Pulmonary arterial hypertension is a key issue of the 4ᵉ Rare disease National Plan, which aims to strengthen access to expert centers and improve patient management. As Dr. Marianne Riou explains, pulmonologist at Strasbourg University Hospitals, “pulmonary hypertension is defined by an abnormal elevation of pressure in pulmonary circulation”.
The specialist specifies that there is a particular form of pulmonary hypertension. “This is pulmonary high blood pressure, or HTAP, a rare disease which, in France, affects around 15 to 50 people per million inhabitants. The problem is that the symptoms of this condition are often not very specific. They are indeed common to other diseases, which makes diagnosis difficult.
“Patients are often diagnosed late, because this pathology is not immediately suspected. This is why it is important that patients and doctors are vigilant in the face of the main signs. “In particular the breathlessness of effort, an unusual fatigue, a persistent and unexplained cough, chest pain, palpitations”.
Give access as quickly as possible to suitable support
In pulmonary high blood pressure (HTAP), early diagnosis remains a real public Health issue. Indeed, the delay between the first symptoms and the diagnosis of HTAP remains greater than 2 years. A deadline heavy of consequences. Because the late the care, the more the patients reach an advanced stage of the disease. It is therefore a real loss of chance for patients, whose prognosis depends largely on the precocity of suitable management.
“In general, the patient consults first for a persistent shortness of breath,” explains Dr. Riou. “The general practitioner seeks to dismiss an acute cause, then he can orient towards a pulmonologist or a cardiologist who will perform a cardiac ultrasound. In the event of suspicion, the patient will then be sent to an expert center for an in -depth diagnosis. “Hence the importance, she insists, for the doctors of first appeal,” to think, in the face of a shortness of breath which persists without obvious cause, to send their patient to a specialist. »»
Faced with this complex journey, Maggy Surace, president of Htapfrance specifies that “The flagship challenge is to raise awareness of cardiologists and pulmonologists from city to disease, so that they think of quickly orienting the patients devoted to patients for whom they have a doubt in the face of an unexplained dyspnea. »» And Dr. Riou to add: “ It is necessary to better train healthcare professionals to reduce deadlines and improve the prognosis. »
The importance of care in an expert center
In France, a network of dedicated expert centers allows patients to quickly access specialized care and suitable treatments. These structures ensure optimal monitoring, in connection with other health professionals.
“For a rare disease like HTAP, management in an expert center is essential,” says Dr. Riou. “The diagnosis is based on a specific examination – right cardiac catheterization – which requires sharp expertise and is not achievable everywhere. In these centers of excellence of which we are fortunate to have in France, the teams are trained in this complex pathology.
Result: a confirmed diagnosis, precise identification of the cause, and treatments adapted to a case -by -case basis. Thanks to the territorial network of these skills and reference centers, distributed throughout the territory, including overseas, each patient can thus benefit from specialized follow-up, near his home and care and access to innovative treatments. »»
“We are very fortunate to have in France a sector of management of rare respiratory diseases of excellence and several treatments which can significantly improve our quality of life. It is therefore crucial that we, sick, be able to access it and this early as possible ”, Add Maggy Surace.
