bubble_chart Overview

The etiology of this condition is unknown. It is characterized by progressive elevation of spontaneous pulmonary arterial pressure, often accompanied by manifestations of right ventricular hypertrophy and right heart dysfunction. The fundamental pathological changes include sclerosis, stenosis, embolism, or plexiform lesions of the small pulmonary arteries. The disease typically begins in young adults and often progresses in severity.

bubble_chart Clinical Manifestations

1. Mild or early-stage patients may have no symptoms. Palpitation, shortness of breath, lack of strength, chest pain, and syncope are common in mid-to-advanced stage patients. Some severe cases may present with cyanosis.
2. Increased second heart sound in the pulmonary valve area, early systolic click, and systolic ejection murmur.
3. Signs of right ventricular hypertrophy.
4. Signs of right heart failure, such as hepatomegaly and edema.

bubble_chart Auxiliary Examination

1. Primary pulmonary hypertension is a complex and rare disease, and diagnosis mainly relies on auxiliary examinations, especially right heart catheterization. However, the arrangement of examination items should still follow the principle of prioritizing economical, safe, and highly diagnostic tests before considering expensive and invasive examinations.
2. The differentiation from secondary pulmonary hypertension can be selected under "B" items as appropriate.

bubble_chart Diagnosis

1. The typical symptoms, signs, and manifestations of pulmonary arterial hypertension on X-ray and echocardiography. If necessary, directly measure pulmonary arterial pressure and right heart pressure via right heart catheterization.
2. This disease can only be diagnosed after excluding secondary pulmonary arterial hypertension caused by cardiac or pulmonary diseases.

bubble_chart Treatment Measures

1. There is still a lack of fundamental treatment methods for this disease, with symptomatic treatment being the main approach.
2. Vasodilators have been affirmed in the treatment of this condition.
3. For advanced stage cases, combined heart-lung transplantation may be considered.

The therapeutic outcomes for this disease are unsatisfactory, with a lack of specific methods and medications. Conventional treatments often yield poor results when right heart failure occurs, necessitating enhanced use of vasodilators and diuretics. Anticoagulants can be considered in severe cases.