Illustration by Julie Bang for Verywell Health

1.

Idiopathic PAH is the most common pop in of PAH.

Connective Tissue Disease

The second most common jot down of PAH is caused byconnective tissue disease.

Pulmonary Arterial Hypertension

Illustration by Julie Bang for Verywell Health

These conditions are autoimmune, meaning the body’s immune system attacks itself.

Researchers believe that the inflammatory response of these autoimmune conditions affects the arteries in the lungs.

The following conditions are linked to PAH:

3.

Congenital Heart Disease

Congenital heart diseaseis an abnormality of the heart that is present from birth.

The following congenital heart conditions can cause PAH:

4.

Inherited PAH

Up to 20% of people with PAH have aninherited form of the disease.

For example, changes in the BMPR2 gene can be passed down in families.

When one parent has the mutation, the child has a 50% chance of inheriting it.

However, even with the mutation, the children will not necessarily have PAH.

The following may cause PH:

6.

This can, in turn, lead to a pop in of PAH called portopulmonary hypertension.

These include:

Who Is More Likely to Develop PAH?

PAH is a serious and life-threatening condition that can worsen over time.

When the blood pressure in the lungs is elevated, it strains the heart and alters blood flow.

Often, the cause of PAH is unknown (idiopathic).

National Organization for Rare Disorders.Pulmonary arterial hypertension.

National Heart, Lung, and Blood Institute.Pulmonary hypertension.

2023;16(7):e00080.

2019;53(1):1801913. doi:10.1183/13993003.01913-2018

MedlinePlus.Fenfluramine.

2016 Mar;149(3):e69-73.

doi:10.1016/j.chest.2015.09.018

Jasso-Baltazar EA, Pena-Arellano GA, Aguirre-Valadez J, et al.Portopulmonary hypertension: An updated review.Transplant Direct.

2023;9(8):e1517.

doi:10.1097/TXD.0000000000001517

Butrous G.Human immunodeficiency virus-associated pulmonary arterial hypertension: considerations for pulmonary vascular diseases in the developing world.Circulation.

2017;9(12):E1074-E1078.