Primary sarcoma of the heart is a rare disease that has an ominous prognosis with either medical or surgical therapy. We report a case of a 25-year-old woman with sarcoma of the heart who received a transplant and is clinically well after 7 years. We believe that transplantation must be considered in this kind of pathology for selected cases.(Ann Thorac Surg 2010;90:635-6) © 2010 by The Society of Thoracic Surgeons P rimary tumors of the heart are rare clinical entities and represent 0.001% to 0.03% of all cardiac surgical cases [1-3]; of these, 25% are malignant tumors, and among them, sarcomas are the most prevalent. Sarcomas of the heart are known to be highly invasive, and when the diagnosis is made, about 80% are already disseminated. Average survival at 9 months is 10% [2].The best therapeutic options for these patients are still being discussed. Total resection is not possible for most patients, and the local recurrence rate is very high. Transplantation has certainly been an option for selected isolated cases, but long-term results and precise indications are still not clear [1].Our patient was a 25-year-old woman who was diagnosed with Hodgkin lymphoma at age 16 years. She was treated with chemotherapy and mediastinal radiotherapy and experienced total remission. As sequela, she had some degree of lung fibrosis and hypothyroidism.In August 2001, the patient was unexpectedly admitted to the hospital with complaints and signs of right-sided heart failure. An echocardiogram showed a tumor mass filling the right ventricular cavity, and a computed tomography scan and cardiac magnetic resonance imaging confirmed those findings and excluded any other locations. Without other diagnosis and given her severe New York Heart Association functional class IV condition, she underwent an open heart operation.A biopsy specimen was obtained from the tumor mass inside the right ventricle, and the pathology report identified sarcoma. Considering the ominous result, partial debulking of the tumor was done, trying to maintain the cavity-filling function of the ventricle without putting the patient at risk with a too-aggressive resection, namely at the septal area where we could create a ventricular septal defect.The patient's postoperative period was without complications. She was referred for chemotherapy and underwent a perfusion of ifosfamide (1500 mg/m 2 ) for 3 days. One month later, she was readmitted with gross rightsided heart failure, and the echocardiogram showed extensive recurrence of the tumor mass, with protrusion of the tumor through the tricuspid valve into the right atrium (Fig 1). At this point, there seemed to be no other medical or surgical options owing to the type of the recurrent tumor involving the heart. Computed tomography scans and liver and bone perfusion scans were able to exclude any metastasis or invasion at distant locations, and cardiac transplantation came as a last option.The patient underwent cardiac transplantation in December 2001 that was performed with a biatrial technique. The tumor i...

Isolated anterior mitral leaflet cleft (not associated with atrio-ventricular septal defect) is a rare cause of congenital mitral regurgitation, and the treatment consists of direct suturing of the cleft. We present a clinical case with this entity.

Background: The appropriateness of rheumatic mitral valve repair remains controversial due to the risks of recurrent mitral dysfunction and need for reoperation. The aims of this study were to determine the overall short-and long-term outcomes of pediatric rheumatic mitral valve surgery in our center. Methods: Single-center, observational, retrospective study that analyzed the results of rheumatic mitral valve surgery in young patients, consecutively operated by the same team, between 1999 and 2014. Results: We included 116 patients (mean age ¼ 12.6 + 3.5 years), of which 66 (57%) were females. A total of 116 primary surgical interventions and 22 reoperations were performed. Primary valve repair was possible in 86 (74%) patients and valve replacement occurred in 30 (26%). Sixty percent of the patients were followed up beyond three months after surgery (median follow-up time ¼ 9.2 months [minimum ¼ 10 days; maximum ¼ 15 years]). Long-term clinical outcomes were favorable, with most patients in New York Heart Association functional class I (89.6%) and in sinus rhythm (85%). Freedom from reoperation for primary valve repair at six months, five years, and ten years was 96.4% + 0.25%, 72% + 0.72%, and 44.7% + 1.34%, respectively. Freedom from reoperation for primary valve replacement at six months, five years, and ten years was 100%, 91.7% + 0.86%, and 91.7% + 0.86%, respectively. Mitral stenosis as the primary lesion dictated early reintervention. Conclusions: Despite the greater rate of reoperation, especially when the primary lesion was mitral stenosis, rheumatic mitral valve repair provides similar clinical outcomes as compared with replacement, with the advantage of avoiding anticoagulation.

Pseudoaneurysms of the ascending aorta are a rare complication of cardiac surgery. However, the poor prognosis associated with this condition if untreated makes early diagnosis and treatment important.We present the case of a 66-year-old woman who had undergone mitral valvuloplasty 12 days previously, who was admitted with a diagnosis of new-onset atrial fibrillation.The transthoracic echocardiogram showed a thrombus in the right atrium and anticoagulation was initiated, followed by antibiotic therapy.After further investigation, the patient was diagnosed with a pseudoaneurysm of the ascending aorta and underwent surgical repair, followed by six weeks of antibiotic therapy.She was readmitted six months later for an abscess of the lower sternum and mediastinum. After a conservative approach with antibiotics and local drainage failed, recurrence of a large pseudoaneurysm compressing the superior vena cava was documented. A third operation was performed to debride the infected tissue and to place an aortic allograft. There were no postoperative complications. © 2012 Sociedade Portuguesa de Cardiologia. Published by Elsevier España, S.L. All rights reserved. PALAVRAS-CHAVEPseudoaneurisma da aorta ascendente; Valvuloplastia mitral; Complicação pós-cirurgia cardíaca Pseudoaneurisma iatrogénico da aorta ascendente: uma complicação esquecidaResumo O pseudoaneunisma da aorta ascendente é uma complicação rara da cirurgia cardíaca. A sua elevada mortalidade torna importante o seu diagnóstico atempado e intervenção precoce.Os autores apresentam o caso de uma doente do sexo feminino, submetida a valvuloplastia mitral 12 dias antes, internada com o diagnóstico de fibrilhação auricular com resposta ventricular rápida. O ecocardiograma transtorácico inicial mostrou imagem sugestiva de trombo na aurícula direita, iniciou-se anticoagulação, seguida de terapêutica antibiótica. Investigação adicional com recurso a TC de tórax com contraste endovenoso permitiu concluir que se tratava de um pseudoaneurisma da aorta ascendente, pelo que a doente foi submetida a correção cirúrgica do mesmo, seguida de seis semanas de terapêutica antibiótica dirigida.A doente foi reinternada seis meses depois por abcesso na porção inferior do externo e mediastino. Após falha da terapêutica conservadora, com antibiótico e drenagem local, com agravamento clínico da doente, documentou-se reaparecimento de pseudoaneurisma de grandes dimensões com compressão da veia cava superior. Foi então submetida a terceira intervenção cirúrgica com desbridamento do tecido infetado e implantação de homoenxerto aórtico. O pós-operatório decorreu sem intercorrências.