Risk of Surgery for Congenital Heart Disease in the Adult: a Multicentered European Study

BACKGROUND: Surgery for congenital heart disease (CHD) has changed considerably during the last three decades. The results of primary repair have steadily improved, to allow treating almost all patients within the pediatric age; nonetheless an increasing population of adult patients requires surgical treatment. The objective of this study is to present the early surgical results of patients who require surgery for CHD in the adult population within a multicentered European study population. METHODS: Data relative to the hospital course of 2,012 adult patients (age > or = 18 years) who required surgical treatment for CHD from January 1, 1997 through December 31, 2004 were reviewed. Nineteen cardiothoracic centers from 13 European countries contributed to the data collection. RESULTS: Mean age at surgery was 34.4 +/- 14.53 years. Most of the operations were corrective procedures (1,509 patients, 75%), followed by reoperations (464 patients, 23.1%) and palliative procedures (39 patients, 1.9%). Six hundred forty-nine patients (32.2%) required surgical closure of an isolated ostium secundum atrial septal defect. Overall hospital mortality was 2%. Preoperative cyanosis, arrhythmias, and NYHA class III-IV, proved significant risk factors for hospital mortality. Follow-up data were available in 1,342 of 1,972 patients (68%) who were discharged home. Late deaths occurred in 6 patients (0.5%). Overall survival probability was 97% at 60 months, which is higher for corrective procedures (98.2%) if compared with reoperations (94.1%) and palliations (86.1%). CONCLUSIONS: Surgical treatment of CHD in adult patients, in specialized cardiac units, proved quite safe, beneficial, and low-risk.

Cardiac Angiosarcoma. A Review

Based on a case of a patient with angiosarcoma (AS) of the right atrium with superior vena cava syndrome associated with urticaria and polyarthralgias, who died soon after surgery, the authors present a brief review of the subject of cardiac AS, an extremely rare pathology, usually diagnosed late due to its non-specific symptomatology. Several topics are discussed, including mechanisms of clinical manifestations caused by blood flow obstruction and valve dysfunction, local invasion with arrhythmias and pericardial effusion, embolic phenomena and constitutional symptoms. Imaging and histopathologic methods of diagnosis are considered, as well as references to cytogenetic analysis. Surgery is the first treatment choice, but heart AS are frequently not completely resectable and concomitant metastases at the time of surgery are common, both usually leading to a dismal prognosis. Chemotherapy, radiotherapy and even heart transplantation do not substantially improve the survival of these patients. Urticaria is not generally assumed by most authors to be associated with malignancy, but there are rare reports of its association with some malignant tumors.

O Varicocelo em Idade Pediátrica

Nos últimos anos o tratamento do varicocelo em idade pediátrica ganhou particular importância devido a evidencia clínica e experimental de atrofia testicular e alterações histo1ógicas em cerca de 50% dos testículos homolaterais e com frequência no contralateral. Estas alterações são progressivas e sobreponíveis às encontradas no adulto inférti1 com varicocelo. Os autores procederam ao estudo retrospectivo de 41 crianças e adolescentes com varicocelo que recorreram a Consulta de Cirurgia Pediátrica do Hospital de Dona Estefânia entre 1 de Janeiro de 1990 e 31 de Dezembro de 1994.O motivo de consulta foi, em quase todos os casos, a existência de uma massa escrotal. As idades estavam compreendidas entre os cinco e os 15 anos, com média etaria de 11,9 anos. O varicocelo localizava-se à esquerda em 39 doentes,à direita em um doente e apenas um caso era bilateral. Todos os casos eram varicocelos primários, sendo mais frequentemente o de grau II (57%). Foram feitas 40 varicocelectomias, 31 das quais (77%) pela técnica de Ivanisevíc. Quatro varicocelos recidivaram ( 10%). Os autores comentam os resultados e tecem algumas considerações sobre a atitude cirúrgica perante um varicocelo diagnosticado em idade pediátrica.