Materiały źródłowe

• #1 Pulmonary hypertension – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697

  When blood vessels in the lungs become thickened, narrowed, blocked or destroyed, it’s harder for blood to flow through the lungs. As a result, blood pressure goes up in the lungs. This condition is called pulmonary hypertension. […] Pulmonary hypertension is caused by changes in the cells that line the lung arteries. The changes can make the artery walls narrow, stiff, swollen and thick. It gets harder for blood to flow through the lungs. […] Pulmonary hypertension is sorted into five groups, depending on the cause. […] Causes include: Unknown cause, called idiopathic pulmonary arterial hypertension. Changes in a gene passed down through families, called heritable pulmonary arterial hypertension. Use of some medicines or illicit drugs, including methamphetamine. Heart condition present at birth, called a congenital heart defect. Other health conditions, including scleroderma, lupus and cirrhosis.

• #2 About Pulmonary Hypertension | Heart Disease | CDC

  https://www.cdc.gov/heart-disease/about/pulmonary-hypertension.html

  Pulmonary hypertension is a condition that affects the blood vessels in the lungs. It develops when the blood pressure in your lungs is higher than normal. […] Some common underlying causes of pulmonary hypertension include high blood pressure in the lungs’ arteries due to some types of congenital heart disease, connective tissue disease, coronary artery disease, high blood pressure, liver disease (cirrhosis), blood clots to the lungs, and chronic lung diseases like emphysema. Genetics also play a role. […] Pulmonary hypertension can happen in association with many other diseases, such as lung disease and heart disease. Heart failure is common in pulmonary hypertension.

• #3 Pulmonary hypertension – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697

  When blood vessels in the lungs become thickened, narrowed, blocked or destroyed, it’s harder for blood to flow through the lungs. As a result, blood pressure goes up in the lungs. This condition is called pulmonary hypertension. […] Pulmonary hypertension is caused by changes in the cells that line the lung arteries. The changes can make the artery walls narrow, stiff, swollen and thick. It gets harder for blood to flow through the lungs. […] Pulmonary hypertension is sorted into five groups, depending on the cause. […] Causes include: Unknown cause, called idiopathic pulmonary arterial hypertension. Changes in a gene passed down through families, called heritable pulmonary arterial hypertension. Use of some medicines or illicit drugs, including methamphetamine. Heart condition present at birth, called a congenital heart defect. Other health conditions, including scleroderma, lupus and cirrhosis.

• #4 Pulmonary Hypertension: Symptoms, Treatment

  https://my.clevelandclinic.org/health/diseases/6530-pulmonary-hypertension-ph

  Pulmonary hypertension has many different causes. Its usually a complication of heart disease or lung disease. But many other diseases and environmental factors can raise your risk for PH. […] Causes of pulmonary hypertension vary widely depending on the type of PH you have. They include a range of diseases and underlying conditions as well as environmental exposures (toxins and drugs). […] There are many causes of pulmonary arterial hypertension. They include: Congenital heart disease. Diet pills like fen-phen (which can cause PAH years after use). Genetic mutations. Glycogen storage diseases. HIV. Liver disease. Lupus. Portal hypertension. Pulmonary capillary hemangiomatosis. Pulmonary veno-occlusive disease. Schistosomiasis. Scleroderma. Recreational drugs like methamphetamine. […] Heart problems are a common cause of pulmonary hypertension. Because the left and right sides of your heart work together, a problem with the left side also affects the right side. The right side of your heart is responsible for pumping blood into your pulmonary arteries. So, left-sided heart problems cause a chain reaction that affects the rest of your heart, your pulmonary arteries and your lungs.

• #5 Pulmonary hypertension – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_hypertension

  The cause is often unknown. […] Risk factors include a family history, prior pulmonary embolism (blood clots in the lungs), HIV/AIDS, sickle cell disease, cocaine use, chronic obstructive pulmonary disease, sleep apnea, living at high altitudes, and problems with the mitral valve. […] The underlying mechanism typically involves inflammation and subsequent remodeling of the arteries in the lungs. […] Pulmonary hypertension is a pathophysiologic condition with many possible causes. […] A distinction was made between primary PH (resulting from a disease of the pulmonary arteries) and secondary PH (resulting secondary to other, non-vascular causes). […] Most recently in 2022, the WHO guidelines were updated by the European Society of Cardiology (ESC) and European Respiratory Society (ERS).

• #6 Types of Pulmonary Hypertension: The WHO Groups – Pulmonary Hypertension Association

  https://phassociation.org/types-pulmonary-hypertension-groups/

  Not all pulmonary hypertension (PH) is the same. PH is a general term used to describe high blood pressure in the lungs from any cause. There are five different groups of PH based on different causes. Sometimes, people refer to the five types of PH as WHO Groups because the World Health Organization originally defined the types. […] WHO Group 1 refers to pulmonary arterial hypertension, which is caused when the arteries in the lungs become narrowed, thickened or stiff. […] Idiopathic PAH is PAH that occurs without a clear cause. Heritable PAH (HPAH) is linked to genes that are inherited from family members. PAH can also develop in association with other medical conditions including congenital heart disease, liver disease, HIV and connective tissue diseases — such as scleroderma and lupus. PAH can even be associated with past or present drug use, such as the use of methamphetamine or certain diet pills.

• #7 Types of Pulmonary Hypertension: The WHO Groups – Pulmonary Hypertension Association

  https://phassociation.org/types-pulmonary-hypertension-groups/

  Not all pulmonary hypertension (PH) is the same. PH is a general term used to describe high blood pressure in the lungs from any cause. There are five different groups of PH based on different causes. Sometimes, people refer to the five types of PH as WHO Groups because the World Health Organization originally defined the types. […] WHO Group 1 refers to pulmonary arterial hypertension, which is caused when the arteries in the lungs become narrowed, thickened or stiff. […] Idiopathic PAH is PAH that occurs without a clear cause. Heritable PAH (HPAH) is linked to genes that are inherited from family members. PAH can also develop in association with other medical conditions including congenital heart disease, liver disease, HIV and connective tissue diseases — such as scleroderma and lupus. PAH can even be associated with past or present drug use, such as the use of methamphetamine or certain diet pills.

• #8 Learn About Pulmonary Arterial Hypertension | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-arterial-hypertension/learn-about-pulmonary-arterial-hypertension

  Pulmonary arterial hypertension (PAH) is a specific type of pulmonary hypertension that is caused when the tiny arteries in your lung become thickened and narrowed. This blocks the blood flow through your lungs which raises the blood pressure in the lungs and causes your heart to work harder to pump your blood through those narrowed arteries. Over time, your heart loses the ability to effectively pump blood throughout your body. […] PAH is unlike other forms of pulmonary hypertension, where high blood pressure in the lungs is caused by underlying heart or lung disease. Researchers believe that PAH occurs when there is injury to the cells that line the blood vessels of the lung, which over time results in this blood vessel disease. If the cause of this change is unknown it is referred to as idiopathic PAH. If the change is believed to be caused by a genetic mutation it is called heritable PAH. Approximately 15-20% of PAH patients have heritable PAH.

• #9 Types of Pulmonary Hypertension: The WHO Groups – Pulmonary Hypertension Association

  https://phassociation.org/types-pulmonary-hypertension-groups/

  Not all pulmonary hypertension (PH) is the same. PH is a general term used to describe high blood pressure in the lungs from any cause. There are five different groups of PH based on different causes. Sometimes, people refer to the five types of PH as WHO Groups because the World Health Organization originally defined the types. […] WHO Group 1 refers to pulmonary arterial hypertension, which is caused when the arteries in the lungs become narrowed, thickened or stiff. […] Idiopathic PAH is PAH that occurs without a clear cause. Heritable PAH (HPAH) is linked to genes that are inherited from family members. PAH can also develop in association with other medical conditions including congenital heart disease, liver disease, HIV and connective tissue diseases — such as scleroderma and lupus. PAH can even be associated with past or present drug use, such as the use of methamphetamine or certain diet pills.

• #10 Pulmonary Arterial Hypertension (PAH): Symptoms and Treatment

  https://my.clevelandclinic.org/health/diseases/23913-pulmonary-arterial-hypertension

  Pulmonary arterial hypertension (PAH) is a condition in which the small blood vessels in your lungs become narrow. […] PAH is one form of pulmonary hypertension. This is a general diagnosis for high blood pressure in your pulmonary arteries from any cause. […] Damage to the lining of the blood vessels in your lungs leads to pulmonary arterial hypertension. Its not always clear what causes this damage. When theres no clear cause, you have whats called idiopathic pulmonary arterial hypertension. […] Other times, there is a clear cause. Known causes of PAH include associated medical conditions, genetic mutations and certain drugs. […] Medical conditions that may lead to the development of PAH include: Congenital heart disease, Glycogen storage diseases, HIV, Liver disease, Lupus, Portal hypertension, Pulmonary capillary hemangiomatosis, Pulmonary veno-occlusive disease, Schistosomiasis, Scleroderma. […] Genetic mutations can also cause pulmonary arterial hypertension. […] Certain drugs can cause PAH, including diet pills like fen-phen and recreational drugs like cocaine and methamphetamine.

• #11 Types of Pulmonary Hypertension: The WHO Groups – Pulmonary Hypertension Association

  https://phassociation.org/types-pulmonary-hypertension-groups/

  Not all pulmonary hypertension (PH) is the same. PH is a general term used to describe high blood pressure in the lungs from any cause. There are five different groups of PH based on different causes. Sometimes, people refer to the five types of PH as WHO Groups because the World Health Organization originally defined the types. […] WHO Group 1 refers to pulmonary arterial hypertension, which is caused when the arteries in the lungs become narrowed, thickened or stiff. […] Idiopathic PAH is PAH that occurs without a clear cause. Heritable PAH (HPAH) is linked to genes that are inherited from family members. PAH can also develop in association with other medical conditions including congenital heart disease, liver disease, HIV and connective tissue diseases — such as scleroderma and lupus. PAH can even be associated with past or present drug use, such as the use of methamphetamine or certain diet pills.

• #12 Pulmonary Hypertension – Pulmonary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pulmonary-disorders/pulmonary-hypertension/pulmonary-hypertension

  Hereditary forms of PAH (autosomal dominant with incomplete penetrance) have been identified; mutations of the following genes have been found: Activin-like kinase type 1 receptor (ALK-1), Bone morphogenetic protein receptor type 2 (BMPR2), Caveolin 1 (CAV1), Endoglin (ENG), Growth differentiation factor 2 (GDF2), Potassium channel subfamily K member 3 (KCNK3), Mothers against decapentaplegic homologue 9 (SMAD9), T-box transcription factor 4 (TBX4). […] Mutations in BMPR2 (the gene coding for bone morphogenetic protein receptor type 2) cause 75% of cases of heritable PAH. […] In about 20% of cases of hereditary PAH, the causative mutations are unidentified. […] A mutation in the eukaryotic translation initiation factor 2 alpha kinase 4 gene (EIF2AK4) has been linked to pulmonary veno-occlusive disease, a form of PAH Group 1.

• #13 Pulmonary Hypertension – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK482463/

  Genetic mutations are associated with various pulmonary hypertension types, including IPAH, HPAH, and hereditary hemorrhagic telangiectasia (HHT) associated with PAH. Individuals with HPAH have an inheritable genetic mutation. People with IPAH have a genetic predisposition, but the mutations are sporadic. These groups are clinically indistinguishable. […] The drugs and toxins known to cause PAH include aminorex, fenfluramine, dexfenfluramine, benfluorex, methamphetamines, dasatinib, and toxic rapeseed oil. Some substances that may give rise to PAH include cocaine, phenylpropanolamine, L-tryptophan, St. John’s wort, amphetamines, interferon – and -, alkylating agents, bosutinib, direct-acting hepatitis C antivirals, leflunomide, and indirubin. […] Various CTDs are also known to cause PAH, including systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, Raynaud disease, and mixed connective tissue disease (MCTD). Among these, systemic sclerosis is the most notorious for causing PAH.

• #14 Pulmonary Hypertension – Pulmonary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pulmonary-disorders/pulmonary-hypertension/pulmonary-hypertension

  Certain medications and toxins are risk factors for PAH. Those definitely associated with PAH are fenfluramine, dexfenfluramine (discontinued in the United States), aminorex (discontinued in the United States), toxic rapeseed oil, benfluorex (not available in the United States), amphetamines, methamphetamines, and dasatinib. […] Similarly, other protein kinase inhibitors have also been linked to drug-induced pulmonary hypertension. […] Patients with hereditary causes of hemolytic anemia, such as sickle cell disease, are at high risk of developing pulmonary hypertension. The mechanism is related to intravascular hemolysis and release of cell-free hemoglobin into the plasma, which scavenges nitric oxide, generates reactive oxygen species, and activates the hemostatic system.

• #15 Types of Pulmonary Hypertension: The WHO Groups – Pulmonary Hypertension Association

  https://phassociation.org/types-pulmonary-hypertension-groups/

  Not all pulmonary hypertension (PH) is the same. PH is a general term used to describe high blood pressure in the lungs from any cause. There are five different groups of PH based on different causes. Sometimes, people refer to the five types of PH as WHO Groups because the World Health Organization originally defined the types. […] WHO Group 1 refers to pulmonary arterial hypertension, which is caused when the arteries in the lungs become narrowed, thickened or stiff. […] Idiopathic PAH is PAH that occurs without a clear cause. Heritable PAH (HPAH) is linked to genes that are inherited from family members. PAH can also develop in association with other medical conditions including congenital heart disease, liver disease, HIV and connective tissue diseases — such as scleroderma and lupus. PAH can even be associated with past or present drug use, such as the use of methamphetamine or certain diet pills.

• #16 Pulmonary Hypertension – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK482463/

  Genetic mutations are associated with various pulmonary hypertension types, including IPAH, HPAH, and hereditary hemorrhagic telangiectasia (HHT) associated with PAH. Individuals with HPAH have an inheritable genetic mutation. People with IPAH have a genetic predisposition, but the mutations are sporadic. These groups are clinically indistinguishable. […] The drugs and toxins known to cause PAH include aminorex, fenfluramine, dexfenfluramine, benfluorex, methamphetamines, dasatinib, and toxic rapeseed oil. Some substances that may give rise to PAH include cocaine, phenylpropanolamine, L-tryptophan, St. John’s wort, amphetamines, interferon – and -, alkylating agents, bosutinib, direct-acting hepatitis C antivirals, leflunomide, and indirubin. […] Various CTDs are also known to cause PAH, including systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, Raynaud disease, and mixed connective tissue disease (MCTD). Among these, systemic sclerosis is the most notorious for causing PAH.

• #17 Pulmonary Hypertension | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-hypertension

  Pulmonary hypertension is a serious condition where there is abnormally high pressure in the blood vessels between the lungs and the heart. […] There are numerous causes of pulmonary hypertension, which can be inherited or acquired as a result of other medical conditions such as heart disease, lung disease, or connective tissue disease. […] Group 1: Pulmonary arterial hypertension (PAH) refers to increased pressure in the vessels caused by obstruction in the small arteries in the lung, for a variety of reasons. These include „idiopathic” (no known cause or underlying condition); drug-related, HIV infection; connective tissue/autoimmune disorders (such as scleroderma) and others. […] Group 2: Pulmonary hypertension due to left-side heart disease. This is when long-term heart disease results in damage to the pulmonary arteries eventually causing PH.

• #18 Pulmonary Hypertension: Symptoms, Treatment

  https://my.clevelandclinic.org/health/diseases/6530-pulmonary-hypertension-ph

  Pulmonary hypertension has many different causes. Its usually a complication of heart disease or lung disease. But many other diseases and environmental factors can raise your risk for PH. […] Causes of pulmonary hypertension vary widely depending on the type of PH you have. They include a range of diseases and underlying conditions as well as environmental exposures (toxins and drugs). […] There are many causes of pulmonary arterial hypertension. They include: Congenital heart disease. Diet pills like fen-phen (which can cause PAH years after use). Genetic mutations. Glycogen storage diseases. HIV. Liver disease. Lupus. Portal hypertension. Pulmonary capillary hemangiomatosis. Pulmonary veno-occlusive disease. Schistosomiasis. Scleroderma. Recreational drugs like methamphetamine. […] Heart problems are a common cause of pulmonary hypertension. Because the left and right sides of your heart work together, a problem with the left side also affects the right side. The right side of your heart is responsible for pumping blood into your pulmonary arteries. So, left-sided heart problems cause a chain reaction that affects the rest of your heart, your pulmonary arteries and your lungs.

• #19 Pulmonary Arterial Hypertension: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/303098-overview

  PAH is categorized by the underlying etiology as follows: […] 1.1 Idiopathic PAH […] 1.2 Heritable PAH […] 1.3 Drug- and toxin-induced PAH […] 1.4 PAH associated with: […] 1.5 PAH with features of venous/capillaries (ie, Pulmonary veno-occlusive disease [POVD] and/or pulmonary capillary hemangiomatosis [PCH]) […] 1.6 Persistent pulmonary hypertension of the newborn (PPHN). […] The prognosis of patients with pulmonary arterial hypertension (PAH) is variable and depends on the etiology, severity, and treatment. […] Overall, although, etiology remains important for prognostication, patients with PAH secondary to connective-tissue disease, PVOD, and familial causes tend to have worse survival than patients with other etiologies of PAH.

• #20 Pulmonary hypertension | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/a-to-z/pulmonary-hypertension/

  Pulmonary arterial hypertension (PAH) is caused by problems with the smaller branches of the pulmonary arteries. In PAH, these changes are usually the core medical problem, rather than a condition that has started somewhere else and then affected the pulmonary arteries. […] PAH can be associated conditions that affect your blood vessels, such as pulmonary veno-occlusive disease a rare condition that causes high blood pressure in the lungs, pulmonary capillary hemangiomatosis when capillaries (tiny blood vessels) grow within the lungs, causing blockages. […] Other conditions or treatments associated with PAH include a condition affecting the structure or composition of your body tissue, such as scleroderma, surgery to artificially connect the aorta to the pulmonary artery, portal hypertension, HIV, certain medications or drugs such as fenfluramine and amphetamines, disorders of the thyroid gland, glycogen storage disorder, splenectomy, sickle cell anaemia.

• #21 Pulmonary Hypertension – Pulmonary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pulmonary-disorders/pulmonary-hypertension/pulmonary-hypertension

  Hereditary forms of PAH (autosomal dominant with incomplete penetrance) have been identified; mutations of the following genes have been found: Activin-like kinase type 1 receptor (ALK-1), Bone morphogenetic protein receptor type 2 (BMPR2), Caveolin 1 (CAV1), Endoglin (ENG), Growth differentiation factor 2 (GDF2), Potassium channel subfamily K member 3 (KCNK3), Mothers against decapentaplegic homologue 9 (SMAD9), T-box transcription factor 4 (TBX4). […] Mutations in BMPR2 (the gene coding for bone morphogenetic protein receptor type 2) cause 75% of cases of heritable PAH. […] In about 20% of cases of hereditary PAH, the causative mutations are unidentified. […] A mutation in the eukaryotic translation initiation factor 2 alpha kinase 4 gene (EIF2AK4) has been linked to pulmonary veno-occlusive disease, a form of PAH Group 1.

• #22 Pulmonary hypertension – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_hypertension

  Mutations in several genes have been associated with this condition these include bone morphogenetic protein receptor type 2 (BMPR2) and eukaryotic translation initiation factor 2 alpha kinase 4 gene (EIF2AK4). […] The pathogenesis of pulmonary arterial hypertension (WHO Group I) involves the narrowing of blood vessels connected to and within the lungs. […] In pulmonary hypertension due to lung diseases and/or hypoxia (WHO Group III), low levels of oxygen in the alveoli (due to respiratory disease or living at high altitude) cause constriction of the pulmonary arteries. […] In chronic thromboembolic pulmonary hypertension, or CTEPH (WHO Group IV), the initiating event is thought to be blockage or narrowing of the pulmonary blood vessels with unresolved blood clots.

• #23 Pulmonary Hypertension – Pulmonary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pulmonary-disorders/pulmonary-hypertension/pulmonary-hypertension

  Hereditary forms of PAH (autosomal dominant with incomplete penetrance) have been identified; mutations of the following genes have been found: Activin-like kinase type 1 receptor (ALK-1), Bone morphogenetic protein receptor type 2 (BMPR2), Caveolin 1 (CAV1), Endoglin (ENG), Growth differentiation factor 2 (GDF2), Potassium channel subfamily K member 3 (KCNK3), Mothers against decapentaplegic homologue 9 (SMAD9), T-box transcription factor 4 (TBX4). […] Mutations in BMPR2 (the gene coding for bone morphogenetic protein receptor type 2) cause 75% of cases of heritable PAH. […] In about 20% of cases of hereditary PAH, the causative mutations are unidentified. […] A mutation in the eukaryotic translation initiation factor 2 alpha kinase 4 gene (EIF2AK4) has been linked to pulmonary veno-occlusive disease, a form of PAH Group 1.

• #24 Pulmonary Hypertension: Symptoms, Treatment

  https://my.clevelandclinic.org/health/diseases/6530-pulmonary-hypertension-ph

  Pulmonary hypertension has many different causes. Its usually a complication of heart disease or lung disease. But many other diseases and environmental factors can raise your risk for PH. […] Causes of pulmonary hypertension vary widely depending on the type of PH you have. They include a range of diseases and underlying conditions as well as environmental exposures (toxins and drugs). […] There are many causes of pulmonary arterial hypertension. They include: Congenital heart disease. Diet pills like fen-phen (which can cause PAH years after use). Genetic mutations. Glycogen storage diseases. HIV. Liver disease. Lupus. Portal hypertension. Pulmonary capillary hemangiomatosis. Pulmonary veno-occlusive disease. Schistosomiasis. Scleroderma. Recreational drugs like methamphetamine. […] Heart problems are a common cause of pulmonary hypertension. Because the left and right sides of your heart work together, a problem with the left side also affects the right side. The right side of your heart is responsible for pumping blood into your pulmonary arteries. So, left-sided heart problems cause a chain reaction that affects the rest of your heart, your pulmonary arteries and your lungs.

• #25 What causes pulmonary hypertension? | Asthma + Lung UK

  https://www.asthmaandlung.org.uk/conditions/pulmonary-hypertension/causes

  According to the NHS and a study from the British Journal of General Practice, this is one of the most common causes of pulmonary hypertension. Research from a study in the American Heart Association journal, indicated that of 50,529 people with PH, 34.2% had group 2 PH (caused by left heart disease). […] In this group, pulmonary hypertension is the secondary condition. The condition causing the pulmonary hypertension is the primary condition. Some common primary conditions that can cause pulmonary hypertension are: chronic obstructive pulmonary disease (COPD), interstitial lung disease (such as pulmonary fibrosis), obstructive sleep apnoea (OSA), obesity hypoventilation syndrome. […] CTEPH occurs when blood clots (pulmonary embolism) block the flow of blood in arteries and blood pressure in the lungs increases.

• #26 What causes pulmonary hypertension? | Asthma + Lung UK

  https://www.asthmaandlung.org.uk/conditions/pulmonary-hypertension/causes

  According to the NHS and a study from the British Journal of General Practice, this is one of the most common causes of pulmonary hypertension. Research from a study in the American Heart Association journal, indicated that of 50,529 people with PH, 34.2% had group 2 PH (caused by left heart disease). […] In this group, pulmonary hypertension is the secondary condition. The condition causing the pulmonary hypertension is the primary condition. Some common primary conditions that can cause pulmonary hypertension are: chronic obstructive pulmonary disease (COPD), interstitial lung disease (such as pulmonary fibrosis), obstructive sleep apnoea (OSA), obesity hypoventilation syndrome. […] CTEPH occurs when blood clots (pulmonary embolism) block the flow of blood in arteries and blood pressure in the lungs increases.

• #27 Pulmonary hypertension – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697

  This is the most common form of pulmonary hypertension. Causes include: Left heart failure. Left-sided heart valve disease, including mitral valve or aortic valve disease. […] Causes include: Scarring of the lungs, called pulmonary fibrosis. Chronic obstructive pulmonary disease, also called COPD. A sleep disorder in which breathing repeatedly stops and starts, called sleep apnea. Being at high altitudes for extended periods of time, if you are at high risk of pulmonary hypertension. […] Causes include: Blood clots in the lungs that don’t go away. Tumors that block the pulmonary artery. […] Causes include: Blood disorders, including polycythemia vera and essential thrombocythemia. Inflammatory disorders such as sarcoidosis. Conditions that affect the body’s ability to break down certain sugars, including glycogen storage disease. Kidney disease. […] Eisenmenger syndrome can lead to pulmonary hypertension. […] The unrepaired hole in the heart causes oxygen-rich blood to mix with oxygen-poor blood. The blood then goes to the lungs instead of going to the rest of the body. This increases pressure in the pulmonary arteries.

• #28 Types of Pulmonary Hypertension: The WHO Groups – Pulmonary Hypertension Association

  https://phassociation.org/types-pulmonary-hypertension-groups/

  WHO Group 2 includes PH due to left heart disease. […] WHO Group 3 includes PH due to chronic lung disease and/or hypoxia (low oxygen levels). […] WHO Group 4 is called chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH can occur when the body is not able to dissolve a blood clot in the lungs. […] WHO Group 5 is where PH is secondary to other diseases in ways that are not well understood. […] Pulmonary hypertension often arises as a complication of an underlying disease. This can be true for most groups and functional classes.

• #29 What causes pulmonary hypertension? | Asthma + Lung UK

  https://www.asthmaandlung.org.uk/conditions/pulmonary-hypertension/causes

  According to the NHS and a study from the British Journal of General Practice, this is one of the most common causes of pulmonary hypertension. Research from a study in the American Heart Association journal, indicated that of 50,529 people with PH, 34.2% had group 2 PH (caused by left heart disease). […] In this group, pulmonary hypertension is the secondary condition. The condition causing the pulmonary hypertension is the primary condition. Some common primary conditions that can cause pulmonary hypertension are: chronic obstructive pulmonary disease (COPD), interstitial lung disease (such as pulmonary fibrosis), obstructive sleep apnoea (OSA), obesity hypoventilation syndrome. […] CTEPH occurs when blood clots (pulmonary embolism) block the flow of blood in arteries and blood pressure in the lungs increases.

• #30 Pulmonary hypertension | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/a-to-z/pulmonary-hypertension/

  Pulmonary hypertension may sometimes be associated with diseases affecting the left side of your heart. […] Because blood goes from the pulmonary arteries to the lungs and then to the left side of the heart, any problem with the left side of the heart could have a backwards effect on this circuit and affect the pulmonary arteries. […] Pulmonary hypertension can also be associated with lung diseases or hypoxia. […] These types of conditions can restrict the amount of oxygen that is getting into the lungs, and therefore into the blood. A low level of oxygen in the blood causes the pulmonary arteries to constrict, increasing the blood pressure and resulting in pulmonary hypertension. […] A foreign object, usually a blood clot, blocking or obstructing a vein, may sometimes cause pulmonary hypertension. […] Other, less common, causes of pulmonary hypertension include sarcoidosis, histiocytosis X, compression of the blood vessels in the lungs.

• #31 Pulmonary Hypertension: Symptoms, Treatment

  https://my.clevelandclinic.org/health/diseases/6530-pulmonary-hypertension-ph

  Lung problems are another common cause of pulmonary hypertension. Some people with left-sided heart disease also have lung disease or a lack of oxygen (hypoxia). […] This form of PH is usually caused by chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH is a condition that involves blood clots and scarring in the arteries in your lungs. […] Many other conditions cause pulmonary hypertension in ways that scientists dont yet understand. Scientists dont know the exact mechanisms that cause these conditions to trigger PH. What they do know is that theres an association between these conditions and PH. That means people with these conditions may face a higher risk of developing PH.

• #32 Pulmonary hypertension | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/a-to-z/pulmonary-hypertension/

  Pulmonary hypertension may sometimes be associated with diseases affecting the left side of your heart. […] Because blood goes from the pulmonary arteries to the lungs and then to the left side of the heart, any problem with the left side of the heart could have a backwards effect on this circuit and affect the pulmonary arteries. […] Pulmonary hypertension can also be associated with lung diseases or hypoxia. […] These types of conditions can restrict the amount of oxygen that is getting into the lungs, and therefore into the blood. A low level of oxygen in the blood causes the pulmonary arteries to constrict, increasing the blood pressure and resulting in pulmonary hypertension. […] A foreign object, usually a blood clot, blocking or obstructing a vein, may sometimes cause pulmonary hypertension. […] Other, less common, causes of pulmonary hypertension include sarcoidosis, histiocytosis X, compression of the blood vessels in the lungs.

• #33 Pulmonary hypertension – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697

  This is the most common form of pulmonary hypertension. Causes include: Left heart failure. Left-sided heart valve disease, including mitral valve or aortic valve disease. […] Causes include: Scarring of the lungs, called pulmonary fibrosis. Chronic obstructive pulmonary disease, also called COPD. A sleep disorder in which breathing repeatedly stops and starts, called sleep apnea. Being at high altitudes for extended periods of time, if you are at high risk of pulmonary hypertension. […] Causes include: Blood clots in the lungs that don’t go away. Tumors that block the pulmonary artery. […] Causes include: Blood disorders, including polycythemia vera and essential thrombocythemia. Inflammatory disorders such as sarcoidosis. Conditions that affect the body’s ability to break down certain sugars, including glycogen storage disease. Kidney disease. […] Eisenmenger syndrome can lead to pulmonary hypertension. […] The unrepaired hole in the heart causes oxygen-rich blood to mix with oxygen-poor blood. The blood then goes to the lungs instead of going to the rest of the body. This increases pressure in the pulmonary arteries.

• #34 Pulmonary Hypertension | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-hypertension

  Group 3: Pulmonary hypertension caused by underlying lung diseases or hypoxemia. The common diseases that cause hypoxia are COPD, emphysema, pulmonary fibrosis, obstructive sleep apnea, lung development abnormalities and chronic high-altitude exposure. […] Group 4: CTEPH (chronic thromboembolic pulmonary hypertension) In some patients, pulmonary embolism, or blood clots, in the lungs arteries can form scar-like tissue, blocking or narrowing the arteries and leading to CTEPH. Some CTEPH patients can be cured with pulmonary thromboendarterectomy (PTE) surgery. […] Group 5: Pulmonary hypertension from numerous other disorders. This group includes any other cause that doesn’t fit under another heading. Examples are blood disorders, systemic disorders like sarcoidosis, metabolic disorders like thyroid diseases and chronic kidney failure or tumors obstructing pulmonary arteries.

• #35 Pulmonary Hypertension: Symptoms, Treatment

  https://my.clevelandclinic.org/health/diseases/6530-pulmonary-hypertension-ph

  Lung problems are another common cause of pulmonary hypertension. Some people with left-sided heart disease also have lung disease or a lack of oxygen (hypoxia). […] This form of PH is usually caused by chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH is a condition that involves blood clots and scarring in the arteries in your lungs. […] Many other conditions cause pulmonary hypertension in ways that scientists dont yet understand. Scientists dont know the exact mechanisms that cause these conditions to trigger PH. What they do know is that theres an association between these conditions and PH. That means people with these conditions may face a higher risk of developing PH.

• #36 Pulmonary hypertension | Canadian Lung Association

  https://www.lung.ca/lung-health/lung-diseases/pulmonary-hypertension

  Certain lung diseases such as COPD, interstitial lung disease or sleep apnea, can cause the pulmonary arteries to tighten, which means less blood flow and higher blood pressure in the lungs. […] Blood clots or scars from blood clots prevent your blood from flowing normally through your lungs. This puts more stress on the right side of your heart and raises pulmonary blood pressure. […] Chronic thromboembolic pulmonary hypertension (CTEPH) is an example of Group 4 PH. CTEPH is caused by recurring pulmonary embolisms (blood clots) forming scar-like tissue in the lungs arteries, blocking or narrowing the arteries. […] Conditions that increase the risk of blood clots in general (for example, obesity, smoking, estrogen-containing birth control and major surgery) can increase the risk of CTEPH. […] PH can occur as a result of other conditions like blood disorders (such as thrombocythemia), sarcoidosis, vasculitis, thyroid disease, kidney disease, sickle cell disease and more. This type of PH can also be caused by tumours putting pressure on the pulmonary arteries.

• #37 Pulmonary hypertension – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697

  This is the most common form of pulmonary hypertension. Causes include: Left heart failure. Left-sided heart valve disease, including mitral valve or aortic valve disease. […] Causes include: Scarring of the lungs, called pulmonary fibrosis. Chronic obstructive pulmonary disease, also called COPD. A sleep disorder in which breathing repeatedly stops and starts, called sleep apnea. Being at high altitudes for extended periods of time, if you are at high risk of pulmonary hypertension. […] Causes include: Blood clots in the lungs that don’t go away. Tumors that block the pulmonary artery. […] Causes include: Blood disorders, including polycythemia vera and essential thrombocythemia. Inflammatory disorders such as sarcoidosis. Conditions that affect the body’s ability to break down certain sugars, including glycogen storage disease. Kidney disease. […] Eisenmenger syndrome can lead to pulmonary hypertension. […] The unrepaired hole in the heart causes oxygen-rich blood to mix with oxygen-poor blood. The blood then goes to the lungs instead of going to the rest of the body. This increases pressure in the pulmonary arteries.

• #38 Pulmonary Hypertension – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK482463/

  Pulmonary hypertension associated with CHD (PH-CHD) is classified into CHD that causes Eisenmenger syndrome, PAH associated with prevalent systemic-to-pulmonary shunts, PAH with small or coincidental defects, and PAH after defect correction. […] Group 3 hypoventilation syndromes comprise conditions causing sleep-disordered breathing. Hypoxia without hypoventilation is seen at high altitudes. Group 4 etiologies include chronic thromboembolic disease, sarcoma, other malignant tumors like renal carcinoma, uterine carcinoma, testicular germ cell tumors, and nonmalignant tumors like leiomyoma, arteritis without CTD, congenital pulmonary artery stenoses, and hydatidosis. These conditions can obstruct the pulmonary artery and cause pulmonary hypertension. […] Chronic hemolytic anemia and myeloproliferative disorders are hematological disorders that cause group 5 pulmonary hypertension. Systemic diseases that can cause pulmonary hypertension include pulmonary Langerhans cell histiocytosis, neurofibromatosis type 1, and sarcoidosis. Glycogen storage diseases and Gaucher disease are metabolic disorders that can cause pulmonary hypertension.

• #39 Pulmonary hypertension | Canadian Lung Association

  https://www.lung.ca/lung-health/lung-diseases/pulmonary-hypertension

  Certain lung diseases such as COPD, interstitial lung disease or sleep apnea, can cause the pulmonary arteries to tighten, which means less blood flow and higher blood pressure in the lungs. […] Blood clots or scars from blood clots prevent your blood from flowing normally through your lungs. This puts more stress on the right side of your heart and raises pulmonary blood pressure. […] Chronic thromboembolic pulmonary hypertension (CTEPH) is an example of Group 4 PH. CTEPH is caused by recurring pulmonary embolisms (blood clots) forming scar-like tissue in the lungs arteries, blocking or narrowing the arteries. […] Conditions that increase the risk of blood clots in general (for example, obesity, smoking, estrogen-containing birth control and major surgery) can increase the risk of CTEPH. […] PH can occur as a result of other conditions like blood disorders (such as thrombocythemia), sarcoidosis, vasculitis, thyroid disease, kidney disease, sickle cell disease and more. This type of PH can also be caused by tumours putting pressure on the pulmonary arteries.

• #40 What causes pulmonary hypertension? | Asthma + Lung UK

  https://www.asthmaandlung.org.uk/conditions/pulmonary-hypertension/causes

  This is a mixed group of rare causes. They are grouped together because their causes are less clear, or because there’s more than one cause. Other rarer causes of pulmonary hypertension include: sarcoidosis, histiocytosis, compression of the blood vessels in the lungs (for example as the result of a tumour).

• #41 Types of Pulmonary Hypertension: The WHO Groups – Pulmonary Hypertension Association

  https://phassociation.org/types-pulmonary-hypertension-groups/

  WHO Group 2 includes PH due to left heart disease. […] WHO Group 3 includes PH due to chronic lung disease and/or hypoxia (low oxygen levels). […] WHO Group 4 is called chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH can occur when the body is not able to dissolve a blood clot in the lungs. […] WHO Group 5 is where PH is secondary to other diseases in ways that are not well understood. […] Pulmonary hypertension often arises as a complication of an underlying disease. This can be true for most groups and functional classes.

• #42 Pulmonary hypertension – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697

  This is the most common form of pulmonary hypertension. Causes include: Left heart failure. Left-sided heart valve disease, including mitral valve or aortic valve disease. […] Causes include: Scarring of the lungs, called pulmonary fibrosis. Chronic obstructive pulmonary disease, also called COPD. A sleep disorder in which breathing repeatedly stops and starts, called sleep apnea. Being at high altitudes for extended periods of time, if you are at high risk of pulmonary hypertension. […] Causes include: Blood clots in the lungs that don’t go away. Tumors that block the pulmonary artery. […] Causes include: Blood disorders, including polycythemia vera and essential thrombocythemia. Inflammatory disorders such as sarcoidosis. Conditions that affect the body’s ability to break down certain sugars, including glycogen storage disease. Kidney disease. […] Eisenmenger syndrome can lead to pulmonary hypertension. […] The unrepaired hole in the heart causes oxygen-rich blood to mix with oxygen-poor blood. The blood then goes to the lungs instead of going to the rest of the body. This increases pressure in the pulmonary arteries.

• #43 Pulmonary Hypertension – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK482463/

  Pulmonary hypertension associated with CHD (PH-CHD) is classified into CHD that causes Eisenmenger syndrome, PAH associated with prevalent systemic-to-pulmonary shunts, PAH with small or coincidental defects, and PAH after defect correction. […] Group 3 hypoventilation syndromes comprise conditions causing sleep-disordered breathing. Hypoxia without hypoventilation is seen at high altitudes. Group 4 etiologies include chronic thromboembolic disease, sarcoma, other malignant tumors like renal carcinoma, uterine carcinoma, testicular germ cell tumors, and nonmalignant tumors like leiomyoma, arteritis without CTD, congenital pulmonary artery stenoses, and hydatidosis. These conditions can obstruct the pulmonary artery and cause pulmonary hypertension. […] Chronic hemolytic anemia and myeloproliferative disorders are hematological disorders that cause group 5 pulmonary hypertension. Systemic diseases that can cause pulmonary hypertension include pulmonary Langerhans cell histiocytosis, neurofibromatosis type 1, and sarcoidosis. Glycogen storage diseases and Gaucher disease are metabolic disorders that can cause pulmonary hypertension.

• #44 Pulmonary Hypertension | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-hypertension

  Group 3: Pulmonary hypertension caused by underlying lung diseases or hypoxemia. The common diseases that cause hypoxia are COPD, emphysema, pulmonary fibrosis, obstructive sleep apnea, lung development abnormalities and chronic high-altitude exposure. […] Group 4: CTEPH (chronic thromboembolic pulmonary hypertension) In some patients, pulmonary embolism, or blood clots, in the lungs arteries can form scar-like tissue, blocking or narrowing the arteries and leading to CTEPH. Some CTEPH patients can be cured with pulmonary thromboendarterectomy (PTE) surgery. […] Group 5: Pulmonary hypertension from numerous other disorders. This group includes any other cause that doesn’t fit under another heading. Examples are blood disorders, systemic disorders like sarcoidosis, metabolic disorders like thyroid diseases and chronic kidney failure or tumors obstructing pulmonary arteries.

• #45 Pulmonary hypertension (differential) | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/pulmonary-hypertension-differential-1?lang=us

  venolobar syndrome […] pulmonary vein atresias […] unilateral pulmonary vein atresia […] pulmonary vein stenosis […] pulmonary vein thrombosis […] partial anomalous pulmonary venous return 4,5 […] left heart […] chronic left heart failure […] mitral valve stenosis […] hypoplastic left heart syndrome […] chest wall and upper airway resulting in chronic hypoxia […] kyphoscoliosis […] obesity without or with hypoventilation syndrome, e.g. Pickwickian syndrome […] fibrothorax […] others 7 […] chronic renal failure 6 […] pulmonary tumoral thrombotic microangiopathy […] fibrosing mediastinitis […] hematological disorders […] chronic hemolytic anemia […] myeloproliferative disorders […] splenectomy […] metabolic disorders […] glycogen storage disease […] Gaucher disease.

• #46 Pulmonary Hypertension – Causes and Risk Factors | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/pulmonary-hypertension/causes

  Family history and genetics: Certain genetic disorders, such as Down syndrome, congenital heart disease, and Gaucher disease, can increase your risk of pulmonary hypertension. A family history of blood clots also increases your risk. […] Lifestyle habits: Unhealthy lifestyle habits such as smoking and illegal drug use can raise your risk of developing pulmonary hypertension. […] Medicine: Some prescribed medicines used to treat cancer and depression may increase your risk of pulmonary hypertension. […] Sex: Pulmonary hypertension is more common in women than in men. Pulmonary hypertension with certain types of heart failure is also more common in women.

• #47 Pulmonary Hypertension | PH | MedlinePlus

  https://medlineplus.gov/pulmonaryhypertension.html

  Pulmonary hypertension can develop on its own or be caused by another condition. Sometimes the cause is unknown or is not clear. […] Some of the possible causes include: Heart diseases, including left-sided heart failure and congenital heart disease […] Lung diseases such as COPD (chronic obstructive pulmonary disease), interstitial lung disease, emphysema, and sleep apnea […] Other medical conditions such as: Liver diseases […] Sickle cell disease […] Pulmonary embolism (blood clots in the lungs) […] Connective tissue disorders like scleroderma. […] Certain factors can make you more likely to develop pulmonary hypertension, such as: Your age. The risk increases as you get older. The condition is usually diagnosed between ages 30 and 60. […] Your environment. Being exposed to asbestos or having certain infections caused by parasites can raise your risk.

• #48 Pulmonary hypertension | healthdirect

  https://www.healthdirect.gov.au/pulmonary-hypertension

  Pulmonary hypertension is due to changes to the blood vessels in your lungs. This may be caused by: […] Pulmonary hypertension can be caused by many things, including: genetic problems, some types of heart disease, autoimmune diseases such as scleroderma, blood clots in your lungs, congenital heart disease, lung diseases such as pulmonary fibrosis, chronic obstructive pulmonary disease and sleep apnoea, liver diseases, chronic kidney failure, HIV/AIDS. […] Things that can raise your risk of pulmonary hypertension include: a family history of pulmonary hypertension and certain other disorders, obesity and obstructive sleep apnoea, pregnancy, living at high altitude, certain toxins, medicines or drugs, smoking.

• #49 Pulmonary Hypertension | PH | MedlinePlus

  https://medlineplus.gov/pulmonaryhypertension.html

  Your family history and genetics. Certain genetic disorders, such as Down syndrome, congenital heart disease, and Gaucher disease, can raise your risk of pulmonary hypertension. So can a family history of blood clots. […] Your lifestyle habits. Smoking and illegal drug use can raise your risk of developing pulmonary hypertension. […] Certain medicines. For example, some medicines used to treat cancer and depression can make you more likely to develop pulmonary hypertension. […] Your sex. Pulmonary hypertension is more common in women than in men.

• #50 Pulmonary hypertension – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_hypertension

  The cause is often unknown. […] Risk factors include a family history, prior pulmonary embolism (blood clots in the lungs), HIV/AIDS, sickle cell disease, cocaine use, chronic obstructive pulmonary disease, sleep apnea, living at high altitudes, and problems with the mitral valve. […] The underlying mechanism typically involves inflammation and subsequent remodeling of the arteries in the lungs. […] Pulmonary hypertension is a pathophysiologic condition with many possible causes. […] A distinction was made between primary PH (resulting from a disease of the pulmonary arteries) and secondary PH (resulting secondary to other, non-vascular causes). […] Most recently in 2022, the WHO guidelines were updated by the European Society of Cardiology (ESC) and European Respiratory Society (ERS).

• #51 Pulmonary Hypertension: Diagnosis and Treatment | AAFP

  https://www.aafp.org/pubs/afp/issues/2016/0915/p463.html

  Pulmonary hypertension is a common, complex group of disorders that result from different pathophysiologic mechanisms but are all defined by a mean pulmonary arterial pressure of 25 mm Hg or greater. […] Pulmonary hypertension is categorized on the basis of pathophysiology, hemodynamics, and therapeutic options. […] Pulmonary hypertension is most prevalent in those with left heart disease (group 2). […] Pulmonary hypertension is also common in patients with lung disease and/or hypoxia (group 3). […] Group 5 is multifactorial in etiology and best characterized in the setting of sickle cell disease, where it is an independent predictor of mortality. […] The mechanisms that increase pulmonary pressures can act primarily on the pulmonary arterial bed or venous bed, either alone or in combination.

• #52 Pulmonary Hypertension: Diagnosis and Treatment | AAFP

  https://www.aafp.org/pubs/afp/issues/2016/0915/p463.html

  Pulmonary arterial hypertension is characterized by progressive narrowing of distal pulmonary arteries attributed to a variety of pathologic insults, such as arterial vasoconstriction, medial hypertrophy, intimal proliferation, and fibrosis. […] Pulmonary hypertension due to left heart disease is primarily a pulmonary venous process and likely results from passive pulmonary venous congestion with vasoconstriction and venous remodeling. […] In pulmonary hypertension due to lung disease and/or hypoxia, increases in pulmonary arterial pressures may arise from destruction of the alveolar capillary bed or chronic hypoxic vasoconstriction. […] Chronic thromboembolic pulmonary hypertension develops following thrombotic macrovascular obstruction with subsequent vasoconstriction and remodeling of the pulmonary arterial bed. […] Regardless of the mechanism, persistently increased pulmonary arterial pressure strains the thin-walled right ventricle. […] Ultimately, right ventricular failure is the most common cause of death in patients with pulmonary hypertension.

• #53 Pulmonary Hypertension: Diagnosis and Treatment | AAFP

  https://www.aafp.org/pubs/afp/issues/2016/0915/p463.html

  Pulmonary arterial hypertension is characterized by progressive narrowing of distal pulmonary arteries attributed to a variety of pathologic insults, such as arterial vasoconstriction, medial hypertrophy, intimal proliferation, and fibrosis. […] Pulmonary hypertension due to left heart disease is primarily a pulmonary venous process and likely results from passive pulmonary venous congestion with vasoconstriction and venous remodeling. […] In pulmonary hypertension due to lung disease and/or hypoxia, increases in pulmonary arterial pressures may arise from destruction of the alveolar capillary bed or chronic hypoxic vasoconstriction. […] Chronic thromboembolic pulmonary hypertension develops following thrombotic macrovascular obstruction with subsequent vasoconstriction and remodeling of the pulmonary arterial bed. […] Regardless of the mechanism, persistently increased pulmonary arterial pressure strains the thin-walled right ventricle. […] Ultimately, right ventricular failure is the most common cause of death in patients with pulmonary hypertension.

• #54 Pulmonary hypertension – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697

  This is the most common form of pulmonary hypertension. Causes include: Left heart failure. Left-sided heart valve disease, including mitral valve or aortic valve disease. […] Causes include: Scarring of the lungs, called pulmonary fibrosis. Chronic obstructive pulmonary disease, also called COPD. A sleep disorder in which breathing repeatedly stops and starts, called sleep apnea. Being at high altitudes for extended periods of time, if you are at high risk of pulmonary hypertension. […] Causes include: Blood clots in the lungs that don’t go away. Tumors that block the pulmonary artery. […] Causes include: Blood disorders, including polycythemia vera and essential thrombocythemia. Inflammatory disorders such as sarcoidosis. Conditions that affect the body’s ability to break down certain sugars, including glycogen storage disease. Kidney disease. […] Eisenmenger syndrome can lead to pulmonary hypertension. […] The unrepaired hole in the heart causes oxygen-rich blood to mix with oxygen-poor blood. The blood then goes to the lungs instead of going to the rest of the body. This increases pressure in the pulmonary arteries.

• #55 About Pulmonary Hypertension | Heart Disease | CDC

  https://www.cdc.gov/heart-disease/about/pulmonary-hypertension.html

  Pulmonary hypertension is a condition that affects the blood vessels in the lungs. It develops when the blood pressure in your lungs is higher than normal. […] Some common underlying causes of pulmonary hypertension include high blood pressure in the lungs’ arteries due to some types of congenital heart disease, connective tissue disease, coronary artery disease, high blood pressure, liver disease (cirrhosis), blood clots to the lungs, and chronic lung diseases like emphysema. Genetics also play a role. […] Pulmonary hypertension can happen in association with many other diseases, such as lung disease and heart disease. Heart failure is common in pulmonary hypertension.

• #56 Pulmonary Hypertension – Causes and Risk Factors | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/pulmonary-hypertension/causes

  The cause of pulmonary hypertension is not always clear. Certain medical conditions can damage, change, or block the blood vessels of the pulmonary arteries, which can lead to pulmonary hypertension. […] Some examples of medical conditions include: […] Left heart diseases, such as left heart failure, which may be caused by high blood pressure throughout your body or coronary heart disease […] Other heart and blood vessel diseases such as congenital (inherited) heart defects […] Lung diseases such as chronic obstructive pulmonary disease (COPD), interstitial lung disease, emphysema, or sleep apnea […] Other medical conditions such as liver disease, sickle cell disease, blood clots in the lungs, or connective tissue disorders like scleroderma. […] Several factors can increase your risk of developing pulmonary hypertension.

• #57 Pulmonary Hypertension – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK482463/

  Genetic mutations are associated with various pulmonary hypertension types, including IPAH, HPAH, and hereditary hemorrhagic telangiectasia (HHT) associated with PAH. Individuals with HPAH have an inheritable genetic mutation. People with IPAH have a genetic predisposition, but the mutations are sporadic. These groups are clinically indistinguishable. […] The drugs and toxins known to cause PAH include aminorex, fenfluramine, dexfenfluramine, benfluorex, methamphetamines, dasatinib, and toxic rapeseed oil. Some substances that may give rise to PAH include cocaine, phenylpropanolamine, L-tryptophan, St. John’s wort, amphetamines, interferon – and -, alkylating agents, bosutinib, direct-acting hepatitis C antivirals, leflunomide, and indirubin. […] Various CTDs are also known to cause PAH, including systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, Raynaud disease, and mixed connective tissue disease (MCTD). Among these, systemic sclerosis is the most notorious for causing PAH.

• #58 Pulmonary hypertension: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000112.htm

  Pulmonary hypertension may be caused by: […] In rare cases, the cause of pulmonary hypertension is unknown. In this case, the condition is called idiopathic pulmonary arterial hypertension (IPAH). […] If pulmonary hypertension is caused by a known medicine or medical condition, it is called secondary pulmonary hypertension.

• #59 Learn About Pulmonary Arterial Hypertension | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-arterial-hypertension/learn-about-pulmonary-arterial-hypertension

  Pulmonary arterial hypertension (PAH) is a specific type of pulmonary hypertension that is caused when the tiny arteries in your lung become thickened and narrowed. This blocks the blood flow through your lungs which raises the blood pressure in the lungs and causes your heart to work harder to pump your blood through those narrowed arteries. Over time, your heart loses the ability to effectively pump blood throughout your body. […] PAH is unlike other forms of pulmonary hypertension, where high blood pressure in the lungs is caused by underlying heart or lung disease. Researchers believe that PAH occurs when there is injury to the cells that line the blood vessels of the lung, which over time results in this blood vessel disease. If the cause of this change is unknown it is referred to as idiopathic PAH. If the change is believed to be caused by a genetic mutation it is called heritable PAH. Approximately 15-20% of PAH patients have heritable PAH.

• #60 Learn About Pulmonary Arterial Hypertension | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-arterial-hypertension/learn-about-pulmonary-arterial-hypertension

  Other conditions that are associated with the development of PAH include: connective tissue disorders like scleroderma and lupus; exposure to certain toxins and drugs, including methamphetamine and cocaine; infections, including HIV and schistosomiasis; cirrhosis of the liver; and congenital heart abnormalities.

• #61 Pulmonary Hypertension | MUSC Health

  https://advance.muschealth.org/library/2021/december/understanding-pulmonary-hypertension

  The fourth group of PH is caused by blood clots in the pulmonary arteries, which restrict blood flow in the lungs and lead to high blood pressure. PH caused by blood clots is curable by removal of the clots. […] The cause of secondary PH is not fully known, although it is linked to other conditions, including sickle cell anemia, metabolic disorder and removal of the spleen. […] Yes. PH is expected to increase as a result of COVID-19, primarily among patients whose lungs were scarred by the disease. COVID-19 also increases chances of blood clots, so some patients may develop PH as a consequence. […] No. PH is incurable and eventually will progress, but the good news is that there has been an exponential increase in the available treatments that can extend life expectancy and allow individuals to lead fulfilling lives if it is diagnosed early. That said, understanding the specific cause of one’s PH is vital since different types of PH are treated differently.

• #62 Pulmonary Hypertension | MUSC Health

  https://advance.muschealth.org/library/2021/december/understanding-pulmonary-hypertension

  For secondary PH, the treatment is to fix the underlying cause. For PAH, treatments include vasodilator medications that open up the blood vessels. These can be administered through an intravenous port, a subcutaneous pump, an inhaler or orally. […] Unfortunately, PAH cannot be avoided, but if diagnosed early, can have as fruitful life unless it is caused by drugs or other foreign bodies. The chances of contracting secondary PH can be reduced by living a healthy lifestyle and avoiding diseases that can cause this secondary process, such as smoking, heart disease and vaccines.

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