World Pulmonary Hypertension Day is commemorated in relation to the death of the first pediatric patient diagnosed with pulmonary arterial hypertension (PAH) secondary to rapeseed oil poisoning in Spain, which occurred more than three decades ago.
Pulmonary Arterial Hypertension is a rare entity, with multiple causes, complex and progressive, caused by an increase in pressure in the lung arteries, which affects the function of the right ventricle and leads to high mortality. In this context, rapid diagnosis and early implementation of specific treatment are decisive in modifying the evolutionary course of the disease.
The average survival in 1981 was only 2.8 years, according to data provided by the first registry of the US National Institute of Health. With the incorporation of specific therapies aimed at dilating the pulmonary vessels, survival improved significantly, reaching approximately 80% at one year and 40% at 5 years. Currently, with more advanced therapeutic strategies and stricter follow-up, one-year survival can reach 95% in certain subgroups.
However, diagnosis remains a challenge, as symptoms (shortness of breath, chest pain, fainting) can be confused with other diseases, generating an average delay in diagnosis of 2 years. On the other hand, about 70% are in advanced stages at the time of confirmation of the disease. Therefore, raising awareness and transmitting information about this pathology to the general population and medical community is essential due to the low survival, the high percentage of hospitalizations and persistent symptoms that affect the daily quality of life of patients.
Due to the complexity of this pathology, multidisciplinary teamwork is essential, including cardiologists, clinicians, rheumatologists, pulmonologists, nurses, psychologists, hemodynamicists, and echocardiographers, among others, as well as adequate training and education for health personnel.
Rapid detection based on suspicion of the clinical picture should be considered in patients with a higher risk of developing the entity (scleroderma, congenital heart disease, portopulmonary hypertension (increased pressure in the liver arteries), related to the HIV virus, and chronic thromboembolic hypertension (due to chronic thrombi in the pulmonary arteries). The Doppler Echocardiogram is the initial tool to determine the probability of suffering from this disease and the placement of a catheter that directly measures the pressures in the pulmonary artery (right heart catheterization), confirms the diagnosis.
Defining the risk of patients is extremely necessary, since it will determine their evolution and will allow medical treatment to be guided according to the severity of the disease.
Finally, it should be noted that this disease is complex and has high mortality, so rapid diagnosis and initiation of specific treatment with approved drugs in PAH is of utmost importance. Currently we have new treatment strategies that act by modulating the proliferation or growth of the pulmonary vessels with encouraging results.
Despite advances, PAH continues to represent a constant challenge in terms of diagnosis, monitoring and treatment, so it is important to raise awareness about this disease and ensure that patients can quickly receive the appropriate treatment to improve survival. Make the invisible visible.
Dr. Guillermina Sorasio
Cardiologist, head of the Pulmonary Hypertension clinic at the Rivadavia CABA Hospital and director of the Heart Failure and Pulmonary Hypertension Council of the Argentine Society of Cardiology 2026.
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