Pulmonary Arterial Hypertension is often a diagnostic and therapeutic challenge. During the last decade, there is a remarkable progress in pharmaceutical therapy which includes active drugs which are medicated orally (Bosentani, amprisentani, sixtasentani, tadalafil, treprostinil), through inhalation (iloprost, treprostinil) or intravenously (epoprostenol), either as monotherapy or together, and which can help the quality of life of patients. Moreover, at patients for whom there is no suitable medication or for whom treatment has failed, surgical and interventional approach still plays an important role. These techniques include pulmonary thromvoendartirektomi (for chronic thromboembolic pulmonary hypertension), lung transplantation and atrial septostomy.
Depending on the cause of P.H., the doctor selects the appropriate treatment for the patient, such as dosage of oxygen, anticoagulants, diuretics, enhance the function of the heart or administration of conventional therapy (competitors Ca). For the cases of patients who do not respond to conventional therapy, recent therapeutic advances offer treatment options that act selectively on pulmonary functio , such as prostanoids, antagonists and phosphodiesterase type 5 antagonists of endothelin receptors with substance bosentan.
The essence of bosentan, which has been successfully tested in adults and children and is taken orally twice a day, improves disease symptoms and quality of life of patients, as well as the life expectancy of sufferers.
In each case, early diagnosis of P.H. and immediate initiation of treatment can improve the quality of life of patients and the progression of the disease can be delayed or even prevented. Worth mentioning that with the new treatments, life expectancy of patients with P.H. has increased.
In any form of Pulmonary Hypertension, the function of the right ventricle is constantly burdened. The walls of the pulmonary arteries thicken and make it difficult to promote the blood to the lung. Eventually, the right ventricle extends (growing) and blood regresses, thereby swollen legs and liver, that in scientific talk means right heart failure. Treating the symptoms with drugs without investigating the cause of this is a medical error, because the discovery of the cause increases the likelihood of successful treatment. The treatment of varicose veins of the legs for example or general venous thrombosis leads to a substantial treatment. On the contrary, healing only the symptoms improves and reassures the patient, but can be definitely a loss of time for a possible treatment.
Assist in removing fluids from the body and are given especially for the right heart failure. The main side effect of chronic use of diuretics, especially in large quantities, has given a negative result and the loss of useful trace elements in the body of the patient.
Those who have right heart failure and hypoxia (lack of oxygen in the blood) at a state of rest need continuous oxygen therapy. Patients with Pulmonary Hypertension who have no resting hypoxemia are not benefited from oxygen therapy.
They are necessary when we have a diagnosis of primary Pulmonary Hypertension associated with local thrombosis, but they also are given in many cases of idiopathic P.H. Even a long term reception has not shown significant side effects.
Based on the response to drugs (in catheterization) of pulmonary artery pressure, patients are divided into:
• Non- Responders (Decrease of pulmonary pressure less than <20 % ) • Moderate Responders (Decrease of pulmonary pressure 20-50 % ) • Bold Responders (Decrease of pulmonary pressure > 50 % )
Calcium antagonists intended for patients who almost normalize the pulmonary pressure during testing and especially for vasodilating patients (patients who keep their vasoconstriction) . Medication without harmful side effects.
Endothelin receptor antagonists (Bosentan)
Act through receptors A and B endothelin. The effect is evident 2-3 months after the start, so in class IV patients should not be administered as monotherapy during this time. A side effect of the use of this product is that there can be an increase in transaminases and there is need of frequent blood tests, and based on them the doctor decides if they are going to follow the treatment.
Produced by the endothelium. Have vasodilating action, they reduce the accumulation of the platelets and the hypertrophy of the vessel wall. There are various means to remedy the lack of prostacyclin, and one of them is intravenous (epoprostenol) has a short life and requires continuous infusion, this makes it extremely effective. There is also the subcutaneous (treprostinil) with a long activity and inhaled (iloprost), but it has a very short time of action. The side effects of these drugs are focused on local physical factors.
The sildenafil (Viagra) is a powerful vasodilator and has been tested with positive results either alone or in combination with prostacyclin. It is given in the majority of the diagnoses as a treatment of P.H. and it is the header of the therapeutic approach of the patient. A medicine with minimal side effects associated with the outcome.
2. Surgical Treatment
Rupture of the atrial septum
Improves cardiac output and is intended for patients who are resistant to the maximum medication often use as a “bridge” for transplantation.
Lung transplantation or heart – lung
Recommended for patients who, despite the maximum medical or surgical treatment, do not improve. Transplantation can be done either in a single lung or both. If we are talking about right heart failure (puncture of the right ventricle), double heart -lung transplantation is the final solution.