- Pulmonary arterial hypertension is a rare, serious disease with vague symptoms.
- The disease is treated with drugs and, in advanced stages, with a lung transplant.
- Approximately 25 patients are added annually, the disease more often affects younger women.
- Narrowed small pulmonary vessels overload the heart’s right ventricle and cause it to fail.
Pulmonary arterial hypertension (PAH) is a rare but serious disease. He has vague symptoms such as shortness of breath, fatigue, dizziness, leg swelling, chest pressure or palpitations. According to the head of the Cardiology Clinic of the Faculty of Medicine of the Comenius University and the National Institute of Heart and Vascular Diseases, Eva Goncalvesová, early diagnosis is absolutely crucial. She informed about it at Wednesday’s press conference.
Goncalves explained that PAH is a disease of the small pulmonary vessels that narrow, and the heart has to work harder to get blood flowing through them. This overloads the right ventricle of the heart and causes it to fail. She added that the disease can be treated pharmacologically, in more advanced stages by lung transplantation. Clinical trials of drugs are also available, which bring new knowledge about the disease and more targeted treatment procedures. Approximately 25 diagnosed patients with this disease increase annually and it mainly affects younger women.
The first step in diagnosing the disease is noticing the symptoms and then seeking a doctor – either a general practitioner, a cardiologist or a pulmonologist. “Of course, he cannot tell from the first contact to the first conversation that you have this rare disease,” stated Goncalvesová. She added that other diseases must be ruled out first, which can delay the diagnosis process. If PAH is suspected, the patient is sent to a specialized center, where the disease is confirmed and the correct treatment is set up. Subsequently, the patient is monitored and controlled, and the treatment is adjusted according to his needs.
President of the Association of Patients with Pulmonary Hypertension Iveta Makovníková explained that even normal activities become an obstacle for patients. “At home, they have a problem with cleaning, a problem with washing windows, they have a problem with vacuuming,” she outlined. She stated that 20 years ago the median survival of patients with PAH was 2.8 years, today with current treatment options it is six years.
On average, a patient waits one to three years for a correct diagnosis, said cardiologist Bibiana Kafková from the East Slovak Institute of Heart and Vascular Diseases. According to her it is also important to raise awareness about PAH not only among patients and the general public, but also among physicians.
