Pulmonary Hypertension – Symptoms and Treatment

Pulmonary Hypertension, as the name implies, is a condition marked by high blood pressure in the arteries of the lungs, and also the right side of the heart. Since the functioning of lungs and heart are closely related, Pulmonary Hypertension (PH) has long-term implications on cardiovascular health as well. It can cause a range of serious complications if not treated in time. While some types of PH do not have a cure, the symptoms can be managed so that the patient leads a normal life.

What happens in Pulmonary Hypertension

The Pulmonary Artery supplies blood to the lungs for its own working as well as for purification of impure blood collected from rest of the body. The Pulmonary artery in turn branches into various capillaries inside the lungs. The Pulmonary Artery (PA) has a central tube called lumen, through which the blood flows, surrounded by layers of muscles that expand and contract in order to push the blood.

In some people, the cells of the lining of the arteries become thick, swollen and stiff. The thickened cells gradually constrict the artery. This increases the pressure in the PA. Since not enough blood is reaching the lungs, the heart pumps harder to make up for this. This again increases the pressure in the PA. The constant strain on the heart to pump harder weakens the heart muscles. If left untreated, this can cause a vicious cycle of increased blood pressure and weakened heart muscles. This is the most common type of PH called as Pulmonary Arterial Hypertension or PAH.

Types of PH and their causes

Pulmonary Arterial Hypertension

Most often, the cause for PAH described above, is unknown (called idiopathic PAH). In many cases, its inherited. Heart conditions present since birth (congenital heart disease) can also trigger PAH later on. Connective tissue disorders such as lupus and scleroderma, viral infections such as HIV and chronic liver disease (cirrhosis) are risk factors for PAH. Prescription diet drugs and illicit drugs such as methamphetamines increase the risk for PAH.

PH caused by left-side heart diseases

Diseases of the mitral valve and aortic valve can trigger PAH. Failure of the left ventricle to pump blood can lead to heart failure or PAH.

PH caused by lung disease

There are smaller arteries and capillaries inside the lungs and these can develop a condition similar to PAH. Causes for this can be lung conditions such as Chronic Obstructive Pulmonary Disease (COPD), sleep apnea and Pulmonary fibrosis where there is scarring in the interstitial cells of the lungs (air sacs). People who live in high-altitude destinations like hill-stations that have lower oxygen levels are at the risk of developing PH.

PH caused by blood clots

Blood clots formed in the lungs (pulmonary embolism) or clots that form in other parts of the body and carried by blood to the lungs, can trigger a phase of PH, till the clots are removed.

PH caused by other health conditions

Chronic Kidney disease can lead to build-up of fluids and toxins in the blood eventually triggering PH. Tumors in the heart or lung that press on the PA create a situation similar to PAH. Sarcoidosis is a condition in which inflammatory cells accumulate in the eyes, skin, lymph nodes and lungs, increasing the risk of PH. Vasculitis is an inflammation of blood vessels which causes them to thicken and when they occur in the arteries of the lungs, it causes PH. Polycythemia Vera is a type of blood cancer that caused blood clots which heighten the risk of PH. Essential thrombocytosis (ET), or primary thrombocythemia is a condition where the body produces too many platelets which increases the risk of blood clots and hence PH. Glycogen storage disease, a rare condition in which the way the body uses and stores glycogen, a form of sugar, gets altered, increasing the risk of PH. Eisenmenger syndrome is a congenital condition caused by a heart condition called as ventricular septal defect. One of the consequences is PH.

Other Risk Factors for PH

• Blood-clotting disorders
• A family history of blood clots in the lungs
• Being overweight
• Exposure to asbestos and fine particles (as in factories, quarries, chemical factories, etc)
• Use of certain weight-loss drugs
• Use of psychedelic drugs such as cocaine
• Selective serotonin reuptake inhibitors (SSRIs) that is used to treat depression and anxiety

Symptoms

• Shortness of breath (dyspnea) that gets worse progressively with time, starting initially while exercising and eventually even while at rest
• Fatigue or tiredness all through the day
• Syncope – Dizziness and fainting
• Angina – A pain or pressure in the chest
• Edema – swelling in the abdomen, ankles and legs
• Cyanosis – A bluish color in the lips and skin
• Racing pulse or heart palpitations

Diagnosis

A wide array of tests is conducted to check for heart, lung and other complications which may be causing PH.

• Genetic test: First and foremost, this is done, to see if the condition is inherited but dormant in the parents and close relatives. This will decide the course of the treatment.
• Blood tests: To check for infections and other health conditions that is causing PH
• Chest X-ray – This looks for enlargement of the right ventricle and pulmonary arteries, a sure sign of PH
• Electrocardiogram – This is done to look for strain or enlargement in the right ventricle and measure the pressure there
• Echocardiogram: This is done to measure the size and thickness of the right ventricle and the pressure in the Pulmonary arteries, both of which are indication of how well the heart valves are working
• Right heart catheterization: This is done once the echocardiogram confirms PH
• CT scan: This looks for blood clots and other abnormalities in the lungs
• MRI scan: This is used to check for blood flow in the right ventricle and arteries of the lungs
• Lung function test: This checks how much air is taken in and how much is let out
• Polysomnogram: This is used to check for the presence of sleep apnea which can cause PH
• Ventilation/Perfusion: This looks for blood clots which could be causing the PH
• Open lung biopsy: If lung cancer is suspected

Treatment

Medication

• Vasodilators: These relax and open out the blood vessels, thereby improving blood flow
• Guanylate cyclase stimulators (GSC): These increase nitric oxide in the body which relaxes the pulmonary arteries and reduces blood pressure in them
• Endothelin receptor antagonists: This reverses the effects of endothelin, a substance that narrows down the arteries
• ED medicines: Drugs used to treat erectile dysfunction are also used to open out blood vessels in the lungs
• Calcium channels blockers (high dosage): These relax the muscles in the walls of blood vessels
• Anti-coagulants: These prevent blood clots from forming in the arteries of the lungs
• Digoxin: This helps the heart beat stronger and pump more blood
• Diuretics: This removes the excess fluid in the body and ease the heart function
• Oxygen therapy: For PH patients living in high-altitude locations

Surgery

• Atrial septostomy: An open-heart surgery where an opening is created between the upper chambers of the heart to relieve the blood pressure
• Lung transplantation: For younger patients suffering from idiopathic PAH, lung transplantation may be the only option

Outlook

If you or any of your dear ones are showing symptoms of PH, do not panic. Rush to a reputed hospital. A pulmonologist or cardiologist will conduct the above tests to confirm or rule out PH and determine severity. Specialists will then choose the right course of treatment for quick recovery and rehabilitation.

 

Kauvery Hospital is globally known for its multidisciplinary services at all its Centers of Excellence, and for its comprehensive, Avant-Grade technology, especially in diagnostics and remedial care in heart diseases, transplantation, vascular and neurosciences medicine. Located in the heart of Trichy (Tennur, Royal Road and Alexandria Road (Cantonment), Chennai, Hosur, Salem, Tirunelveli and Bengaluru, the hospital also renders adult and pediatric trauma care.

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