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We report the case of a 3-year-old female patient, who, since birth, had cyanosis difficult to explain with usual diagnostic tests. The only findings on physical examination were cyanosis and clubbing of her fingers. Chest computerized tomography showed images of excessive attenuation in the right lung, which resembled arteriovenous fistulae that were later confirmed on cardiac catheterization. The fistulous trajectories were then embolized with 7 Gianturco coils, which resulted in an immediate increase in the arterial saturation of blood oxygen.

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PURPOSE: To validate the hypothesys that a psycological preparation for children who will undergo cardiac surgery may improve the outcome. METHODS: Sixty patients, with ages ranging between 3 and 10 years, submitted to heart surgery for treatment of congenital heart defects, were evaluated. They were divided in 2 groups: experimental and control. A questionnaire was designed for collecting data about psychological and clinical aspects of each patient. RESULTS: The following data was found to be of statistical significance: acceptance of peripheral vein puncture in the surgical group (chi 2 = 11.59, p < 0.05), calm awakening following general anesthesia (chi 2 = 9.64 p < 0.05), cooperation with the physiotherapy staff (chi 2 = 13.30, p < 0.05), coping with parents absence (chi 2 = 9.64, p < 0.05), acceptance of fluid restriction (chi 2 = 17.78, p < 0.05) and cooperation with removal of stitches and pacemaker electrodes (chi 2 = 19.20, p < 0.05). There was not statistical significance on demand of sedation, cooperation at removal of the orotracheal tube and during examination, necessity of reintubation and occurrence of clinical complications. However, the prepared group showed a slight tendency to have less postoperative complications (20%) than the control (27%). CONCLUSION: It was found that children who had adequated psychologic preparation prior to the correction of congenital heart defects had better psychological results with the imposed trauma.

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A simple technique is proposed for restoring aortic flow in an interrupted aortic arch simulating a type B interruption that does not require aortic cross-clamping, circulatory arrest, or the ligation of major aortic branches. Side-to-side anastomosis between the left carotid and subclavian arteries together with division of the ductus arteriosus and pulmonary artery banding proved effective for relieving aortic arch stricture in that location.

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The occurrence of horseshoe malformation of the lungs is very rare, especially in association with anomalies of the pulmonary arteries such as a pulmonary sling. We describe a case which, as far as we know, is the first example of this association.

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The influence of palliation on survival was studied in 191 consecutive infants, presenting at under 1 year of age, with double-inlet ventricle (1973 to 1988, median follow-up 8.5 years). Palliative operations were performed on 154 occasions in 121 patients (63%). Survival after a systemic-pulmonary arterial shunt (n = 57) and banding of the pulmonary trunk (n = 35) was comparable (84% and 77% at 1 year, 62% and 45% at 5 years), but those who underwent repair of aortic arch obstruction fared worse (n = 18, 44% and 22% at 1 and 5 years, p less than 0.001). The remainder did not undergo an operation because of balanced physiology (n = 17, 9% of entire group), complex anatomy (n = 32, 15%), or irreversible low output (n = 19, 12%). Palliative surgery, overall, had a deleterious effect on immediate survival (greater than 1 month relative risk 6.6, p less than 0.001), but, in the survivors, medium-term outcome was improved (greater than 6 months, 0.68, p less than 0.05). This effect was most marked for those undergoing a systemic-pulmonary artery shunt (less than 1 month, 2.52; greater than 6 months, 0.43); by contrast, after banding of the pulmonary trunk, with or without additional repair of the aortic arch repair, medium-term risk was not altered (greater than 6 months, 1.13 and 0.91, respectively). These data will assist the clinician in making decisions concerning the management of infants with double-inlet ventricle and in the judicious use of palliative surgery.

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Survival before definitive operations was studied in 191 infants with double-inlet ventricle presenting before 1 year of age (1973 to 1988, median follow-up 8.5 years). The morphologic spectrum was broad, with a great prevalence of associated lesions. The actuarial survival rate before definitive repair was 57% at 1 year, 43% at 5 years, and 42% at 10 years, worse than prior reports because of the younger age at entry into our series. Analysis of univariate risk factors established that right atrial isomerism (18% of the group, relative risk 2.9), common atrioventricular orifice (42%, 2.0), pulmonary atresia (20%, 3.4), obstruction of the systemic outflow tract (18%, 2.5), and extracardiac anomalous pulmonary venous connection (13%, 3.1) were strongly associated with poorer survival. Pulmonary stenosis (40%, 0.35), balanced pulmonary blood flow (9%, 0.40), and presentation at an older age (3%, 0.42 to 0.18) were beneficial (p less than 0.05 to 0.0001). Multivariate analysis allowed the creation of patient-specific curves for prediction of survival for different anatomic and physiologic variants of double-inlet ventricle. A simple additive index was then derived from the multivariate Cox coefficients to enable stratification of risk for these morphologic subgroups of patients and so assist in the making of clinical decisions in infancy.

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The fate of 191 infants with double-inlet ventricle was studied to determine the influence of morphologic characteristics at presentation and subsequent management on the potential for, and timing of, definitive repair by the Fontan operation or ventricular septation. At presentation, 136 patients (71%) were potential candidates for a Fontan procedure. Actuarial survival was better than for those deemed unsuitable for either definitive option (n = 55; 68% versus 28% at 1 year; p less than 0.001), but still, only 78 patients (57%) were known to be alive and suitable candidates at 2 years of age. This was largely due to death after presentation with low cardiac output (n = 19) and at palliative operation (20 of 98 surgically treated patients). The adverse events of late sudden death (n = 14) and the development of new features precluding a Fontan operation (n = 18) mostly occurred before 4 years of age (n = 22). Patients requiring no operation and those who underwent a systemic-pulmonary arterial shunt fared better than those who underwent isolated banding of the pulmonary trunk (9/13 and 25/42 alive and suitable versus 14/33; p less than 0.05), and than those who required aortic arch repair together with banding (1/12; p less than 0.01), because of the development of subaortic stenosis in the latter group. In contrast, only 43 patients (23% of all 191 patients) had morphologic features that were additionally compatible with future ventricular septation. Actuarial survival free of adverse events for these 43 patients was similar to that of the 136 patients considered suitable for a Fontan operation. Thus management in infancy must be aimed at maintaining potential for a future Fontan operation, which itself should not be delayed, for most patients, beyond 3 years of age, because of the prevalence of adverse events with increasing age.

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Hearts from 29 children with complete transposition were examined postmortem to analyze the pattern of the coronary arteries and the origin of the sinus node artery. Four patterns of origin and distribution of the coronary arteries were found. In all, the coronary arteries arose from one or both of the facing sinuses, which were termed sinus 1 and sinus 2. The patterns were left coronary artery from sinus 1 and right coronary artery from sinus 2; anterior descending artery from sinus 1 and right coronary artery and circumflex artery from sinus 2; left coronary and right coronary from sinus 2; and circumflex artery from sinus 2 and right coronary artery from sinus 1. This study highlights the patterns of the coronary arteries in complete transposition and categorizes the variability using a simple classification. It also emphasizes the surgical significance of variations in distribution of the sinus node artery.

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Foram estudados 27 casos portadores de miocardiopatia hipertrofica registrados na Instituicao com diagnostico efetuado pelo ecocardiograma modulo "M" e confirmado, em alguns deles, pela angiocardiografia. A idade media foi 58 anos e nao houve predominancia de um sexo (relacao 1,07:1).A dor toracica foi referida por 85% dos pacientes, tendo carater anginoso em apenas 29% deles. Detectou-se um sopro sistolico em 48% dos casos e um "click" sistolico em 29,6%. O eletrocardiograma convencional mostrou-se alterado em 85% dos pacientes e o teste de esforco em 40%. O ecocardiograma evidenciou um padrao de hipertrofia concentrica do ventriculo esquerdo em 16 pacientes e de hipertrofia septal assimetrica nos 11 restantes com 15% desses mostrando sinais de obstrucao a ejecao ventricular esquerda. Com os nitratos houve piora dos sintomas em 74% dos pacientes e os bloqueadores beta-adrenergicos ou dos canais lentos do calcio diminuiram ou aboliram os sintomas em 95% dos casos

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Foram revisadas as prescricoes para pacientes indigentes internados em um hospital-escola, tendo sido observado que estas sao feitas em um numero semelhante a de outros meios. Ha semelhancas tambem em relacao aos grupos farmacologicos mais prescritos. Nao se observaram diferencas quanto ao numero de medicamentos prescritos em relacao a patologia que ocasionou a baixa hospitalar ou a idade do paciente

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