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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.
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@#Objective To analyze the clinical effects of allograft orthotopic heart transplantation. Methods The clinical data of 36 patients with allograft orthotopic heart transplantation performed in the Heart Centre of Nanjing First Hospital from January 1, 2014 to January 1, 2019 were retrospectively analyzed. There were 31 males and 5 females, aged 23-65 (46.2±8.8) years. Protopathy diseases of recipients included dilated cardiomyopathy in 33 patients, end-stage coronary heart disease in 2 patients, and end-stage valvular heart disease in 1 patient. Heart transplantations were performed through double vena cava anastomosis. Immune induction during operations was treated with a combination therapy of both bariximab and methylprednisolone. Postoperatively, all patients were treated with a new triple immunosuppression protocol: FK506+cellcept+prednisone. Results During the perioperative period, 1 patient died of severe infection. For 8 patients with heart failure, after adjustment and intra-aortic balloon pump, the cardiac function of all the 8 patients improved. For 5 patients with renal failure, after continuous renal replacement therapy, the renal function of all the patients returned to normal. One patient died of graft failure after 1 year of follow-up. The follow-up time for each patient postoperatively differed from 3 to 49 months with an average time of 16±4 months while the 1-year survival rate was 97.1% (34/35). Among them, 10 patients were marginal donors, with no significant differences between conventional donors and them. Conclusion For end-stage heart diseases, heart transplantation is one of the effective treatment methods in China with fine early- and middle-term curative effects. Reasonable application of intra-aortic balloon pump, continuous renal replacement therapy and other adjuvant treatments and the new triple immunosuppression protocol can significantly improve the success rate of heart transplantation, reduce the occurrence of acute and chronic rejections. The application of marginal donors can alleviate the current situation of shortage of donors to some extent.
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Objective To investigate the early application value of echocardiography (UCG)after orthotopic heart transplantation (OHT). Methods A total of 29 patients were monitored by UCG early after OHT. On the 1st,7th,14th,30th day after operation,the left ventricular end-diastolic diameter (LVDd)and right ventricular end-diastolic diameter (RVDd),interventricular septal thickness (IVST),left ventricular posterior wall thickness (LVPWT)and the tricuspid regurgitation area (TRA)were measured,and the left ventricular ejection fraction (LVEF ) and left ventricle Tei index (LV-Tei ) were calculated. The blood pressures (BP),central venous pressure (CVP)of the patients were recorded simultaneously. Results The LVDd were larger on the 7th,14th and 30th day after operation,while the RVDd were smaller,compared with those on the 1st day after operation (all in P<0.05). The IVST,LVPWT were lower on the 1th,14th and 30th day after operation,compared with those on the 7th day after operation (all in P<0.05). The BP,CVP,LV-Tei were all significantly lower on the 1st,14th and 30th day after operation,compared with those on the 7th day after operation (P<0.05 ). There was no significant difference in LVEF between each time points(P>0.05 ).The tricuspid regurgitation was detected in 3-4 d after operation by UCG,and the TRA reached the peak value [(9.2 ±2.5)cm2]in 5-8 d after operation,then gradually decreased to (4.7 ±2.4)cm2 at 1 month after operation. Conclusions Early phase after OHT,the structure and function of transplanted heart can be monitored effectively by echocardiography,and it can timely provide multiple parameters as the basis of diagnosis and treatment for clinical doctors.
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Fulminant myocarditis has been defined as the clinical manifestation of cardiac inflammation with rapid-onset heart failure and cardiogenic shock. We report on the case of a 23-yr-old woman with pathology-proven fulminant lymphocytic myocarditis presenting shock with elevated cardiac troponin I and ST segments in V1-2, following sustained ventricular tachycardia and a complete atrioventricular block. About 55 min of intensive cardio-pulmonary resuscitation, with extracorporeal membrane oxygenation support, bridged the patient to orthotopic heart transplantation. The explanted heart revealed diffuse lymphocytic infiltration and myocyte necrosis in all four cardiac chamber walls. Aggressive mechanical circulatory support may be an essential bridge for recovery or even transplantation in patients with fulminant myocarditis with shock.
Subject(s)
Female , Humans , Young Adult , Combined Modality Therapy/methods , Extracorporeal Membrane Oxygenation/methods , Heart Transplantation , Myocarditis/complications , Shock/diagnosis , Treatment OutcomeABSTRACT
Orthotopic heart transplantation was performed on two critical patients with end-stage dilated cardiomyopathy on Oct.14, 1996 and March 20, 1997 respectively.One case of pre-operative hepatic-renal failure died of systemic organs failure and severe circulation failure in oper-ation, and the other case of severe pulmonary hypertension preoperatively died of right heart fail-ure and pulmonary hypertension crisis on the seventh day after operation.Operating time and indi-cations of heart transplantation, preoperative evaluation for the patients with pulmonary hyperten-sion, and management of right heart failure after heart transplantation were detailedly discussed.
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Orthotopic heart transplantation was successfully performed on one patients with endstage dilated cardiomyopathy.The patient has been survived for more than 1 year until today and is now living well.The immunosuppressive therapy and the acute rejective surveillance for 1 year after operation were detailedly introduced and discussed in this paper.In the second week and sixteenth week after operation,two episodes of acute cardiac rejection occurred and were promptly treated well with methylprednisolone.