Congenital structural abnormalities like pulmonary atresia, are compatible with intrauterine life, but once the baby is born, this defect causes life-threatening clinical manifestations. The patent ductus arteriosus plays a key role in the survival of these patients.Methods
We present the case of a newborn eutrophic, who in a few hours after birth has progressive desaturation, until SaO2=55%. Reviewing intrauterine images show right ventricular hypertrophy and tricuspid regurgitation. Echocardiographic evaluation shows pulmonary atresia with intact ventricular septum. The baby receives oxygen and continuous infusion with Prostaglandin. When we consider interventional or surgical therapeutic alternatives, the interventional option is chosen.Methods
Due to complications such as infection or the degration of the patient general condition, the transfer to a specialised cardiology unit is delayed. There is the opportunity to perform pulmonary valvotomy and ballon dilatation by transcatheter intervention to decompress the right ventricular, however despite a mild haemodynamic improvement there is no significant improvement of right ventricular function. Therefore, performing ductus arteriosus stenting under diuretics, vasodilatory and antiplatelet drugs, is the best solution with the possibility of correction that is termed ‘1½ ventricle circulation’ in the future.Results
General condition of the infant remains clinically stable, with slow weight gain and naso-gastric probe suppression after one month. Reassessment of the echocardiographic images at eight weeks show atrial septal defect with left-right shunt and restrictive stented patent ductus arteriosus. Right ventricular function has significantly improved which is suitable for biventricular correction. Also, the tricuspid valve regurgitation has reduced considerably.Conclusions
1. Ducto-dependent congenital heart disease after birth, will have dramatic implications if patent ductus arteriosus close.Conclusions
2. Pulmonary artery atresia with intact ventricular septum is a heart cyanogenic disease requiring prompt intervention. Maintenance of ductal patency is the first step in saving patients.Conclusions
3. General condition in infants is unpredictable, therefore to gain favourable results, an interdisciplinary team is required.