Pulmonary hypertension refers to a hemodynamic and pathophysiological state in which pulmonary artery pressure is elevated above a certain threshold and can lead to right heart failure, either as an independent disease, a complication, or a syndrome. Pulmonary hypertension is a common and frequent disease with a high rate of disability and death, and should be given high priority.
English name: pulmonary hypertension
Department: Respiratory Medicine
Prevalent groups: people with congenital heart disease, connective tissue disease, portal hypertension, pulmonary disease, chronic pulmonary embolism, HIV infection and other underlying diseases, people taking diet pills, central appetite suppressants, people with a family history of idiopathic pulmonary hypertension or hereditary pulmonary hypertension
Common symptoms: dyspnea, fatigue, weakness, reduced exercise tolerance, syncope, angina pectoris or chest pain, hemoptysis, hoarseness
Pulmonary hypertension is classified into five major categories based on pathological manifestations, hemodynamic characteristics and clinical management strategies: ① arterial pulmonary hypertension; ② pulmonary hypertension due to left heart disease; ③ pulmonary hypertension due to hypoxia and/or pulmonary disease; ④ chronic thromboembolic pulmonary hypertension; ⑤ pulmonary hypertension due to multiple mechanisms and/or unknown mechanisms (see Table 1 for classification).
Table 1. Classification of pulmonary hypertension at the 2013 World Congress on Pulmonary Hypertension in Nice
Clinical manifestations
The symptoms of pulmonary hypertension are nonspecific and may be asymptomatic in the early stages and may manifest as the disease progresses as follows.
1. dyspnea
It appears earliest and is the most common. It is manifested as shortness of breath after progressive activity, and can also occur at rest in severe cases.
2. Fatigue, weakness, and reduced exercise tolerance
Related to reduced cardiac output and insufficient tissue perfusion.
3. Syncope
Decreased cardiac output leads to insufficient blood supply to brain tissue.
4.Angina pectoris or chest pain
Due to right heart ischemia, associated with reduced coronary perfusion due to right ventricular hypertrophy and inadequate relative blood supply to the myocardium.
5.Hemoptysis
Caused by rupture of pulmonary precapillary microangioma.
6.Heartness of voice
Due to pulmonary artery dilatation compressing the recurrent laryngeal nerve.
7.Symptoms of right heart failure
Lack of appetite, nausea, vomiting, epigastric distension, edema of both lower limbs, perineum, lumbosacral region, thoraco-abdominal fluid, cyanosis of lips, fingertips and auricles, and neurological symptoms.
8, some types of pulmonary hypertension will also have symptoms of the original disease
Such as connective tissue disease-related pulmonary hypertension may have hair loss, photosensitivity, oral ulcers, arthritis, etc.
Examination
1.Laboratory tests
Autoantibodies, liver function and hepatitis virus markers, HIV antibodies, thyroid function tests, blood gas analysis, prothrombin time and activity, BNP or NT-proBNP.
2. Electrocardiogram
Suggests right ventricular overload, hypertrophy and right atrial dilatation.
3.Chest radiograph
Signs suggestive of pulmonary hypertension include: right lower pulmonary artery transverse diameter ≥15mm, protrusion of pulmonary artery segment ≥3mm, dilated central pulmonary artery, loss of peripheral pulmonary vessels forming “stump sign”, enlarged right atrium and right ventricle, and increased cardiothoracic ratio.
4.Echocardiography
Echocardiography is used to estimate pulmonary artery pressure and exclude other causes, such as precardiac disease and valvular disease, etc. It can also evaluate right heart function and determine prognosis.
5.Pulmonary function measurement
It is used to clarify airway and lung parenchymal lesions, with emphasis on carbon monoxide diffusion capacity.
6.Pulmonary ventilation/perfusion scan
To help determine the presence or absence of pulmonary embolism.
7.High resolution CT and enhanced CT
Provide more detailed imaging information of lung parenchyma and pulmonary vessels.
8.Magnetic resonance imaging
Can directly assess right ventricular morphology, size and function, and also non-invasively assess some right heart hemodynamic characteristics.
9.Polygraphic sleep monitoring
Used to rule out hypoxic pulmonary hypertension.
10.Cardiopulmonary exercise test
Can evaluate cardiac function, gas exchange capacity, maximum oxygen consumption and EqCO2 can be used to predict prognosis.
11.6 minutes walking distance
An important method to evaluate the exercise tolerance of patients.
12.Right heart catheterization and acute vasodilation test
Right heart catheterization is the gold standard for the diagnosis of pulmonary hypertension and can accurately obtain the hemodynamic characteristics of the pulmonary circulation and the right heart system. The acute vasodilatation test is used to determine whether the patient responds to calcium blocker therapy.
13.Pulmonary arteriography
To rule out pulmonary embolism, pulmonary artery tumor, etc.
14.Thoracoscopic lung biopsy
Not recommended to be performed routinely.
Diagnosis
1.Identify people at risk for pulmonary hypertension: People with underlying diseases listed in the classification table are at high risk for pulmonary hypertension, such as those with congenital heart disease, connective tissue disease, portal hypertension, lung disease, chronic pulmonary embolism, HIV infection and other underlying diseases, those taking diet pills, central appetite suppressants, and those with a family history of idiopathic pulmonary hypertension or inherited pulmonary hypertension.
2.Screening for pulmonary hypertension: echocardiography.
3.Confirmation of pulmonary hypertension: right heart catheterization.
Treatment
1.General measures
Rehabilitation/exercise and exercise training, psychosocial support, contraception, vaccination.
2.Supportive treatment
Anticoagulants, diuretics, digitalis, oxygen absorption.
3.Targeted drug therapy
At present, drugs that have been approved by the Chinese State Food and Drug Administration for targeted treatment of pulmonary hypertension include.
(1) Bosentan Indications: Used for patients with class 1 and 4 pulmonary arterial hypertension.
Precautions.
(1) Aggravate water and sodium retention and edema.
(2) There is a possibility of liver damage, liver function tests should be performed prior to drug administration and liver function should be checked once a month during drug administration.
③It is contraindicated in those who are pregnant or will become pregnant. Women with the possibility of pregnancy should have a pregnancy test once a month. This drug also affects the effect of hormonal contraceptives and other forms of contraception should be used.
④Check hemoglobin level before use and in the 1st and 3rd month after use, and every 3 months thereafter.
(2) Anrisentan Indications: For patients with class 1 and 4 pulmonary arterial hypertension.
Precautions.
(1) Risk of fetal malformation. Strict contraception is required during the use of the drug. It is prohibited in pregnant and lactating women.
②Risk of hepatic damage, liver function tests should be performed prior to drug administration and liver function should be checked once a month during drug administration.
(3) Adverse reactions: fluid retention, heart failure, hypersensitivity reactions, anemia, etc.
(3) Iloprost Indications: For patients with class 1 and 4 pulmonary arterial hypertension.
Precautions.
(1) Adverse reactions: vasodilation, headache, cough, hypotension.
②Dose reduction should be considered in cases of abnormal liver function and renal failure.
(3) Bleeding disorders, pregnancy and lactation are prohibited, and attention to contraception.
(4) Treprostinil Indications: For patients with class 1 and 4 pulmonary arterial hypertension.
Precautions: Adverse effects include pain, diarrhea, jaw pain, edema, vasodilation, and nausea.
4.Interventional treatment
Pulmonary hypertension associated with congenital heart disease: interventional occlusion therapy can be performed if there is an indication.
Chronic thromboembolic pulmonary hypertension and aortitis involving the pulmonary arteries: for those with indications, pulmonary vascular balloon dilation and stent placement are feasible.
Balloon atrial septal stoma: for patients with pulmonary hypertension who have failed to receive the best combination of drugs, and end-stage patients with mRAP >20 mmHg and arterial oxygen saturation <85% at rest are contraindicated.
5.Surgical treatment
Pulmonary artery thromboendarterectomy: It is the preferred treatment for chronic thromboembolic pulmonary hypertension, with indications of cardiac function grade III or IV, mean pulmonary artery pressure above 30 mmHg, pulmonary vascular resistance >300 dyn・s/cm, and thrombus located in arteries above the pulmonary segment that can be reached by surgery.
Lung transplantation: Lung transplantation is recommended for patients with pulmonary arterial hypertension who have failed to respond to drug therapy.
Prevention
Primary prevention: for the general population, promote a healthy lifestyle, quit smoking, limit alcohol, and use diet pills carefully.
Secondary prevention: For high-risk groups, especially those with underlying diseases listed in the classification table, such as congenital heart disease, connective tissue disease, portal hypertension, lung disease, chronic pulmonary embolism, HIV infection, taking diet pills, central appetite suppressants, and those with a family history of idiopathic pulmonary hypertension or hereditary pulmonary hypertension, attention should be paid to monitoring, active control and treatment of primary diseases, and timely detection of pulmonary Hypertension.
Tertiary prevention: For patients with pulmonary hypertension, the prognosis should be improved, active treatment should be given, and factors that aggravate pulmonary hypertension such as pregnancy, colds, and heavy physical activity should be avoided.