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Management of Congenital Heart Disease (CHD)

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

Deadline for manuscript submissions: 20 August 2026 | Viewed by 3504

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Special Issue Editors

Dr. Sarah Nordmeyer

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Guest Editor

Department of Diagnostic and Interventional Radiology, Tübingen University Hospital, Tübingen, Germany
Interests: cardiac magnetic resonance imaging; aortic valve stenosis; virtual treatment planning; myocarditis

Dr. Marie Schafstedde

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Guest Editor

1. Department of Congenital Heart Disease—Pediatric Cardiology, German Heart Center Charité, 13353 Berlin, Germany
2. Institute of Computer-Assisted Cardiovascular Medicine, German Heart Center Charité, 13353 Berlin, Germany
Interests: precisine medicine; virtual therapy planning; in-silico modeling; congenital heart disease

Special Issue Information

Dear Colleagues,

Congenital heart defects affect approximately 1% of all newborns. Owing to significant advances in diagnosis, surgical techniques, and perioperative care, more than 90% of affected individuals now reach adulthood. This remarkable progress has shifted the clinical focus towards lifelong management and the optimisation of long-term outcomes.

This Special Issue invites high-quality submissions spanning the full spectrum of congenital heart disease, including innovations in imaging, intervention, surgery, and personalised care. We particularly encourage contributions that explore emerging technologies such as 3D modelling, virtual planning, and molecular diagnostics, as well as research aimed at refining patient pathways and enhancing quality of life.

We warmly welcome your valuable contributions to this evolving and impactful field.

Dr. Sarah Nordmeyer
Dr. Marie Schafstedde
Guest Editors

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Keywords

congenital heart disease
imaging
diagnostics
surgical innovations
minimally invasive interventions

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Published Papers (5 papers)

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Research

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15 pages, 1314 KB

Open AccessArticle

Clinical Benefit of Percutaneous Treatment of Fontan Pathway Obstructions

by Anastasia Schleiger, Julia Moosmann, Damian Schaffner, Marie Schafstedde, Jan Brühning, Paul Spiesecke, Hans Peter Müller, Stanislav Ovroutski, Felix Berger and Peter Kramer

J. Clin. Med. 2026, 15(6), 2240; https://doi.org/10.3390/jcm15062240 - 16 Mar 2026

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Abstract

Background/Objectives: Fontan pathway obstructions are a common complication during long-term follow-up after Fontan surgery. The clinical or hemodynamic benefit of percutaneous treatment of Fontan stenoses is poorly understood. In this study we analyzed the feasibility and clinical effects of percutaneous treatment of Fontan pathway obstructions. Methods: From April 2019 to December 2024 35 Fontan patients received percutaneous treatment of Fontan pathway obstructions by stent implantation. Indications for treatment included clinical signs of lower venous congestion or PLE and/or a moderate-to-severe morphologic pathway stenosis independent of clinical sequelae. Median follow-up time after the intervention was 1.5 years [IQR 0.7; 2.2]. Results: Median patient age was 20.3 years [IQR 16.3; 26.8]. Interventional success was defined as a significant increase in median indexed Fontan conduit cross sectional area and was achieved in all patients by expanding from 98.4 mm²/m² [IQR 80.7; 115.5] to 145.1 mm²/m² [IQR 134.8; 167.0, p < 0.001]. In symptomatic patients, a significant clinical improvement was detected 6 months after the intervention including an amelioration of physical capacity assessed by NYHA classification (p = 0.039) and cardiopulmonary exercise testing (VO_2peak; p = 0.025). Global assessment of Fontan-associated liver disease (FALD) showed improvement during follow-up (p = 0.035). No peri- or postprocedural complications occurred. Conclusions: Percutaneous treatment of Fontan pathway obstruction has a high interventional success rate with a significant increase in indexed cross-sectional area. During follow-up, improvement of patients’ clinical condition and global signs of FALD were observed. The impact of percutaneous Fontan pathway obstruction relief on reversing or delaying the progression of FALD requires further investigation. Full article

(This article belongs to the Special Issue Management of Congenital Heart Disease (CHD))

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16 pages, 2265 KB

Open AccessArticle

Development and Validation of an Interpretable Model for Predicting Postoperative Hyperlactatemia in Young Children Following Congenital Heart Surgery

by Yuchan Chen, Wenxin Ge, Lixin Hu, Jiaqi Chen and Yajun Chen

J. Clin. Med. 2026, 15(5), 1846; https://doi.org/10.3390/jcm15051846 - 28 Feb 2026

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Abstract

Objectives: Postoperative hyperlactatemia (POHL) is a common complication after pediatric cardiac surgery, yet its perioperative risk factors remain unclear. This study developed and internally validated an interpretable machine learning (ML) model to identify young children at risk for POHL. Methods: We retrospectively analyzed 3224 children aged 0 to 36 months from 2018 to 2023. Four ML models, including logistic regression (LR), random forest (RF), support vector machine (SVM), and eXtreme Gradient Boosting (XGBoost), were trained and validated. Model performance was assessed using discrimination, calibration, and classification metrics, and decision curve analysis evaluated clinical utility. SHapley Additive exPlanation (SHAP) provided both global and local interpretability. Results: Of the 3224 children, 731 (22.7%) developed POHL, with a median age of 5 months. The RF model performed best (AUC, 0.821; 95% CI, 0.787–0.854; sensitivity, 69.7%; specificity, 84.1%; Brier score, 0.146). SHAP analysis identified 8 key predictors of POHL. Established factors included cardiopulmonary bypass duration, lowest bypass temperature, epinephrine dose, and RACHS-1 category. Novel contributors comprised low body weight, reduced left ventricular end-diastolic diameter, plasma transfusion, and continued mechanical ventilation within the first 24 postoperative hours. Conclusions: We developed and internally validated an interpretable RF model that integrates established and novel predictors to estimate POHL risk in young children after cardiac surgery. Pending external validation, it may support earlier risk recognition and more personalized perioperative management in this high-risk pediatric population. Full article

(This article belongs to the Special Issue Management of Congenital Heart Disease (CHD))

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21 pages, 2026 KB

Open AccessArticle

Early Detection of Acute Kidney Injury After Congenital Heart Surgery—Using Urine Proteomics to Identify New Biomarker Candidates: A Prospective Clinical Study

by Raphael Seiler, Alexa Leona Herre, Marieluise Kirchner, Matthias Ziehm, Philipp Mertins, Felix Berger, Joachim Photiadis, Giang Tong, Liliya Brankova, Katharina R. L. Schmitt and Jana Lücht

J. Clin. Med. 2025, 14(22), 8253; https://doi.org/10.3390/jcm14228253 - 20 Nov 2025

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Abstract

Background: Acute kidney injury (AKI) is a frequent complication following congenital heart surgery and is associated with increased morbidity and mortality. Early recognition is crucial, yet standard clinical biomarkers result in delayed detection. Urine, which can be collected non-invasively, offers unique insights into kidney function and systemic responses. Methods: This prospective clinical study aimed to identify novel urinary biomarkers for the early detection of AKI, using high-accuracy proteome profiling. Patients with congenital heart disease undergoing cardiac surgery at Deutsches Herzzentrum der Charité were included in the study. Urine samples were collected at four timepoints: preoperatively and immediately postoperatively, and then again at six and 24 h post-surgery. Samples were analyzed using high-accuracy mass spectrometry. Linear models were applied to identify proteins associated with AKI. Results: A total of 67 patients with a median age of two years were included, of whom thirteen (19%) developed an AKI. Fifteen potential urinary biomarkers were identified. The most promising early indicators of AKI directly after surgery across all age groups were Chitotriosidase-1 (AUC 0.79; 95% CI:0.64–0.94), Kallikrein-1 (AUC 0.76; 95% CI:0.76–0.89), and Carbonic anhydrase 3 (AUC 0.73; 95% CI:0.6–0.87). Conclusions: High-accuracy mass spectrometry urine proteome profiling enabled the identification of potential new AKI biomarkers directly after congenital heart surgery. Utilization of the urinary markers Chitotriosidase-1, Kallikrein-1 and Carbonic anhydrase 3 has the potential to enable earlier detection of patients at risk for AKI. Further validation in larger, age-stratified pediatric cohorts is required to confirm the diagnostic utility of the identified urinary biomarker candidates. Full article

(This article belongs to the Special Issue Management of Congenital Heart Disease (CHD))

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10 pages, 220 KB

Open AccessArticle

Bronchoscopy in the Pediatric Cardiovascular Patient with Persistent Respiratory Pathology

by Ana-Belen Ariza-Jimenez, Delia Valverde Montoro, Pilar Caro Aguilera, Estela Perez Ruiz and Francisco Javier Perez Frias

J. Clin. Med. 2025, 14(18), 6606; https://doi.org/10.3390/jcm14186606 - 19 Sep 2025

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Abstract

Background: Patients with congenital heart disease can associate malformations. The most frequent complications are those related to the airways, which produce prolonged cardiovascular postoperative. Objectives: Describe pathology, bronchoscopy indications, and findings in patients with heart pathology and persistent breath failure to improve prognosis and determine an early treatment. Methods: Retrospective descriptive study of bronchoscopies performed during 24 years in pediatric patients with congenital heart disease with surgery indication and persistent respiratory symptomatology. Results: We performed 199 fibrobronchoscopies in 144 patients, with an average of 1.4 fibrobronchoscopies per patient. A total of 58% were male. The mean age was 27.5 months (5 days–13 years). Valvular disease was the most frequent congenital heart disease, followed by the transposition of large vessels. The most frequent indications were stridor (42.7%) and persistent atelectasis (24.6%), followed by extubation failure (12.4%) and pump output (6.2%). The majority of the findings were found in the upper airway (56%), with a clear predominance of malacias (32%), while in the lower airway, extrinsic compression was highlighted (42%). Conclusions: Flexible fiberoptic bronchoscopy is a useful and rapid method for the diagnosis of airway malformations associated with congenital heart diseases that may have a relevant role in its management and prognosis. Full article

(This article belongs to the Special Issue Management of Congenital Heart Disease (CHD))

Review

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15 pages, 621 KB

Open AccessReview

Pulmonary Valve Replacement: Update on Timing and Ventricular Remodelling

by Almudena Ortiz-Garrido, Monika Różewicz Juraszek, Dominik Daniel Gabbert, Jill Jussli-Melchers and Inga Voges

J. Clin. Med. 2026, 15(3), 1295; https://doi.org/10.3390/jcm15031295 - 6 Feb 2026

Viewed by 655

Abstract

Chronic pulmonary regurgitation (PR) after the repair of tetralogy of Fallot (TOF) and other right ventricular outflow tract (RVOT) interventions leads to progressive right ventricular (RV) dilatation, altered ventricular–ventricular interaction, and an increased risk of arrhythmia and heart failure. Pulmonary valve replacement (PVR), whether surgical or transcatheter, effectively eliminates or reduces PR and is associated with short- and mid-term improvement in RV size, symptoms, and electrocardiographic markers. However, the optimal timing of intervention remains unresolved: operating late can result in irreversible myocardial damage and arrhythmogenic substrates, whereas operating early can lead to repeated reinterventions, the impact of which on hard outcomes is uncertain. This review summarizes contemporary evidence on ventricular remodelling after PVR, focusing on cardiovascular magnetic resonance (CMR) and echocardiographic markers, and critically appraises proposed criteria for timing PVR. Classic CMR-derived thresholds (RV end-diastolic volume index [RVEDVi] 150–170 mL/m², RV end-systolic volume index [RVESVi] 80–90 mL/m²) and QRS duration cut-offs are discussed alongside emerging markers of risk, including the RV mass-to-volume ratio, diffuse myocardial fibrosis (extracellular volume fraction), strain imaging, and diastolic dysfunction. Meta-analyses show consistent reverse remodelling and symptomatic benefit after PVR, but no conclusive survival benefit has been demonstrated, and data on arrhythmic outcomes remain conflicting. Key gaps include (i) the lack of prospective randomized or carefully matched comparative studies of “early” versus “deferred” PVR; (ii) limited understanding of how myocardial fibrosis, RV hypertrophy, and diastolic dysfunction interact with volume load and timing to influence long-term outcomes; (iii) under-representation of adult and older adult TOF cohorts; and (iv) insufficient integration of multiparametric risk scores and machine-learning approaches into clinical decision-making. Future research should prioritize multicentre longitudinal cohorts with standardized imaging, electrophysiological and clinical endpoints, incorporate advanced imaging techniques (e.g., strain, 3D late gadolinium enhancement, and T1 mapping), and explore precision-medicine strategies to individualize PVR timing. Full article

(This article belongs to the Special Issue Management of Congenital Heart Disease (CHD))

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