A 3-year-old boy, who underwent heart transplantation when he was 1, after an uneventful follow-up, with no signs of rejection and with always well-dosed immunosuppressive therapy, has been showing signs of heart failure for about 4 months. Constrictive pericarditis was diagnosed for which he underwent surgical total pericardiectomy 1 month ago. A huge fibrotic plaque was removed from the anterior right ventricular wall. The biopsy showed a fibrotic reaction of the subepicardial layer with a periarteriolar inflammatory reaction. After a short period of hemodynamic improvement, he experienced a cardiac arrest during an invasive procedure. He was treated by a rescue ECMO, from which he was weaned after 7 days. He performed an MRI which showed epicardial fibrosis with the persistence of a constricting pattern. A cath lab procedure showed mildly elevated pulmonary pressures with a positive response to NO and hyperoxia. The baby is currently listed for retransplantation. The fear is that it may develop a constricting picture even after retransplantation. Any similar experiences in the community? Therapeutic suggestions? Thanks a lot to those who will help us.
Constrictive pericarditis after Heart Transplantation is rare and usually developed from one week to even 1 year of Transplantation. Usually Diagnosed on basis of high index of clinical suspicion and Doppler echo. H/o Postoperative Pericardial effusion or use of providine iodine or drying of pericardium are risk for developing Constrictive pericarditis
Thank you very much for your answer. The problem is quite different because the child has already received a pericardiectomy and that is no more a problem. Now there is evidence of a constrictive epicarditis mimicking the clinical effect of the pericarditis. We are sequencing the genome to disclose any problem with the immune system that can preclude the success of retransplantation. In the meantime, the child is back on ECMO for heart failure. We are now waiting for the results of the tests on the immune system to decide to go to HTx or not.
Any experience with constructive epicarditis???
We have a mutation in the STAT3 gene. According to our specialists, it is possible to hope for a good response to tocilizumab therapy. Obviously, no one can predict the effect on a retransplanted heart. The patient is now on ECMO and we are planning to switch to biVAD because the hemodynamic is not so stable. Do you think it's worth going on with heart retransplantation?
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