Pulmonary hypertension is a clinical and pathophysiological syndrome characterized by abnormally high blood pressure in the pulmonary arteries. Common symptoms include shortness of breath after activity, fatigue, chest pain, dizziness, and lower extremity edema. The cause of pulmonary hypertension has not yet been fully elucidated, and may be related to cardiac disease, pulmonary disease, and genetics, etc. It is necessary to address the cause of the condition with treatments that include general therapy, supportive care, medication, and surgery.
Definition
Pulmonary hypertension is a common pulmonary vascular disease, which is a clinical and pathophysiologic syndrome of elevated pulmonary vascular resistance and pulmonary artery pressure due to various causes.
Classification
Clinical classification
Pulmonary hypertension is classified into the following 5 major categories, which in turn contain multiple subcategories, as described below.
Arterial pulmonary hypertension
This includes pulmonary hypertension that is idiopathic, hereditary, persistent in the newborn, drug- and toxin-related, disease-associated, chronically effective on calcium channel blockers, and has significant pulmonary venous/pulmonary capillary involvement (pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis).
Disease-related pulmonary hypertension further includes connective tissue disease, human immunodeficiency virus (HIV) infection, portal hypertension, congenital heart disease, and schistosomiasis.
Pulmonary hypertension due to left heart disease
Includes heart failure with preserved ejection fraction, reduced ejection fraction, valvular heart disease, congenital/acquired cardiovascular disease causing post-capillary pulmonary hypertension.
Pulmonary hypertension due to lung disease and/or hypoxia
Includes obstructive lung disease, restrictive lung disease, other coexisting obstructive and restrictive lung disease, hypoxemia not due to lung disease, and disorders of pulmonary development.
Pulmonary hypertension due to chronic thromboembolic pulmonary hypertension and/or other obstructive lesions of the pulmonary arteries
Includes chronic thromboembolic pulmonary hypertension, other obstructive pulmonary artery disease (e.g., malignant tumors such as pulmonary artery sarcoma or hemangiosarcoma, pulmonary vasculitis, congenital stenosis of the pulmonary arteries, parasites).
Unspecified and/or multifactorial causes of pulmonary hypertension
Includes hematologic disorders (e.g., chronic hemolytic anemia, myeloproliferative disorders), systemic and metabolic disorders (e.g., tuberculosis, Gaucher’s disease, glycogen storage disorders), complex congenital heart disease, other (e.g., fibrous mediastinitis).
Hemodynamic classification
Pre-capillary pulmonary hypertension
Mean pulmonary artery pressure (mPAP) ≥25 mmHg and pulmonary artery wedge pressure (PAWP) ≤15 mmHg.
Clinical classifications include: arterial pulmonary hypertension, pulmonary hypertension due to lung disease and/or hypoxia, pulmonary hypertension due to chronic thromboembolic pulmonary hypertension and/or other obstructive lesions of the pulmonary arteries, and pulmonary hypertension of unspecified and/or multifactorial origin.
Post-capillary pulmonary hypertension
Clinical classification includes: pulmonary hypertension due to left heart disease; pulmonary hypertension due to unspecified and/or multifactorial causes.
Simple: mPAP ≥25 mmHg and PAWP >15 mmHg and pulmonary vascular resistance (PVR) ≤3 WU.
Mixed: mPAP ≥25 mmHg and PAWP >15 mmHg and PVR ≤3 WU.
Morbidity
We lack epidemiologic data on pulmonary hypertension in the general population.
In the UK, the prevalence of pulmonary hypertension is 97/1 million, with a female to male ratio of 1.8:1.
Of the arterial pulmonary hypertension, congenital heart disease-related pulmonary hypertension, hereditary pulmonary hypertension, and drug and toxicant-related pulmonary hypertension are common.
Causes
Causes
Pulmonary hypertension is caused by structural or functional changes in the pulmonary vasculature due to a variety of etiologic factors and different pathogenic mechanisms, and its occurrence is related to the following factors.
Disease factors
Cardiac diseases: e.g. congenital heart disease (Eisenmenger syndrome, etc.), heart valve disease, cardiac insufficiency, etc.
Lung diseases: e.g. chronic obstructive pulmonary disease, pulmonary fibrosis, abnormal lung development, pulmonary embolism, etc.
Connective tissue diseases: such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, etc.
Hematologic diseases: such as myelodysplasia, chronic hemolytic anemia, etc.
Metabolic diseases: e.g. glycogen storage disease, Gaucher disease, thyroid disease.
Infectious diseases: e.g. acquired immunodeficiency syndrome (AIDS), schistosomiasis.
Other diseases: e.g. nodular disease, vasculitis, tumor, chronic renal insufficiency on dialysis, portal hypertension, etc.
Environmental factors
Hypoxia, e.g. living in highland areas (high altitude) for a long time.
Drugs and toxins
e.g. Amirex, fenfluramine, toxic rapeseed oil, etc.
Genetic factors
Genetic mutations are associated with the development of some patients with arterial pulmonary hypertension, and all hereditary pulmonary hypertension is inherited in a monogenic autosomal dominant manner.
BMPR2 is the most common causative gene for arterial pulmonary hypertension.
Others
The etiology of idiopathic pulmonary hypertension is still unknown. It is currently believed to be related to genetic factors, autoimmunity, and endothelial and smooth muscle dysfunction of the pulmonary vasculature.
Symptoms
Main Symptoms
Pulmonary arterial hypertension is a slowly developing process with no obvious characteristic manifestations. It mainly manifests as symptoms related to progressive right heart insufficiency, often triggered by exertion, and the common manifestations are as follows.
Shortness of breath: the most common, with the development of the disease may also appear at rest.
Dry cough or hemoptysis: dry cough is common, hemoptysis may be small or massive.
Fatigue: This is characterized by extreme tiredness or fatigue and an inability to function properly due to lack of energy. Fatigue may appear suddenly or gradually.
Dizziness or fainting.
Chest pain and tightness in the chest.
Edema of the ankles, lower extremities, and even the abdomen and whole body.
Cyanosis: purple lips and skin.
Other symptoms
Pestle-like fingers (toes): Hypertrophy and pestle-like enlargement of the ends of the fingers or toes.
The thickened pulmonary artery compresses the recurrent laryngeal nerve, which can cause hoarseness.
Weight loss.
Complications
Anemia: pallor, easy fatigue, dizziness, etc.
Arrhythmia: the heart beats too fast, too slow or has an irregular rhythm.
Heart failure: especially right heart failure, often characterized by lack of appetite, nausea, vomiting, epigastric pain, low urine output, and dyspnea.
Pulmonary hemorrhage: hemoptysis, cough, dyspnea, etc.
Liver damage: elevated right heart pressure may cause damage to the liver, with manifestations such as abdominal distension, jaundice, and abdominal distension.
Pericardial effusion: chest tightness, palpitations, dyspnea, abdominal distension, edema, etc.
Consultation
Department of Medicine
Department of Respiratory Medicine
If symptoms such as shortness of breath that cannot be relieved, coughing up blood, chest tightness, palpitations, etc. occur, it is recommended to consult a doctor promptly.
Cardiovascular Medicine
If abnormal changes in the heart are detected by auxiliary examinations, consult the Department of Cardiovascular Medicine.
Emergency Medicine
For sudden severe chest pain, dyspnea, coughing up blood, etc., it is recommended to go to the Emergency Department or call 120 emergency immediately.
Preparation for medical treatment
Preparation for medical consultation: registration, preparation of documents, common problems
Tips for seeking medical treatment
Pulmonary arterial hypertension is often associated with other primary diseases, and it is important to consult a doctor promptly and actively treat the primary disease after the discovery of pulmonary arterial hypertension.
Preparation List
Symptom list
Pay special attention to the time of onset of symptoms, special manifestations, etc.
What are the main symptoms? Are there signs of shortness of breath, dry cough, coughing up blood?
Are there signs of edema?
Any signs of dyspnea, dizziness?
What are the triggers and relievers of the symptoms?
How long have they lasted?
Medical History Checklist
Is there a family history of related medical conditions?
Are there any other medical conditions?
What medications have you been taking recently?
Have you measured your own blood pressure recently? What were the results?
Checklist
Results of the last six months, which can be brought to the doctor’s office
Blood Tests
Blood biochemistry
Electrocardiogram
Lung Function Test
Chest X-ray
Chest CT
Pulmonary Arteriography
Right heart catheterization
Medication List
Medications used in the last 3 months, if available in boxes or packages, bring with you to the doctor’s office
History of use of appetite suppressant-type weight loss drugs, antidepressants, antitumor drugs, and other medications.
History of exposure to toxic canola oil.
History of drug use (cocaine, etc.).
Clinical manifestations
Symptoms
The main manifestations are fatigue after activity, shortness of breath, chest tightness, chest pain and syncope, and in some patients, lower limb edema, hemoptysis, hoarseness and other symptoms.
Signs
Enlargement of the right heart can lead to bulging of the precordial region.
Severe right heart insufficiency may be characterized by jugular venous filling or distention, hepatomegaly, lower extremity edema, pleural and abdominal effusion, jaundice and cyanosis.
Right ventricular hypertrophy may lead to subxiphoid elevated beats and the appearance of the third heart sound, suggesting elevated right ventricular diastolic filling pressures and right heart insufficiency; the fourth heart sound of the right ventricle, prancing rhythm, may be heard in some patients.
Laboratory tests
They are mainly used for the diagnosis of primary disease.
They mainly include routine blood tests, biochemical tests, B-type brain natriuretic peptide (BNP) or N-terminal-B-type brain natriuretic peptide precursor (NT-proBNP), D-dimer, thyroid function, rheumatoid immunity antibody, hepatitis and HIV antibody, homocysteine, coagulation function, arterial blood gas analysis, and so on.
Imaging
Echocardiography
Echocardiography can be used to estimate the likelihood of pulmonary hypertension, identify the cause of the disease and evaluate cardiac function.
Precautions: Follow the doctor’s requirements to maintain a fixed position during the examination, avoid moving around, and generally breathe calmly.
Chest X-ray
It helps to screen for the cause of the disease, but does not exclude pulmonary hypertension.
The examination shows signs of bulging pulmonary artery segments, dilated central pulmonary artery, and enlarged right atrium and right ventricle.
Caution: Remove jewelry or metal objects, such as necklaces and earrings, before the test.
Chest CT
It can clearly show the morphology of the pulmonary arteries and their branches, and measure the degree of widening of the pulmonary arteries and their branches; it can also observe whether there are interstitial lesions and their degree, lesions in the lung parenchyma and mediastinum, thickening of the walls of the main pulmonary arteries and the right and left pulmonary arteries, and pressure on the lymph nodes.
Note: Remove jewelry or metal objects, such as necklaces and earrings, before the examination.
Nuclide Pulmonary Ventilation/Perfusion Imaging
This is an important test for determining the presence of stenosis or occlusive lesions of the pulmonary arteries (including embolic disease).
Precautions: The imaging agent will be injected intravenously during the examination. Drinking plenty of water after the examination will help to expel the imaging agent.
Pulmonary Arteriography
It is mainly used to understand the morphology of pulmonary blood vessels and blood perfusion, and is also commonly used to identify other pulmonary vascular diseases such as pulmonary vascular blockage, stenosis, occlusion and pulmonary arteriovenous malformation.
Precautions: Fasting from food and water for 6 to 8 hours before the operation. After the examination, local pressure bandage should be applied to the puncture site, and the right lower limb should be braked for 6 hours.
Cardiovascular magnetic resonance imaging
It can directly evaluate the size, morphology and function of the right ventricle and assess the blood flow non-invasively.
Remove jewelry or metal objects, such as necklaces and earrings, before the examination.
Abdominal Ultrasound
Helps to understand the structure and function of the abdominal organs and provides a basis for screening for the cause of pulmonary hypertension.
Right heart catheterization
A standardized method for diagnosing and evaluating pulmonary hypertension.
The test is invasive, so it is not usually performed unless other tests are performed that do not lead to a definitive diagnosis. The test involves inserting a catheter under local anesthesia into a large vein in the neck, arm, or groin, and then inserting it through the right atrium and right ventricle of the heart and into the pulmonary artery to measure the pressure in the pulmonary artery.
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Precautions
If any discomfort occurs during the examination, inform the medical staff promptly.
For venous puncture, the limb should be braked for 4~6 hours; for arterial puncture, pressure bandage should be applied after 15~20 minutes of hemostasis, and the wound should be pressurized with a 1kg sandbag for 6~8 hours, and the limb should be braked for 24 hours.
Other examinations
Electrocardiogram: Electrocardiogram can provide important information for diagnosis, differential diagnosis and prognosis judgment of pulmonary arterial hypertension, but it cannot be used as the basis for diagnosis or exclusion of pulmonary arterial hypertension. Abnormal ECG is mostly seen in severe pulmonary hypertension.
Pulmonary function tests: help to detect underlying airway or lung disease.
6-minute walk test: objectively assesses how far a patient can walk and observes whether oxygen levels drop during exercise. This test is the most important test for evaluating a patient’s activity tolerance, and is also an important method for making judgments about prognosis and treatment effects.
World Health Organization (WHO) Cardiac Function Class: Helps to assess the severity of the disease and prognosis.
Cardiac function class I: physical activity is not limited. Daily physical activity does not cause disproportionate dyspnea or weakness, chest pain, or near syncope.
Cardiac Class II: Mild limitation of physical activity. There is no discomfort at rest, and daily physical activity causes disproportionate dyspnea or fatigue, chest pain, or near-syncope.
Cardiac Class III: Significant limitation of physical activity. No discomfort at rest, less than daily physical activity causing excessive dyspnea or fatigue, chest pain, or near syncope.
Cardiac Class IV: Symptoms occur with any physical activity. Dyspnea and/or malaise also occurs at rest and worsens with physical activity; these patients have signs of right heart failure.
Acute vasovagal response test: the purpose of the test is to screen patients who are effective on oral high-dose calcium channel blockers.
Sleep apnea monitoring: monitoring the patient’s brain activity, heart rate, blood pressure, blood oxygen levels, and other factors while sleeping. It can help diagnose whether obstructive sleep apnea is causing pulmonary hypertension.
Lung biopsy: Rarely used, a procedure under general anesthesia to determine the cause by removing a small sample of tissue from the lungs.
Genetic testing: If there is a family history of pulmonary hypertension, genetic screening can be beneficial in determining if it is familial.
Diagnostic Criteria
The hemodynamic diagnostic criteria for pulmonary hypertension are: mean pulmonary artery pressure ≥ 25 mmHg (1 mmHg = 0.133 kPa) measured by right heart catheterization at sea level and at rest.
In normal adults, the mean pulmonary artery pressure at rest is (14.0±3.3) mmHg, with the upper limit not exceeding 20 mmHg.
Differential Diagnosis
Since the symptoms of pulmonary hypertension are not specific, the diagnosis needs to be combined with relevant auxiliary examinations. After the auxiliary examination is clear that there is pulmonary arterial hypertension, it is mainly the differential diagnosis of the cause of pulmonary arterial hypertension, especially for idiopathic pulmonary arterial hypertension must exclude other secondary factors.
Patients with idiopathic pulmonary hypertension with reduced activity tolerance and peripheral edema need to be differentiated from left heart failure, pericardial disease, liver disease and vena cava lesions.
Left heart failure
Similarities: shortness of breath after activity.
Differences: Patients with left heart failure often cough up frothy sputum, sit-up breathing, and sweating profusely. History of left heart disease.
Differences: In constrictive pericarditis, the peritoneal effusion appears earlier and is more severe than lower extremity edema, and there is often a history of tuberculosis, acute pericarditis, and trauma. Pericardial constriction is seen on imaging.
Cirrhosis
Similarities: loss of appetite, malaise, dyspepsia, abdominal distension.
Differences: Patients with cirrhosis may also have jaundice, itching, bleeding tendency, dark complexion, and dilated capillaries on the face.
Vena cava obstruction
Similarities: weakness, shortness of breath, jugular vein raging (superior vena cava obstruction), swelling of lower limbs (inferior vena cava obstruction).
Differences: superior vena cava obstruction may present with headache, physical examination reveals signs such as swelling of the upper limbs, conjunctival congestion, tortuous superficial veins in the chest and abdomen, etc. Inferior vena cava stenosis or obstruction manifests as hyperpigmentation, dilatation of the superficial veins in the lower half of the body, difficulty in walking, and lumbar and abdominal pain.
Treatment
There is no cure for pulmonary hypertension, but the condition can be managed with some treatment, which may help improve symptoms and slow the progression of pulmonary hypertension.
Treatment of pulmonary hypertension is usually complex and requires treatment of the primary disease if it is caused by another condition.
It is important to note that all medications should be used as prescribed by the doctor and should not be adjusted or discontinued.
General Treatment
Physical activity and professionally directed rehabilitation exercises: Patients should be encouraged to move within the limits of their symptoms. Mild dyspnea is acceptable, but patients should avoid exercises that cause severe dyspnea, exertional dizziness, or chest pain.
Family planning to avoid pregnancy: Strict contraception is recommended for female patients of childbearing age.
Postmenopausal hormone therapy: If intolerable menopausal symptoms are present, the use of estrogen under medical supervision may be considered.
Vaccination: Patients with pulmonary arterial hypertension are prone to pneumonia and can be vaccinated against influenza and pneumonia.
Psychosocial support: Anxiety and depression can occur in many patients, and psychotherapy can be provided if necessary.
Travel precautions: WHO cardiac class performance is poor (class III, IV) or arterial oxygen partial pressure <60mmHg need to be cautious flight or have oxygen support during the flight. Patients should avoid altitudes higher than 1500-2000 meters without oxygen therapy.
Elective surgery: Patients with pulmonary hypertension are at increased risk when undergoing elective surgery and require careful selection of anesthesia and avoidance of general anesthesia, especially general anesthesia requiring tracheal intubation. Appropriate perioperative drug therapy is selected for surgery.
Supportive therapy
Oxygen therapy
Oxygenation is recommended when peripheral oxygen saturation is <91% or arterial partial pressure of oxygen is <60 mmHg to achieve oxygen saturation >92%.
Anticoagulation
Commonly used drugs include warfarin.
Lifelong anticoagulation is required in patients with chronic thromboembolic pulmonary hypertension. Long-term anticoagulation may be considered in patients with idiopathic pulmonary hypertension, hereditary pulmonary hypertension, and weight-loss drug-associated pulmonary hypertension if there are no contraindications to anticoagulation.
Patients with comorbid Eisenmenger syndrome and pulmonary artery thrombosis in situ require anticoagulation as appropriate.
In general, patients on long-term intravenous prostaglandin therapy without contraindications to anticoagulation require anticoagulation to avoid the risk of thrombosis.
During anticoagulation, coagulation should be reviewed regularly to avoid the risk of bleeding.
Other medications
Diuretics: commonly used diuretics include hydrochlorothiazide, furosemide, torasemide, bumetanide and spironolactone. They reduce the load on the heart and can also be used to reduce fluid accumulation in the lungs, lower limbs and abdomen.
Digoxin: enhances myocardial contractility and decreases ventricular rate.
Dopamine: It is the preferred positive inotropic drug for patients with severe right heart failure (cardiac function class IV) and acute right heart failure.
Iron: Iron deficiency can cause a decrease in exercise tolerance and an increase in morbidity and mortality in patients. Therefore intravenous iron may be considered in patients with iron deficiency.
Specific drug therapy
Calcium channel blockers
Applicable to patients with acute pulmonary vasodilatation test positive pulmonary hypertension.
Commonly used are nifedipine, diltiazem, and amlodipine. Verapamil may increase the rate of sudden death in idiopathic pulmonary hypertension and is therefore contraindicated.
To avoid serious adverse effects (e.g., hypotension, syncope, and right heart failure), calcium channel blockers are contraindicated in patients who have not undergone a vasovagal response test or in patients with a negative test.
Prostacyclin Analogs
Prostacyclin analogs are synthesized primarily by endothelial cells and cause significant dilation of the vascular bed.
Commonly used drugs include eptifibatrol, iloprost, troglitazone, and beclomethasone.
Epoprostenol requires continuous administration by an infusion pump, and iloprost can be administered intravenously, orally, and by spray.
Endothelin receptor antagonists
Endothelin is a substance in the walls of blood vessels that causes endothelial narrowing, and such drugs can reverse the effects of endothelin.
Commonly used drugs include bosentan, macitentan, and amlisentan.
Bosentan can be used for WHO cardiac function class III and IV idiopathic pulmonary hypertension or pulmonary hypertension caused by scleroderma.
Amrisentan can be used to treat patients with symptomatic pulmonary hypertension of WHO cardiac class II or III.
Phosphodiesterase V Inhibitors
The pulmonary vasculature contains a large amount of phosphodiesterase type V. These drugs work by inhibiting this enzyme to dilate the pulmonary vasculature.
Commonly used drugs are sildenafil, tadalafil, vardenafil and so on.
Sildenafil, commonly known as Viagra, is often used to treat erectile dysfunction, but it can also dilate the pulmonary arteries, making it easier for blood to flow through them.
Soluble Guanylate Cyclase Agonists
increase nitric oxide (NO) in the body, which relaxes the pulmonary arteries and reduces their internal pressure.
Liothyronine is a new soluble guanylate cyclase agonist indicated for the treatment of pulmonary hypertension and chronic thromboembolic pulmonary hypertension.
Prostacyclin Receptor Agonists
Sileptapag targets and activates prostacyclin receptors, thereby helping to relax blood vessels in the lungs.
Combination therapy
Combination therapy refers to the concomitant use of more than one therapeutic agent specific for pulmonary hypertension, such as endothelin receptor antagonists, phosphodiesterase type V inhibitors, prostaglandin analogs, and other newer agents.
Monotherapy or oral drug combinations should be used for low or intermediate risk patients, and combination therapy (including intravenous prostacyclin analogs) should be performed for high risk patients.
According to the WHO cardiac function classification recommendations for patients with pulmonary arterial hypertension, the starting combination therapy regimen for targeted agents is aniricetam + tadalafil.
Surgical treatment
Balloon septostomy
Atrial septostomy is recommended after adequate medical treatment and no significant improvement.
The procedure creates an opening between the right and left atria to relieve pressure on the right side of the heart as a palliative treatment.
Serious complications may occur after surgery, and atrial septostomy may be used as a transitional treatment before lung transplantation.
Lung or combined heart-lung transplantation
Commonly used methods: unilateral lung transplantation, double lung transplantation, living lung lobe transplantation and heart-lung transplantation, which has been maturely used abroad in the treatment of patients with pulmonary arterial hypertension.
Indications: patients who have been adequately treated with internal medicine without obvious efficacy.
At present, double-lung transplantation is the general choice for patients with pulmonary arterial hypertension at home and abroad.
Rehabilitation
Although patients with pulmonary hypertension often experience fatigue, it is important to keep exercising and avoid activities such as weight lifting.
A safe exercise program should be developed in consultation with a physician. Sometimes a 6-minute walk may be required, and the patient should not be alone but accompanied during this assessment.
Patients may need to spend more time warming up before exercising.
When exercising start with minimal intensity and if you feel tired you have reached your limit for the day and should stop.
Some shortness of breath may be felt during exercise, but shortness of breath should be urgently sought if it does not resolve after 5 minutes of rest.
When you start exercising, you can exercise 3 to 5 days a week, depending on the situation, for a total of 30 minutes a day (including warm-up, exercise and rest periods).
Other treatments
Gene therapy has been reported abroad, but is not widely used in clinical practice.
Pulmonary hypertension due to left heart disease can be treated etiologically, such as the application of antihypertensive drugs (e.g., angiotensin-converting enzyme inhibitors, beta-blockers, etc.).
Blood transfusion or hydroxyurea for sickle cell disease.
Heart valve repair.
Nitric oxide (NO) inhalation therapy is not currently recommended due to the inability to monitor inhalation concentrations and the number of side effects.
Management of special cases
Acute right heart failure
Control fluid retention, salt restriction, weight monitoring.
Continuous low-flow oxygenation, assisted by ventilator if necessary.
Use diuretics appropriately to avoid hypotension.
Short-term application of positive inotropic drugs such as dobutamine or phosphodiesterase inhibitors (milrinone).
Pregnancy
In principle, pregnancy is not recommended in female patients of childbearing age.
Clinical classification and risk-stratified diagnosis of patients with this disease in combination with pregnancy are important in determining prognosis.
Termination of pregnancy is first recommended in early pregnancy, and manual negative pressure suction or forceps under general anesthesia is the better choice.
If cardiac function is classified as grade I-II and the patient strongly wants to continue the pregnancy, the patient and her family can be informed of the risks of pregnancy and then closely monitored in a qualified center.
Patients with mild pulmonary hypertension and cardiac function class I can terminate the pregnancy at 34-35 weeks of gestation, and a few patients can terminate the pregnancy at 37 weeks of gestation.
Congenital heart disease
Congenital heart diseases such as atrial septal defect, ventricular septal defect, and arterial duct failure may cause pulmonary hypertension.
They can be treated with medications, interventions, and surgery.
In ventricular septal defects, the presence of Eisenmenger’s syndrome mostly eliminates the need for surgery.
Prognosis
Cure
Pulmonary hypertension is not usually curable, let alone self-resolving, and often progresses slowly, requiring early intervention to achieve the best clinical outcome.
The mortality rate of patients with arterial hypertension in the intensive care unit (ICU) has been shown to be as high as 41%, with a poor prognosis.
A 2011 study in China showed that the 1-year and 3-year survival rates for idiopathic pulmonary hypertension were 92.1% and 75.1%, respectively.
World Health Organization cardiac function class remains a strong predictor of patient survival.
In patients with untreated idiopathic pulmonary hypertension or hereditary pulmonary hypertension, previous data have shown that patients with cardiac function class IV have a median survival of 6 months.
Patients with cardiac function class III have a median survival of 2.5 years.
Patients with cardiac function classes I and II have a median survival of 6 years.
Median Survival: The survival time of patients with the same disease in chronological order that lies in the middle of the total number (e.g., 50th out of 99 patients).
Prognostic Factors
Patients with idiopathic pulmonary hypertension/hereditary pulmonary hypertension harboring a BMPR2 mutation have an earlier onset of disease, a more severe clinical phenotype, and a worse prognosis than patients who do not harbor the mutation.
In patients with idiopathic pulmonary hypertension, a significant decrease in carbon monoxide diffusion (<45% of predicted value) on pulmonary function tests is often indicative of a significant decrease in cardiac output and a poor prognosis; lower partial pressure values of carbon dioxide are indicative of more severe hyperventilation and a poorer prognosis.
Patients with a positive acute vasovagal response test have a better prognosis than those with a negative test.
Hazards
In children, pulmonary hypertension can limit mobility, affect growth and development, and be life-threatening in severe cases.
Pulmonary hypertension can also cause related complications, such as thrombosis, arrhythmia, and pulmonary hemorrhage.
Poorly controlled pulmonary hypertension can lead to right heart failure, which is ultimately life-threatening.
Suffering from pulmonary hypertension during pregnancy can be life-threatening to both the mother and the fetus.
Daily
Daily Management
Control salt intake
Control the amount of salt when cooking and avoid eating pickled foods such as olive greens, sauerkraut, pickled cucumbers, cured fish and meat.
Condiments, such as ketchup and soy sauce, should be avoided in excess.
Don’t eat salted snack foods such as potato chips, tortilla chips, pretzels, crackers and salted nuts; some instant breakfast drinks and canned foods should also be avoided.
Use lemon juice, small amounts of cayenne pepper and garlic for flavoring.
Avoid excessive intake of animal fats. Choose fresh vegetables, fruits, and high-fiber foods such as bananas, oranges, broccoli, whole grains, and bran.
Monitor fluid intake
Keep a daily record of the amount of fluids consumed, such as soups, juices, etc., for several weeks after diagnosis.
Weight gain is usually one of the earliest signs of increased fluids. Weigh yourself daily. If your weight increases by 1.5 to 2 kilograms a day, or if the weight gain persists for more than two days, you need to take it seriously and seek medical attention if necessary.
Others
Quit smoking and avoid second-hand smoke.
Avoid sitting in hot tubs or saunas, as well as avoiding high-altitude travel.
Many patients find that bending over often aggravates their symptoms. Products you commonly use can be placed in the kitchen, bathroom, bedroom and laundry room and kept at eye level to avoid breathing difficulties while performing the action.
Pregnancy puts stress on the body and can be life-threatening for people with pulmonary hypertension. Therefore, before becoming pregnant, you should consult with your doctor in detail about the risks involved.
People with pulmonary hypertension may feel anxious and/or depressed. If you have problems dealing with your situation, talk to other patients, family members, friends and consult your doctor if necessary.
Follow-up and review
Patients with pulmonary hypertension should be followed up regularly so that the doctor can understand the progress of the disease and make timely adjustments to the treatment plan.
Stable patients should be reviewed every 3 to 6 months, and unstable patients or patients on combination therapy should be reviewed every 1 to 3 months.
Echocardiography may be needed for review.
Prevention
Avoid weight loss medications that can cause pulmonary hypertension.
Avoid exposure to drugs and toxic substances (toxic canola oil).
Those with congenital heart disease should have early intervention.
Stop smoking and limit alcohol consumption.
Avoid schistosomiasis or hepatitis virus infections.
Pay attention to safe sexual behavior.
Those with chronic cardiopulmonary disease, connective tissue disease, chronic liver and kidney disease should undergo relevant screening regularly.
Exercise appropriately to enhance physical fitness and get vaccinated against influenza or pneumonia if necessary.