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Reverse Remodeling in Pediatric Myocarditis With DCM Phenotype Impact of rASD and PAB
Thushiha Logeswaran1, Hakan Akintürk2, Dietmar Schranz3
1Department of Pediatric Cardiology, Intensive Care Medicine and Congenital Heart Disease, Pediatric Heart Center, Justus-Liebig-University, Giessen, Germany.
Insights
A novel treatment strategy combining left atrial decompression and pulmonary artery banding (PAB) shows promise for myocardial recovery in young children with myocarditis-associated dilated cardiomyopathy (DCM). This approach may improve outcomes for pediatric heart failure patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Pediatric Critical Care
Background:
- Myocarditis can progress to dilated cardiomyopathy (DCM) in children, leading to end-stage heart failure.
- The immature myocardium has age-dependent regenerative capacity, suggesting potential for intervention.
Purpose of the Study:
- To evaluate a staged, pathophysiology-guided strategy for myocardial recovery in infants and young children with myocarditis-associated DCM.
- The strategy combines left atrial decompression and pulmonary artery banding (PAB).
Main Methods:
- Retrospective analysis of 31 children under 3 years with biopsy-proven myocarditis and DCM phenotype.
- Standardized multimodal assessment including echocardiography, cardiac MRI, hemodynamic assessment, and endomyocardial biopsy.
- Interventions included creation of restrictive atrial communication and surgical PAB in selected patients.
Main Results:
- Patients presented with severe heart dysfunction (ejection fraction 24% ± 6.2%, elevated filling pressures).
- 90% overall survival at a median 2-year follow-up; 19% required heart transplantation.
- Among patients selected for PAB, 76% achieved sustained functional recovery.
Conclusions:
- A staged strategy of left atrial decompression and PAB can promote myocardial recovery in select pediatric patients with myocarditis-associated DCM.
- This approach offers a potential therapeutic option for young children with severe heart dysfunction.
Background:
Myocarditis progressing to a dilated cardiomyopathy (DCM) phenotype carries a high risk of end-stage heart failure in early childhood.
Objectives:
Given the age-dependent regenerative capacity of the immature myocardium, we evaluated a staged, pathophysiology-guided strategy combining left atrial decompression and pulmonary artery banding (PAB) to promote myocardial recovery in selected infants and young children.
Methods:
We retrospectively analyzed 31 children <3 years (55% female; median age 312 days) with biopsy-proven myocarditis and a DCM phenotype treated between 2013 and 2024. All underwent standardized multimodal assessment, including echocardiography, cardiac magnetic resonance imaging, invasive hemodynamic assessment, and endomyocardial biopsy.
Results:
At presentation, all patients were in Ross functional class > III; median BNP was 2,258 pg/mL (IQR: 770-4331), and 90% required inotropic support. The mean echocardiography-derived left ventricular (LV) ejection fraction at admission was 24% ± 6.2% with marked LV dilatation (mean z-score +5). The median LV end-diastolic pressure (was 20 mm Hg (IQR: 15-23), indicating elevated filling pressures. A restrictive atrial communication (restrictive atrial septal defect or patent foramen ovale dilatation) was created in 17 patients for atrial decompression. Seventeen patients met the predefined criteria for surgical PAB, including severely reduced LV ejection fraction (mean 19% ± 6%), marked LV dilatation (z-score +6 ± 1.6), and preserved right ventricular function. During a median follow-up of 2 years (IQR: 1-7) overall survival was 90%. Heart transplantation was required in 19%; mechanical circulatory support in 6%. Among patients selected for PAB, 76% achieved sustained functional recovery.
Conclusions:
A staged, pathophysiology-guided strategy incorporating left atrial decompression and PAB may promote myocardial recovery in selected young children with myocarditis-associated DCM.
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