Pulmonary hypertension (PH) is a rare lung disorder that causes constricting and clogging of the pulmonary arteries that are used to conduct blood from the heart to the lungs. Because the disease makes it difficult for the heart to properly pump the blood, the organ needs to work harder, under stress, and it becomes enlarged and weakened. In the long-term, patients with pulmonary hypertension are at higher risk of serious illness including fatal right heart failure and death.
There is currently no cure for pulmonary hypertension, but there are treatments approved by the U.S. Food and Drug Administration (FDA) to help in the management of its symptoms. If not treated, the prognosis of the disease is only 2.8 years on average, which explains the importance of early and proper diagnosis followed by treatment, as described in the article “Pulmonary hypertension: Past, present and future.”
Several signs and symptoms may indicate PH, but a physician needs to conduct tests and exams to confirm it.
Signs And Symptoms That Can Lead To Diagnose Pulmonary Hypertension
The first symptoms associated with the disease include shortness of breath (dyspnea), fatigue, dizziness or fainting spells (syncope), pressure or pain in the chest, swelling (edema) in the ankles, legs or abdomen (ascites), bluish color in the lips and skin (cyanosis), and irregular heart beat. In early stages, signs of the disease can be barely noticed for months or years until they worsen with disease progression.
Through assessment of the symptoms’ aggressiveness, a physician can diagnose the disease and its stage.
The World Health Organization (WHO) defines Class I PH as no presence of symptoms; Class II PH when the patient experiences fatigue, shortness of breath or chest pain during normal activity but not at rest; Class III PH if the patient feels comfortable at rest but has symptoms when physically active; and Class IV when the patient experiences symptoms even at rest.
Tests And Exams Conducted To Diagnose Pulmonary Hypertension
When a patient is experiencing the kind of symptoms that indicate pulmonary hypertension, the first exams to be conducted for diagnosis are blood tests that verify the presence of substances that show pulmonary hypertension or complications; chest X-ray to check for enlarged pulmonary arteries or right heart ventricle; and Doppler echocardiogram to analyze the heart with sound waves and without a surgery.
A transesophageal echocardiogram allows the physician to see a clear picture of the heart and lungs; a standard echocardiogram and a right heart catheterization is a reliable method to directly measure the pressure in the main pulmonary arteries and right ventricle.
In addition, some exams that be requested to confirm the diagnosis or the stage of the disease. An exercise echocardiogram can help determine how well the heart works under stress, while a pulmonary function test measures the amount of air that the lungs can hold.
A perfusion lung scan, with the use of radioactive substances, may also be used to analyze the blood flow in the lungs. Computerized tomography (CT) scan, magnetic resonance imaging (MRI), and open-lung biopsy are also options.
When cases of pulmonary hypertension seem to run in the family, the physician may also request genetic tests.
Difficulties And Limitations To Diagnose Pulmonary Hypertension
Diagnosing pulmonary hypertension is particularly challenging in early stages because the progression of the disease can be slow and go unnoticed during routine physical examinations.
The study “Pulmonary hypertension: diagnostic and therapeutic challenges” demonstrated that echocardiography is the best screening tool, but that it limited by the reliability of pulmonary arterial pressures. The researchers concluded additionally that pulmonary hypertension is especially difficult to diagnose when patients already struggle with other chronic conditions such as chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), and sleep disordered breathing.
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