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Current Challenges in Adult Congenital Heart Diseases
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Current Challenges in Adult Congenital Heart Diseases

A Special Issue of Journal of Clinical Medicine (ISSN 2077-0383) belonging to the section "Cardiology".

Deadline for manuscript submissions: 20 March 2027 | Viewed by 2279

Editor

Prof. Dr. Agata Bielecka-Dabrowa

E-Mail Website
Guest Editor
1. Department of Cardiology and Congenital Diseases of Adults, Polish Mother's Memorial Hospital Research Institute (PMMHRI), 93-338 Lodz, Poland
2. Department of Preventive Cardiology and Lipidology, Medical University of Lodz, Lodz, Poland
Interests: heart failure; body mass compartments, sarcopenia, obesity, echocardiography; congenital heart diseases; cardiovascular diseases in pregnancy; hypertension
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Advances in neonatal cardiac surgery and significant improvements in medical therapy over recent decades have reshaped the landscape of congenital heart disease (CHD). As a result, the population of adults living with CHD (ACHD) now exceeds that of children, creating a rapidly growing and uniquely complex patient group. This demographic shift brings new challenges that extend well beyond childhood and into every stage of adult life.

Among the most pressing issues is the transition from pediatric to adult-centered care, which is complicated by a shortage of ACHD specialists and loss to follow-up. Many adults with congenital heart disease experience diminished quality of life due to residual lesions, late complications, and psychosocial burdens. Pregnancy poses additional risks and requires careful preconception counseling and coordinated management. Furthermore, heart failure and arrhythmias are leading causes of morbidity and mortality in this population. These conditions often develop earlier in ACHD patients than in patients with acquired heart disease. Addressing these challenges demands truly multidisciplinary care, drawing on cardiology, cardiac surgery, imaging, electrophysiology, mental health, reproductive medicine, and beyond.

This Special Issue invites original research and comprehensive reviews that will enhance clinicians’ understanding and support their everyday practice in managing adults with congenital heart disease.

Prof. Dr. Agata Bielecka-Dabrowa
Guest Editor

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Keywords

  • adult congenital heart disease
  • quality of life
  • pregnancy in congenital heart disease
  • heart failure
  • arrhythmia
  • septal defect
  • congenital valve stenosis
  • coarctation of the Aorta
  • Fontan circulation
  • Tetralogy of Fallot

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Published Papers (3 papers)

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Research

13 pages, 253 KB  
Article
Predictors of Higher Peak Exercise Oxygen Uptake in a Cohort of Adult Patients with Fontan Circulation
by Andrzej Wittczak, Mateusz Kobierecki, Maciej Banach and Agata Bielecka-Dabrowa
J. Clin. Med. 2026, 15(10), 3805; https://doi.org/10.3390/jcm15103805 - 15 May 2026
Viewed by 398
Abstract
Background/Objectives: Percent achieved of predicted peak exercise oxygen uptake (%VO2pred) is a prognostic factor for patients with Fontan circulation. The main purpose of this study was to determine predictors of higher %VO2pred in a cohort of adult Fontan patients. [...] Read more.
Background/Objectives: Percent achieved of predicted peak exercise oxygen uptake (%VO2pred) is a prognostic factor for patients with Fontan circulation. The main purpose of this study was to determine predictors of higher %VO2pred in a cohort of adult Fontan patients. Methods: Medical records of 50 adult Fontan patients who underwent cardiopulmonary exercise testing were reviewed. All patients were divided into two groups according to the mean value of %VO2pred and, in separate analysis, according to the morphology of the systemic ventricle. Spearman’s rank correlation was used to examine the relationship between %VO2pred and blood biomarkers. Regression analyses were used to identify predictors of %VO2pred. Results: The median age of all patients was 22 years, and 50% were female. The systemic ventricle was dominant right in 23 patients. Negative correlations were found between %VO2pred and N-terminal prohormone of brain natriuretic peptide, mean cell hemoglobin concentration, and ferritin, and positive correlations were found between %VO2pred and total protein, total iron-binding capacity. Higher chronotropic index [CI] (β = 0.31; p = 0.009), higher maximal diastolic blood pressure during CPET [DBPmax] (β = 0.4; p = 0.001), and lower serum concentration of high-sensitivity troponin T [hsTnT] (β = −0.31; p = 0.007) were significantly and independently associated with %VO2pred. Conclusions: Higher CI and DBPmax and lower hsTnT were identified as independent predictors of %VO2pred in this cohort. These findings suggest that the absence of chronotropic incompetence is a positive predictor of exercise capacity. Furthermore, hsTnT shows potential as a useful biomarker in this population. Further studies are needed to validate these parameters for clinical assessment and risk stratification in Fontan patients. Full article
(This article belongs to the Special Issue Current Challenges in Adult Congenital Heart Diseases)
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Graphical abstract

14 pages, 1257 KB  
Article
Left Ventricular Hypertrabeculation and Ventricular Arrhythmias
by Michele Alfieri, Samuele Principi, Alessandro Barbarossa, Federico Paolini, Lorenzo Torselletti, Francesca Coraducci, Sara Belleggia, Francesca Coretti, Paolo Compagnucci, Giulia Stronati, Michela Casella, Antonio Dello Russo and Federico Guerra
J. Clin. Med. 2026, 15(9), 3464; https://doi.org/10.3390/jcm15093464 - 1 May 2026
Viewed by 506
Abstract
Background/Objectives: Excessive trabeculation of the left ventricle, previously known as left ventricular non-compaction (LVNC), is a rare phenotypic trait whose mechanisms and pathogenesis still remain conflictual. Its presentations may range from heart failure to embolism and, most importantly, ventricular arrhythmias (VAs). This [...] Read more.
Background/Objectives: Excessive trabeculation of the left ventricle, previously known as left ventricular non-compaction (LVNC), is a rare phenotypic trait whose mechanisms and pathogenesis still remain conflictual. Its presentations may range from heart failure to embolism and, most importantly, ventricular arrhythmias (VAs). This study aims to find novel predictive factors for the occurrence of potentially fatal VAs in patients with left ventricular hypertrabeculation. Methods: All consecutive patients meeting the echocardiographic (Chin, Jenny or Stöllberger) and/or MRI criteria (Petersen) for hypertrabeculation were prospectively enrolled from October 2009 to December 2023. The primary outcome was a composite of sudden cardiac death, sustained ventricular tachycardias (sVTs), ventricular fibrillation (VF) or appropriate implantable cardioverter defibrillator (ICD) interventions. The secondary outcome was a composite of cardiovascular death and cardiovascular hospitalizations. Results: Overall, 64 patients (41 males, mean age 46 ± 19 years old) were enrolled and followed for a median time of 2.2 years. Six patients (9.4%) experienced a composite outcome at eight years, three with previous sVTs and three with previous non-sustained VTs (nsVTs). The strongest predictor of the primary endpoint was the anamnesis of nsVTs and sVTs before LVNC diagnosis. In addition, nsVTs and sVTs were significantly associated with the secondary outcome. Conclusions: Hypertrabeculation of the left ventricle is a complex and poorly understood condition whose status of cardiomyopathy is currently challenged. In our population, patients with a trabecular pattern experienced a high incidence of VAs, cardiovascular death and hospitalizations. VAs before LVNC diagnosis were predictive of the outcome independently from systolic function. Full article
(This article belongs to the Special Issue Current Challenges in Adult Congenital Heart Diseases)
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11 pages, 226 KB  
Article
Cardiorenal Biomarkers and Cerebrovascular Risk in Patients with Congenital Heart Disease
by Efrén Martínez-Quintana and Fayna Rodríguez-González
J. Clin. Med. 2026, 15(6), 2440; https://doi.org/10.3390/jcm15062440 - 23 Mar 2026
Viewed by 658
Abstract
Background/Objectives: Adults with congenital heart disease (CHD) have a substantially higher risk of ischemic stroke than the general population. Circulating biomarkers such as N-terminal pro B-type natriuretic peptide (NT-pro-BNP), high-sensitivity C-reactive protein (hs-CRP), and microalbuminuria have been associated with adverse cardiovascular outcomes [...] Read more.
Background/Objectives: Adults with congenital heart disease (CHD) have a substantially higher risk of ischemic stroke than the general population. Circulating biomarkers such as N-terminal pro B-type natriuretic peptide (NT-pro-BNP), high-sensitivity C-reactive protein (hs-CRP), and microalbuminuria have been associated with adverse cardiovascular outcomes in CHD, but their role in predicting cerebrovascular events remains uncertain. Methods: Prospective cohort study including 372 adults with CHD [median age 34 years (IQR 23–42); 57.8% male] followed at a tertiary center between 2017 and 2022. Baseline assessments included demographic characteristics, CHD anatomical complexity, cardiovascular risk factors, NT-pro-BNP, hs-CRP, lipid profile, and 24-h urinary albumin excretion. The primary endpoint was incident ischemic stroke during a median follow-up of 6.3 years (IQR 3.9–8.3). Univariable Cox proportional hazards models were used to identify predictors of stroke. Results: During follow-up, 13 patients (3.5%) experienced ischemic stroke. Patients with stroke were significantly older [51 (46–64) vs. 30 (23–40) years; p < 0.001] and had a higher prevalence of dyslipidemia (61.5% vs. 15.0%; p < 0.001). NT-pro-BNP levels were markedly higher in patients with stroke [369 (218–604) vs. 64 (21–172) pg/mL; p < 0.001]. No significant differences were observed between groups in renal function parameters, hs-CRP, thyroid-stimulating hormone, or urinary albumin excretion rate. In Cox analyses, older age and dyslipidemia were the strongest predictors of stroke (p < 0.001). Arterial hypertension, diabetes mellitus, and higher NT-pro-BNP levels were also associated with increased stroke risk (p < 0.05), whereas CHD anatomical complexity, NYHA functional class, and cyanosis were not. Conclusions: In adults with CHD, ischemic stroke was mainly associated with traditional cardiovascular risk factors and elevated NT-pro-BNP levels rather than anatomical disease complexity or functional status. Full article
(This article belongs to the Special Issue Current Challenges in Adult Congenital Heart Diseases)
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