Transcatheter stenting of arterial duct in duct-dependent congenital heart disease

Introduction. Critical congenital heart diseases (CHD) are mostly duct-dependent and require stable systemic-pulmonary communication. In order to maintain patency of the ductus arteriosus (DA), the first line treatment is Prostaglandin E1 and the second step is the surgical creation of aortic-pulmonary shunt. To reduce surgical risk in neonates with the critical CHD, transcatheter stenting of DA can be performed in selected cases. Case Outline. A four-month old infant was diagnosed with the pulmonary artery atresia with ventricular septal defect (PAA/VSD). The left pulmonary artery was perfused from DA, and the right lung through three major aortopulmonary collaterals (MAPCAs). A coronary stent was placed in the long and critically stenotic DA, with final arterial duct diameter of 3.5 mm, and significantly increased blood supply to the left lung. After the procedure, the infant’s status was improved with regard to arterial oxygen saturation, feeding and weight gain. During the follow-up, one year later, aortography revealed in-stent stenosis. The left pulmonary artery, as well as the branches, was well-developed and the decision was made to proceed with further surgical correction. Conclusion. Stenting of DA can be an effective alternative to primary surgical correction in selected patients with duct-dependent CHD

The role of modern imaging techniques in the diagnosis of malposition of the branch pulmonary arteries and possible association with microdeletion 22q11.2

Malposition of the branch pulmonary arteries is a rare malformation with two forms. In the typical form, pulmonary arteries cross each other as they proceed to their respective lungs. The "lesser form" is characterised by the left pulmonary artery ostium lying directly superior to the ostium of the right pulmonary artery, without crossing of the branch pulmonary arteries. Malposition of the branch pulmonary arteries is often associated with other congenital heart defects and extracardiac anomalies, as well as with 22q11.2 microdeletion. We report three infants with crossed pulmonary arteries and one adolescent with "lesser form" of the malformation. The results suggest that diagnosis of malposition of the branch pulmonary arteries could be challenging if based solely on echocardiography, whereas modern imaging technologies such as contrast computed tomography and magnetic resonance angiography provide reliable establishment of diagnosis. In addition, we performed the first molecular characterisation of the 22q11.2 region among patients with malposition of the branch pulmonary arteries and revealed a 3-megabase deletion in two out of four patients

The role of modern imaging techniques in the diagnosis of malposition of the branch pulmonary arteries and possible association with microdeletion 22q11.2

Malposition of the branch pulmonary arteries is a rare malformation with two forms. In the typical form, pulmonary arteries cross each other as they proceed to their respective lungs. The "lesser form" is characterised by the left pulmonary artery ostium lying directly superior to the ostium of the right pulmonary artery, without crossing of the branch pulmonary arteries. Malposition of the branch pulmonary arteries is often associated with other congenital heart defects and extracardiac anomalies, as well as with 22q11.2 microdeletion. We report three infants with crossed pulmonary arteries and one adolescent with "lesser form" of the malformation. The results suggest that diagnosis of malposition of the branch pulmonary arteries could be challenging if based solely on echocardiography, whereas modern imaging technologies such as contrast computed tomography and magnetic resonance angiography provide reliable establishment of diagnosis. In addition, we performed the first molecular characterisation of the 22q11.2 region among patients with malposition of the branch pulmonary arteries and revealed a 3-megabase deletion in two out of four patients