ATS GUIDELINES Bundle

Approach Caution Advised • Creation or enlargement of an ASD can be done using a balloon atrial septostomy, static balloon dilation, an atrial flow regulator device, a stent, or surgery. • For children with a moderate to large ASD, an individualized approach to closure or partial closure should be taken, considering underlying disease, age, severity of PH, and need for other procedures. • For children with severe PH, a preexisting patent foreman ovale or small ASD should not undergo percutaneous or surgical closure. • Children with markedly elevated right atrial pressure or pulmonary vascular resistance are at risk for excessive right-to-left shunting from creating or enlarging an ASD. • For precautionary measures during ASD-intervention, immediate ECMO cannulation should be considered as a backup. • A transcatheter or surgical approach can be considered based on center expertise. • A transcatheter approach should be pursued if the patent ductus arteriosus (PDA) was recently closed or if there is a prominent aortic ampulla. • An anterior approach is considered the best surgical approach for a pulmonary-to-systemic shunt, to minimize complications and for future consideration of lung transplant. • PDA recanalization and stenting can be feasible weeks to months after echocardiographic closure. • There is insufficient data to support transcatheter creation of a pulmonary-to- systemic shunt when the PDA has been chronically closed or an alternate path from the left pulmonary artery to the aorta must be created. • For children on ECMO, pulmonary-to- systemic shunt should only be considered for palliative indications at a highly qualified center with expertise and experience. • For surgical pulmonary-to-systemic shunts, a lateral approach is considered a relative contraindication to lung transplant. • Children with PH should be considered for lung transplantation at a pediatric-capable program with experience and expertise caring for this high-risk patient population. • Early consultation or early referral to a lung transplant program facilitates transplant education for the patient and caregivers, addresses potential barriers to transplant, and optimizes the best chance for a successful outcome. • Bilateral lung transplantation is the best surgical approach for transplantation in children with PH especially those with more severe disease that requires ECMO support. • Combined heart-lung transplantation for PH is reserved for: ▶ Uncorrectable congenital heart disease ▶ Coexisting left ventricular dysfunction ▶ Technical issues » Massive right heart enlargement in young children » Donor lung constraints » Lower likelihood of airway caliber compromise with tracheal versus bi- bronchial anastomoses in small children • Children with PH, especially those on ECMO at time of lung transplant, are at high risk for primary graft dysfunction (PGD), so mitigation strateg y for PGD includes supportive care and early initiation of ECMO with the need to balance benefits and risks.

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