Natural and Unnatural History of Isolation of Pulmonary Artery of Ductal Origin (IPADO)

Objectives: Unilateral isolation of pulmonary artery of ductal origin is rare with a reported incidence of 1:200,000 live births (in the absence of a congenital heart defect). We report our experience in this specific patient group with regard to the treatment options and possible complications.

Methods: From 2002 to 2019 IPADO was diagnosed in our department in 14 patients. Seven patients had associated complex conotruncal heart defects, while in the remaining seven, it occurred solitary. Among those with “only” IPADO a left-sided aortic arch with isolation of the right pulmonary artery was present in four (57%), while three patients had a right-sided aortic arch with isolation of the left pulmonary artery (43%); only two patients (29%) were diagnosed in the neonatal period (median age at diagnosis 0.67 years). One female with delayed diagnosis presented with hemoptysis at 9 years of age. Treatment for resuscitation of the isolated pulmonary artery was initiated in five children: Primary ductal stenting in three patients and surgical shunt creation in two patients. In two patients (29%) with late diagnosis, no treatment was possible due to extreme hypoplasia of the pulmonary vascular bed. Among patients with associated conotruncal malformations, IPADO was detected in the neonatal period in five of seven (71%) patients. In these five patients, surgery to reconnect the isolated pulmonary artery was performed together with surgery for the associated conotruncal defect. Late resuscitation of the LPA by implantation of a left BT-shunt was unsuccessful in a 19-year-old patient due to development of pulmonary hypertension and hemoptysis, requiring an interventional occlusion of the shunt. In the remaining patient reconnection was not attempted because of severe hypoplasia of the pulmonary artery. Our patients with early treatment showed good development of the affected pulmonary arteries, following surgical and interventional reconnection both in isolated cases and those with complex CHD.

Conclusion: Early detection of IPADO in the neonatal period or infancy offers the possibility of resuscitation of the affected pulmonary artery. While IPADO associated with CHD is detected early, children with isolated IPADO are still at increased risk to remain undetected due to the paucity of clinical symptoms until they develop complications of the natural course.