Primary cardiac tumours can be surgically treated with good short- and long-term results. Mortality and morbidity are mainly due to the status of preoperative patients'. An accurate follow-up is mandatory in order to detect the recurrence of a cardiac tumour such as to exclude the presence or the development of extracardiac tumours that we found frequently associated with the myxoma.
Primary cardiac tumours are relatively rare in the paediatric population, and they may occur with different signs and symptoms in foetal or post-natal life. The clinical manifestations of cardiac tumours in foetal life may include arrhythmias, congestive heart failure and hydrops. In post-natal life, cardiac tumours may cause cyanosis, respiratory distress, myocardial dysfunction, valvular insufficiency, arrhythmias, inflow or outflow tract obstructions and sudden death. Surgical treatment is essential when symptoms are present, while the role of medical therapy can merely be palliative. Results are various and related to the patients' and tumour characteristics. Primary benign heart tumours mainly have a good prognosis, while malignant neoplasms usually have a poor prognosis; in both cases, however, a strict follow-up is always mandatory in order to detect the recurrence of cardiac neoplasms after surgery.
The treatment outcome for patients affected by primary malignant heart tumors remains poor. Aggressive surgery alone does not provide good results in terms of survival rate. A new multidisciplinary approach is mandatory to improve long-term survival.
SummaryCombined orthotopic heart and liver transplantation (CHLT) is a lifesaving procedure for patients with end-stage heart-liver disease. We reviewed the long-term outcome of patients who have undergone CHLT at the University of Bologna, Italy. Fifteen patients with heart and liver failure were placed on the transplant list between November 1999 and March 2012. The pretransplant cardiac diagnoses were familial amyloidosis in 14 patients and chronic heart failure due to chemotherapy with liver failure due to chronic hepatitis in one patient. CHLT was performed as a single combined procedure in 14 hemodynamically stable patients; there was no peri-operative mortality. The survival rates for the CHLT recipients were 93%, 93%, and 82% at 1 month and 1 and 5 years, respectively. Freedom from graft rejection was 100%, 90%, and 36% at 1, 5, and 10 years, respectively, for the heart graft and 100%, 91%, and 86% for the liver graft. The livers of eight recipients were transplanted as a "domino" with mean overall 1-year survival of 93%. Simultaneous heart and liver transplantation is feasible and was achieved in this extremely sick cohort of patients. By adopting the domino technique, we were able to enlarge the donor cohort and include high-risk patients.
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