Advances in Pediatric and Congenital Heart Disease Management

The year 2025 saw continued innovation in congenital and pediatric cardiology, driven by updated practice guidelines, multicenter research, and the translation of bench-to-bedside discoveries. Key areas of progress included pediatric heart failure (HF), pediatric pulmonary hypertension (PH), Fontan circulation, and adult congenital heart disease (ACHD).

Pediatric Heart Failure: Updated Guidelines

The International Society for Heart and Lung Transplantation (ISHLT) released updated guidelines for the management of pediatric HF, representing the first revision since 2014. These guidelines incorporate advancements in diagnostics, pharmacotherapy, and mechanical support strategies tailored to pediatric physiology.

Key updates include standardized evaluation protocols utilizing biomarker levels (such as N-terminal pro–B-type natriuretic peptide), advanced imaging like 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. Recommendations for angiotensin receptor–neprilysin inhibitors and cautious leverage of sodium-glucose cotransporter-2 (SGLT2) inhibitors in select pediatric populations under institutional oversight were also introduced.

For advanced HF, the guidelines outline criteria for ventricular assist device implantation and heart transplant, considering growth and neurodevelopmental factors. They also address perioperative management, immunosuppression, and long-term follow-up, stressing the importance of multidisciplinary care and family-centered decision-making.

Pediatric Pulmonary Hypertension: Interventional Strategies

The American Thoracic Society (ATS) issued clinical practice guidelines on interventional strategies for children with progressive PH despite optimal therapy. The guidelines, based on systematic reviews and expert opinion, offer conditional recommendations for:

• Atrial septostomy: Advised for palliative symptom relief in children with progressive PH and right ventricular (RV) failure.
• Pulmonary-to-systemic 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, and advised for those supported on extracorporeal membrane oxygenation with persistent RV failure.

The panel emphasized the limited evidence and potential risks associated with these interventions, advocating for multidisciplinary decision-making and shared decision-making with families.

Fontan Circulation: Conduit vs. Lateral Tunnel

The FORCE investigators published a multicenter comparison of extracardiac conduit (ECC) versus lateral tunnel (LT) Fontan operations at 15 years of follow-up. Both techniques demonstrated excellent overall survival, but differences emerged in morbidity. ECC operations were associated with lower rates of atrial arrhythmias, likely due to reduced 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 highlights the need for lifelong surveillance for arrhythmias, thromboembolism, and organ dysfunction regardless of the surgical approach.

SGLT2 Inhibitors in Fontan Failure

A study explored the off-label use of SGLT2 inhibitors in patients with single-ventricle physiology and Fontan circulatory failure. SGLT2 inhibitors were generally well-tolerated, with modest symptomatic improvements reported, such as better fluid balance and exercise tolerance. However, objective hemodynamic or biomarker changes were inconsistent, emphasizing the need for prospective trials to confirm efficacy and long-term safety.

Adult Congenital Heart Disease: A Unified Framework

A multisociety guideline for the management of ACHD provided an updated framework for the care of the growing population of adults with congenital heart disease. The guideline emphasizes lifelong, multidisciplinary care and structured transition from pediatric to adult services. Key updates include guidance on HF management in systemic RVs and left ventricles, refined arrhythmia care, pregnancy and contraception counseling, and mental health/neurocognitive assessment.

Looking Ahead

Research in 2025 focused on systems-level advancements in pediatric and congenital heart disease, allowing for standardized care, individualized therapy, and sustained outcomes across the lifespan. Updated guidelines in interventional cardiology and other innovative research are anticipated in 2026.