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Perspectives on Congenital Heart Disease from Children to Adults
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Perspectives on Congenital Heart Disease from Children to Adults

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Cardiology".

Deadline for manuscript submissions: 30 June 2025 | Viewed by 2380

Special Issue Editor

Prof. Dr. Raffaele Giordano

E-Mail Website
Guest Editor
Department of Advanced Biomedical Science, Division of Adult and Pediatric Cardiac Surgery, University of Naples Federico II, Via Pansini 5, Napoli, Naples, Italy
Interests: cardiovascular sciences; adult and pediatric cardiac surgery; congenital heart disease
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Although a small percentage of specialists are dedicated to the management of congenital heart disease, this Special Issue focuses on congenital heart disease in newborns and adults. Over the decades, advances in the treatment of these malformations have led to an increase in the lifespan of this population. As a result, today, an important part of patients treated for congenital heart disease are adults. The aim of this Special Issue is to cover the latest developments in the diagnosis and management of fetuses, newborns, children, adolescents, and adults and how these patients should be treated. Moreover, we also aim to describe the results of the follow-up and whether there are any special needs, from short- to long-term. We welcome original or other manuscripts covering all aspects of congenital heart disease, including embryology and anatomy, physiology and pharmacology, pathology, genetics, radiology, clinical aspects, investigative cardiology, electrophysiology and echocardiography, and cardiac surgery.

Prof. Dr. Raffaele Giordano
Guest Editor

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. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly 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 2600 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
  • pediatric cardiac surgery
  • pediatric cardiology
  • children
  • adult congenital heart disease

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Related Special Issue

Published Papers (4 papers)

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13 pages, 1170 KiB  
Article
Long-Term Outcomes After Arterial Switch Operation for dextro-Transposition of the Great Arteries—30-Year Single-Center Experience
by Johanna Schlein, Clemens Ungerböck, Daniela Tertschnig, Alexandra Kaider, Barbara Karner, Clemens Atteneder, Erhan Urganci, Paul Werner, Eva Base, Peter Murin and Daniel Zimpfer
J. Clin. Med. 2025, 14(9), 3160; https://doi.org/10.3390/jcm14093160 - 2 May 2025
Viewed by 241
Abstract
Background/Objectives: The arterial switch operation (ASO) has been performed in the neonatal period as an anatomical correction for d-transposition of the great arteries since the 1980s. As the population of adult congenital heart disease patients grows, it is essential for healthcare providers [...] Read more.
Background/Objectives: The arterial switch operation (ASO) has been performed in the neonatal period as an anatomical correction for d-transposition of the great arteries since the 1980s. As the population of adult congenital heart disease patients grows, it is essential for healthcare providers across various disciplines to comprehend the complexities of these patients. We report on outcomes up to the third decade after ASO. Methods: All patients who underwent ASO from May 1985 to December 2020 were included, and a retrospective chart review with follow-up until December 2021 was conducted. Additionally, vital status verification (90.3% complete) was performed through the national health insurance. Survival analysis and competing risk analysis were performed to determine outcomes in the third decade after ASO. Results: One-hundred-ninety-five patients (72.3% male; median age at surgery 6 days; median weight at surgery 3.4 kg) underwent ASO from May 1985 to December 2020. Patients with a prenatal diagnosis showed a lower rate of preoperative cardiac decompensation (p = 0.017). Early in-hospital mortality was 8.7%, and no early in-hospital deaths occurred in the study era from 2006 to 2020. Four late deaths occurred, and the Kaplan–Meier estimated survival of the 178 hospital survivors was 98.3% ± 1.2% at 10 years, 96.8% ± 1.9% at 20 years and 92.4% ± 4.7 at 30 years. The cumulative incidence of left ventricular outflow tract (LVOT) reoperation after ASO was 1.3% at 10 years, 3.4% at 20 years and 11.3% at 30 years. The cumulative incidence of right ventricular outflow tract (RVOT) reoperation after ASO was 7.2% at 10 years and 17.5% at 20 and 30 years. Conclusions: Overall long-term survival of the hospital survivors is good. The reoperation rate on the LVOT is favorable. Percutaneous interventions, if feasible after the Lecompte maneuver, might pose an option to delay the more common reoperations on the RVOT, though further studies are needed to determine their long-term impact. Full article
(This article belongs to the Special Issue Perspectives on Congenital Heart Disease from Children to Adults)
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18 pages, 7580 KiB  
Article
The Influence of Social Determinants of Health on the Survival of Heart Transplants in the Pediatric Age: An Analysis of a Mexican Cohort and Its Comparison with Latin America and the Caribbean
by Horacio Márquez-González, Alejandro Bolio-Cerdán, Sergio Ruiz-González, Julio Erdmenger-Orellana, Carlos Alcántara-Noguez, Ma Pueblito Patricia Romero-Cárdenas, Diana Avila-Montiel and Solange Gabriela Koretzky
J. Clin. Med. 2025, 14(5), 1506; https://doi.org/10.3390/jcm14051506 - 24 Feb 2025
Viewed by 602
Abstract
Background/Objectives: A heart transplantation (HT) is the definitive treatment for heart failure. There is a difference in the success between national HT programs in developed countries and those in Central America, South America, and the Caribbean (LAC), and social determinants of health (SDHs) [...] Read more.
Background/Objectives: A heart transplantation (HT) is the definitive treatment for heart failure. There is a difference in the success between national HT programs in developed countries and those in Central America, South America, and the Caribbean (LAC), and social determinants of health (SDHs) can directly influence this. The objectives of this study were to describe the survival since the beginning of the HT program of a national pediatric institute in Mexico City and to compare it with the results of a systematic review of LAC. Methods: A cohort study of a pediatric hospital (which performed 42% of the pediatric HTs in Mexico) was performed since the beginning of the HTs program in 2001. Clinical variables related to the transplants were identified, and the SDHs were divided into three categories: personal, family, and community. A systematic literature review was performed using keywords and a search in the medical indexes of LAC countries. The statistical analysis included descriptive statistics and a bivariate survival analysis. A risk calculation was estimated using the hazard ratio (HR) of the SDHs. Results: A total of 38 HTs were performed, the median age was 7 (4–16) years, and 22 (58%) were men. The leading cause was cardiomyopathy in 20 (53%) cases. The first-year survival rate was 76.3 per 100 HTs. The SDHs that increased the risk of death were suboptimal immunosuppression, the persistence of malnutrition, parental education, the distance from the center, the socioeconomic level, and the absence of transitional care. Conclusions: This cohort of pediatric patients with HTs resulted in the identification of risk variables of personal and community SDHs for mortality in the first and fifth years. Chronic rejection occurred in 50%, and the absence of transitional care to adulthood was the variable with the highest risk. The systematic review identified Mexico as the country with the second-highest frequency of HTs, and our cohort represented 42% of the total number of transplants in the country. Numerically, LAC has a lower frequency of transplants and survival in the first year compared to other developed countries, possibly due to a gap associated with organizational justice. Full article
(This article belongs to the Special Issue Perspectives on Congenital Heart Disease from Children to Adults)
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16 pages, 7788 KiB  
Article
Transforming Cardiac Imaging: Can CT Angiography Replace Interventional Angiography in Tetralogy of Fallot?
by Ali Nazım Güzelbağ, Serap Baş, Muhammet Hamza Halil Toprak, Demet Kangel, Şenay Çoban, Selin Sağlam and Erkut Öztürk
J. Clin. Med. 2025, 14(5), 1493; https://doi.org/10.3390/jcm14051493 - 23 Feb 2025
Viewed by 502
Abstract
Background: Tetralogy of Fallot (TOF) is a complex congenital heart condition characterized by four major anatomical abnormalities. Accurate preoperative imaging is critical for optimal surgical outcomes, with transthoracic echocardiography (TTE), computed tomography angiography (CTA), and conventional catheter angiography (CCA) being the primary diagnostic [...] Read more.
Background: Tetralogy of Fallot (TOF) is a complex congenital heart condition characterized by four major anatomical abnormalities. Accurate preoperative imaging is critical for optimal surgical outcomes, with transthoracic echocardiography (TTE), computed tomography angiography (CTA), and conventional catheter angiography (CCA) being the primary diagnostic tools. This study aimed to compare the diagnostic utility of TTE, CTA, and CCA in preoperative evaluations of TOF patients, focusing on anatomical parameters, imaging accuracy, and patient outcomes. Methods: A retrospective, single-center analysis included TOF patients under one year of age who underwent complete repair between January 2021 and December 2024. Preoperative imaging with TTE, CTA, and CCA was analyzed for parameters including pulmonary artery diameters, Nakata index, McGoon ratio, and Z-scores. Radiation exposure, procedure duration, contrast volume, and complications were documented. Statistical analyses were performed to assess the comparative accuracy and safety of these modalities. Results: All patients underwent TTE (n = 127), while CTA was performed in 86 patients and CCA in 41 patients. Among 127 patients, 62% were male, with a mean age of 5.81 ± 2.15 months. On TTE, CTA and CCA provided statistically similar measurements of the pulmonary annulus, main pulmonary artery, and branch diameters, with no significant differences in the Nakata index and McGoon ratio. CTA had a shorter procedure duration (3.1 ± 0.58 min) and lower radiation dose (1.19 ± 0.22 mSv) compared to CCA (20.73 ± 11.12 min; 5.48 ± 1.62 mSv). CTA successfully identified major aortopulmonary collateral arteries (MAPCAs) in 10% of patients and detected additional pulmonary pathologies, such as subsegmental atelectasis in 12%. Access site complications were observed in 10% of CCA cases but were absent in CTA evaluations. Conclusions: CTA emerges as a highly effective and non-invasive alternative to CCA for preoperative assessment of TOF, offering comparable anatomical accuracy with significantly reduced procedural risks, radiation exposure, and contrast volume. Combining TTE and CTA provides comprehensive diagnostic coverage, minimizing the need for invasive procedures and enhancing surgical planning. These findings underscore the evolving role of CTA in the management of congenital heart disease, contributing to improved patient safety and outcomes. Full article
(This article belongs to the Special Issue Perspectives on Congenital Heart Disease from Children to Adults)
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16 pages, 831 KiB  
Systematic Review
Aortopexy for Tracheomalacia in Children: A Systematic Review and Meta-Analysis
by Andrea Zanini, Stefano Mazzoleni, Luigi Arcieri, Raffaele Giordano, Stefano Marianeschi and Francesco Macchini
J. Clin. Med. 2025, 14(4), 1367; https://doi.org/10.3390/jcm14041367 - 18 Feb 2025
Viewed by 609
Abstract
Introduction: Aortopexy is the most common surgical option for the treatment of severe tracheomalacia (TM) in children. Despite being described over 75 years ago, several aspects of the procedure remain controversial. Materials and Methods: A systematic review of aortopexy was conducted following the [...] Read more.
Introduction: Aortopexy is the most common surgical option for the treatment of severe tracheomalacia (TM) in children. Despite being described over 75 years ago, several aspects of the procedure remain controversial. Materials and Methods: A systematic review of aortopexy was conducted following the PRISMA guidelines. All original articles describing at least one case of aortopexy performed in children for the treatment of TM were included. Patients’ characteristics, surgical indications, approaches and details were collected. Outcomes were reviewed, and potential factors associated with procedural success were investigated. Results: Of 243 papers, 17 were included in this review, encompassing a total of 473 patients who underwent aortopexy. Of these, 65.3% were male, with a mean age at surgery of 12.2 months (5 days–18 years). Primary TM accounted for 7.9% of cases, while 92.1% were secondary to other anomalies, including esophageal atresia (54.1%), aberrant innominate artery (16.1%) and vascular rings (8.3%). There is a general consensus on the surgical indication for TM with severe symptoms, although the definition of severity is not well established. Overall success was 84%, but 40.8% of patients experienced some persistent symptoms. Sternotomy and thoracotomy were the most successful approaches (92.6% and 84.2%, respectively). Thoracoscopy had a similar success rate to sternotomy when pericardiotomy, thymectomy and pledgeted suture were performed (86.4% vs. 92.6% p = 0.41). Conclusions: Aortopexy is associated with good outcomes, but no evidence-based guidelines are currently available. Success appears to be associated with specific surgical steps rather than the surgical approach. Prospective studies are desirable for establishing precise guidelines. Full article
(This article belongs to the Special Issue Perspectives on Congenital Heart Disease from Children to Adults)
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