What are the other Names for this Condition? (Also known as/Synonyms)

• PAH Associated with Other Conditions
• Pulmonary Arterial Hypertension and Other Conditions

What is Pulmonary Arterial Hypertension Associated with Other Conditions? (Definition/Background Information)

• Pulmonary arterial hypertension (PAH) is a specific type of hypertension that is caused by increased blood pressure within the pulmonary (lung) arteries. When PAH is suspected to be the result of a specific associated condition, it is termed Pulmonary Arterial Hypertension Associated with Other Conditions
• In general, pulmonary hypertension (PH) is a disorder characterized by very high blood pressure (above normal) in the lungs due to a variety of causes. It should not be confused with systemic hypertension, otherwise known as ‘high blood pressure’
• The World Health Organization (WHO) has clinically classified pulmonary hypertension according to certain groups. Pulmonary Arterial Hypertension Associated with Other Conditions forms part of WHO group 1 (sub group 1.4)
• Accordingly, the following conditions are listed as being associated with pulmonary arterial hypertension:
  • Connective tissue diseases
  • HIV infection
  • Portal hypertension
  • Congenital heart diseases
  • Schistosomiasis
• The signs and symptoms of Pulmonary Arterial Hypertension Associated with Other Conditions may include shortness of breath, chest pain, fatigue, and dizziness. The signs and symptoms also depend on the underlying causative condition
• Following a diagnosis of Pulmonary Arterial Hypertension Associated with Other Conditions, the treatment measures may involve the use of medications, such as calcium channel blockers, and bringing about certain lifestyle modifications, among others. Treating the underlying cause forms an important component of PAH management
• The prognosis of Pulmonary Arterial Hypertension Associated with Other Conditions may be improved if diagnosed promptly and treated appropriately. However, the overall prognosis depends on the type of underlying cause and its severity; the prognosis may vary on a case-by-case basis

Who gets Pulmonary Arterial Hypertension Associated with Other Conditions? (Age and Sex Distribution)

The age, gender, and racial/ethnic distribution depends on the underlying cause of Pulmonary Arterial Hypertension Associated with Other Conditions.

What are the Risk Factors for Pulmonary Arterial Hypertension Associated with Other Conditions? (Predisposing Factors)

The risk factors for Pulmonary Arterial Hypertension Associated with Other Conditions are dependent on the respective risk factors of the underlying/associated conditions. These may include:

• A history of connective tissue disease, such as systemic sclerosis, Raynaud’s disease, systemic lupus erythematosus, mixed connective tissue disease, and rheumatoid arthritis. Currently, there are no known risk factors for acquiring connective tissue diseases
• A history of congenital heart disease, such as septal defects, Tetralogy of Fallot, and Eisenmenger syndrome
• The risk factors for HIV infection include:
  • Individuals who have a history of sexually-transmitted diseases
  • Having sex with those who are infected with HIV infection
  • Victims of sexual assault
  • Individuals who have unprotected sex
  • Men and women who have sex with multiple partners or who have sex for money
  • Men-men sex
  • Injection drug users who share needles
• A history of portopulmonary hypertension (typically in those with chronic liver disease): The risk factors include alcohol use, diabetes mellitus, infection with hepatitis B, C, or D viruses, or individuals with autoimmune hepatitis
• Schistosomiasis (especially with hepatosplenic involvement) risk factors that include:
  • Residing in or traveling to an area where the infection is endemic
  • Conducting domestic, recreational, occupational, or agricultural activities in and around contaminated fresh waterbodies such as ponds, lakes, and streams
  • Having poor waste disposal and water treatment facilities
  • Schistosomes can also infect other warm-blooded animals such as pets and livestock. Such infected animals frequently facilitate the spread of infection to other areas

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pulmonary Arterial Hypertension Associated with Other Conditions? (Etiology)

The cause of Pulmonary Arterial Hypertension Associated with Other Conditions depends on a variety of underlying risk factors that include certain infections, disorders, and cardiovascular status.

The causative conditions associated with pulmonary arterial hypertension include:

• Connective tissue diseases
• HIV infection
• Portal hypertension
• Congenital heart diseases
• Schistosomiasis

What are the Signs and Symptoms of Pulmonary Arterial Hypertension Associated with Other Conditions?

The signs and symptoms of Pulmonary Arterial Hypertension Associated with Other Conditions depends on a set of several factors that include age of the individual, overall health of the individual, underlying risk factors, and associated health conditions. The signs and symptoms can be directly related to pulmonary hypertension, and to the underlying risk factors and associated conditions.

The common signs and symptoms exhibited by individuals with PAH Associated with Other Conditions are similar to most individuals with pulmonary hypertension. These include:

• Progressive shortness of breath (especially while exercising), is the most common symptom
• Fatigue (frequent tiredness)
• Chest pain
• Dizziness and fainting
• Swelling of the ankles/legs
• Palpitations
• Bluish lips and skin, observed during the later stages
• Irregular heartbeat (arrhythmias)
• Fluid in the abdomen (ascites)

The signs and symptoms related to the associated condition may also be noted. Often, the detection of the associated condition may help in determining the cause of pulmonary arterial hypertension.

How is Pulmonary Arterial Hypertension Associated with Other Conditions Diagnosed?

To diagnose Pulmonary Arterial Hypertension Associated with Other Conditions, a diagnosis of pulmonary hypertension (PH) must first be established. This involves certain preliminary diagnostic procedures. Depending on the results of these tests, confirmation will be made with a right-heart catheterization.

After a diagnosis is made, the healthcare provider may inquire about the presence of any associated conditions, such as connective tissue disease, congenital heart disease, HIV infection, portal hypertension, or schistosomiasis. This can help in diagnosing and differentiating PAH Associated with Other Conditions from other types of pulmonary hypertension. 

The preliminary procedures may include:

• A complete physical examination of the patient 
• A thorough checking of the patient’s medical history and a detailed checking of the family history of the patient
• Blood tests:
  • Blood tests help rule out other diseases, such as HIV infection, liver disease, rheumatoid or connective tissue disease, and thyroid disease
  • They also help in checking the blood oxygen levels
  • The measurement of brain natriuretic peptide (BNP) can be used to assess the strain on one’s heart
• Chest X-ray: Chest X-rays can reveal structural signs of pulmonary hypertension by identifying changes in the pulmonary arteries or enlargement of the right side of the heart
• Electrocardiogram (ECG): An electrocardiogram checks the electrical impulses of the heart. There are certain identifiable patterns on an ECG that may indicate pulmonary hypertension. However, ECG is not specific enough to diagnose the condition by itself, so a combination of tests may be recommended by the healthcare provider
• Echocardiogram: In this procedure, a sonogram of the heart is taken and used to measure overall functioning as well as measure the pressure within the chambers of the right heart. An echocardiogram is also often times used to monitor a patient’s condition after diagnosis and during treatment
• Pulmonary function tests: These tests help measure the quality of breathing and check the functioning of the lungs (such as how much air is breathed in and out, as well as the quality of oxygen exchange)
• Exercise tolerance test (six-minute walk test): This helps to measure the patient’s ability to exercise
• Nuclear scan (ventilation/perfusion scan or V/Q scan): This tool helps identify any new or chronic blood clots in the vessels of the lungs that could be causing pulmonary hypertension
• Tests and exams related to the underlying/associated conditions, as necessary, may be undertaken

The gold standard for pulmonary hypertension diagnosis and management is right-heart catheterization and acute vasodilator challenge.

• Right-heart catheterization:
  • If pulmonary hypertension is suspected based off preliminary tests and procedures, a right-heart catheterization test is ordered to help confirm the diagnosis
  • This test can accurately quantify right-heart pressures (measure the pressure within the chambers of the right heart), especially the pressure inside the pulmonary arteries
  • During this procedure, a very small catheter is inserted into a large vein (either within the patient’s groin or neck) and passed into the patient’s heart and vasculature to measure the internal blood pressures 
• Vasodilator study (acute vasodilator challenge):
  • While this test is not used to necessarily “diagnose” pulmonary hypertension, it is used to evaluate the patient for possible therapeutic management
  • Like right-heart catheterization, a catheter is placed within the pulmonary artery to test for vasodilation (or relaxation) in response to a class of medications called calcium channel blockers (CCBs)
  • The response and dosing of CCBs can be evaluated with this procedure

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pulmonary Arterial Hypertension Associated with Other Conditions?

The complications of Pulmonary Arterial Hypertension Associated with Other Conditions depends on a set of several factors that include the age of the individual, overall health of the individual, underlying health conditions, and promptness of diagnosis. 

Following are the possible complications that may arise due to the condition:

• Right-sided heart failure with hepatic congestion (due to deposition of stones in the liver)
• Pedal edema: Swelling of the leg or ankle
• Pleural effusions: Building-up of excess fluid around the lungs
• Worsening dyspnea (shortness of breath) upon exertion

Complications due to underlying conditions/disorders may be additionally noted

How is Pulmonary Arterial Hypertension Associated with Other Conditions Treated?

The treatment of Pulmonary Arterial Hypertension Associated with Other Conditions depends on a set of several factors that include the severity of the signs and symptoms, age of the individual, overall health of the individual, underlying/associated health condition. A treatment of the underlying condition, such as connective tissue disease, congenital heart disease, HIV infection, portal hypertension, or schistosomiasis, is essential.

PAH Associated with Other Conditions is best treated at a medical center with appropriate expertise. Several medical treatments are highlighted below:

• Conventional medical therapies:
  • Calcium channel blockers (CCBs): These are a group of medications that help vasodilate (or relax) the arteries. However, CCBs are only appropriate for patients demonstrating a favorable response to the vasodilator study
  • Inotropic agents: These are medications that assist in the pumping of the heart 
  • Diuretics, salt limitation, and weight monitoring may be recommended, to limit excess strain on the heart
  • Supplemental oxygen may help an individual meet the ‘oxygen needs’ for normal daily activities
  • Blood thinners: These medications help prevent blood clots, especially in the lung blood vessels
  • Treating underlying diseases or conditions
• Oral, inhaled, subcutaneous, or intravenous treatment options:
  • Endothelin receptor antagonists (ERAs) help in preventing pulmonary blood vessels from narrowing which helps keep the pressures towards normal levels
  • Phosphodiesterase inhibitors (PDE 5 Inhibitors), prostacyclin analogues, and soluble guanylate cyclase (sGC) stimulators, all aid in allowing vessels of the lungs to vasodilate (or relax)
• Additional treatment options may include:
  • Lifestyle modifications, such as nutrition, exercise, avoiding tobacco, and limiting alcohol consumption, are beneficial
  • Judicious exercising may be effective in limiting deconditioning (by building stamina), improving the quality of life, and exercising capacities. Isometric exercises, such as heavy weightlifting, should be avoided
  • Other treatments, depending on the etiology (cause) of pulmonary hypertension, may include various cardiothoracic procedures
  • Lung transplantation may be required with advanced forms of the disorder

How can Pulmonary Arterial Hypertension Associated with Other Conditions be Prevented?

Pulmonary Arterial Hypertension Associated with Other Conditions may be prevented or controlled if appropriate treatment of the underlying associated disorder is considered.

The non-modifiable risk factors for pulmonary hypertension (PH), such as age, gender, and genetics (family history), cannot be controlled; and therefore, it may not be possible to prevent PH that develops from these factors. However, an early and effective treatment is critical and imperative.

• For those with a pre-existing condition that is a risk factor for pulmonary arterial hypertension, seeking medical attention at the onset of symptoms may help avoid worsening of the condition and complications 
• Avoidance of recreational drugs, various toxic agents, and inappropriate weight loss drugs is important

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

What is the Prognosis of Pulmonary Arterial Hypertension Associated with Other Conditions? (Outcomes/Resolutions)

The prognosis of Pulmonary Arterial Hypertension Associated with Other Conditions differs from one individual to another, depending on the severity of the condition and its associated cause. 

• For some cases of pulmonary arterial hypertension (PAH), the disorder has no cure, but earlier and focused treatment can help improve outcomes. In other types, it may be possible to cure PAH, if the underlying condition is addressed promptly
• Pulmonary Hypertension is a negative prognostic sign in many medical conditions, meaning that the presence of Pulmonary Hypertension makes the overall health outcomes worse. For example, in individuals who have COPD or heart failure, the development of PH worsens the overall outcomes
• Individuals with advanced heart disease, a suboptimal 6-minute walk test (exercise tolerance test to measure one’s ability to exercise without significant signs and symptoms), and with conditions, such as advanced liver disease and connective tissue disease, have poor prognosis

Additional and Relevant Useful Information for Pulmonary Arterial Hypertension Associated with Other Conditions:

The following DoveMed website links are useful resources for additional information:

https://www.dovemed.com/diseases-conditions/acute-human-immunodeficiency-virus-hiv-infection/

https://www.dovemed.com/diseases-conditions/schistosomiasis/

https://www.dovemed.com/diseases-conditions/tetralogy-fallot/

https://www.dovemed.com/diseases-conditions/aortic-valve-stenosis/

https://www.dovemed.com/diseases-conditions/liver-cirrhosis/

https://www.dovemed.com/diseases-conditions/systemic-lupus-erythematosus/

https://www.dovemed.com/diseases-conditions/scleroderma/