A. How pulmonary hypertension is diagnosed Your child’s doctor will perform a physical examination, listen to the heart and lungs and make other observations. Some of the following tests can help confirm the diagnosis: Blood tests – help distinguish familial pulmonary hypertension from other causes of pulmonary hypertension Radiological tests (such as CT) – help determine the extent and possible causes of the disease Chest X-ray – a diagnostic method that uses hidden rays to show the inner tissues, bones and organs on film. Electrocardiogram – records the electrical activity of the heart and can detect abnormal heart rhythms and cardiac hypertrophy Echocardiogram – uses sound waves to determine the structure and function of the heart, and can show the activity of the heart and valves, with the possibility of measuring the pressure inside the heart Pulmonary function tests – A series of tests to measure your child’s breathing and lung capacity, as well as to scan for blood flow and airflow through the lungs by inhalation or injection of a radioactive substance. Cardiac catheterization – enables a more detailed examination of the structures within the heart. A small, bendable tube is inserted from a blood vessel at the base of the thigh to the heart in a quiet state to measure blood pressure and oxygen saturation in the heart, pulmonary arteries and aorta. The injection of contrast media shows the structures of the heart and lungs more clearly. The injection of drugs can further clarify the cause and select a treatment. Second, how to treat pulmonary hypertension pulmonary hypertension is a serious life-threatening condition, although there is no cure, there are still several treatment options available. For primary pulmonary hypertension (PPH), treatment includes: 1. inhaled oxygen helps to increase oxygen levels in the blood; 2. nitric oxide (NO) helps to reduce pulmonary vascular resistance and improve cardiac function; 3. calcium channel blockers relieve pulmonary artery constriction and promote the heart’s pumping ability; 4. intravenous prostacyclin helps to relax constricted pulmonary blood vessels and reduce pulmonary hypertension; 5. endothelin antagonists , prostacyclin analogs and phosphodiesterase inhibitors reduce pulmonary hypertension; 6. Anticoagulants prevent pulmonary thrombosis; 7. Diuretics help drain the kidneys; 8. Digoxin helps maintain the heart’s pumping capacity; 9. Lung transplantation may be considered for patients who do not respond to medications —- Single, double or heart-lung transplantation. For secondary pulmonary hypertension, treatment of the primary cause is most effective. All the therapies listed above are available and in combination with treatment of the relevant etiology may help to relieve pulmonary hypertension.