Commentary from Dr. Charlotte Van Dorn (Rochester, MN), section editor of Pediatric Cardiology Journal Watch:
Take Home Points:
- Pulmonary hypertension mortality has been decreasing over the last 2 decades in children and adults, likely secondary to increased awareness, more accurate diagnoses, better risk stratification and early initiation of combination pharmacotherapy.
- The lower limit of normal mean pulmonary arterial pressure (mPAP) was decreased from 24 mmHg to 20 mmHg.
- A subgroup of children with idiopathic pulmonary arterial hypertension (IPAH) are positive responders to acute vasoreactivity testing (AVT) and would be classified as PAH long-term responders to calcium channel blockers (CCB).
- PAH and pulmonary veno-occlusive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH) are now considered a spectrum of pulmonary vascular disease (PVD).
- Diagnostic methods and variables and their application to pediatric PH have been updated including echocardiography, cardiac MRI, CT and cardiac catheterization.
- New treatment algorithms, including medications for pediatric PH and PAH-CHD as well as drug-drug interactions are available.
This is an updated, comprehensive and practical guideline for healthcare providers providing care to children and young adults with pulmonary hypertension (PH) and pulmonary vascular disease (PVD). Methodology included an executive writing group consisting of 22 pediatricians, 7 adult congenital heart disease physicians, 1 adult pulmonologist and 1 thoracic surgeon. Specific features of this guideline include new patient groups such as PH associated with congenital heart disease (PAH-CHD), persistent PH of the newborn period (PPHN) as well as PH associated with bronchopulmonary dysplasia (BPD) or chronic lung disease (CLD). The guidelines also include new recommendations for the treatment of acute PH in the intensive care unit (ICU). This is also the first time the challenges of addressing PH in middle to low income regions are addressed.
