What question did this study set out to answer?

To explore the challenges of managing patients with failed Fontan physiology and the role of paediatric palliative care.

January 10, 2026Cardiology in the Young0 citationsOpen Access

“Failing Fontans:” an emerging challenge in paediatrics

THTracy HillsVanderbilt University Medical Center EMErika MejiaRobert H. Lurie Comprehensive Cancer Center of Northwestern University RMRandal MayUniversity of Oklahoma Health Sciences Center

Key Result

Paediatric palliative care plays a crucial role in managing patients with failed Fontan physiology as they age beyond 50 years post-surgery.

Key Points

To explore the challenges of managing patients with failed Fontan physiology and the role of paediatric palliative care.
Case report highlighting clinical approaches to palliative care for patients with failed Fontan physiology.
Discussion of various support strategies tailored to the specific needs of this population.
Emphasized the need for individualized palliative care approaches in managing failed Fontan patients.
Identified gaps in current management strategies that require attention and innovation.

Structured PICO

Population

19-year-old male with hypoplastic left heart syndrome with left pulmonary artery atresia status post Fontan palliation at age 3, who developed failed Fontan physiology characterized by elevated venous pressures, biventricular heart failure, cirrhosis, and multifocal hepatocellular carcinoma.

Intervention

Paediatric palliative care integration, including symptom management (e.g., home milrinone) and concurrent hospice enrollment.

Early integration of paediatric palliative care is crucial for managing the complex medical, psychosocial, and end-of-life needs of adolescents and young adults with failing Fontan physiology.

Main Result

Absolute Event Rate: 0% vs 0%

Abstract

Abstract As staged palliative surgery for patients with hypoplastic left heart syndrome ages to greater than 50 years in practice, providers are increasingly faced with the challenges of managing patients with “failed Fontan” physiology. In this brief case report, we discuss different ways paediatric palliative care can support this population.

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