[Compression of both lungs by severe right heart dilatation in congenital pulmonary atresia]

Neonatal cyanosis due to pulmonary atresia is seen in one-third of all cyanotic neonates with congenital malformation of the heart. In both types of this disorder survival is dependent on patency of the ductus arteriosus. Consequently the first days of life most often show dramatic changes in clinical status, sometimes similar to that in patients with transposition of the great arteries. Intensive care treatment of the baby, rapid diagnostic management and early operative intervention may improve the poor prognosis. Because of the numerous cardiopulmonary and diagnostic complications, only 50 percent of patients will reach 4 weeks of age if not operated. We describe an unusual case of type 2 pulmonary atresia with intact ventricular septum in which pulmonary complications led to irreversible cardiac failure. Aneurysmatic enlargement of the right atrium caused compression atelectasis of nearly all parts of the lungs, followed by a unilateral pneumothorax after postnatal emergency ventilation. Clinical and circulatory status deteriorated. Successful pharmacological or operative therapy was impossible at this point. We want to stress upon the fact that - despite the hopeless situation of our patient - we were able to establish diagnosis by improvised procedures, which can be performed in every ICU.