The Turkish Journal of Pediatrics 2023 , Vol 65 , Num 2
Successful treatment of post-pericardiotomy syndrome via C1 inhibitor replacement therapy in a hereditary angioedema patient with Marfan syndrome
Ezgi Topyıldız 1 ,Handan Duman Şenol 1 ,Figen Gülen 1 ,Esen Demir 1 ,Nihal Mete Gökmen 2
1 Division of Pediatric Allergy and Clinical Immunology, Department of Pediatrics, Ege University Faculty of Medicine, İzmir
2 Division of Allergy and Clinical Immunology, Department of Internal Medicine Allergy, Ege University Faculty of Medicine, İzmir, Türkiye
DOI : 10.24953/turkjped.2022.637 Background. Hereditary angioedema with C1 inhibitor deficiency (HAE-C1INH) is caused by dysfunctional C1-INH protein due to mutations in the SERPING1 gene encoding C1-INH. Marfan syndrome is a genetic connective tissue disease that affects the cardiovascular and ocular systems along with the skeletal system. In this case, we present the successful treatment of post-pericardiotomy syndrome unresponsive to classical therapy, which has not been described in the literature. The syndrome developed in a patient with hereditary angioedema (HAE) who underwent open heart surgery due to cardiac involvement in Marfan syndrome.

Case. A nine-year-old male HAE-C1INH patient underwent open heart surgery secondary to cardiac involvement caused by Marfan syndrome. To prevent HAE attacks, 1000 units of C1 inhibitor concentrate therapy were given 2 hours before and 24 hours after the operation. Post-pericardiotomy syndrome was diagnosed on the postoperative second day and ibuprofen 15 mg/kg/day (3 weeks) was started. Since there was no response to classical treatment on the 21st postoperative day, C1 inhibitor concentrate treatment was planned as 1000 units/ dose for 2 days a week considering a prolonged hereditary angioedema attack. In the second week of treatment, complete recovery was achieved for pericardial effusion with a total of 4 doses.

Conclusions. We emphasize that in patients with hereditary angioedema undergoing this treatment, care should be taken in terms of complications that may be associated with the disease even if short-term prophylaxis is given before operations and that longer-term use of C1 inhibitor concentrate has a place in treatment. Keywords : hereditary angioedema, Marfan syndrome, post-pericardiotomy syndrome, C1 inhibitor replacement therapy

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