Summary:We report a right atrial myxoma which suddenly developed in a thalassemic patient after allogeneic bone marrow transplantation. The tumor was first detected by echocardiography on day +47 after transplant and the patient underwent surgical removal of the myxoma on day +103. The post-operative course was uneventful, and at more than 3 years from the event, he is alive and well, cured from his congenital disease, with no detectable intra-cardiac tumor. The onset of the myxoma in the early post-transplant period and the extremely high velocity of growth suggest a possible relationship of this condition with the immunosuppressive status. Keywords: myxoma; BMT; thalassemia Bone marrow transplantation (BMT) is a situation which may allow the development of rare and sometimes unrecognised complications 1,2 due to the conditioning regimen, immunosuppression, graft-versus-host disease (GVHD) etc. Cardiac myxoma is a rare form of heart tumor whose origin is still under discussion. 3,4 Here, we report the occurrence of a right atrial myxoma in a young thalassemic patient in the early period after BMT.
Case reportThe patient was a 17-year-old male suffering from homozygous B thalassemia, referred to our Unit in August 1994 to be evaluated for allogeneic BMT. Following the Pesaro classification system 5 he was evaluated as a class 3 patient. A complete clinical and echocardiographic evaluation was performed before the transplant; twice in his home town and once in Pesaro. Diagnostic conclusions were consistent between the three different cardiologists and showed mild left ventricle dilatation (5.3 cm) with normal myocardial wall thickness and normal regional and global systolic function. No morphological abnormalities were observed in the right heart. The patient underwent BMT on 1 September 1994 from his HLA-identical brother. Preparation for BMT was performed following the scheme at that time in use for class 3 patients 6 (busulfan 14 mg/kg over 4 consecutive days followed by cyclophosphamide 120 mg/kg over 4 days and anti-lymphocytic globulin 2 ml/kg from day −8 to day −2). The early post-BMT course was uneventful and the patient was discharged on day +16 with documented allogeneic engraftment. Because of severe metabolic problems (fasting serum glucose: 44 mmol/l, serum triglycerides: 33 mmol/l) he was readmitted to the ward on day +34. During hospitalization he experienced two episodes of symptomatic hypotension (blood pressure 80/40 mm Hg). Echocardiographic examination performed on 18 October (day +47) revealed 'a large intra-cardiac tumor (oval in shape with maximum diameter = 3.1 cm) with ultrasound density similar to the myocardium, immotile, attached to the lateral wall of the right atrium, below the tricuspid anulus, near to the apex of the central venous catheter, without doppler findings of valve orifice stenosis' (Figure 1). After initial anticoagulant treatment, a MRI scan (Figure 2) was performed (27 October, day +56) which confirmed the presence of tumor, excluded a thrombotic origin, but did not pe...
False aneurysm occurring after replacement of ascending aorta by a vascular prosthesis is a rare, but life-threatening complication. In spite of advances in endovascular techniques, surgery remains the treatment of choice in the majority of cases. We report the case of a huge pseudoaneurysm caused by late dehiscence of the right coronary ostium-aortic tubular graft anastomosis, occurred 30 years after replacement of aortic valve and ascending aorta by classical Bentall operation. A fistula originating from the aneurysmal sac extended across the sternum into the thoracic subcutaneous soft tissues and gave rise to a pulsatile mass well appreciable on the anterior chest wall. The surgical treatment, consisting of partial resection of the aortic tubular graft and sternal reconstruction was effective and uneventful.
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