Effects Of Diagnosis And Race On The Prevalence Of Pulmonary Hypertension
Table 3 indicates the presence or absence of pulmonary hypertension in diagnostic groups of patients, with PH defined as measured mPAP by RHC > 25 mmHg. Patients with CTD-IP had a higher prevalence of PH when compared to patients with IPF or non-IPF IIP , and a higher prevalence of PH when compared to the IPF and non-IPF IIP patients when combined into an IIP group as a whole . The prevalence of PH in the IPF subgroup , the combined IIP group , and the CTD-IP group was 29%, 29%, and 64%, respectively.
When Should I Go To The Emergency Department
Go to the emergency department or call 9-1-1 if you are experiencing:
- Hickman catheter complications with intravenous prostacyclins, such as infection, catheter displacement/leakage of solution or blood, IV pump malfunction
- New chest pain or discomfort that is severe, unexpected, and is accompanied by shortness of breath, sweating, nausea, or weakness
- Fast, sustained heart rate â especially if you are short of breath
- Shortness of breath that is NOT relieved by rest
- Sudden weakness or paralysis in your arms or legs
- Sudden onset of severe headache
- Fainting spells with loss of consciousness
The Exact Cause Of Pah Is Unknown But Physiological Changes Are Key To Its Development And Progression
PAH is diagnosed when mean, or average, blood pressure leaving the arteries on the right side of the heart is measured at 25 mmHg or higher. No one knows exactly what kicks off the chain of events that sets PAH in motion, but experts do know a lot about how it progresses.
“It starts with constriction of these vessels and leads to remodeling of the vessel wall,” says Alessandro Maresta, M.D.Alessandro Maresta, M.D.,Vice President and Head of Global Medical Affairs, Actelion Pharmaceuticals, Vice President and Head of Global Medical Affairs for Actelion Pharmaceuticals, a Swiss biotech company that Johnson & Johnson acquired in June of this year. “As the walls of the blood vessels thicken, the lumenthe space where blood flows throughgets smaller, and the pressure goes up. As the disease progresses, pressure continues to rise, but the right ventricle of the heart is not designed to pump blood against such high pressure.”
As the condition worsens, “patients tend to become very tired and short of breath,” says Nazzareno Galié, M.D., professor of cardiology and Director of the Specialization School of Cardiology, University of Bologna, Italy. “Some people might notice heart palpitations or feel chest pressure, especially when exercising.”
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Nocturnal Oxygen Therapy In Patients With Isolated Sleep
COPD patients who are hypoxaemic while awake will also be hypoxaemic during sleep. In most COPD patients, hypoxaemia does, in fact, worsen during sleep and especially during REM sleep, as mentioned above. It follows that it is particularly important to oxygenate these patients during sleep. It is sometimes recommended to add 1L·min1 oxygen flow to the daytime resting prescription .
Some COPD patients do not have significant hypoxaemia when awake but are hypoxaemic during sleep with a mean nocturnal transcutaneous Sa,O2< 8890%. It has been hypothesised that sleep-related hypoxaemia, occurring in COPD patients without significant daytime hypoxaemia, could lead to permanent PH . If this was the case, prescription of nocturnal oxygen therapy to these patients would be justified. In fact, this hypothesis has not yet been demonstrated .
The present data, related to the evolution of pulmonary haemodynamics, are not sufficient for justifying the use of nocturnal oxygen in COPD patients who do not qualify for conventional oxygen therapy according to current criteria , .
What Raises The Risk Of Pulmonary Hypertension

Several factors can increase your risk of developing pulmonary hypertension.
- Age: Pulmonary hypertension can occur at any age, but your risk increases as you get older. The condition is usually diagnosed between ages 30 and 60.
- Environment: You may be at an increased risk of pulmonary hypertension if you have or are exposed to Asbestos or certain infections caused by parasites.
- 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.
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What Causes Pulmonary Hypertension
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:
- Other medical conditions such as liver disease, sickle cell disease, blood clots in the lungs, or connective tissue disorders like scleroderma
Drugs For Pulmonary Hypertension
Several drugs, either in isolation or in combination with each other, can support the management of pulmonary hypertension. For example:
- Diuretics: These help a person reduce fluid retention and swelling. They are also known as water pills.
- Digoxin: A person can use these to manage heart failure. Digoxin helps control heart rate and rhythm and increases the amount of blood that the heart can pump with each heartbeat.
- Blood thinning drugs such as warfarin: These can help reduce the risk of blood clots, which are more likely in people with pulmonary hypertension.
- Calcium channel blockers such as diltiazem: These help the blood vessels widen to improve blood flow.
- Phosphodiesterase-5 inhibitors: These also increase blood flow by making blood vessels wider. Sildenafil is one example of a PDE5 inhibitor.
- Endothelin receptor antagonists such as bosentan and ambrisentan: These limit the constriction of arteries that can develop when a person has too much natural peptide.
Inhaled options, such as iloprost or treprostinil , can help relieve breathlessness.
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Can You Prevent Pulmonary Hypertension
Prevention is not always possible since the cause of pulmonary hypertension is not always clear. Your doctor may suggest a preventative screening if you have a known risk factor or medical condition that causes pulmonary hypertension. Your doctor may also recommend prevention strategies to help you lower your risk of developing pulmonary hypertension. Talk to your doctor about your risk factors and concerns to learn more.
How Is Pulmonary Hypertension Diagnosed
Signs of pulmonary hypertension can be similar to the signs of many other health problems. This makes it hard to diagnose. Your doctor will probably run tests to estimate the blood pressure in your pulmonary arteries. He or she will also want to find out how well your heart and lungs are working. These tests may include:
- A chest X-ray
- A breathing test called a lung function test
- An echocardiogram
Your doctor may also need to do other tests to find out whether another medical condition is causing your pulmonary hypertension. These could include:
- Blood tests
- A chest CT scan
- A chest MRI
If your doctor determines that you have pulmonary hypertension, he or she will want to see how severe it is. For this, they may order an exercise test. These tests measure your activity level and how well your lungs and heart work while you are exercising. These tests can also be done during treatment to see how well the treatment is working.
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Factors That Can Affect Pulmonary Hypertension
While pulmonary hypertension has no cure, you can live an active, fulfilling life by working with your doctor to manage your symptoms. Talk to your doctor before taking over-the-counter medicine, as some can worsen symptoms of pulmonary hypertension or interfere with prescriptions and keep a list of medicines with you. Ask whether you should get a pneumonia vaccination or flu shot. Additionally, pregnancy can pose serious risks for women with pulmonary hypertension, so be sure to discuss birth control options with your physician.
The Worst Advice You Can Give To A Pulmonary Hypertension Patient
Ive spoken previously about the day that I was diagnosed with pulmonary hypertension. There are a few moments that stick out from that day that will probably stay engraved in my mind for eternity. When I was first diagnosed, the doctor promptly gave me a life expectancy. It couldnt have come at a worse time. I was 25 and just starting my career after working less than ideal jobs since the age of 15. I was obviously devastated to hear that my life was ending during a time it felt like it was just beginning. It felt like someone crumpled up everything I worked so hard for and threw it in the trash. As I started to cry my doctor said, Any one of us could die at any time. I could die in a car accident. This was the first time I heard this expression, but sadly, it wasnt the last time.
As the days, weeks, months, and even two years have dragged on, numerous people have told me that anyone could die at any point, that the future is promised to no one. While I am aware of everyones mortality, living with a fatal illness is a very different experience than the advice I have been given. I know that the people who have said that anyone could die at any moment and the future is promised to no one are attempting to comfort me. However, these pieces of advice are often coming from a very privileged source. The doctors who have told me this are able to work full days and have a family. The people my age who have told me this are purchasing their first homes and traveling.
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Group : Pulmonary Hypertension Associated With Lung Conditions Or Lack Of Oxygen
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:
- obesity hypoventilation syndrome.
These conditions reduce the amount of oxygen getting into your lungs. When theres a low level of oxygen, your pulmonary arteries get narrower. This increases the lung blood pressure. Lung diseases can also damage blood vessels directly.
Healthy pulmonary artery and pulmonary artery in someone with a lung condition
Case Presentation And Ethical Statement

A 14-year-old male was referred to us from a peripheral hospital, where he had then presented with complaints of cough, bloody sputum, and nausea. At the time, he was afebrile and had no difficulty in breathing. A chest CT-Scan returned typical results. Nonetheless, he was started on a Cefaclor. Four days during treatment, his general condition worsened it was noted that he was febrile with the highest temperature at around 39.0 deg. Celsius, with associated chills. They then added Clindamycin, Brufen, and Ganciclovir to his treatment regimen. A day later, the patient started having trouble breathing, which was worse when lying flat , and he was also noted to be confused. However, despite treatment with now two antibiotics and antiviral coverage, his general condition continued to deteriorate. A repeat chest CT scan was then done, and it showed multilobar pneumonia that was more severe on the left upper lobe. An echocardiogram was also done and showed moderate PH. Due to his worsening clinical state despite all the medical intervention, he was referred to our EICU for admission and further management.
He presented at our EICU with complaints of cough, difficulty in breathing that was worse when lying flat, bloody sputum, fevers and chills, chest pains which were pleuritic in nature, chest tightness, nausea, and decreased urine output. It should be noted that during his presentation, we did not observe any bouts of confusion that were initially stated.
CT-scan of the chest
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Taking Care Of Yourself
One of the best things you can do for yourself is to stay active, even if you have shortness of breath. Regular exercise, like taking a walk, will help you breathe better and live better. Talk to your doctor first to find out what kind of exercise is best for you, and how much you should do. Some people may need to use oxygen when they exercise.
Get plenty of rest, too. Pulmonary hypertension makes you tired, so get a good night’s sleep and take naps when you need to.
Just like anyone else, it’s good for you to eat a healthy diet with lots of fruits, vegetables, and whole grains. That’s important for your overall health.
Symptoms Of Reduced Pumping Capability
The most prominent symptoms are:
- Extreme weakness and fatigue
- Muscle weakness and muscle wasting
- Lethargy and inanition
- Extreme weight loss
Obviously, symptoms like this are not compatible with a long life. Unless the cardiac function can be improved, or unless cardiac transplantation or a ventricular assist device can be used, once a person with heart failure develops these kinds of symptoms, death usually follows relatively soon.
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Treatment And Prognosis Of Pulmonary Hypertension And Pneumonia
Pneumonia is the cause of death in patients who suffer from pulmonary hypertension in seven percent of the cases, since the lungs cannot tolerate pulmonary infections, according to the study Guidelines on diagnosis and treatment of pulmonary arterial hypertension. This is why early detection and proper treatment are important in patients with pulmonary hypertension and pneumonia. Vaccination is recommended to prevent it, while any persistent fever in patients with IV catheter for continuous administration of epoprostenol should raise the suspicion of infection.
Following diagnosis, patients are usually administered antibiotic therapy to treat infections. Children are particularly at risk since their reactive pulmonary vascular bed is greater than the adults, which means that an infection affecting the respiratory can cause ventilation/perfusion mismatching from alveolar hypoxia, which can cause severe consequences, including death. Therefore, children are recommended to be hospitalized to have antibiotic therapy if diagnosed with pneumonia.
Research Into Pulmonary Hypertension
Pulmonary hypertension is an area of intense research, particularly with respect to:
- the correct type of medication to use in the different groups of pulmonary hypertension, and in which combinations
- management of right heart failure, and possible new treatment targets or therapies
- treatments at the molecular level, such as monoclonal antibodies, which may be useful tools to slow disease progression
- further genetic markers that may identify people at high-risk of developing pulmonary hypertension.
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Pregnancy And Pulmonary Hypertension
If you have pulmonary hypertension, before considering conception, please talk to your specialist. Pregnancy in women with pulmonary hypertension is very high-risk, both to the mother and the baby. Many of the medications used to treat pulmonary hypertension are toxic to developing foetuses or untested in pregnant women. Unfortunately, for many women with pulmonary hypertension, carrying a baby to term may not be possible.
Can Pneumonia Lead To Pulmonary Hypertension
Pulmonary hypertension is defined as the presence of a PAPm greater than 25 mm Hg measured by cardiac catheterization.
Pulmonary hypertension is seen relatively frequently in intensive care units. Despite this, its transcendence has only recently been recognized.
It Manifests Itself In Two Forms: as the aggravation of a pre-existing condition or as a de novo process that complicates an acute condition .
In this last situation the diagnosis is not easy, since the values of pressure that are detected are not very high, because the right ventricle is a chamber of volume, with thin walls and has no possibility of generating values greater than 50 mm Hg systolic pressure or 40 mm Hg mean pressure. Subjects with higher values have some previous disease that has conditioned right ventricle so that it can sustain higher pressures.
The Pathogenesis Of Acute Pulmonary Hypertension Is Essentially Based On:
On the other hand, pneumonia is an acute respiratory disease, of infectious origin, which compromises the pulmonary parenchyma caused by the invasion of pathogenic microorganisms .
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Adempas Not For Use In Patients With Pulmonary Hypertension Caused By Idiopathic Interstitial Pneumonia
EMA recommendation follows early termination of a clinical trial in these patients
The European Medicines Agency recommends that Adempas should not be used in patients with ‘symptomatic pulmonary hypertension associated with idiopathic interstitial pneumonia’ or PH-IIP . Adempas is not authorised for use in PH-IIP patients. The recommendation follows the early termination of a phase II clinical trial called RISE-IIP which was investigating the effects of Adempas in this patient population. Preliminary results showed an increased number of deaths and serious adverse events, including breathing problems and lung infections, with Adempas compared with placebo . The available data do not indicate a clinically significant benefit from Adempas treatment in these patients.
The product information for Adempas will be updated to add a contraindication for PH-IIP to help ensure that Adempas is not used in these patients. The population for which Adempas is currently authorised is different from that included in the RISE-IIP study. The benefits of Adempas continue to outweigh its risks in its authorised uses .
Information for patients
Information for healthcare professionals
More about the medicine
Adempas has been authorised in the EU since 27 March 2014. It is used to increase the ability to carry out physical activity in adults with the following forms of pulmonary hypertension :
More about the procedure
What Is Primary Pulmonary Hypertension

Primary pulmonary hypertension is high blood pressure in the lungs. Its a rare lung disorder in which the blood vessels in the lungs narrow and the pressure in the pulmonary artery rises far above normal levels. The pulmonary arteries carry blood from your body to the lungs where carbon dioxide is traded for oxygen.
Pulmonary hypertension is a serious, chronic disease that can lead to heart failure if its not treated.
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