Newly Diagnosed With PH – Pulmonary Hypertension News


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Navigating a PH Diagnosis
Pulmonary hypertension (PH), a chronic and progressive disease associated with high blood pressure in the pulmonary arteries, can be very difficult to detect and diagnose in routine physical exams. Its symptoms can develop over a long period of time and mimic those of other heart and lung conditions.
PH usually develops in individuals between the ages of 20 and 60, but it can occur at any age. Pulmonary arterial hypertension (PAH), in particular, affects women more often than men.
PH symptoms are generally not apparent at the onset of the disease, and this may be the case for years to come. Patients affected with PH often go years without a formal diagnosis. This is often due to milder symptoms that come and go with exercise or other physical exertion.
Because PH is rare, being diagnosed with the disease can be overwhelming for many individuals. Becoming educated is a good place to start on your PH journey. Whether you are a patient or a caregiver, knowing as much as possible about the disease will help you to be a more active participant in your or your loved one’s healthcare.
Did You Know?
According to cardiologist Sanjay Ghandi, MD, congenital heart defects may lead to several complications later in life, including PH. Heart defects can greatly undermine blood flow, contributing to the development of heart disorders and other diseases. Nonetheless, it is not possible to predict when these heart defect complications might arise, if at all.
CTEPH | Pulmonary Hypertension News | illustration of doctor and patient talking
Classification of PH
There is a wide range of potential causes for PH, and they can affect disease symptoms and its treatment and prognosis.
The World Health Organization divides PH patients into five groups, according to the underlying cause of their disease. These groups include the following:
Group 1 includes PH associated with the narrowing of the small blood vessels in the lungs. This is called PAH and includes cases in which the underlying cause of the narrowing is not known, or idiopathic PAH.
There are several other subgroups in group 1, including the following:
Group 2 refers to PH due to left heart disease, also known as pulmonary venous hypertension. Long-term problems with the left side of the heart can lead to changes in the pulmonary arteries and cause PH.
These may include:
Group 3 includes PH resulting from lung diseases or a shortage of oxygen in the body, known as hypoxia)
Causes of pulmonary hypoxic hypertension include various lung diseases, such as chronic obstructive pulmonary disease (COPD) and diffuse parenchymal lung disease.
Sleep-disordered breathing, a group of diseases that affect breathing during sleep, like obstructive sleep apnea, are also included in this group. Other causes include repeated exposure to high altitudes, lung developmental abnormalities, and alveolar hypoventilation disorders (marked by a buildup of carbon dioxide and a reduction of oxygen in the lungs’ tiny air sacs).
 
Group 4 refers to PH caused by blood clots obstructing the pulmonary arteries. Clots are the body’s response to bleeding and injuries but can occur without an apparent cause.
Pulmonary embolism occurs when blood clots travel to the lungs from the legs or other parts of the body. These clots restrict blood flow through the lungs, which causes a localized increase in blood pressure in the pulmonary arteries and a reduction in the level of oxygen transported to the rest of the body. The increase in blood pressure means the heart must work harder to pump blood, which can weaken the heart muscles.
This group can also be referred to as chronic thromboembolic PH (CTEPH).
Group 5 includes causes of PH that do not fit into any of the other four groups. These are widely split into four categories:
Did You Know?
A study published in 2019 found that certain biomarkers may help in diagnosing specific types of PH. Levels of markers known as GDF-15 and suPAR associated with mechanical stress and damage in the heart — were highest among individuals with Group 2 disease, while H-FABP and BNP — linked to inflammation — were predominantly elevated in individuals with Group 2 or Group 3 PH. Additional research is necessary to determine how these biomarkers and others might aid in quicker diagnosis of PH.

Breathing reserve | Pulmonary Hypertension News | congenital heart disease | illustration of lungs

Early Signs of PH
Because PH is a progressive disease, patients may not notice early symptoms or may attribute them to another condition. Initial symptoms, such as shortness of breath, are typically brought on by exercise or strenuous physical activity. If left untreated, the symptoms can steadily worsen.
Doctors may suspect PH when an individual demonstrates complications such as shortness of breath, called dyspnea, fatigue, dizziness or fainting, and/or chest pressure or pain. Swelling in the ankles, legs, and abdomen, a bluish color in the lips and skin, or an irregular heartbeat are other symptoms that may indicate PH.
Visit the Pulmonary Hypertension News forums to connect with others in the PH community.
Did You Know?
Results from a study published in 2018 show that patients with newly diagnosed PH who didn’t previously exhibit symptoms already experience a significant reduction in exercise capacity. Researchers used the incremental shuttle walk distance test, or ISWD, to determine if patients’ exercise capacity was diminished. Further research is needed to determine if the ISWD test might help in detecting PH earlier.
How Is PH Diagnosed?
Because PH symptoms occur in other, less serious conditions, medical professionals sometimes discount the possibility of a patient having PH or attribute the symptoms to other illnesses. As such, PH is considered a medical zebra.
Misdiagnoses for PH are common, and patients can sometimes go years without an official PH diagnosis. However, because PH is a progressive disease, early diagnosis is essential to control its progression and provide the best quality of life possible for patients.
To diagnose PH, a physician will use a patient’s medical and family histories, a physical exam, and the results of various tests.
Did You Know?
A 2019 study found that PH patients in the U.K. experience delayed diagnosis, resulting in considerable financial and emotional implications. Almost half of the study’s participants were diagnosed a year or more after experiencing their first PH-related symptoms. Furthermore, 40% had visits with four or more healthcare professionals before receiving a final diagnosis of PH.
Methods to Diagnose PH
To confirm a diagnosis of PH and determine its cause, doctors usually start by analyzing a patient’s medical history and requesting numerous tests. The disorder may be inherited in some cases, so information about the medical history of family members also can be important in making a diagnosis.
Below are tests doctors may request to help diagnose PH:
Electrocardiogram
An electrocardiogram is among the first tests to analyze the heart in PH patients. This noninvasive test records the electrical activity of the heart, enabling the assessment of its rhythm and structural alterations.
Lung Function Tests
Spirometry is commonly used to measure pulmonary function. During this noninvasive test, patients blow into a simple device with a mouthpiece called a spirometer. The aim is to measure the amount of air a person can breathe in and out, as well as how fast a patient exhales.
Chest X-rays
A chest X-ray is an imaging technique used to analyze the internal structure of the heart, lungs, and chest. It is used to identify signs such as dilation of the right heart or pulmonary arteries.
Polysomnogram
A polysomnogram is a test conducted while the patient is sleeping that measures a person’s brain activity, heart rate, blood pressure, oxygen levels, and other factors such as eye movement. It is used to analyze if a low level of oxygen occurs during sleep.
Echocardiogram
This procedure allows physicians to evaluate heart enlargement, any thickening of the heart walls, and the pressure in the pulmonary arteries, as well as the potential existence of abnormal fluid around the heart. The procedure’s images of the heart also can help to detect congenital heart disease.
Lung Ventilation or Perfusion Scan
During this procedure, a tracer is injected into a vein in the patient’s arm to show the air and blood flow in the lungs. This test is used to determine if blood clots are in the lungs’ vessels and causing PH.
Chest CT Scan
This test offers pictures of chest structures — bones, heart, lungs, blood vessels — particularly showing the heart’s size and determining the presence of blood clots in the pulmonary arteries.
Chest MRI
This procedure uses a magnetic field and radio wave energy to produce pictures of the body. It aims to analyze the function and structure of the right heart ventricle and blood flow in the lungs to help detect signs of PH or its underlying conditions.
Blood Tests
Blood tests also can help determine the cause of PH and exclude the possibility of other disorders.
Abdominal Ultrasound
An abdominal ultrasound is a noninvasive method used to assess the organs and structures within the abdomen. This test helps to identify clinical conditions related to PH, such as liver cirrhosis and portal hypertension.
Right Heart Catheterization
During this procedure, a thin, flexible tube called a catheter is inserted into a vein in the neck, arm, or groin and passed through to the right ventricle and pulmonary artery. The method aims to measure the pulmonary arteries’ pressure and show the heart’s capacity to pump blood.
Exercise Tests
These methods can be used to reach a diagnosis but also are helpful after a diagnosis is confirmed to help determine the severity and stage of the disease.
Ask an expert survey graphic

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Did You Know?
An Austrian study found that four noninvasive parameters may help to diagnose PH. Specifically, a combination of non-right axis ECG deviation, low levels of the heart disease marker NT-proBNP (N-terminal pro brain natriuretic peptide), good oxygen saturation of the blood, and WHO functional class 1 or 2 could rule out the possibility of PH with a probability of 96%. Because the invasive right heart catheterization procedure is necessary for a definitive diagnosis of PH, identifying noninvasive ways to improve the diagnostic process would be useful for clinicians.
Illustration of person under stress

You’ve Been Diagnosed With PH; Now What?
Being diagnosed with a rare, progressive disease is life-changing for both patients and their families. Newly diagnosed individuals sometimes experience depression and anxiety following their diagnosis, and all experience a period of adjustment as they adapt to their new normal.
Understanding PH is crucial and allows patients and their caregivers to have some sense of stability in anticipating what to expect going forward.
Ultimately, every PH journey will be different, but having a community of individuals who are navigating similar issues offers patients some comfort and hope.
Dear newly diagnosed Mike, 
You’re probably a little scared. Certainly, a level of uncertainty is creeping in around you. The doctors are discharging you from the hospital with a diagnosis of pulmonary hypertension, a rare disease that affects the lungs and heart. “PH” for short and, no, it has nothing to do with the acids and bases scale you learned about in chemistry class. Your PH diagnosis helps to clear the fog for you; it explains why you can’t make it to the end of the street without gasping for breath. Yes, you’re getting some answers, but that just kicks open a revolving door of new questions.
Getting sick, particularly with a rare disease that doesn’t have a cure, is not an easy turn in the road. It’s not even a nice symmetrical fork in the road. It can feel like a cliff and put you on edge in a way you couldn’t have anticipated. Standing on the edge of that cliff can feel incredibly isolating, leaving you feeling like your life has come to an abrupt stop. You’re going to hear a lot about embracing your “new normal” and get all kinds of advice about managing a disease you still haven’t quite wrapped your head around.
You’re going to feel anger, frustration, anxiety, loss, and hopelessness — and these sentiments can spike in intensity on any given day. You will have successes and failures when speaking with a therapist. You’ll start taking an oxygen concentrator everywhere you go. You will attend respiratory rehabilitation and a support group, and you’ll feel out of place at first.
Over the next two years, you will make strides in living your life with PH. And while the conference will be affirming in many aspects, it will also inspire you to do more to put your health first and push you to be the biggest and loudest advocate for your care. For example, you will learn about the importance of developing an emergency plan. You will hear about the need to conserve energy when you have it, and you’ll get reassurance that other people feel exhausted after an activity such as changing sheets on a bed. You will remember (again) to exercise more.
Mike, I know you’re still adjusting to your diagnosis, and your world feels like a spinning carnival ride right now. But guess what? You’re not on this ride alone. You will meet long-term survivors, patients who have been PHighting for decades, and along with your community, they will be a source of hope for many more days to come.
Columnist Mike Naple, in a letter to his newly diagnosed self
Explaining PH to a Loved One
Coming to terms with a PH diagnosis is difficult for patients, and having to explain their disease to loved ones adds another layer of uneasiness.
Some patients’ PH symptoms may be obvious; others may be mistaken for symptoms of other illnesses. Nonetheless, because the disease is so rare, patients sometimes struggle to describe it.
Columnist Colleen Steele reflects on her experience with her son’s PH diagnosis, providing some advice for others. She writes: 
“People would ask questions that we didn’t know how to answer or comment in ways that left us uncertain as to how to react. This inspired us to do more research and to seek out others in the same situation.”
PH education is your best weapon.
Playing devil’s advocate doesn’t mean letting repeat verbal offenders, well-meaning naysayers, and parking lot complainers get off without a hitch. Seeing things from their perspective can help calm you before hitting them with your best line of defense: PH education.
The PH Association website offers many free resources you can download and share. My family was quick to share the PH wallet fact card and additional information with those who seemed open to learning more than PH basics.
I also encourage you to invite family members and caregivers to join the PH forums, where we will answer their questions and concerns with the utmost patience and compassion. We can help you by helping them understand the ins and outs of PH.”
The progressive, incurable nature of PH makes being diagnosed with the disease hard to process. Many people aren’t familiar with PH and its symptoms, so the more that loved ones can research about the disease, the greater the burden that is lifted off newly diagnosed individuals.
Understanding patients’ needs and present limitations also helps to support them as they adjust during an unsettling time. Because some patients may experience depression and anxiety following a PH diagnosis, caregivers might consider recommending support groups to their loved ones.
In a column reflecting on her PH diagnosis, columnist Jen Cueva asks her husband what advice he would offer to a partner or caregiver of someone newly diagnosed with PH? His response is as follows: “Breathe. Talk to someone. Things will get complicated. Be prepared to go through many emotions. You’ll be mad, sad, happy, confused, all at once. You find yourself asking why on many occasions. Be ready for the bell and fight like hell. Your partner, family member, whoever is going through PH needs you more now than ever before.”
Did You Know?
A California study found that support groups for PH positively affected health-related outcomes such as management of symptoms and adherence to medication. While support group participation was not shown to improve quality of life, it did improve other aspects such as self-healing, confidence in self-care, and patients’ understanding of the disease. Attending support groups also allowed patients to gain a better understanding of common PH procedures, such as right heart catheterization.
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This site is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

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