Table 1. Potential Interventions for Children with Severe PH:
Considerations for Patient Selection, Approach, and
Caution Advised
Patient Selection
ASD Intervention • Presence of right heart failure in a child with disease
progression.
• Children with impaired systemic blood flow due to reduced
left heart filling that leads to syncope are more likely to
respond favorably to creation or enlargement of an ASD.
• For children with PH on ECMO without reversible cause
for deterioration, ASD intervention can be an alternative
strateg y to avoid peripheral ECMO as a bridge to lung
transplant.
Pulmonary-to-systemic
Shunt
• Presence of suprasystemic pulmonary artery pressures, where
the purpose of the pulmonary-to-systemic shunt is to be a
pressure-unloading shunt for the right ventricle to improve
its function.
• In urgent situations, a pulmonary-to-systemic shunt is a
better option than lung transplant due to wait times for
transplantation.
• Compared to lung transplant, complexity of care after a
pulmonary-to-systemic shunt may be less burdensome on
families.
Lung Transplantation • Considerations for referral for lung transplantation are
outlined in Table 2. Early referral is recommended due to the
limited availability of suitable organs for children.
• ECMO is not a contraindication for lung transplant if the
following criteria are met:
▶ Single-organ (lung ) failure
▶ Neurologically intact with minimal sedation
▶ Actively participating in physical rehabilitation, preferably
ambulating if age appropriate
▶ Receiving primarily enteral nutritional support.
• For consideration of ECMO prior to lung transplant, it is
preferable that the patient is already on the transplant wait
list at time of initiation to reduce wait list mortality.
