Ten cases of pulmonary atresia with intact ventricular septum are presented. The groups in which these patients are classified are described, thinking that the mentioned classification is a simplification of the problem. After discussion of the dates apported by clinic, thorax X-Rays and electrocardiogram, authors come to the conclusion that they are not specific of this cardiopathy. Contribution of hemodinamic and angiocardiographic characteristics, paying special attention to the blood flow to lung vascular tree and in existance of intramyocardium sinusoid are shown. Description of the general tendencies of the most accepted surgical techniques in bibliography for resolution of both groups of patients is made.