Pulmonary Hypertension and Congenital Heart Disease
Pulmonary hypertension associated with congenital heart disease can result from various heart and blood vessel abnormalities that develop before a person is born.
Related Conditions
Some types of PH are secondary to an associated condition, such as sleep apnea, lupus or sarcoidosis. Having an associated condition can increase a person's risk for PH. Sometimes PH may improve when the underlying condition is successfully treated.
Many pulmonary hypertension patients are diagnosed with PH in association with one or more related conditions. Related or associated conditions are other diseases or conditions that may raise the risk of pulmonary hypertension.
Research shows that up to 30% of scleroderma patients, 20-40% of sickle cell patients and one of every 200 HIV patients develop at least mild PH. Other conditions commonly associated with PH include mixed connective-tissue disease, congenital heart disease, chronic obstructive pulmonary disease, hereditary hemorrhagic telangiectasia, sleep apnea, liver disease and lupus. PH has also been linked to the use of certain drugs and toxins, including fenfluramine, dexfenfluramine and methamphetamines.
Learn more about these conditions by visiting the pages below.
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- Pulmonary Hypertension and Chronic Obstructive Pulmonary Disease
Pulmonary hypertension associated with chronic obstructive pulmonary disease refers to progressive lung diseases caused by reduced air flow in and out of the lungs.
- Pulmonary Hypertension and Hereditary Hemorrhagic Telangiectasia
Hereditary hemorrhagic telangiectasia is a genetic disorder that causes abnormalities of blood vessels, usually resulting in excessive bleeding. It often is difficult to detect pulmonary hypertension in persons with HHT and there are also cases where PH is diagnosed first before a person realizes they have HHT.
- HIV-Associated Pulmonary Hypertension
Human immunodeficiency virus is a chronic infection known to be associated with Group 1 PH or pulmonary arterial hypertension.
- Pulmonary Hypertension and Interstitial Lung Disease
There are many types of interstitial lung disease, which are related to the network of lung tissue that supports air sacs in the lungs. These lung diseases can lead to Group 3 pulmonary hypertension.
- Liver Disease-Associated Pulmonary Hypertension
Portopulmonary hypertension is a type of Group 1 PH that occurs as a result of portal hypertension and advanced liver disease. Patients with PoPH experience similar symptoms as patients with other forms of pulmonary arterial hypertension.
- Lupus-Associated Pulmonary Hypertension
In rare cases, people with the autoimmune condition, systemic lupus erythematosus, develop Group 1 pulmonary hypertension or PAH.
- Pumonary Hypertension Related to Meth Use
People who use methamphetamine have an increased risk of heart and lung diseases including Group 1 PH or pulmonary arterial hypertension.
- Pulmonary Hypertension and Sarcoidosis
Sarcoidosis, an inflammatory disease that can affect many organs and in particular the lungs, increases the risk for sarcoidosis-associated pulmonary hypertension.
- Pulmonary Hypertension and Schistosomiasis
Schistosomiasis is a parasitic disease caused by a flatworm present in fresh water sources in the Global South. Some of the flatform’s eggs may migrate to the lung, causing inflammation that leads to pulmonary arterial hypertension.
- Pulmonary Hypertension and Scleroderma
Group 1 pulmonary hypertension affects up to 15% of those with scleroderma, an autoimmune disease that targets connective tissues and blood vessels. Because the complication is so common, all scleroderma patients should be screened annually for PAH, even if they do not have any symptoms.
- Pulmonary Hypertension and Sickle Cell Disease
Pulmonary hypertension is an increasingly recognized complication of sickle cell disease, a type of inherited anemia. Studies show that 6 to 11% of adults with sickle cell disease develop PH.
- Pulmonary Hypertension Related to Sjögren’s
Sjögren’s is the third most common autoimmune disease and causes inflammation of the salivary, tear and respiratory glands. Associated with other connective tissue disorders, Sjögren’s can cause lung damage that may lead to Group 1 pulmonary hypertension.
- Pulmonary Hypertension Related to Sleep Apnea
Mild-to-moderate pulmonary hypertension is a common complication of sleep apnea, a sleep disorder in which breathing repeatedly stops and starts while one is asleep.