[Agensis of the pulmonary valve with tetralogy of Fallot].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to J I Azcuna.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Four cases between one day of life and seven years are presented. Two of them were males and two females. All of them had cardiac pulsation palpable to the right of the sternum, one with some bulging of the left hemitorax. In two cases a systolic murmur was heard over the second intercostal space associated with fixed splitting of the second sound and the other case presented recurrent infections processes of the lung dying ultimately from bronchoneumonia. On the simple X-ray displacement of the mediastine towards the right side, dextroposition of the heart, right pulmonary hyoplasia, enlargement of the right atrium and image in "scimitar" (obvious in two cases and possibly existed in the other two) was observed. In three cases a lung scan was carried out which showed a marked decrease in perfusion of the right lung. Angiocardiography in all cases as able to demonstrate anomalous venous drainage of the right lung to inferior vena cava directly.
In this article we try to show the value of radiography and angiocardiography in primary endocardial fibroelastosis. The 20 CASEs studied in this report, were angiocardiographically and/or necropsically proved. In spite of different opinions, we think that it is possible to reach an approximate diagnosis of this condition if we use the clinical and angiohemodinamic examinations. On the other hand, it is necessary to point out that pathology is characteristic. The most important radiological signs were cardiomegaly, together with venocapilar congestion, both of variable magnitude, and dependent on the degree of cardiac insufficiency. By means of angiocardiography we could appreciate a great dilatation and hypertrophy of the left ventricle and a reduction of the mobility and distensibility of this cavity along the cardiac cycle.