Pulmonary hypertension (PH) is a life threatening, progressive disease in which there is elevated blood pressure in the arteries supplying the lungs. The blood vessels affected with pulmonary hypertension are pulmonary artery, pulmonary vein and pulmonary capillaries. The blood flow from the heart to the lungs is disrupted due to constriction in blood vessels causing elevated pulmonary arterial pressure. Normal pressures in pulmonary arteries are 15 to 30 mm Hg systolic pressure and 4 to 12 mm Hg diastolic pressure. If the pressure is greater than 30/12 mm Hg, then it is known as pulmonary hypertension.
What Are The Diagnostic Approaches For Pulmonary Hypertension?
The diagnosis of pulmonary hypertension is difficult to make as the symptoms of pulmonary hypertension often overlap with other diseases of heart and lung such as fatigue, chest pain, dizziness, shortness of breath, tachycardia. Therefore, it is not diagnosed until it becomes very severe leading to poor prognosis of the disease. The different diagnostic approaches include pulmonary function tests, certain imaging tests such as right heart catheterization, echocardiogram (heart ultrasound), chest X-ray, computed tomography, electrocardiogram, exercise stress test and nuclear medicine lung scan. The treatment plan is based on the results of these tests as these tests also help in finding out the cause of disease.
Is there a Cure for Pulmonary Hypertension?
Pulmonary hypertension is a progressive disease, which is life threatening if left untreated with a survival rate of only 2 to 3 years in the absence of management. There have been advances in treatment modalities with the survival rate rising to 5 to 10 years and more medications in the market. Unfortunately, there is no definitive treatment/cure for pulmonary hypertension. The aim of treatment is focused on slowing/delaying the progress of the disease by improving the quality of life of the patient with medications. The goal of treatment is to treat the underlying cause and reduce symptoms of elevated pulmonary pressure in blood vessels in order to improve quality of life of the patients.
There are various treatment options that include:
Prostacyclin Analogue reduces blood pressure by relaxation of pulmonary blood vessels. The relaxation of blood vessels causes increase in blood flow to the lungs, thus reducing workload on heart. The prostacyclin used is treprostinil, which can be taken orally (Orenitram), inhaled (Tyvaso), IV (Remodulin), and subcutaneously.
Endothelin Receptor Antagonists (ERAs) are group of medications that prevent blood vessel narrowing or vasoconstriction. They are usually taken orally and the various ERAs used are ambrisentan (Letairis), macitentan (Opsumit) and bosentan (Tracleer).
Phosphodiesterase-5 inhibitors (PDE 5 inhibitors cause vasodilatation of the pulmonary blood vessels by augmentation of cGMP and NO. PDE 5 inhibitors used are sildenafil (Revatio) and tadalafil (Adcirca) in oral form.
Selective IP Receptor Agonist helps in relaxing blood vessels in the lungs by activating prostacyclin receptor. The example is selexipag (Uptravi) taken orally.
Soluble Guanylate Cyclase (sGC) relaxes pulmonary blood vessels by increasing interaction of sGC with NO. Example includes riociguat (Adempas).
Intravenous medications that are used are Flolan (epoprostenol), Remodulin and Veletri for symptomatic relief of chest pain and shortness of breath. Ventavis and Tyvaso are used in inhaled form for relief of shortness of breath.
Conventional treatment options include calcium channel blockers that help in decreasing of blood pressure. Blood thinners (anticoagulants) that prevent clotting of blood are also used in the form of warfarin (Coumadin). Digoxin aids in better pumping of the heart by increasing the force of contraction of heart muscle. Elevated blood pressure in pulmonary vessels (capillaries) causes leakage of fluid in the interstitial spaces and other parts of the body leading to swelling. Diuretics are used for removal of extra fluid and to reduce swelling. Patient can also be put on artificial oxygen if needed.
Severe cases of PH that are not responsive to medications can consider lung transplant options or atrial septostomy (making holes between right and left heart), which carry their own risk and side effects. Newer therapies such as stem cell therapy are also under the research.
- Pulmonary Infarction or Lung Infarction: Causes, Symptoms, Signs, Tests, Treatment
- Primary Pulmonary Hypertension: Who Is At Risk, Is It Serious Disease, Symptoms, Diagnosis, Treatment
- Pulmonary Arterial Hypertension: Causes, Who Is At Risk, Is It A Serious Condition, Symptoms, Treatment
- What is Pulmonary Embolism: Causes, Symptoms, Signs, Risk Factors
- Pulmonary Hemorrhage: Symptoms, Treatment, Causes, Prognosis, Epidemiology, Pathophysiology, Complications