Clinical Applications of Novel Tools to Personalize the Follow-Up and Predict the Outcomes in Congenital Heart Disease

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

Deadline for manuscript submissions: closed (20 March 2024) | Viewed by 994

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Guest Editor
Department of Pediatric Cardiology, Cardiac Surgery and Heart Lung Transplantation, Bambino Gesu Children’s Hospital and Research Institute, IRCCS, Rome, Italy
Interests: pulmonary valve replacement; tetralogy of Fallot; genetic abnormalities; QRS fragmentation; cardiopulmonary stress test in tetralogy of Fallot; congenital heart disease
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Guest Editor
Pediatric Cardiology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
Interests: arrhythmias; heart and lung transplantation, biomarkers, artificial intelligence

Special Issue Information

Dear Colleagues,

In recent years, there has been growing interest in identifying tools that can be used by clinicians to help predict the outcome of patients with congenital heart disease (CHD). In fact, although a clinical assessment is fundamental to understand the patient's state of health, the use the artificial intelligence to standardize, for example, biventricular volumes analysis with cardiovascular magnetic resonance imaging (MRI) can save our clinicians time and decrease contour errors, such as of the right ventricle measurement which remains difficult to standardize (even with MRI). In addition, nowadays, clinicians can go into detail in cardiac shape modeling using machine learning to visualize abnormal mechanisms underlying dysfunction in heart disease, which was not previously possible with other methods. Similarly, the use of 4D flow MRI in bicuspid aortic valve disease allows clinicians to understand the anomalous direction of the blood flow over time highlighting patients at risk of developing a progressive dilatation of the ascending aorta. Furthermore, being able to calculate the aortic wall shear stress provides precise data regarding the distensibility in different aortic regions of interest (ROI), thereby hypothesizing which site is most at risk of aortic dissection. Lately, clinicians have been using strain analysis either with echocardiography or with cardiac MRI to anticipate the worsening of cardiac performance before the common method of estimating ejection fraction to predict adverse events in CHD. Finally, at present, we are also considering the use of biomarker-based risk models (interleukin -8 (IL-8), chemokine ligand 3 (CCL3)) to predict persistent multiple organ dysfunctions after congenital heart surgery.

Thus, the aim of this issue is to encourage researchers to present original works on how these new tools can be used in clinical practice to optimize the management of patients with CHD and reduce the incidence of adverse events.

Dr. Benedetta Leonardi
Dr. Giorgia Grutter
Guest Editors

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Keywords

  • machine learning
  • artificial intelligence
  • strain analysis
  • biomarkers
  • aortic wall shear stress
  • 4D flow

Published Papers (2 papers)

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Research

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10 pages, 628 KiB  
Article
Predicting Long-Term Childhood Survival of Newborns with Congenital Heart Defects: A Population-Based, Prospective Cohort Study (EPICARD)
by Makan Rahshenas, Nathalie Lelong, Damien Bonnet, Lucile Houyel, Babak Choodari-Oskooei, Mithat Gonen, Francois Goffinet and Babak Khoshnood
J. Clin. Med. 2024, 13(6), 1623; https://doi.org/10.3390/jcm13061623 - 12 Mar 2024
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Abstract
Backgroud: Congenital heart defects (CHDs) are the most frequent group of major congenital anomalies, accounting for almost 1% of all births. They comprise a very heterogeneous group of birth defects in terms of their severity, clinical management, epidemiology, and embryologic origins. Taking this [...] Read more.
Backgroud: Congenital heart defects (CHDs) are the most frequent group of major congenital anomalies, accounting for almost 1% of all births. They comprise a very heterogeneous group of birth defects in terms of their severity, clinical management, epidemiology, and embryologic origins. Taking this heterogeneity into account is an important imperative to provide reliable prognostic information to patients and their caregivers, as well as to compare results between centers or to assess alternative diagnostic and treatment strategies. The Anatomic and Clinical Classification of CHD (ACC-CHD) aims to facilitate both the CHD coding process and data analysis in clinical and epidemiological studies. The objectives of the study were to (1) Describe the long-term childhood survival of newborns with CHD, and (2) Develop and validate predictive models of infant mortality based on the ACC-CHD. Methods: This study wasbased on data from a population-based, prospective cohort study: Epidemiological Study of Children with Congenital Heart Defects (EPICARD). The final study population comprised 1881 newborns with CHDs after excluding cases that were associated with chromosomal and other anomalies. Statistical analysis included non-parametric survival analysis and flexible parametric survival models. The predictive performance of models was assessed by Harrell’s C index and the Royston–Sauerbrei RD2, with internal validation by bootstrap. Results: The overall 8-year survival rate for newborns with isolated CHDs was 0.96 [0.93–0.95]. There was a substantial difference between the survival rate of the categories of ACC-CHD. The highest and lowest 8-year survival rates were 0.995 [0.989–0.997] and 0.34 [0.21–0.50] for “interatrial communication abnormalities and ventricular septal defects” and “functionally univentricular heart”, respectively. Model discrimination, as measured by Harrell’s C, was 87% and 89% for the model with ACC-CHD alone and the full model, which included other known predictors of infant mortality, respectively. The predictive performance, as measured by RD2, was 45% and 50% for the ACC-CHD alone and the full model. These measures were essentially the same after internal validation by bootstrap. Conclusions: The ACC-CHD classification provided the basis of a highly discriminant survival model with good predictive ability for the 8-year survival of newborns with CHDs. Prediction of individual outcomes remains an important clinical and statistical challenge. Full article
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Review

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18 pages, 1583 KiB  
Review
Repaired Tetralogy of Fallot: Have We Understood the Right Timing of PVR?
by Benedetta Leonardi, Marco Perrone, Giuseppe Calcaterra, Jolanda Sabatino, Isabella Leo, Martina Aversani, Pier Paolo Bassareo, Alice Pozza, Lilia Oreto, Sara Moscatelli, Nunzia Borrelli, Francesco Bianco and Giovanni Di Salvo
J. Clin. Med. 2024, 13(9), 2682; https://doi.org/10.3390/jcm13092682 - 02 May 2024
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Abstract
Despite many advances in surgical repair during the past few decades, the majority of tetralogy of Fallot patients continue to experience residual hemodynamic and electrophysiological abnormalities. The actual issue, which has yet to be solved, is understanding how this disease evolves in each [...] Read more.
Despite many advances in surgical repair during the past few decades, the majority of tetralogy of Fallot patients continue to experience residual hemodynamic and electrophysiological abnormalities. The actual issue, which has yet to be solved, is understanding how this disease evolves in each individual patient and, as a result, who is truly at risk of sudden death, as well as the proper timing of pulmonary valve replacement (PVR). Our responsibility should be to select the most appropriate time for each patient, going above and beyond imaging criteria used up to now to make such a clinically crucial decision. Despite several studies on timing, indications, procedures, and outcomes of PVR, there is still much uncertainty about whether PVR reduces arrhythmia burden or improves survival in these patients and how to appropriately manage this population. This review summarizes the most recent research on the evolution of repaired tetralogy of Fallot (from adolescence onwards) and risk factor variables that may favor or delay PVR. Full article
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