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Prophylactic Pulmonary Artery Banding in Pediatric Dilated Cardiomyopathy: An Additional Therapeutic Option
Elena Panaioli1, Diala Khraiche1, Margaux Pontailler1
1Unité Médico-Chirurgicale de Cardiologie Congénitale et Pédiatrique, Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Universitaire Necker-Enfant-Malades, 75015 Paris, France.
Insights
Pulmonary artery banding (PAB) shows promise for infants with severe dilated cardiomyopathy (DCM) unresponsive to medical treatment. This procedure can improve heart function and reduce mortality, offering an alternative to heart transplantation.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Congenital Heart Disease
Background:
- Dilated cardiomyopathy (DCM) is a leading cause of mortality in children.
- Heart transplantation is often the only treatment for severe pediatric DCM.
- Pulmonary artery banding (PAB) is an emerging strategy for DCM management.
Purpose of the Study:
- To evaluate the clinical outcomes of PAB in pediatric DCM patients.
- To assess left ventricle reverse remodeling (LVRR) after PAB.
- To determine PAB's efficacy as a bridge or alternative to heart transplantation.
Main Methods:
- Retrospective analysis of 10 pediatric patients with severe DCM who underwent PAB.
- Clinical follow-up and echocardiography at 6 months and 1 year post-surgery.
- Selective PAB for heritable DCM or DCM with congenital left ventricle aneurysm (CLVA).
Main Results:
- Zero in-hospital mortality; two late deaths due to end-stage heart failure.
- Improved modified Ross class in 8 surviving DCM patients.
- Significant LVRR observed: LV end-diastolic diameter Z-score decreased (8.4 to 2.8), and LV ejection fraction increased (23.8% to 44.5%).
Conclusions:
- PAB is a viable option for infants and toddlers with severe DCM refractory to medical therapy.
- PAB facilitates significant LVRR and may improve clinical status in select pediatric DCM cases.
- PAB can be considered an important therapeutic tool in managing pediatric DCM.
Abstract:
Dilated cardiomyopathy (DCM) is the most common childhood cardiomyopathy and is associated with considerable early mortality. Heart transplantation is often the only viable life-saving option. Pulmonary artery banding (PAB) has been recently proposed as a bridge or alternative to transplantation for DCM. In our cohort, PAB was selectively addressed to heritable DCM or DCM with congenital left ventricle aneurysm (CLVA). This study aimed to describe the clinical evolution and left ventricle reverse remodeling (LVRR) over time (6 months and 1 year after surgery). Ten patients with severe DCM received PAB between 2016 and 2021 and underwent clinical and postoperative echocardiography follow-ups. The median age at PAB was <1 year. The in-hospital mortality was zero. Two patients died two months after PAB of end-stage heart failure. The modified Ross class was improved in the eight survivors with DCM and remained stable in the two patients with CLVA. We observed a positive LVRR (LV end-diastolic diameter Z-score: 8.4 ± 3.7 vs. 2.8 ± 3; p < 0.05; LV ejection fraction: 23.8 ± 5.8 to 44.5 ± 13.1 (p < 0.05)). PAB might be useful as part of the armamentarium available in infants and toddlers with severe DCM not sufficiently responding to medical treatment with limited probability of spontaneous recovery.
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