Pulmonary hypertension (PH) is a chronic and progressive disease associated with high blood pressure in the blood vessels, known as the pulmonary arteries, that supply the lungs.
It is very difficult to detect and diagnose PH in routine physical exams, given that its symptoms can develop over a long period of time and mimic those of other heart and lung conditions.
To confirm a diagnosis of PH and determine its cause, doctors usually start by analyzing a patient’s medical history and requesting numerous tests.
Patient’s history and physical exam
Physicians will start the diagnostic process by collecting information about the symptoms a patient is experiencing.
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 implicate PH.
Medicines being taken and other conditions the patient may have also are discussed in consultation, as is whether any relatives have PH. 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.
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. Particularly, the procedure can detect abnormal heartbeats and the enlargement of or strain on the lower right heart chamber, called the right ventricle.
Chest X-Rays
A chest X-ray is an imaging technique for the analysis of 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.
The images also can help reveal other lung and heart conditions that can result in the development of PH. Notably, the degree of abnormalities found on this exam typically does not correlate with the severity of PH.
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.
Pulmonary function also may be assessed via the capacity of the lungs to properly deliver oxygen to the blood, known as diffusing capacity of the lung for carbon monoxide. In addition, lung volume testing measures the volume of air in the lungs, including after a normal breath.
Polysomnogram
A polysomnogram is a test, done 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.
This test can help diagnose some sleep disorders such as obstructive sleep apnea, a condition characterized by repetitive episodes of momentary stops in breathing during sleep, which may be one of the causes of PH.
Echocardiogram
An echocardiogram is a common procedure that uses sound waves to create a moving image of the heart.
With the results of an echocardiogram, physicians are able to examine the right-side chambers (ventricle and atrium) of the heart, as well as its valves. By doing so, physicians can 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 can be done at rest while lying down, and during or after exercise on a treadmill or stationary bike to check heart function during activity.
The images of the heart can help detect congenital heart disease. As with other tests used for diagnosis, an echocardiogram can be used to evaluate treatment efficiency.
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. It is particularly helpful in the sreening of chronic thromboembolic pulmonary hypertension, a rare form of 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. The procedure may reveal signs of the condition responsible for secondary PH, such as chronic obstructive pulmonary disorder, or COPD, or pulmonary fibrosis.
A test called CTÂ angiography may be combined with a CT scan, wherein a special dye is injected into the vein to produce pictures of blood vessels and tissues.
Chest MRI
This procedure uses a magnetic field and radio wave energy to produce pictures of the body. It aims at analyzing 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. Such tests can help rule out such conditions as HIV infection, thyroid and liver disease, scleroderma, or another autoimmune diseases. Notably, autoimmune conditions are the result of the immune system’s attack on the body’s own tissues.
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 — an advanced stage of scarring of the liver — and portal hypertension. This type of hypertension is characterized by increased pressure within the vein that carries blood from the digestive organs to the liver.
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.
Right heart catheterization also is used later during treatment to assess the efficacy of different therapies.
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.
An exercise test can be a six-minute walk test or a cardiopulmonary test. The walk test is used to measure the distance a person can walk during that defined period of time, and the cardiopulmonary exercise test measures the capacity of the lungs and heart to function as the patient exercises on a treadmill or bicycle.
Last updated: June 17, 2021
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