Multi-disciplinary team of the treatment of heart transplantation for Danon disease
10.3969/j.issn.1674-7445.2021.01.010
- VernacularTitle:心脏移植治疗Danon病的多学科综合诊疗
- Author:
Hengqiang LIN
1
;
Sheng LIU
;
Jie HUANG
;
Juan DU
;
Lei CHEN
;
Hongyue WANG
Author Information
1. Department of Adult Cardiac Surgery, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100037, China
- Publication Type:Research Article
- Keywords:
Danon disease;
Multi-disciplinary team (MDT);
Orthotopic heart transplantation;
Lysosomal-associated membrane protein 2;
Hypertrophic cardiomyopathy;
Heart failure;
Preexcitation syndrome;
Intra-aortic balloon pump (IABP)
- From:
Organ Transplantation
2021;12(1):64-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To improve the understanding of Danon disease and the efficacy of heart transplantation by multi-disciplinary team (MDT) pattern. Methods Prior to heart transplantation, MDT consultation was performed on one case of Danon disease, an extremely rare X-chromosome dominant genetic disease. The content of consultation included surgical indication, preoperative preparation and postoperative precaution, as well as the role of MDT in the treatment of Danon disease was summarized. Results Preoperative echocardiography showed that the patient presented withdilated-phase of hypertrophic cardiomyopathy complicated with heart failure, which was considered as Danon disease with end-stage heart failure. After MDT consultation, the patient received the preoperative treatment including anti-heart failure, reduction of pulmonary artery pressure, intra-aortic balloon pump (IABP) assistance, liver protection, strengthening nutritional support, etc. The patient underwent orthotopic heart transplantation after a suitable donor was matched. The patient developed muscle weakness in the limbs 5 days after operation, which was gradually mitigated after reducing the dose of glucocorticoid. At postoperative 48 days, the patient was discharged in good condition and continually treated with triple immunosuppressive regimen after discharge. Reexamination at postoperative 6 months revealed that the patient was in good health without any abnormality in electrocardiogram and echocardiography images. Conclusions Danon disease is extremely rare and MDT pattern may enhance the efficacy and safety of treatment by heart transplantation.
