Treatment and Management of Cardiopulmonary Failure in the Pediatric Population

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Cardiology".

Deadline for manuscript submissions: closed (25 February 2026) | Viewed by 1266

Special Issue Editor


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Guest Editor
Division of Pediatric Cardiology, Department of Pediatrics, University of Tennessee Health Science Center, Memphis, TN 38163, USA
Interests: congenital heart disease; patent ductus arteriosus; hypoplastic left heart syndrome; exercise physiology; pediatric echocardiography; interventional echocardiography; hypertension; pulmonary hypertension
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Special Issue Information

Dear Colleagues,

Cardiopulmonary failure in the pediatric population, driven by congenital heart disease, cardiomyopathy, acute myocarditis, severe respiratory failure, sepsis, and post-cardiac surgery complications, remains a leading cause of morbidity and mortality. Advances in ventilation strategies, extracorporeal life support (ECLS/ECMO), pediatric ventricular assist devices (VADs), pulmonary hypertension therapies, and perioperative critical care are rapidly changing outcomes. The Special Issue will synthesize current evidence, highlight innovations, address resource variability, and set priorities for research and practice across neonates through adolescents.

Dr. Ranjit Philip
Guest Editor

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Keywords

  • heart transplant
  • myocarditis
  • cardiomyopathy
  • tracorporeal life support (ECMO)
  • pulmonary hypertension

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Published Papers (1 paper)

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Review

18 pages, 861 KB  
Review
Sotatercept in Children with Pulmonary Hypertension—A Narrative Review
by Johanna Schulz, Veronika C. Stark, Lars Harbaum, Rainer Kozlik-Feldmann, Thomas S. Mir, Fridrike Stute and Jakob Olfe
Children 2026, 13(4), 465; https://doi.org/10.3390/children13040465 - 28 Mar 2026
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Abstract
Background/Objectives: Pulmonary arterial hypertension (PAH) is a rare but life-threatening disease that presents particular therapeutic challenges in children. It is characterized by pulmonary vasoconstriction and vascular remodeling, leading to right ventricular strain and eventually right heart failure. Although advances in pharmacotherapy have improved [...] Read more.
Background/Objectives: Pulmonary arterial hypertension (PAH) is a rare but life-threatening disease that presents particular therapeutic challenges in children. It is characterized by pulmonary vasoconstriction and vascular remodeling, leading to right ventricular strain and eventually right heart failure. Although advances in pharmacotherapy have improved outcomes, treatment options remain limited. This review aims to evaluate the potential role of sotatercept, a novel fusion protein recently approved for adult PAH, and to assess the translatability of adult data to the pediatric population. Methods: A narrative synthesis of preclinical studies and randomized controlled trials was conducted to summarize the current evidence on sotatercept. In addition, pathophysiological, developmental, and therapeutic differences between adult and pediatric PAH were critically examined to assess relevance and applicability to younger patients. Results: Clinical trials in adults (PULSAR, STELLAR, ZENITH, HYPERION) confirm sotatercept’s efficacy on background therapy, with significant reductions in pulmonary vascular resistance, improvements in 6 min walk distance, enhanced right ventricular function, and risk reductions in clinical worsening events. However, extrapolation to pediatric PAH faces challenges including etiological differences (e.g., PAH-CHD predominance, PPHN in infants), age-inappropriate endpoints (e.g., 6MWD infeasible in young children), variable growth-related pharmacokinetics, and compensatory RV physiology delaying overt failure. Safety concerns are manageable in adults but raise pediatric-specific alarms: activin inhibition’s theoretical tumorigenic potential (dual tumor suppressor/promoter role), pubertal/fertility disruption (FSH suppression, gonadal maturation delay), and skeletal growth interference—unproven clinically yet demanding long-term monitoring. The ongoing MOONBEAM trial will provide initial pharmacokinetic/safety data in children. Conclusions: Sotatercept represents a promising, first-in-class therapeutic option for PAH with the potential to transform disease management. Nevertheless, dedicated pediatric studies are crucial to confirm safety, efficacy, and appropriate dosing and to define its role in the long-term treatment of children with PAH. Full article
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