Case report: MRI evaluation of congenital coronary artery fistulae.

Congenital coronary artery fistula is a rare disease and MRI is a promising technique that may be useful to demonstrate the coronary artery tree. We report three patients who underwent cardiac MRI to investigate right coronary artery fistulae. On clinical examination, a continuous murmur was heard along the left sternal border, and chest X-ray showed moderate cardiomegaly with enlargement of right chambers in all patients. Transthoracic Doppler echocardiography showed fistulae in two cases; the third case was not demonstrated by transthoracic or transoesophageal echocardiography. MRI demonstrated the course of the fistulous vessels in all patients. All patients underwent surgical closure of their coronary artery fistulae. MRI may show detailed anatomy of congenital coronary artery fistulae and may be useful as an additional non-invasive method in their investigation.

[Pulmonary valve agenesis. Clinico-surgical evaluation of 32 patients]. / Agenesia de valva pulmonar. Avaliação clínico-cirúrgica de 32 pacientes.

PURPOSE: To study the surgical and clinical evolution of 32 cases with absent pulmonary valve to propose the ideal period of time for surgical correction. METHODS: Clinical and laboratorial analysis were performed in 32 infants, under 12 months of age, between 1980 an 1993, in an evolutive character. From the clinical viewpoint, hypoxic and/or congestive features were considered in previous and late periods related to surgical repair. Laboratorial studies as ECG (cavities overload), chest X-ray (cardiac size and pulmonary vascular markings) and echocardiogram (associated defects, pressure gradients and anatomical aspects of pulmonary arteries) were also analyzed. Cardiac catheterization was performed in 15 patients. RESULTS: Early cyanosis in 84% of cases and "to and for "murmur in 90% of them facilitate clinical diagnosis in whom tetralogy of Fallot was associated in 30 patients. Refractory respiratory and cardiac insufficiency were responsible for operative indication in 12 patients, half of them, operated on under 12 months of age, died. Survival patients were repaired between two to 11 years old. Four deaths occurred early in life, before any surgical consideration and the 16 remaining patients will electively be considered for an opportune repair. CONCLUSION: Conservative clinical treatment is indicated, waiting for a more rigid bronchial wall can support the pressure of the dilated pulmonary arteries. This way, surgical repair is postponed for at least two years of age.

[Agenesis of the right pulmonary artery with severe pulmonary hypertension, attenuated by surgical correction]. / Agenesia da artéria pulmonar direita com acentuada hipertensão pulmonar, atenuada por correção cirúrgica.

A 22 months old infant with agenesis of right pulmonary artery, without other associated congenital heart defects, is reported. Reconstruction of pulmonary arteries by connecting them through a 7mm Goretex tube was performed, with clear clinical improvement in view of disappearance of signs of cardiac insufficiency and of hypoxemia. Contralateral pulmonary hypertension, at systemic level, decreased to 77% in the immediate postoperative period. So, this technique becomes the first option, before considering these cases for cardiopulmonary transplantation.

[Goldenhar syndrome. Report of 5 cases in association with cardiac malformations]. / Síndrome de Goldenhar. Relato de cino casos em associação com malformações cardíacas.

The paper presents five cases of the Syndrome described by Goldenhar, which consists in oculo-auriculo-vertebral alterations. Classic descriptions relate the presence of epibulbar dermoid, pre-auricular appendixes, mandible hypoplasia and vertebral anomalies. Excepting by some vertebral alterations, all the other anomalies were common to the five cases. This syndrome may be accompanied by congenital cardiopathy in a percentage varying from 15 to 50%, depending on the publications. The reason of hospitalization was the real presence of congenital cardiopathy of important clinical repercussion. Three patients had Tetrad of Fallot, one transposition of the great arteries, and the last one total annomalous pulmonary venous connection, which died due to clinical complications. The four patients were submitted to surgical correction of their cardiac defects, being the face defects programmed to the corrected later on.

Aneurisma solitário de artéria pulmonar no curso de endocardite infecciosa. Relato de caso / Solitary aneurysm of the pulmonary artery in the course of infectious endocarditis. A case report

E relatado o caso de uma paciente de 7 anos de idade, portadora de comunicacao interventricular, com moderada repercussao hemodinamica. Apresentou, no curso de endocardite infecciosa por S. aureus, formacao de aneurisma solitario do ramo lobar medio de arteria pulmonar direita. Sao feitos comentarios dos aspectos clinicos, laboratoriais, etiopatogenia e conduta adotada no caso

Importância da ecocardiografia bidimensional na detecçäo e avaliaçäo dos derrames pericárdicos / The importance of two-dimensional echocardiography in the detection and evaluation of pericardial effusion

Os derrames pericárdicos, principalmente septados, podem apresentar sérias dificuldades para sua detecçäo e quantificaçäo pela ecocardiografia convencional. A ecocardiografia bidimensional, em auxílio a modalidade M, permite a detecçäo mais correta dos derrames generalizados, pela regiäo apical e subxifóide. Foram estudados 22 pacientes portadores de derrame pericárdico, dos quais quatro eram septados e oito eram pós-operatórios. Estabeleceram-se as diferenças ecocardiográficas entre os diversos tipos e, em 10 casos, relacionou-se com o aspecto radiológico e com o volume de líquido extraído pela punçäo pericárdica ou pela pericariotomia, mostrando uma razoável correlaçäo (r = 0,76). Conclui-se que a ecocarddiografia bidimensional permite a detecçäo de derrames pericárdicos, especialmente os septados situados fora do alcance do sistema convencional, assim como a sua quantificaçäo, com boa confiabilidade

Cor triatriatum. Estudo clinico de seis casos. / Cor triatriatum. Clinical study of 6 cases

Os autores estudam 6 casos de cor triatriatum quanto aos dados clinicos, exames fisico e subsidiarios, incluindo o valor inestimavel representado pelo ecocardiograma em 3 dos casos, bem como a definicao diagnostica dada pelo estudo hemodinamico e cineangiocardiografico realizado em 4. Comentam-se, ainda, os achados operatorios de 4 casos e de necropsia de 1, evidenciado o bom prognostico daqueles que foram operados