Definition
Pulmonary hypertension - also called idiopathic pulmonary arterial hypertension - is a rather rare disorder that manifests itself with a prolonged pathological increase in blood pressure ONLY in the lungs and in the right side of the heart, in the absence of an identifiable cause. Pulmonary hypertension is a serious disease whose symptomatology tends often to progressively and rapidly degenerate.
Causes
In the context of pulmonary hypertension, the blood fails to flow normally into the lungs, due to a narrowing or occlusion of the pulmonary arteries and capillaries: pulmonary hypertension has repercussions in the heart which, forced to overwork, s 'weakens.
- Hypothetical risk factors: obstructive sleep apnea, low levels of oxygen in the blood for a long time, COPD, pulmonary embolism, HIV infections, congestive heart failure, pulmonary fibrosis, some heart valve diseases, rheumatic diseases.
Symptoms
Shortness of breath and stunning during sport are the first signs that should alert a patient suffering from pulmonary hypertension. Other recurrent symptoms associated with this rare disease include swollen ankles, cyanosis, weakness, chest pain, fatigue, fainting, tachycardia, dizziness.
Information on Pulmonary Hypertension - Drugs for the Treatment of Pulmonary Hypertension is not intended to replace the direct relationship between health professional and patient. Always consult your doctor and / or specialist before taking Pulmonary Hypertension - Medicines for the Treatment of Pulmonary Hypertension.
drugs
Before starting to analyze the drugs and possible treatments to lighten the symptoms of pulmonary hypertension, we schematically report the mechanism that triggers the disorder, which will help to better understand the choice of therapeutic strategies.
Narrowing of the pulmonary arteries → the blood is struggling to flow into the lungs → ↑ pressure → the right ventricle is forced to overstep to pump blood into the lungs → swelling of the ventricle → weakening of the heart and heart failure
Although a complete solution to pulmonary hypertension has not yet been identified, the current treatment options aim to lighten the symptoms, improve the patient's quality of life and, above all, block or in any case curb the inexorable progression of the disease.
First of all it is essential to intervene on the factors that predispose or accentuate pulmonary hypertension, such as (in particular) apneas, lung diseases and heart valve disorders.
It will be the doctor who chooses which drug is most suitable for the patient, depending on the cause (hypothesized or ascertained, when detectable) and the obvious symptoms; some patients need to take blood thinners to reduce the risk of blood clots forming in the veins of the lower limbs or in the pulmonary arteries.
Low levels of oxygen in the blood are observed in many pulmonary hypertension patients: in these cases, it is necessary to intervene promptly with an appropriate therapy (oxygen supplementation).
Patients suffering from pulmonary hypertension should stop smoking, avoid going to places of high altitude, refrain from heavy and lifting physical activities, and undergo the annual influenza and pneumococcal vaccine.
In cases of severity, it is conceivable to have a heart, lung, or both.
First-line drugs for the treatment of pulmonary hypertension:
- Ambrisentan (Ex. Volibris): the drug belongs to the class of endothelin receptor inhibitors; the active ingredient exerts its therapeutic activity excellently in the context of pulmonary hypertension by blocking the hormone endothelin receptors, responsible for constricting blood vessels. By making the activity of the hormone vain, ambrisentan dilates the vessels and promotes a reduction in altered lung pressure. The recommended dose suggests taking 5 mg of the drug orally, once a day, with food or fasting.
- Bosentan (eg. Tracleer): the drug, like the previous one, is an inhibitor of endothelin-1. For adults who weigh more than 40 kg, suffering from pulmonary hypertension, it is advisable to start taking the drug at a dose of 62.5 mg, twice a day, for a month. The maintenance dosage plan is to increase the dose up to 125 mg twice a day. For affected individuals weighing less than 40 kg, it is advised to maintain the 62.5 mg dose, both to start therapy and to maintain. The drug may induce liver toxicity.
- Iloprost (Es. Ventavis): available in the form of a nebulizer, the drug is a prostacyclin analogue able to determine a dilation of blood vessels, thus reducing blood pressure and improving the symptoms derived from pulmonary hypertension. It is recommended to start therapy by inhaling the drug with an inhaler at a dose of 2.5 micrograms; follow the therapy with 5 microgram doses. If the dose of 5 mcg is too strong for the patient, it is advisable to return to the initial dose and continue the therapy according to this treatment plan. It is recommended not to administer more than 6-9 sprays a day.
- Sitacentan sodium (Ex. Thelin): the drug belongs to the class of endothelin-1 inhibitors. It is recommended to take one 100 mg tablet once a day, with or without food. It is advisable to take the drug approximately at the same time. In case of treatment failure after 3 months of treatment, it is recommended to change medication. The drug trade was suspended in 2011 due to severe side effects in the liver.
- Sildenafil (eg Revatio): the drug is widely used in therapy for the treatment of erectile dysfunction; however, being a 5-phosphodiesterase inhibitor, it is also used to reduce the symptoms of pulmonary hypertension because, by modulating the effect of nitric acid on vascular tone, it is a good, relatively selective, vasodilator of the pulmonary arteries. It is recommended to take the drug at a dose of 20 mg, orally, three times a day, at a distance of at least 4-6 hours between one dose and the other. Alternatively, it is possible to take 10 mg (equivalent to 12.5 ml) by intravenous bolus injection, three times a day: the dosage just described determines the same therapeutic effect as an oral dose of 20 mg. In general, this second indication is reserved for patients previously treated with oral Sildenafil and temporarily unable to take the drug by mouth.
- Nitrogen oxide (Ex. INOmax): drug to be inhaled, indicated for the treatment of neonatal pulmonary hypertension, especially when associated with hypoxic respiratory failure. Generally, the drug is not used in monotherapy, but with other specific drugs; furthermore, the patient is normally also subjected to assisted ventilation, in order to improve oxygenation. The active ingredient is diluted in nitrogen gas at a concentration of 400 ppm. For precise dosage: consult a doctor.
- Tadalafil (Ex. Adcirca): the drug belongs to the class of phosphodiesterase type 5 inhibitors, able, therefore, to block the enzyme. It is recommended to take the drug at a dose of one 40 mg tablet, twice a day. Reduce the dosage in case of mild or moderate renal or hepatic deficits. On the other hand, therapy with this drug is not recommended in case of serious deficiencies in the kidneys or liver.
Parallel therapies for the control of pulmonary hypertension symptoms
Some specialists recommend that you also take standard drugs used in therapy for the treatment of hypertension; particularly indicated are the calcium channel blockers (or calcium channel blockers) and diuretics.
Anticoagulant therapy to prevent complications of pulmonary hypertension
- Isosorbide dinitrate (eg Carvasin, Dinike, Nitrosorbide): the drug is a nitrate, also used for the treatment of angina pectoris; it is used as a second choice for the treatment of symptoms related to pulmonary hypertension. Start therapy with a dose of 40 mg every 8-12 hours. Continue with a maintenance therapy at a dose of 40-80 mg, every 8-12 hours.
- Epoprostenol (eg Flolan, Epoprostenol PHT): promotes vasodilation and inhibits platelet aggregation. For this reason, the drug is used in therapy to control symptoms of pulmonary hypertension. Initiate therapy with a drug dose of 2 ng / kg / min; gradually increase the dose in increments of 2 ng / kg every 15 minutes. Do not exceed 8.6 ng / kg / min. consult your doctor for more information.
Hypotheses and hopes for the treatment of pulmonary hypertension
Scientists are directing their research for the treatment of pulmonary hypertension towards the testing of new drugs, such as serotonin antagonists, soluble guanylate cyclase stimulants, tyrosine kinase inhibitors and vasoactive intestinal peptide. We will have to wait a few more years before finding the truly miraculous drug - therefore completely decisive - for pulmonary hypertension.
