Pulmonary Artery Hypertension (PAH): Early recognition is key to management.
Pulmonary Hypertension (PAH) is a disease often under-recognised and dreaded both by doctors and patients. PAH was associated with a life expectancy of 2-2.5 years just 2 decades ago. The recent advances in treating this disease has brought up the life expectancy but the problem still remains as the disease is unrecognised and treatment is sought after very late in the disease.
An accurate and timely diagnosis of PAH and identification of the specific underlying cause of PAH is essential to ensure that patients can be treated as early as possible with interventions that are appropriate for their diagnosis.
Many effective PH-targeted therapies are available and when used in patients with appropriate types of PH, these therapies can lead to significant improvements in functional capacity, quality of life and outcome
The incidence of PAH ranges from 2-4/million population but can be very high in patients with Cardiac illness, Chronic Kidney Disease and illnesses of the Lung. Patients developing fibrosis of lung post COVID-19 are at high risk of developing PAH.
On the occasion of world PAH day let us try and understand the disease.
What is pulmonary hypertension?
Pulmonary hypertension happens when the pressure in the blood vessels leading from the heart to the lungs is too high.
With pulmonary hypertension, the blood vessels to the lungs develop an increased amount of muscle in the wall of the blood vessels. The heart pumps blood from the right ventricle to the lungs to get oxygen. Because the blood does not have to travel very far, the pressure in this side of the heart and in the artery taking blood from the right ventricle to the lungs is normally low—usually much lower than systolic or diastolic blood pressure.
When the pressure in this artery gets too high, the arteries in the lungs can narrow and then the blood does not flow as well as it should, resulting in less oxygen in the blood.
What causes pulmonary hypertension?
The most common causes include Heart Diseases, Valvular Heart Disease, Failing Left Side Of Heart, Chronic Lung Illness, Connective Tissue Diseases, liver disease. Sometimes no cause for Pulmonary Hypertension can be found out. In few cases Genetics also play a role.
What are the risk factors for pulmonary hypertension?
Pulmonary hypertension happens at all ages, including children, and its incidence increases with age. Pulmonary hypertension is more common among women, elderly aged 75 or above, patients with lung and cardiac illness.
Shortness of breath, initially while exercising and eventually while at rest
Dizziness or fainting spells (syncope)
Chest pressure or pain
Swelling (edema) in ankles, legs and abdomen (ascites)
Bluish coloration of lips and skin (cyanosis)
Racing pulse or heart palpitations
The signs and symptoms of pulmonary hypertension develop slowly. One may not notice them for months or even years. Symptoms get worse as the disease progresses.
How is pulmonary hypertension treated?
There is no cure for pulmonary hypertension very late in the course of disease. However, there are many different types of treatments which can control the disease to large extent especially when initiated early on.
Oral medicines- calcium channel blockers, sildenafil
Oxygen therapy, Inhaled medications
Targeted therapy – Bosentan, Ambrisentan
How can I prevent pulmonary hypertension?
While not all pulmonary hypertension can be prevented, one can take steps to prevent it by making healthy lifestyle changes and managing high blood pressure, coronary heart disease, chronic liver disease, and abstaining from tobacco use.
What tests are used for detection of PAH?
The tests may vary based on the cause of PAH in a given patient. A simple 2D echocardiography which is an ultrasound of the heart can confirm its presence and should be done very early whenever PAH is suspected.