Pediatric Pulmonary Hypertension

At the time of initial PH diagnosis, a comprehensive history and physical examination, combined with diagnostic testing for assessment of PH pathogenesis/classification and formal assessment of cardiac function, should be performed before the initiation of therapy at an experienced center. ( B , I )

Imaging to diagnose pulmonary thromboembolic disease, peripheral pulmonary artery stenosis, pulmonary vein stenosis, pulmonary veno-occlusive disease (PVOD), and parenchymal lung disease should be performed at the time of diagnosis. (B, I)

After a comprehensive initial evaluation, serial echocardiograms should be performed. More frequent echocardiograms are recommended in the setting of changes in therapy or clinical condition. (B, I)

Cardiac catheterization is recommended before initiation of PAH-targeted therapy. (B, I)

Exceptions may include critically ill patients requiring immediate initiation of empirical therapy. (B, I)

Cardiac catheterization should include acute vasoreactivity testing (AVT) unless there is a specific contraindication. (A, I)

The minimal hemodynamic change that defines a positive response to AVT for children should be considered as a ≥20% decrease in PAP and pulmonary vascular resistance (PVR)/systemic vascular resistance (SVR) without a decrease in cardiac output. (B, I)

Repeat cardiac catheterization is recommended within 3 to 12 months after initiation of therapy to evaluate response or with clinical worsening. (B, I)