decreased.

Prof. Dr. P. Syamasundar Rao
Dr. Arpit Agarwal
Prof. Dr. Harinder R. Singh
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

  • congenital heart disease
  • cardiac MRI
  • cardiac catheterization
  • echocardiography
  • fetal echocardiography
  • transesophageal echocardiography
  • cardiac computed tomography
  • cardiac angiography
  • three-dimensional printing
  • cardiac surgery
  • pediatric cardiology

Published Papers (12 papers)

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Editorial

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4 pages, 175 KiB  
Editorial
Advances in the Diagnosis and Management of Congenital Heart Disease in Children
by P. Syamasundar Rao
Children 2023, 10(4), 753; https://0-doi-org.brum.beds.ac.uk/10.3390/children10040753 - 21 Apr 2023
Cited by 3 | Viewed by 1203
Abstract
The last five decades have witnessed an inordinate number of advances in the diagnosis and management of congenital heart defects (CHDs), as reviewed elsewhere [...] Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)
3 pages, 164 KiB  
Editorial
Advances in the Diagnosis and Management of Congenital Heart Disease in Children
by P. Syamasundar Rao and Arpit Agarwal
Children 2022, 9(7), 1056; https://0-doi-org.brum.beds.ac.uk/10.3390/children9071056 - 15 Jul 2022
Cited by 1 | Viewed by 1136
Abstract
The last five decades have witnessed an inordinate number of advances in the diagnosis and management of congenital heart defects (CHDs), as reviewed elsewhere [...] Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)

Research

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15 pages, 995 KiB  
Article
Overshoot of the Respiratory Exchange Ratio during Recovery from Maximal Exercise Testing in Young Patients with Congenital Heart Disease
by Marco Vecchiato, Andrea Ermolao, Emanuele Zanardo, Francesca Battista, Giacomo Ruvoletto, Stefano Palermi, Giulia Quinto, Gino Degano, Andrea Gasperetti, Massimo A. Padalino, Giovanni Di Salvo and Daniel Neunhaeuserer
Children 2023, 10(3), 521; https://0-doi-org.brum.beds.ac.uk/10.3390/children10030521 - 07 Mar 2023
Cited by 4 | Viewed by 1577
Abstract
Introduction: The overshoot of the respiratory exchange ratio (RER) after exercise is reduced in patients with heart failure. Aim: The present study aimed to investigate the presence of this phenomenon in young patients with congenital heart disease (CHD), who generally present reduced cardiorespiratory [...] Read more.
Introduction: The overshoot of the respiratory exchange ratio (RER) after exercise is reduced in patients with heart failure. Aim: The present study aimed to investigate the presence of this phenomenon in young patients with congenital heart disease (CHD), who generally present reduced cardiorespiratory fitness. Methods: In this retrospective study, patients with CHD underwent a maximal cardiopulmonary exercise testing (CPET) assessing the RER recovery parameters: the RER at peak exercise, the maximum RER value reached during recovery, the magnitude of the RER overshoot and the linear slope of the RER increase after the end of the exercise. Results: In total, 117 patients were included in this study. Of these, there were 24 healthy age-matched control subjects and 93 young patients with CHD (transposition of great arteries, Fontan procedure, aortic coarctation and tetralogy of Fallot). All patients presented a RER overshoot during recovery. Patients with CHD showed reduced aerobic capacity and cardiorespiratory efficiency during exercise, as well as a lower RER overshoot when compared to controls. RER magnitude was higher in the controls and patients with aortic coarctation when compared to those with transposition of great arteries, previous Fontan procedure, and tetralogy of Fallot. The RER magnitude was found to be correlated with the most relevant cardiorespiratory fitness and efficiency indices. Conclusions: The present study proposes new recovery indices for functional evaluation in patients with CHD. Thus, the RER recovery overshoots analysis should be part of routine CPET evaluation to further improve prognostic risk stratifications in patients with CHD. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)
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8 pages, 1098 KiB  
Article
Quality Improvement in a Pediatric Echocardiography Laboratory: A Collaborative Process
by Danielle Sganga, Sarina Behera, Meaghan J. Beattie, Katie Jo Stauffer, Aubrey Burlinson, Leo Lopez and Elif Seda Selamet Tierney
Children 2022, 9(12), 1845; https://0-doi-org.brum.beds.ac.uk/10.3390/children9121845 - 28 Nov 2022
Cited by 2 | Viewed by 1439
Abstract
Transthoracic echocardiography (TTE) is an essential tool for diagnosis and management of congenital heart disease. Pediatric echocardiography presents unique challenges including complex anatomy, variable patient cooperation and provider expertise. Diagnostic errors inevitably occur. We designed a collaborative and stepwise quality improvement (QI) process [...] Read more.
Transthoracic echocardiography (TTE) is an essential tool for diagnosis and management of congenital heart disease. Pediatric echocardiography presents unique challenges including complex anatomy, variable patient cooperation and provider expertise. Diagnostic errors inevitably occur. We designed a collaborative and stepwise quality improvement (QI) process to address diagnostic errors within our laboratory. We retrospectively reviewed medical records to identify diagnostic TTE errors in 100 consecutive cardiac surgery patients ≤ 5 years old (July 2020–January 2021). We identified 18 diagnostic errors. Most errors had minor impact (14/18), and 13 were preventable or possibly preventable. We presented these results to our sonographers and faculty and requested input on preventing and managing diagnostic errors. Our root cause analysis based on their responses yielded 7 areas for improvement (imaging, reporting, systems, time, environment, people, QI processes). Our faculty and sonographers chose QI processes and imaging as initial areas for intervention. We defined our SMART goal as a 10% reduction in diagnostic errors. We implemented interventions focused on QI processes. On initial follow up in May 2022, we identified 7 errors in 70 patients (44% reduction in error rate). Utilizing a stepwise and team-based approach, we successfully developed QI initiatives in our echocardiography laboratory. This approach can serve as a model for a collaborative QI process in other institutions. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)
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7 pages, 1253 KiB  
Communication
Cardiothoracic CTA in Infants Referred for Aortic Arch Evaluation—Retrospective Comparison of Iomeprol 350, Ioversol 350, Iopromide 370 and Iodixanol 320
by Marian Pop
Children 2021, 8(11), 949; https://0-doi-org.brum.beds.ac.uk/10.3390/children8110949 - 21 Oct 2021
Cited by 9 | Viewed by 2398
Abstract
Background: Computed tomography angiography (CTA) in infants is considered one of the most challenging radiological examinations due to difficulties in balancing start delay, contrast agent (CA) volume and flow in order to achieve optimal opacification of the large vessels. This study aimed to [...] Read more.
Background: Computed tomography angiography (CTA) in infants is considered one of the most challenging radiological examinations due to difficulties in balancing start delay, contrast agent (CA) volume and flow in order to achieve optimal opacification of the large vessels. This study aimed to compare the contrast enhancement achieved by four CAs when taking into consideration CA injection parameters and patient characteristics. Methods: We performed a retrospective assessment of forty-eight consecutive cardiothoracic CTAs performed for aortic arch evaluation on children aged 0–1 year. All examinations were performed using the same 64-slice scanner and power injector using the bolus tracking technique. Axial 0.6 mm slices were used to measure large vessel enhancement using regions of interest at the level of the main pulmonary artery, ascending and descending aorta. The recorded variables included anthropometric measurements, CA type, flow rate, volume, and the average Hounsfield unit (HU) values of the blood pool. Descriptive statistics are presented as averages and standard deviations (SD) for normal distributed data or otherwise as medians and interquartile ranges (IQRs). Results: We found no statistically significant differences between age and anthropometric parameters when looking at different CAs. The median CA volume was 7 (IQR, 7–9) mL with the average flow rate of 0.94 (SD, 0.23) mL/s. Ascending aorta average HU values were 605.9 (SD, 177.23) for Iomeprol 350, 626 (SD, 183.83) for Ioversol 350, 530.83 (SD, 175.56) for Iopromide 370 and 354.91 (SD, 115.81) for Iodixanol 320. The difference in HU value for Iodixanol 320 compared to the other CAs was statistically significant. Similar differences were found for the other vascular structures. Conclusion: In CTA of infants suspected of aortic arch hypoplasia/coarctation, Iodixanol 320 provided up to 40% less enhancement of the great vessels when compared to Iomeprol 350, Ioversol 350 and Iopromide 370. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)
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Review

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15 pages, 5266 KiB  
Review
Mitral Atresia with Normal Aortic Root
by P. Syamasundar Rao
Children 2022, 9(8), 1148; https://0-doi-org.brum.beds.ac.uk/10.3390/children9081148 - 30 Jul 2022
Cited by 1 | Viewed by 1769
Abstract
Mitral atresia with normal aortic root is a rare complex congenital heart defect (CHD) and constitute less than 1% of all CHDs. In this anomaly, the mitral valve is atretic, a patent foramen ovale provides egress of the left atrial blood, either a [...] Read more.
Mitral atresia with normal aortic root is a rare complex congenital heart defect (CHD) and constitute less than 1% of all CHDs. In this anomaly, the mitral valve is atretic, a patent foramen ovale provides egress of the left atrial blood, either a single ventricle or two ventricles with left ventricular hypoplasia are present, and the aortic valve/root are normal by definition. Clinical, roentgenographic and electrocardiographic features are non-distinctive, but echo-Doppler studies are useful in defining the anatomic and pathophysiologic components of this anomaly with rare need for other imaging studies. Treatment consists of addressing the pathophysiology resulting from defect and associated cardiac anomalies at the time of initial presentation, usually in the early infancy. These children eventually require staged total cavo-pulmonary connection (Fontan) in three stages. Discussion of each of these stages were presented. Complications are observed in-between the stages of Fontan surgery and following completion of Fontan procedure. Attempts to monitor for early detection of these complications and promptly addressing the complications are recommended. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)
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8 pages, 1641 KiB  
Review
ALARA in Pediatric Electrophysiology Laboratory
by Harinder R. Singh
Children 2022, 9(6), 866; https://0-doi-org.brum.beds.ac.uk/10.3390/children9060866 - 10 Jun 2022
Cited by 3 | Viewed by 1711
Abstract
The effects of radiation on patients and the providers are dose-dependent and cumulative. Pediatric patients are not only more sensitive to radiation but also may undergo more procedures and diagnostic tests throughout their lifetime. As providers, the endeavor is to cause no harm [...] Read more.
The effects of radiation on patients and the providers are dose-dependent and cumulative. Pediatric patients are not only more sensitive to radiation but also may undergo more procedures and diagnostic tests throughout their lifetime. As providers, the endeavor is to cause no harm and it behooves us to either eliminate or minimize the radiation exposure to patients without affecting the efficacy and outcomes of the diagnostic or therapeutic modalities. Pediatric electrophysiologists have taken the lead in attempting to minimize the radiation exposure to patients and staff with innovative and advanced techniques. The techniques range from minimizing the exposure to radiation with better understanding and applications of the physics associated with fluoroscopic imaging to using alternative imaging modalities that do not use radiation. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)
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28 pages, 5020 KiB  
Review
Transcatheter Device Therapy and the Integration of Advanced Imaging in Congenital Heart Disease
by Abhay A. Divekar, Yousef M. Arar, Stephen Clark, Animesh Tandon, Thomas M. Zellers and Surendranath R. Veeram Reddy
Children 2022, 9(4), 497; https://0-doi-org.brum.beds.ac.uk/10.3390/children9040497 - 02 Apr 2022
Cited by 3 | Viewed by 3039
Abstract
Transcatheter device intervention is now offered as first line therapy for many congenital heart defects (CHD) which were traditionally treated with cardiac surgery. While off-label use of devices is common and appropriate, a growing number of devices are now specifically designed and approved [...] Read more.
Transcatheter device intervention is now offered as first line therapy for many congenital heart defects (CHD) which were traditionally treated with cardiac surgery. While off-label use of devices is common and appropriate, a growing number of devices are now specifically designed and approved for use in CHD. Advanced imaging is now an integral part of interventional procedures including pre-procedure planning, intra-procedural guidance, and post-procedure monitoring. There is robust societal and industrial support for research and development of CHD-specific devices, and the regulatory framework at the national and international level is patient friendly. It is against this backdrop that we review transcatheter implantable devices for CHD, the role and integration of advanced imaging, and explore the current regulatory framework for device approval. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)
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19 pages, 33787 KiB  
Review
Role of Cross-Sectional Imaging in Pediatric Interventional Cardiac Catheterization
by Yousef Arar, Abhay Divekar, Stephen Clark, Tarique Hussain, Roby Sebastian, Mehar Hoda, Jamie King, Thomas M. Zellers and Surendranath R. Veeram Reddy
Children 2022, 9(3), 300; https://0-doi-org.brum.beds.ac.uk/10.3390/children9030300 - 22 Feb 2022
Cited by 4 | Viewed by 2894
Abstract
Management of congenital heart disease (CHD) has recently increased utilization of cross-sectional imaging to plan percutaneous interventions. Cardiac computed tomography (CT) and cardiac magnetic resonance (CMR) imaging have become indispensable tools for pre-procedural planning prior to intervention in the pediatric cardiac catheterization lab. [...] Read more.
Management of congenital heart disease (CHD) has recently increased utilization of cross-sectional imaging to plan percutaneous interventions. Cardiac computed tomography (CT) and cardiac magnetic resonance (CMR) imaging have become indispensable tools for pre-procedural planning prior to intervention in the pediatric cardiac catheterization lab. In this article, we review several common indications for referral and the impact of cross-sectional imaging on procedural planning, success, and patient surveillance. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)
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12 pages, 2793 KiB  
Review
Utility of Three-Dimensional Printed Model in Biventricular Repair of Complex Congenital Cardiac Defects: Case Report and Review of Literature
by Lauren Gabriel Betancourt, Si Hui Wong, Harinder R. Singh, Daniel Nento and Arpit Agarwal
Children 2022, 9(2), 184; https://0-doi-org.brum.beds.ac.uk/10.3390/children9020184 - 01 Feb 2022
Cited by 4 | Viewed by 1798
Abstract
Heterotaxy is a rare syndrome associated with cardiac complexity, anatomic variability and high morbidity and mortality. It is often challenging to visualize and provide an accurate diagnosis of the cardiac anatomy prior to surgery with the use of conventional imaging techniques. We report [...] Read more.
Heterotaxy is a rare syndrome associated with cardiac complexity, anatomic variability and high morbidity and mortality. It is often challenging to visualize and provide an accurate diagnosis of the cardiac anatomy prior to surgery with the use of conventional imaging techniques. We report a unique case demonstrating how the use of three-dimensional (3D) cardiac printed model allowed us to better understand the anatomical complexity and plan a tailored surgical approach for successful biventricular repair in a patient with heterotaxy syndrome. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)
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Other

7 pages, 646 KiB  
Brief Report
Impact of Extubation Time on Feeding Outcomes after Neonatal Cardiac Surgery: A Single-Center Study
by Jeffrey W. Kepple, Meghan Kendall and Laura A. Ortmann
Children 2023, 10(3), 592; https://0-doi-org.brum.beds.ac.uk/10.3390/children10030592 - 20 Mar 2023
Cited by 2 | Viewed by 1323
Abstract
This study aimed to examine the impact of timing of extubation on feeding outcomes in neonates after surgery for congenital heart disease. This was a single-center retrospective study between December 2014 and June 2020. Patients were divided into three categories: extubated in the [...] Read more.
This study aimed to examine the impact of timing of extubation on feeding outcomes in neonates after surgery for congenital heart disease. This was a single-center retrospective study between December 2014 and June 2020. Patients were divided into three categories: extubated in the OR (immediate), extubated in the intensive care unit (ICU) between 0 and 3 days post-procedure (early), and extubated >3 days post-procedure (delayed). Comparing the immediate and early groups, we found no difference in time to first enteral feed (1.3 days (1.0–3.4) vs. 2.3 days (1.1–3.3), p = 0.27). There was no difference in time to first oral feed (2.0 days (1.1–4.5) vs. 3.1 days (1.8–4.4), p = 0.34) and time to goal feed (6.0 days (3.2–8.3) vs. 6.9 days (5.0–9.0), p = 0.15)). There was no difference in all oral feeds at one year: 88% vs. 98%, p = 0.16. The delayed extubation group performed significantly worse on all measures. Immediate and early extubation displayed no differences in feeding outcomes and length of stay in this study, while delayed extubation performed worse on all measures. Thus, we believe that clinicians should emphasize extubation within 3 days post-surgery to improve feeding outcomes while minimizing time hospitalized. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)
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Graphical abstract

8 pages, 498 KiB  
Perspective
Advances in the Prenatal Management of Fetal Cardiac Disease
by Chetan Sharma, Joseph Burns, Krittika Joshi, Monesha Gupta, Harinder Singh and Arpit Agarwal
Children 2022, 9(6), 812; https://0-doi-org.brum.beds.ac.uk/10.3390/children9060812 - 31 May 2022
Cited by 4 | Viewed by 1681
Abstract
Advances in the field have improved the prenatal management of cardiovascular diseases over the past few decades; however, there remains considerable challenges in the approach towards patient selection as well as the applicability of available therapies. This review aims to discuss the current [...] Read more.
Advances in the field have improved the prenatal management of cardiovascular diseases over the past few decades; however, there remains considerable challenges in the approach towards patient selection as well as the applicability of available therapies. This review aims to discuss the current knowledge, outcomes and challenges for prenatal intervention for congenital heart disease. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Congenital Heart Disease in Children)
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