I. Pre-treatment preparation for patients with pulmonary hypertension
1.Medical history taking
(1) Present medical history; the following points need to be clarified: onset of disease, pulmonary hypertension diagnosis and treatment, cardiopulmonary function (feeding difficulties, activity and breathing difficulties), cyanosis, complications (chest pain, syncope, hemoptysis and lung infection).
(2) Past history.
Drug history: history of weight loss medications taken or not. Appetite suppressants such as fenfluramine, aminobenzoline and phentermine; central excitatory drugs such as amphetamines, methamphetamines; contraceptives such as estrogen, thus excluding drug-induced pulmonary hypertension.
History of infectious and contagious diseases: exclude schistosomiasis and AIDS as causes.
History of other diseases: history of hepatitis, unexplained hematocrit and hemolytic anemia; unexplained neurological long-term thyroid disease, connective tissue disease and heart (cardiac systolic and diastolic function and valvular disease) and lung (slow-onset lung, interstitial lung, apnea syndrome, etc.) disease history. Myelodysplastic syndrome, vasculitis, etc.
(3) Family history to exclude hereditary factors causing (bone forming protein type II receptor gene variant related). Ask for a history of similar disease episodes with at least two people in the family affected. In male patients one should ask about any history of habitual abortion in their mother or sisters. Note any history of familial venous thromboembolism.
(4) Personal history: history of drug use, unclean sexual intercourse and homosexuality. Any history of toxic oil, contaminated canola oil, long-term exposure to printing oil and other volatile industrial oils, etc.
(5) Marital history: history of habitual abortion, because habitual abortion is an important clinical feature of antiphospholipid antibody syndrome, which causes embolic pulmonary hypertension.
(6) Surgical history: any history of pulmonary artery annuloplasty, septal stoma and combined cardiopulmonary transplantation, etc.
2. Physical examination
Special facial features, cyanosis, oxygen saturation (especially the lower extremities should not be neglected), murmurs, right heart function conditions such as edema of the extremities, effusion, venous anger, etc.
3.Auxiliary examination
(1) Cardiac ultrasound: To confirm the diagnosis of pulmonary hypertension associated with precordial disease and to exclude pulmonary hypertension caused by those with mitral stenosis. The degree of right heart dilatation, main pulmonary artery diameter, ventricular septal motion, and changes in ejection fraction of the right and left ventricles are observed to assess the condition and prognosis.
(2) Electrocardiogram: To clarify whether there is right ventricular hypertrophy, etc.
(3) Chest radiograph.
(4) Laboratory tests.
Autoantibodies: including anti-SSA, anti-SSB, anti-Sm, anti-Scl-70, anti-RNP, anti-Jo-1, anti-adhesion point antibodies, antiphospholipid antibodies and ANCA, etc. Exclusion of rheumatic diseases causing pulmonary hypertension.
Liver function and hepatitis markers to rule out hepatitis causing it.
HIV antibodies: exclude HIV infection leading to.
Thyroid disease: autoimmune thyroiditis can cause pulmonary hypertension must be ruled out.
Blood gas analysis: Patients with pulmonary hypertension have hypoxia and hypocapnia.
Prothrombin time and activity: Patients with pulmonary hypertension have a hypercoagulable state of blood.
Blood routine: clarify the presence or absence of anemia, and differentiate from chronic hemolytic anemia and myelodysplastic syndrome if necessary.
4. Right heart catheterization and acute pulmonary vasodilatation test.
Diagnostic gold indicator. Clarify pulmonary artery systolic pressure, diastolic pressure, mean pressure, small pulmonary artery wedge pressure, pulmonary vascular resistance, cardiac output. Definition of pulmonary vasodilation test: mean pulmonary artery pressure less than 40 mmHg after inhalation or injection of specific acute vasodilating drugs (adenosine, iloprost) in children with a mean pulmonary artery pressure decrease greater than 10%, cardiac output in the normal range, and pulmonary vascular resistance less than 6 wood./u.m^2.
5. 6-minute walk test: to assess exercise tolerance.
II. Diagnosis and treatment process.
After the patient is admitted to the hospital
1, clear diagnosis: take medical history, cardiac ultrasound, electrocardiogram and chest X-ray.
2, clarify the cause of pulmonary hypertension: familial, drug, left-to-right shunt congenital heart disease-related pulmonary hypertension, liver, thyroid, connective tissue disease, rheumatic diseases, chronic hemolytic anemia, long-term hypoxia or respiratory diseases related to pulmonary hypertension and chronic thromboembolic pulmonary hypertension, other tumors, metabolic diseases and other factors leading to pulmonary hypertension.
3, the tests: blood routine, liver and kidney function, BNP, coagulation routine, electrolytes, if necessary, sent to PICU for blood gas analysis. Patients without preexisting heart disease should have a full set of hepatitis, HIV screening, rheumatology, plasma D-dimer, protein C activity, antithrombin III activity measurement, and pulmonary vascular enhancement CT scan. Patients with anemia should have a complete set of erythrocyte morphology and anemia lysis.
4.After admission, perform 6-minute walk test as appropriate.
5.Right heart catheterization and pulmonary arteriography are required to clarify the diagnosis of pulmonary hypertension.
6.Treatment
(1) Basic treatment: Remove the causative factors. Rest, control intake and output, maintain electrolyte acid-base balance.
(2) Positive inotropic drugs: digoxin, small doses of dobutamine, milestone
(3) Diuretic as appropriate
(4) Pulmonary vasodilators and targeted drugs: NO inhalation, vantave inhalation, prostaglandin I2 class drug bosentan orally, andrisentan orally, sildenafil, vardenafil, tadalafil orally, etc.
(5) Anticoagulation: Warfarin to maintain INR between 1.5 and 2.0.
(6) Mechanical support: IABP, mechanical ventilation
(7) Surgical and interventional procedures: atrial septal stoma, lung transplantation, etc.