PH Life Care Program - A New Initiative of Child Heart Foundation Provides Financial Assistance for People with Pulmonary Hypertension

By Child Heart Foundation - June 10, 2019

Pulmonary Arterial Hypertension

Pulmonary Hypertension (PH) is a severe, rare lung disease characterized by high blood pressure in the pulmonary arteries. Due to the condition, the pulmonary arteries, which are responsible for transporting the blood from the right heart ventricle to the lungs, become narrowed and thickened. In order to properly pump the blood, the heart needs to work harder, which can lead to enlargement and weakening of the organ, as well as potential right heart failure. The World Health Organization recognizes 5 groups of pulmonary hypertension (PH), categorized by pathogenesis or comorbidity: 1-pulmonary arterial hypertension 2-left-heart disease, 3-lung disease and hypoxia 4-chronic thromboembolic disease, and 5-miscellaneous. 

  • Pulmonary hypertension is a substantial global health issue.
  • All age groups are affected with rapidly growing importance in elderly people, particularly in countries with ageing populations.
  • Present estimates suggest a pulmonary hypertension prevalence of about 1% of the global population, which increases up to 10% in individuals aged more than 65 years.
  • In almost all parts of the world, left-sided heart and lung diseases have become the most frequent causes of pulmonary hypertension.
  • According to the Centre for Disease Control and Prevention the number of deaths from PH has increased over the past one decade.
  • 80% of patients live in developing countries where PH is associated with Congenital Heart Disease with an estimated prevalence ranging from 10 to 52 cases per million.
Healthy Pulmonary arteries of the lungs are elastic. They expand and contract with evert heartbeat. In cases of Pulmonary Hypertension the arteries stiffen and become thick. This results in increasing resistance to blood passing through thereby leading to increasing pressure. Higher pulmonary may lead to symptoms of pulmonary hypertension. The symptoms of pulmonary hypertension directly affects the functioning of the heart. The common symptoms may include: 
  • Shortness of breath 
  • Low oxygen levels 
  • Chest pain or pressure 
  • Fatigue 
  • Heart palpitation
  • Swelling of the ankles or abdomen 
  • Heart failure in advanced cases 

The diagnosis of Pulmonary Hypertension may be difficult and is generally diagnosed at an advanced stage. One the reasons been that the symptoms of pulmonary hypertension is misinterpreted with other health ailments such as asthma, lung diseases etc. When diagnosing pulmonary hypertension, an ultra sound of the heart or echocardiogram can provide an estimate of the pressure in the heart. Only a procedure called right heart catheterization (RHC) can directly measure blood pressure in the lungs and determine if pulmonary hypertension is present. A right heart catheterization can also be used to determine if pulmonary hypertension is responsive to intravenous vasodilator medication. This will help determine whether or not a person is a candidate for chronic medication treatment. 

Other test are performed to screen associated diseases, including blood test, EKG, Chest X rays, pulmonary function testing and a test for lung blood clots called ventilation/perfusion scan. A six minute walk is typically performed to assess a person’s exercise capacity and need to oxygen therapy. 

Treatment of Pulmonary Hypertension is directed towards improving symptoms, improving exercise capacity and delaying progression of the disease. The treatment used for pulmonary hypertension depends upon its underlying cause. Pulmonary Hypertension can improve with treatment of associated hearts or lung disease.

Important non medication treatment includes: 
  • Oxygen therapy 
  • Quitting smoking 
  • Removal of deleterious drugs 
  • Routine exercise (with the guidance of your pulmonary hypertension doctor)

People whose pulmonary blood pressure responds to intravenous vasodilator treatment during right heart catheterization may be candidates for calcium channel blocker therapy. People with idiopathic pulmonary arterial hypertension that does not respond to vasodilator challenge during right heart catheterization or who do not improve with calcium channel blocker treatment, or people who have inherited or connective – tissue associated pulmonary hypertension are candidates of oral medications. 

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