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Primary Pulmonary Hypertension -Procedures, Side effects, Recovery time, Costs and alternatives

Last Updated: Oct 30, 2024

What is Primary Pulmonary Hypertension?

Primary pulmonary hypertension, also known as pulmonary arterial hypertension, is an uncommon type of high blood pressure. The effects can be seen in the pulmonary arteries and the capillaries. The functions of these blood vessels are to carry blood to lungs from the right ventricle of the heart. The pressure in the pulmonary arteries and other small blood vessels increase the heart has to work at a much faster rate.

This leads to the weakening of muscles in the heart. The ultimate result of this is heart attacks that lead to death. With primarypulmonary hypertension, the rise in blood pressure is caused by changes in the cells that line your pulmonary arteries. These changes can cause the walls of the arteries to become stiff and thick, and extra tissue may form. The blood vessels may also become inflamed and tight. To diagnose your condition, your doctor may review your medical and family history, discuss your signs and symptoms, and conduct a physical examination. Doctors may order several tests to diagnose pulmonary hypertension, determine the severity of your condition and find out the cause of your condition. Based on the condition the doctor may treat the condition with medicines or there may be a need of surgery. The early stages of this disease do not show any harmful or noticeable symptoms. But as the symptoms grow stronger, the condition of the patient becomes worse. Some common symptoms of this disease are:

  • Chest pressure
  • Increase in pulse rate
  • Pain in chest
  • Fainting and dizziness
  • Breathing difficulties
  • Heart palpitations

How is the treatment done?

To diagnose your condition, your doctor may review your medical and family history, discuss your signs and symptoms, and conduct a physical examination. Doctors may order several tests to diagnose pulmonary hypertension, determine the severity of your condition and find out the cause of your condition. There are various techniques to diagnose this medical state. Primary pulmonary hypertension requires one or more tests to get diagnosed. Some tests for the diagnosis of the same are mentioned below:

  • Electrocardiogram: This is used to check the signs for strain and abnormal heart rhythms.
  • Echocardiogram: This technique is used in order to check the structure and function of the heart. The pressure at the pulmonary artery is also measured.
  • Chest X-ray: The enlargement of pulmonary arteries and the lower right chamber of the heart is diagnosed.
  • CT Scan or MRI Scan: Narrowing or damage of pulmonary arteries and blood clots are checked using CT Scan.
  • Right heart catheterization: The blood pressure in the pulmonary arteries and the right ventricles is measured.

Even the advancement in science and medicine has no treatment for this disease. The treatments options available can reduce give a relive from the symptoms, but a complete cure is not possible in this case. Basically, you can minimize the chances of complications and earn some extra life. There is no permanent cure for this problem, but for reducing the symptoms the doctors prescribe few medicines which may help the patient to overcome this condition. Few medications which the doctors prescribe are given below:

  1. Medication
    • Prostacyclin therapy: This is for the dilation of blood vessels.
    • Soluble guanylate cyclase: These are stimulators, which are also used for dilating the blood vessels.
    • Endothelin receptor antagonists: The major activity of these is to block all the functions of endothelin. This substance causes narrowing of the blood vessel.
    • Anticoagulants: Anticoagulants are preferably used for preventing the formation of blood clots.
  2. Apart from medications, there are many other methods as well.
  3. Surgery
    • Surgery for primary pulmonary hypertension is known as Atrial Septostomy. Under severe conditions, the doctors will prefer surgery for the patient. During the surgery, the pressure from the right side of the heart is removed, and a lung or heart transplantation is required in order to replace the damaged organ. If the lung is affected more due to the primary pulmonary hypertension then the doctor will recommend a lung transplant. Whereas in case of a heart having serious issues a heart transplant will be recommended to the patient.
    • Changes in lifestyle Having a proper diet, exercising daily and other daily routine habits can make a great difference in the treatment of this disease. Adopting healthy habits will contribute to the reduction of complications.

Who is eligible for the treatment? (When is the treatment done?)

The person who is diagnosed with the symptoms of primary pulmonary hypertension is eligible for the treatment. On the other hand in case of surgery sometimes the patient is not eligible for treatment because of some other health reasons like problem with anesthesia etc.

Who is not eligible for the treatment?

The person who is healthy and do not have any symptoms of primary pulmonary hypertension is not eligible for the treatment.

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Are there any side-effects of primary pulmonary hypertension treatment ?

There are various stages of this disease. Basically, the symptoms have been divided into four categories. Based on the categories the side-effects can be seen.
  1. Class 1 - There are no visible side-effects in this stage. The condition does not affect patient’s physical growth in any way. No side-effects seen during regular work or rest.
  2. Class 2 - From this stage, the side-effects are visible in a manner. The effect can be seen on the physical activities of the patient.
  3. Class 3 - A stage up and the side-effects also gains a height. This stage completely limits the physical activities of the patient. The exertion can be felt during work time, but no clue during rest periods.
  4. Class 4 - Restricts all the physical activities of the patient. Symptoms become quite noticeable and can be seen during the rest periods as well.

Some medicines like Treprostinil (Tyvaso, Remodulin, Orenitram) and Sildenafil and tadalafilcauses headache, diarrhea andnausea and in case of pregnancy the Soluble guanylate cyclase (SGC) stimulator medications should not be taken because it causes dizziness in the patient.Warfarin also has same side effects. And surgeries like Atrial septostomy can have serious complications, including heart rhythm abnormalities (arrhythmias). And also in transplantation include rejection of the transplanted organ and serious infection. In case any of these side effects persists or there any other health issues arising during or after treatment, it is advised to kindly consult the health specialist immediately.

What are the Post-treatment guideline

There is no permanent treatment or cure for this disease. The only way is to suppress the symptoms. But these symptoms can come back if proper medications are not taken by the patient. So as a post-treatment guideline, it is necessary for the patient to take their medicines on time. Maintaining a healthy lifestyle is also important for suchpatients. Regular exercises, healthy eating and sleeping habits can help in improving the condition of the patient.

How long doesit take to recover?

Primary pulmonary hypertension is a disease with no cure there are ways to minimize the symptoms of the disease. According to studies, it has been noted that the patients who are untreated and suffering from the disease have a probability of 68 percent of survival at the initial stage. As the disease advances the survival rate drops up to 48 percent in three years and then to 34 percent as the time passes. But, if the patient has undergone the treatment, then their life expectancy increases by seven years at most. Hence to live a much longer life, it is important for the patients to undergo the treatment. The recovery time for the patient varies from patient to patient based on his or her health condition.Recovery period from this treatment is near about one month to three months. And in surgical cases the recovery period is about two months to six months.

What is the treatment price in India?

As we discussed earlier in this article, the disease is incurable. Hence with the passage of time, the disease also keeps progressing. So the continuous medication and treatment is a must for this disease. Hence the price cannot be fixed for the treatment. The price of the treatment in India is different from person to person and place to place because some cases are serious, so the cost of the treatment is high. And in the surgical cases the cost are very high. The cost of medicines is started from rupees 1000 to rupees 45000 and in case of surgery the cost of the treatment is INR 1 lakh to INR 7 lakh or above. Depends upon which hospital do you choose for the treatment.

Are the results from the treatment permanent?

The results of the primary pulmonary hypertension ‎ is totally dependent upon the patient condition because if the patient follow the proper guidelines after the treatment like diet, lifestyle changes, exercise and medication on time then the result of this treatment is permanent while on the other hand if a person cannot follow the guideline of doctor properly then the results are not permanent and it may occur any time.

What are the alternatives to the treatment?

There are no alternatives for the treatment of this disease as the main cure does not exist. But proper care can lead to a reduction of the symptoms. Yoga and Meditation might give a hand.

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Written ByDr. Ashok Mishra MBBSGeneral Physician
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