Pulmonary Hypertension, a rare disease with three thousand cases in Italy
Pulmonary Hypertension is a rare, disabling disease that affects approximately 60 in one million people and there are currently about three thousand cases in Italy. Early diagnosis is essential in order to improve a patient’s prognosis and quality of life.
Characteristics of Pulmonary Hypertension
With pulmonary hypertension, the arteries in the lungs are narrowed, blocked or destroyed making it difficult for blood to flow through and, thus, raising blood pressure in the lungs. Reduced blood flow makes work harder for the right side of the heart, as it tends to expand and is not able to pump an adequate supply of blood to the lungs.
There are two types of chronic pulmonary hypertension:
Primary: an abnormal increase in pulmonary artery pressure that leads to increased pulmonary vascular resistance and, consequently, right heart failure
Secondary: caused by other diseases
Secondary forms can be caused by:
Both men and women can be affected by pulmonary hypertension, but the disease is 2-3 times more common in women than men, especially affecting women between the ages of 30 and 50 years old. The disease, however, can occur at any age and affects people of all races.
The problem of late diagnosis
Today, early treatment of a disease allows a patient to go on living an almost normal life. Pulmonary hypertension, however, is not always so easy to diagnose given the similarities of the first symptoms to other disorders, for example, shortness of breath (dyspnea) depending on the level of activity, fatigue (asthenia), dizziness and fainting. Symptoms in more advanced stages of the disease include a dry cough and hoarseness, chest pain (angina) and haemoptysis. Thus, non-specific symptoms in the initial phases of the disease often delay diagnosis, yet early diagnosis is fundamental for significantly improving prognosis and quality of life. An accurate diagnosis includes the following tests:
Echocardiogram- the most useful instrument in making a preliminary diagnosis
Right-heart catheterization- essential in confirming a patient’s diagnosis of pulmonary hypertension, establishing hemodynamic parameters and performing pulmonary vascular reactivity tests
Ventilation/perfusion scan- useful in diagnosing the type of pulmonary hypertension associated with chronic pulmonary thromboembolism
CT chest scan and angiography- x-rays that show the location and extension of obstructive lesions for eventual surgery
There is a multi-disciplinary approach to the management -from diagnosis to treatment- of this type of disease that is only carried out in a few specialized centers such as Domedica, which has created specific Patient Support Programs for the disease.
A new type of oral therapy
Medical treatment has two objectives: to treat and prevent the decompensation of the right ventricle and prevent the progression of pulmonary lesions, thus slowing down the progression of the disease, improving symptoms and prolonging survival. In more serious cases, a lung transplant or a heart-lung transplant is possible. Medication used to treat the disease has been administered through long-term infusions. Since 2018, however, a new oral therapy has been made available, as shown in the results of a study published by the New England Journal of Medicine. The new drug has already been approved by the U.S. Food and Drug Administration and the European Medicines Agency. This new oral therapy has simplified the management of the disease in that it involves taking the medication orally twice a day. The new therapy, however, does not substitute infusions. Invasive therapy is still administered when the symptoms of pulmonary arterial hypertension enter an advanced stage and become incapacitating. Oral therapy is prescribed in the initial stages of the disease when symptoms are still subtle. The early use of oral drugs can reduce progression of the disease by 40%.
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