Pulmonary hypertension is a chronic and progressive disease characterized by high blood pressure in the pulmonary arteries, the blood vessels of the lungs.
There are many potential causes of the disease that, along with how early the disease is diagnosed and when treatment is started in the patient, can affect prognosis.
There is currently no known cure for pulmonary hypertension, but research is ongoing, and with the continual improvement of diagnostic techniques and therapies, it is becoming possible to diagnose the disease earlier to manage the symptoms for longer.
Pulmonary hypertension is a progressive disease, which means it worsens over time, although the speed varies based on the individual and the class of pulmonary hypertension. If left untreated, the prognosis is poor and the disease is often fatal within a few years, emphasizing the need for a quick and accurate diagnosis.
The survival of patients with pulmonary hypertension used to be around three to five years, and it could take two or more years to correctly diagnose. But the life expectancy of patients with pulmonary hypertension has improved dramatically since the disease was first identified in 1891. This is because of the continual improvement of diagnostic techniques and the development of specialized therapies for pulmonary hypertension, beginning with the introduction of Flolan (epoprostenol GM) in 1995.
Improved diagnostic techniques result in faster and more accurate diagnoses, which then results in treatment beginning at earlier stages of the disease. Early treatment can significantly improve a patient’s quality of life and can improve the overall life expectancy.
For example, a study observing more than 1,000 patients with pulmonary hypertension in Taiwan determined that 62.6 percent of patients were now surviving 10 years or more after diagnosis. Researchers concluded that the mortality rate of patients with pulmonary hypertension was more than three times higher than in the control group of healthy people.
There is currently no exact method of accurately predicting prognosis following a diagnosis of pulmonary hypertension. However, there are some known risk factors associated with a better or worse outcome.
Potential indicators of a poorer prognosis include:
- A higher functional classification at diagnosis, which indicates a later stage of the disease.
- Comorbidities, or the presence of additional conditions, such as diabetes.
- Presence of pericardial effusion, or abnormal accumulation of fluid around the heart.
Some types of pulmonary hypertension, such as scleroderma-associated pulmonary arterial hypertension and chronic obstructive pulmonary disease (COPD), are also associated with a poorer life expectancy.
Prognosis of group 4 pulmonary hypertension
Group 4 pulmonary hypertension, or chronic thromboembolic pulmonary hypertension (CTEPH), is the only type of pulmonary hypertension that can be cured. CTEPH is caused by blood clots in the pulmonary arteries, and many patients are able to undergo a surgical procedure, called pulmonary thromboendarterectomy (PTE) to remove these clots. Around 85 to 90 percent of patients will experience significantly fewer or no symptoms of CTEPH following the procedure.
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