The dangers of severe primary pulmonary hypertension

Primary pulmonary arterial hypertension poses a great threat to human health and life. Many patients can only survive for an average of 2 to 3 years after discovering these symptoms. Therefore, it is more important to detect it as early as possible and take treatment measures as soon as possible.

1. Primary pulmonary hypertension, a rare occlusive disease of unknown cause involving medium and small pulmonary arteries, causing right ventricular failure or fatal syncope 2 to 5 years after diagnosis.

2. The incidence rate between men and women is 1:5, and it is more common in women of childbearing age. The average age at diagnosis is 35 years old, and the younger the patient, the worse the prognosis. Almost all cases have intimal hyperplasia and the resulting vascular stenosis. More advanced cases have multiple medial hypertrophy and hyperplasia. Irreversible plexiform damage and necrotizing arteritis (reticular arteriopathy). The same clinical course of vascular damage is also seen in some patients with cirrhosis and in some patients taking anorexia-suppressing drugs, which are no longer marketed in the United States.

3. More than 95% of cases have progressive exertional dyspnea. Precordial pain and syncope during exertion are rare. Many patients have Raynaud's phenomenon and joint pain, which usually appear several years before the onset of obvious primary pulmonary hypertension.

4. Thirty years ago, the prognosis for patients with primary pulmonary hypertension was disastrous, with an average survival of only 2 to 3 years after the onset of symptoms. Recently, through acute trials and long-term treatment with vasodilators, it has been found that the condition of about 20% of patients has been alleviated or stopped progressing, which has improved the prognosis to a certain extent; the success of single lung transplantation has significantly improved the long-term survival rate of organ transplantation, all of which bring hope to the treatment of patients with primary pulmonary hypertension.

5. Persistent fetal circulation is often a serious condition. Severely ill children have symptoms of left heart failure in addition to heart failure. The mortality rate is as high as 50%. Some children have a trend of natural remission, while some children's conditions continue to deteriorate after treatment, and they suffer from obvious hypoxia and eventually die of acidosis. But in general, the effect of drug treatment for most children is relatively satisfactory, and the course of the disease after treatment is about several days to half a month.