In this section you will find a complete catalogue of the different diseases related to pulmonary hypertension.Our goal is to provide you with reliable and detailed information so you can better understand these conditions, their symptoms, diagnoses, and treatment options.
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Detecting pulmonary hypertension (PH) can be tricky, as its most common symptoms—shortness of breath, fatigue, and dizziness—commonly occur alongside many other medical conditions.
When a doctor suspects the presence of HP in a patient, he or she will perform a review of medical and family history, in addition to a physical examination and specific tests.
Initial Medical Evaluation
Before ordering more advanced studies, the specialist performs a physical examination, in which he or she looks for:
Swollen or visible veins in the neck.
Irregular heartbeats.
Swelling in the extremities or abdomen (edema).
Subsequently, a series of diagnostic tests are recommended to determine the type of HP, its possible cause, and the most appropriate treatment.
Diagnostic Tests
Diagnosis may require various studies, such as:
Assessment of lung function.
Stress tests (such as a six-minute walk or a cardiopulmonary study).
Blood test.
Echocardiography.
However, the only way to confirm PH with certainty is through right heart catheterization (RHC).
Right heart catheterization is the gold standard for accurately diagnosing PH.
Tests to Determine the Type of Pulmonary Hypertension
There are different forms of pulmonary arterial hypertension (PAH), and identifying the specific type is key to guiding treatment.
Blood Tests and Immunology
The doctor may order a blood test to assess the patient's overall health and detect markers associated with PH. These tests help identify:
Function of organs such as the liver, kidneys, and thyroid.
Connective tissue diseases.
Signs of anemia or cardiac stress.
Blood oxygen and carbon dioxide levels (arterial blood gas).
Abdominal Ultrasound
If liver disease is suspected, an abdominal ultrasound is recommended to examine organs such as the liver, kidneys, spleen, and pancreas.
In some cases, portopulmonary hypertension (PPH) develops as a result of advanced liver disease, presenting with symptoms similar to those of group 1 PAH.
Once a diagnosis of HP is confirmed, doctors perform additional tests to personalize treatment and assess disease progression.
Studies to Evaluate Heart Function
Right Heart Catheterization
The CCD is a fundamental test in which a catheter is inserted into a vein (in the neck or groin) to guide it to the heart and measure the pressure in the pulmonary arteries.
This procedure allows for detailed analysis of blood flow and cardiac function. Blood samples can also be obtained for further studies.
In some cases, wedge pressure is measured, which provides information about the function of the left side of the heart. This helps differentiate between the different types of PH.
Right heart catheterization is the only test that can accurately confirm PAH.
Although pulmonary hypertension has no cure, there are treatments that can help control the disease and improve quality of life.
Therapeutic strategies include:
Oral medications.
Drugs inhaled or administered subcutaneously.
Intravenous therapy in advanced cases.
Oxygen therapy and diuretics in some patients.
Treatment of underlying diseases (in HP groups 2 and 3).
In certain cases, lung or heart transplant.
For patients with group 4 PH, pulmonary thromboendarterectomy surgery may be an option to remove clots in the pulmonary arteries.
If surgery is not feasible, balloon pulmonary angioplasty or specific drug treatments may help.
Each patient requires a personalized approach, so it is essential to consult with a PH specialist to determine the best treatment option.
Main Medications for PH
Oral Therapies
Endothelin receptor blockers:
Ambrisentán, Bosentán, Macitentan.
Phosphodiesterase type 5 (PDE-5) inhibitors:
Sildenafil, Tadalafil.
Other medications:
Treprostinil oral, Selexipag, Riociguat.
Inhaled Drugs
Prostacyclin analogues:
Iloprost, Inhaled Treprostinil, Tyvaso Dry Powder Inhaler.
Subcutaneous Therapy
Subcutaneous treprostinil.
Intravenous Treatment
Drugs for hospital use:
Epoprostenol, Treprostinil.