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During the last decades, advances in diagnosis and treatment of congenital heart disease have allowed many individuals to reach adulthood. Due mainly to the great diagnostic diversity and to the co-morbidities usually present in this age group, these patients demand assistance in a multidisciplinary facility if an adequate attention is aimed. In this paper we reviewed, based in the international literature and also on the authors’ experience, the structural conditions that should be available for these patients. We highlighted aspects like the facility characteristics, the criteria usually adopted for patient transfer from the paediatric setting, the composition of the medical and para- medical staff taking into account the specific problems, and also the model of outpatient and in-hospital assistance. We also emphasized the importance of patient data storage, the fundamental necessity of institutional support and also the compromise to offer professional training. The crucial relevance of clinical research is also approached, particularly the development of multicenter studies as an appropriate methodology for this heterogeneous patient population. ResumoDurante as últimas décadas, os avanços verificados no diagnóstico e tratamento das cardiopatias congênitas têm permitido que muitos indivíduos cheguem à idade adulta. Devido principalmente à grande diversidade diagnóstica e também às comorbidades habitualmente presentes nesse grupo etário, esses pacientes necessitam ser atendidos numa unidade multidisciplinar, se o objetivo for proporcionar uma assistência adequada. Neste trabalho revisamos, com base na experiência dos autores e na literatura internacional, as condições estruturais que devem estar disponíveis para esses pacientes. Procuramos ressaltar aspectos como as características da unidade, o critério usualmente adotado para transferência desses pacientes da unidade pediátrica, a composição das equipes médica e paramédica levando em consideração os problemas específicos dos pacientes e também o modelo de assistência ambulatorial e hospitalar. Enfatizamos, ainda, a importância do armazenamento dos dados dos pacientes, a necessidade fundamental de apoio institucional e a importância de oferecer treinamento profissional. A relevância da pesquisa clínica é também abordada, particularmente a importância da confecção de estudos multicêntricos, como uma metodologia apropriada para essa heterogênea população de pacientes.

OBJECTIVE: To report the institution experience with the surgical treatment of adults with congenital heart disease due to the increasing number of these patients and the need for a better discussion of the subject. METHODS: Retrospective analysis describing demographic data, risk factors and results. RESULTS: 191 patients between 16 and 74 years old were operated on. Primary correction was done in 171 cases, 93 (55%) for atrial septal defect repair. Among 20 (12%) reoperations, pulmonary valve replacement was done in six cases. The mean intensive care and hospital stay were 2.7 and 8.5 days respectively, significantly greater for the reoperated cases (P=0.001). The mean bypass and clamping times were 68.6 and 44.7 minutes respectively, greater for the reoperated cases (P

We report the case of an adolescent referred with initial diagnosis of pulmonary hypertension. Non-invasive investigation disclosed a sinus venous atrial septal defect with pulmonary hypertension. The hemodynamic study confirmed diagnosis, and also showed extrinsic compression of left main coronary artery by pulmonary trunk. Surgical closure of the defect in addition to pulmonary trunk plasty were undertaken. Two years after the surgery the patient is well, with clinical signs of mild pulmonary hypertension, and showing no evidence--also on echocardiogram--of left coronary artery trunk obstruction.

Operation, Fontan, protein loss. In 1971, Fontan and Baudet were the first to describe a surgical technique for the successful separation of the systemic and pulmonary circulations in a patient with tricuspid atresia1. Soon after, Kreutzer suggested a similar procedure for a patient with the same condition2. This innovative procedure became known as “the Fontan operation”, which, especially in the 1970’s and 1980’s, provided a better prognosis for patients with functionally univentricular hearts. Since these early impressive reports, several modifications of the techniques have been described as an attempt to further improve the function of both circulations3-5.

The objective of this paper was to describe the outcomes in patients submitted to arterial switch operation and to analyze the predictors of in-hospital mortality and further need of re-operation at a single-center institution. Between September 1995 and January 2014, 128 consecutive arterial switch operations were performed. Surgical mortality during this period was analyzed retrospectively, and a follow-up analysis of the survivors was conducted. Surgical era, cardiopulmonary bypass time (p = 0.001), and diagnosis category (p = 0.025) influenced in-hospital mortality. The estimated overall survival for the 91 hospital survivors was 96.8, 96.4, and 96.2 % at 5, 10, and 15 years, respectively. The median follow-up time was 67 months (range 0.71-222 months). Three patients (5 %) presented severe aortic regurgitation. Right ventricle outflow tract systolic gradient by echocardiography was above 60 mmHg in 2 %. Late re-interventions occurred in 12 (13 %) patients with mean time of 64 ± 34 months after the initial procedure. Actuarial freedom from re-interventions at 5, 10, and 15 years was 96.4, 69.7, and 61.9 %, respectively. Arterial switch operation remains the procedure of choice in patients with transposition of great arteries. It can be performed even in middle-volume institutions, leading to the same middle- and long-term outcomes of high-volume institutions. Early high mortality rate may occur due not only to learning curve, but also to cardiopulmonary bypass time and ventricular septal defect closure.

A teenager with longstanding arterial hypertension was admitted for acute pneumonia treatment. New onset atypical chest pain for the last months and aortic valve regurgitation were also present. A dissecting aneurysm of the ascending aorta with moderate aortic valve regurgitation was evidenced by laboratorial diagnostic. Aneurismectomy with aortic valve preservation and coronary artery reimplantation was carried out.

Background: Retention of early-taught knowledge and fundamental clinical skills, albeit essential in medical education, has not been extensively investigated. This study is aimed at assessing prospectively the retention of knowledge and skills related to communication with patients and the physical examination by fourth year medical students after an introductory course in basic pediatric cardiology. Methods: Three cohorts of fourth year medical students aged 21-26 years volunteered for the study. All students (N = 130) were assessed immediately after the teaching period and after six months (N = 42) and after one year (N = 21) later. Assessments included a 40 multiple-choice question (MCQ) test and an 8-station objective structured clinical examination (OSCE) focused on communication with patients, physical examination and interpretation of diagnostic tests (chest radio- graphs and electrocardiograms). Cases were portrayed by either real, rehearsed patients or standardized patients. Student performance was assessed by trained staff members using structured checklists. Results: For all aspects of knowledge assessed, scores obtained in the second and the third assessment were significantly lower than those verified in the first assessment. There were no significant differences between the three assessments re- garding overall clinical performance and data for communication with patients and physical examination. Concerning di- agnostic tests interpretation, scores obtained in the second and third assessment were significantly lower than those veri- fied in the first assessment. Paired analysis of the results obtained for the 21 students who took the three assessments showed similar results, except for a trend for improvement in clinical skills. Conclusions: Medical student retention of clinical sciences knowledge follows a pattern similar to that found in other scientific domains, characterized by progressive decay after initial acquisition. In contrast, fundamental clinical skills, such as communication with patients and physical examination are substantially retained, which could be ascribed to con- tinuous practice. These findings should be taken into account for devising instructional strategies for enhancing student knowledge and clinical skills maintenance.

We report a case of congenital coarctation of the lower thoracic aorta. The patient, a 15-year-old man, presenting with the signs of classical coarctation, had the diagnosis confirmed by an aortography. A good surgical result was achieved by means of resection of the internal shelf and aortoplasty using a bovine pericardium patch. One year after the operation the patient has normal blood pressure with good femoral pulses.

Coronary recanalization with thrombolytic agents is a new therapeutic approach to the treatment of acute myocardial infarction that can be beneficial even to patients in cardiogenic shock. Although few cases have been reported in the literature, treatment of acute occlusion of the left main coronary artery (LMCA) has been made possible by myocardial reperfusion. This communication concerns a patient with acute LMCA occlusion who was successfully treated by thrombolytic therapy with streptokinase followed by revascularization of the myocardium seventy-two hours after reperfusion was achieved.

Traumatic rupture of the interventricular septum is a rare condition usually presenting in infancy and childhood. This report describes two cases: a 3-year-old boy operated on soon after the diagnosis was made, and a 15-year-old girl operated on 12 years following the causative accident. Both patients had the diagnosis confirmed by cardiac catheterization. The typical appearance of a traumatic ventricular septal defect (VSD) was seen during surgical repair that was successfully carried out in both patients. The protracted clinical course of the second patient, eventually requiring corrective intervention, contributes to the understanding of natural history of traumatic VSD.