Pulmonary Hypertension: Overview

What is pulmonary hypertension?

Pulmonary hypertension (PH) is a rare disorder of high blood pressure in the blood vessels that line the lungs. Pulmonary blood pressure rises when arteries and capillaries in the lungs become narrowed, blocked, or destroyed, making it hard for blood to flow through the lungs. Because the vessels of the lung and the heart are physically connected, this makes blood pressure in the heart rise and forces the heart to work harder than normal.

If the condition goes untreated, the heart cannot push hard enough against the lung pressures, which may eventually lead to heart failure.

What causes pulmonary hypertension in children?

There are several reasons a child could have pulmonary hypertension. Congenital heart defects and lung disease are common causes of hypertension in children.

Other causes include:

  • Sleep apnea
  • Altitude effects
  • Blood clotting disorders
  • Autoimmune diseases
  • Liver disease
  • Familial disease
  • Persistent Pulmonary Hypertension of the Newborn (PPHN)

In the above cases, the pulmonary hypertension is secondary because the rise in blood pressure was the result of another condition. This is known as associated pulmonary arterial hypertension (APAH), previously known as secondary pulmonary hypertension.

Other times, there is no underlying reason causing the blood pressure increase. This is called idiopathic pulmonary arterial hypertension (IPAH), previously known as primary pulmonary hypertension (PPH). Idiopathic pulmonary hypertension tends to affect girls more than boys. Children of any age can develop the condition.

Learn about our world-renown Pulmonary Hypertension Program.