Children's Hospital Colorado
Pulmonary Hypertension Program

Pulmonary Hypertension

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What is pediatric pulmonary hypertension?

Pediatric pulmonary hypertension (PH) is a rare disorder of high blood pressure in the blood vessels of the lungs. Pulmonary blood pressure rises when arteries and capillaries in the lungs become narrowed, blocked or destroyed, making it harder for blood to flow through the lungs. Because the vessels in the lungs and heart are 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 has difficulty pushing against the pressure from the lungs, which may eventually lead to heart failure.

What causes pediatric PH?

In some cases, there is no underlying cause of the high blood pressure in the lungs. This type of PH is called idiopathic pulmonary arterial hypertension (IPAH). There are also genetic forms of PH, called heritable pulmonary arterial hypertension (HPAH). In some cases, PH is the result of other conditions, which is known as associated pulmonary hypertension.

Causes of associated pulmonary hypertension include:

Some forms of PH will improve or resolve with proper treatment. Most forms of PH require lifelong treatment.

Groups of pulmonary hypertension

There are five groups of pulmonary hypertension:

  • Group 1 pulmonary hypertension is referred to as pulmonary arterial hypertension (PAH) and affects the small arteries in the lungs, making them narrow, stiff and thick. Conditions may include genetic mutations, congenital heart disease, persistent pulmonary hypertension of the newborn (PPHN), and others.
  • Group 2 pulmonary hypertension is due to issues between blood vessels and the left side of the heart or differences in the left side of the heart, such as a weakened left heart muscle or issues with left heart valves. These issues cause blood to back up into the lungs resulting in high pressure.
  • Group 3 pulmonary hypertension is due to lung diseases such as bronchopulmonary dysplasia (BPD), lung hypoplasia (small lungs), congenital diaphragmatic hernia (CDH), sleep disordered breathing, developmental lung disease, interstitial lung disease or pulmonary fibrosis.
  • Group 4 chronic thromboembolic pulmonary hypertension (CTEPH) is due to chronic blood clots or blockages in the pulmonary arteries.
  • Group 5 pulmonary hypertension can be due to multiple factors, including thyroid, blood disorders, metabolic disorders and complex congenital heart disease.

Next steps

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    Learn more about the Pulmonary Hypertension Program
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Get to know our pediatric experts.

Dunbar Ivy, MD

Dunbar Ivy, MD

Cardiology - Pediatric

Jessica MacLean, FNP

Jessica MacLean, FNP

Family Medicine

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Benjamin Frank, MD

Benjamin Frank, MD

Cardiology - Pediatric, Pediatrics

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Charles Simpkin, DO

Charles Simpkin, DO

Cardiology - Pediatric, Pediatrics

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