RESUMEN
Objective:To analyze the case characteristics and outcomes of 12 idiopathic giant cell myocarditis(IGCM)cases after heart transplantation(HT).Methods:From June 2004 to May 2022, clinical data were retrospectively reviewed for 12 cases with pathologically confirm IGCM after HT at Fuwai Hospital.General characteristics, clinical manifestations, pathological examinations and postoperative follow-ups are recorded.Results:From June 2004 to May 2022, a total of 1 143 HT operations are performed at Fuwai Hospital and 12 cases of IGCM(1.05%)are confirmed by postoperative pathology.The age is(47.6±7.3)years.There are 5 boys and 7 girls.Initial presenting manifestations are congestive heart failure(7 cases, 58.3%)and arrhythmia(4 cases, 33.3%). Median time from symptom onset to HT is 6 months.All of them are undiagnosed pre-operation.And dilated cardiomyopathy(5 cases, 41.7%)and arrhythmogenic right ventricular cardiomyopathy(3 cases, 25%)are confirmed.The follow-up period is(4~142)months post-HT.One death occurred during perioperative period and another is due to heart failure at 68 months post-HT.Only 1 case of grade 1R transplant heart rejection occurrs at 9 years post-HT and there is no case of recurrence.According to Kaplan-Meier survival analysis, cumulative survival rates of 1/5/10 years post-HT in IGCM patients are 91.7%, 91.7% and 73.3% respectively.No significant difference exist in survival rate for other etiologies post-HT(Log-rank P=0.265). Conclusions:HT is efficacious for end-stage IGCM.Regular and sufficient postoperative immunosuppression is vital for preventing heart transplant rejection and recurrent IGCM.Most IGCM patients have a decent prognosis post-HT.
RESUMEN
Idiopathic granulomatous myocarditis is extremely rare, particularly since the introduction of drugs effective against tuberculosis (TB), viruses, fungi and the effective treatment of sarcoidosis. Here is a case of a 65-year-old female prisoner having history of sudden collapse and ultimately death. Autopsy fi ndings of various viscera on histopathological examination show granulomatous pathology, that is, in spleen, liver and in the left ventricular wall of heart. Ziehl-Neelsen staining of the sections show the absence of acid fast bacilli, negative for fungal staining as most of the granulomas are noncaseating type with presence of giant cells having no asteroid body and Schuamann body, real-time polymerase chain reaction for TB is negative. Idiopathic giant cell myocarditis is a disease of relatively young adults, that is, between 3rd and 4th decade of life. So, this case is strongly considered to be a case of sudden death due to myocarditis as a result of idiopathic systemic granulomatous pathology, a rare case in in literature.