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Beta1-adrenergic receptor antibodies in children with dilated cardiomyopathy
Manuela Camino1, Maria Dolores Morales2
1Pediatric cardiologist. Hospital Universitario Gregorio Marañón. Madrid, Spain, manuelacamino59@gmail.com.
Insights
Autoantibodies to beta-1 adrenergic receptors are found in children with dilated cardiomyopathy (DCM). Immunoadsorption therapy may improve heart function in pediatric DCM patients with these antibodies.
Area of Science:
- Cardiology
- Immunology
Background:
- Autoantibodies targeting the beta-1 adrenergic receptor are implicated in adult dilated cardiomyopathy (DCM).
- Previous studies suggest that removing these autoantibodies can improve cardiac function in adults.
Observation:
- This study investigated the presence and impact of beta-1 adrenergic receptor autoantibodies in pediatric DCM patients.
- Seventeen children diagnosed with DCM, exhibiting ventricular dysfunction, were assessed for these antibodies.
Findings:
- Autoantibodies to beta-1 adrenergic receptors were detected in 8 out of 17 pediatric DCM patients.
- Patients with high antibody titers often had poor clinical outcomes, requiring transplantation.
- Conversely, patients with low antibody titers showed significant recovery of heart function after immunoadsorption (IA).
- One patient treated with immunoglobulin and IA demonstrated improved LVEF over 5 years.
Implications:
- Beta-1 adrenergic receptor autoantibodies are present in children with DCM, suggesting a shared pathogenic mechanism with adult DCM.
- Immunoadsorption therapy presents a potential therapeutic strategy for improving heart failure in pediatric DCM associated with these autoantibodies.
Abstract:
Autoantibodies to beta-1 adrenergic receptor have been reported in adult patients with dilated cardiomyopathy (DCM). Removal of these antibodies has a positive hemodynamic effect. Our aim was to investigate whether these antibodies are present in children with DCM and explore the potential hemodynamic benefit of immunoadsorption (IA). Seventeen children with DCM were tested for these antibodies. The etiology of DCM was genetic (n=5), myocarditis (n=4), DCM and congenital heart block (n=3), DCM associated to maternal lupus (n=1), DCM and Wolff Parkinson White Syndrome (n=1), and idiopathic (n=3). All patients evidenced ventricular dysfunction. Antibody testing was positive in 8 patients, 7 received IA. Three patients with high titers had a poor clinical outcome and needed transplantation. Two patients with low titers exhibited a full recovery of heart function. One patient with multiple myocarditis episodes was treated with immunoglobulin IgG and IA ; after 5 years this patient presented a LVEF of 40 percent. Beta-1 adrenergic receptors autoantibodies are present in children with DCM. Immunoadsorption therapy may help improve heart failure in this context.
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