The pace of innovation in congenital and pediatric cardiology continued to accelerate in 2025, shaped by practice‑defining guidelines, multicenter investigations, and growing bench‑to‑bedside translation. This year's highlights span four pillars: pediatric heart failure (HF), pediatric pulmonary hypertension (PH), Fontan circulation, and adult congenital heart disease (ACHD).

Pediatric Heart Failure

Pediatric HF Guidelines ¹

The 2025 International Society for Heart and Lung Transplantation (ISHLT) Guidelines for the Management of Pediatric HF provide an updated, evidence-based framework for managing HF in infants, children, and adolescents. This revision, the first since 2014, reflects advances in diagnostics, pharmacotherapy, and mechanical support strategies tailored to pediatric physiology.

Key updates include standardized evaluation protocols to guide therapy by incorporating biomarker levels (e.g., N-terminal pro–B-type natriuretic peptide), advanced imaging modalities such as echocardiographic strain and cardiac magnetic resonance imaging, and functional assessments. The guidelines emphasize structured titration of guideline-directed medical therapy (GDMT), including angiotensin-converting enzyme inhibitors, beta-blockers, and mineralocorticoid-receptor antagonists. The guidelines also introduce recommendations for angiotensin receptor–neprilysin inhibitors and cautious use of sodium-glucose cotransporter-2 (SGLT2) inhibitors in select pediatric populations under institutional oversight.

For advanced HF, the guidelines outline criteria for ventricular assist device implantation and heart transplant, balancing timing with growth and neurodevelopmental considerations. They also address perioperative management, immunosuppression strategies, and long-term follow-up, highlighting the importance of multidisciplinary care teams and family-centered decision-making.

In short, these guidelines provide clinicians with more tools to consistently identify HF severity, tailor therapy, and monitor patients using updated images and evaluations methods that were not available in 2014.

Pediatric Pulmonary Hypertension

Interventions After Optimal Pharmacotherapy Guideline ²

The 2025 American Thoracic Society (ATS) clinical practice guideline on Interventional Strategies for Children With Progressive PH Despite Optimal Therapy addresses interventional strategies for pediatric PH when optimal pharmacotherapy fails (phosphodiesterase-5 inhibitors, endothelin-receptor antagonists, prostacyclin pathway agents). A multidisciplinary panel, using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach, issued five conditional recommendations with very low certainty based on systematic reviews and expert opinion.

The key recommendations are:

• Atrial septostomy. Advised for children with progressive PH and right ventricular (RV) failure as a palliative measure to improve symptoms by decompressing the right heart.
• Pulmonary-to-systemic shunt (e.g., Potts shunt). Conditional recommendation for patients with suprasystemic RV pressure unresponsive to medical therapy.
• Lung transplant. Recommended for children with RV failure not improving with medical management.
• In patients supported on extracorporeal membrane oxygenation. Lung transplant is advised when RV failure persists without reversible causes. The guideline recommends against shunt creation in children receiving extracorporeal support due to risk/benefit concerns.

The panel emphasized that evidence is limited. Interventions carry notable risks, including procedural morbidity and resource constraints. The recommendations encourage multidisciplinary decision-making and shared decision-making with families. Both guideline documents outline gaps in evidence, calling for future pediatric-specific trials, standardized outcome reporting, and expanded registries.

Fontan Circulation

Extracardiac Conduit vs. Lateral Tunnel: 15‑Year Multicenter Outcomes ³

The FORCE (Fontan Outcomes Registry using CMR Examinations) investigators published reports of multiple studies in 2025. One study was a multicenter comparison at 15 years of extracardiac conduit (ECC) versus lateral tunnel (LT) Fontan operations. Data from multiple institutions included survival rates, incidence of arrhythmias, thromboembolic events, reinterventions, and end-organ complications. Both techniques demonstrated excellent overall survival, but differences emerged in morbidity profiles. Patients who underwent ECC operations showed lower rates of atrial arrhythmias than did those who underwent LT operations, likely due to reduced atrial suture lines and minimized atrial manipulation. Reintervention rates were similar, primarily for conduit or fenestration management. Liver dysfunction and protein-losing enteropathy occurred in both groups without significant difference. The study results emphasize that, although ECC operations may offer rhythm advantages, both techniques require lifelong surveillance for arrhythmias, thromboembolism, and organ dysfunction. Additionally, the importance of counseling families about the long-term complications of Fontan regardless of surgical approach is also highlighted in the study.

SGLT2 Inhibitors in Single‑Ventricle/Fontan Failure: Signal Generation ⁴

A study by Konduri et al. explored the off-label use of SGLT2 inhibitors in patients with single-ventricle physiology and Fontan circulatory failure. The retrospective analysis included pediatric and young adult patients prescribed dapagliflozin or empagliflozin as adjunct therapy to standard HF management. The rationale was based on potential benefits observed in adult HF, such as improved diuresis, reduced preload, and metabolic modulation. Overall, SGLT2 inhibitors were well tolerated, with few discontinuations. Reported adverse events were mild, including urinary tract infections and transient kidney changes; no severe complications occurred. Clinicians noted modest symptomatic improvements, such as better fluid balance and exercise tolerance, although objective hemodynamic or biomarker level changes were inconsistent. The investigators conclude that SGLT2 inhibitors may offer a promising adjunct for Fontan failure but emphasize the need for prospective trials to confirm efficacy and long-term safety.

Adult Congenital Heart Disease

2025 Guideline on ACHD: A Unified Framework for Lifelong Care ⁵

The 2025 multisociety Guideline for the Management of ACHD provides an updated, comprehensive framework for ACHD, addressing the growing population of >2 million individuals in the United States. The guideline emphasizes lifelong, multidisciplinary care and structured transition from pediatric to adult services. The key updates include:

• HF management in systemic RVs and left ventricles, including GDMT adaptation, pacing strategies, and inclusion of Fontan‑specific HF pathways.
• Arrhythmia care with refined indications for catheter ablation, device therapy, and anticoagulation stratified by anatomy/physiology.
• Pregnancy and contraception counseling with risk modeling and delivery planning, emphasizing ACHD team involvement.
• Mental health and neurocognitive assessment, exercise testing to enable participation, and structured transition/continuity frameworks to reduce care drop‑off.

The guideline emphasizes the complexity of the ACHD population and the need to provide lifelong systems of care that can offer consistent and comprehensive medical management.

In 2025, research in pediatric and congenital heart disease (CHD) focused on systems‑level advancement. These advances allow clinicians to standardize care, individualize therapy, and sustain outcomes across the lifespan. Similar advances for other pediatric and CHD subspecialities are on the horizon, and the authors anticipate that 2026 will bring updated guidelines in interventional cardiology and other innovative research that can empower clinicians to better understand and care for this unique population.

References

1. Irving C, Azeka E, Adorisio R, et al. The International Society for Heart and Lung Transplantation guidelines for the management of pediatric heart failure (update from 2014). J Heart Lung Transplant. 2025;44(10):e21-e71. doi:10.1016/j.healun.2025.06.003
2. Hayes D Jr, Jennerich AL, Coleman RD, et al. Interventional strategies for children with progressive pulmonary hypertension despite optimal therapy: an official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2025;211(2):157-173. doi:10.1164/rccm.202410-1901ST
3. Seese L, Schiff M, Morell VO, et al. Multicenter comparison of long-term outcomes: extracardiac conduit Fontan vs lateral tunnel Fontan at 15-year follow-up. Ann Thorac Surg. 2025;120(6):1115-1123. doi:10.1016/j.athoracsur.2025.07.059
4. Konduri A, West C, Lowery R, et al. Experience with SGLT2 inhibitors in patients with single ventricle congenital heart disease and Fontan circulatory failure. Pediatr Cardiol. 2025;46(1):81-88. doi:10.1007/s00246-023-03332-5
5. Gurvitz M, Krieger EV, Fuller S, et al. 2025 ACC/AHA/HRS/ISACHD/SCAI guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. Published online December 18, 2025. doi:10.1016/j.jacc.2025.09.006