Patients with severe PH should consider surgery for any indication carefully, discussing with the care team their risk and exploring nonsurgical options. Cardiothoracic surgical and liver transplantation services might have highly specific criteria for treating patients with PH, but other essential and nonessential surgeries require individualized risk stratification. Surgery for patients with severe PH and right ventricular dysfunction should be performed at a center equipped to handle high-risk patients.
Other preventive measures. Patients with PAH should6,10:
remain current with immunization against influenza virus, SARS-CoV-2, and pneumococcal pneumonia
avoid high altitudes
use supplemental oxygen during air travel to keep arterial oxygen saturation > 91%.
Lung transplantation. Patients eligible for transplantation who (1) are at intermediate-to-high risk or high risk or (2) have a REVEAL (Registry to EValuate Early And Long-term pulmonary arterial hypertension disease management) risk score > 7, and who have had an inadequate response to oral combination therapy, should be referred for evaluation for lung transplantation. Placement on the list for lung transplantation is also recommended for patients at high risk of death and who have a REVEAL risk score ≥ 10 despite medical therapy, including a subcutaneous or IV prostacyclin analogue.6
PH in infants and children
The Pediatric Task Force of the 6th WSPH has applied the new definition proposed for adult PH (> 20 mm Hg mPAP) to children and infants > 3 months of age (see “Pulmonary hypertension in the pediatric population,” at left32-36).
SIDEBAR Pulmonary hypertension in the pediatric population
The onset of pulmonary hypertension (PH) in children can occur at any age and be of quite different causes than in adults. In newborns, pulmonary pressure drops rapidly during the week after delivery; in some cases, however, pressures remain elevated (> 20 mm Hg) despite healthy lungs. These asymptomatic newborns require close monitoring.32
Etiology. Pediatric PH can be persistent or transient. Prominent causes of persistent or progressive PH in children are pulmonary arterial hypertension (PAH) associated with congenital heart disease and developmental lung disease, such as bronchopulmonary dysplasia and idiopathic PAH. Major categories of congenital heart disease that cause PH are shunting lesions and left heart disease associated with elevated atrial pressure. Other causes are rare.33
Persistent PH of the newborn (PPHN) and PH due to diaphragmatic hernia are common causes of transient PH.34 In PPHN, pulmonary vascular resistance remains abnormally high after birth, resulting in right-to-left shunting of the circulation that, in turn, leads to hypoxemia unresponsive to usual measures. In most cases, signs of respiratory distress and hypoxia are noted within the first 24 hours of life. The most common cause of PPHN is infection.35
Evaluation. The typical diagnostic work-up of suspected pediatric PH is similar to what is undertaken in the adult population—varying, however, according to the specific suspected cause. As in adults, right heart catheterization remains the gold standard of diagnosis, and should be conducted at a pediatric PH expert center. As with adult patients, infants and children with PH should be managed by a multidisciplinary expert team.
Management. PAH-targeted medications (see “What are the options for managing PH?”) are used to treat PAH in children.36
CORRESPONDENCE Madhavi Singh, MD, 1850 East Park Ave., Suite 207, State College, PA 16803; msingh1@pennstatehealth.psu.edu
