Pulmonary Hypertension

By Dr. Munesh Tomar in Cardiology

May 19 , 2023 | 3 min read

What is Pulmonary Hypertension?

Pulmonary hypertension (PH) means high blood pressure in blood vessels (pulmonary arteries) supplying the lungs leading to pressure load on the right side of the heart and, ultimately, heart failure.

Depending on the cause, there are five different groups of pulmonary hypertension. It is important to classify as treatment might differ.

Classification of Pulmonary Hypertension

  • Group I: Pulmonary arterial hypertension (PAH): This group includes PH that is caused by abnormalities in the pulmonary arterial system, such as narrowing, thickening, or increased stiffness of the pulmonary arteries. It can be idiopathic, hereditary, drug-induced or secondary to congenital heart disease (Eisenmenger syndrome), connective tissue disorders, and some infections. Persistent pulmonary hypertension of newborns (PPHN) is also categorised under group I.

  • Group II: PH due to left heart disease: This group includes PH that is caused by left-sided heart diseases such as heart failure, valvular heart disease, or congenital heart defects.

  • Group III: PH due to hypoxia and/or lung disease: It includes PH that is caused by diseases such as sleep apnea, chronic obstructive pulmonary disease (COPD), etc.

  • Group IV: Chronic thromboembolic pulmonary hypertension (CTEPH): This group includes PH, which is caused by blood clots in the lungs, which can lead to persistent obstruction of the pulmonary arteries.

  • Group V: PH with unclear: This group includes PH with no clear underlying cause or PH that is caused by a combination of factors.


What are Signs and Symptoms of Pulmonary Hypertension?

In the early stage, the patient may be asymptomatic or have symptoms related to the primary disease. However, with disease progression, the pressure in the lungs increases and puts a pressure load on the right heart. Complaints generally progress over a period of time, and generally, most patients are diagnosed in 2 to 3 years. Pulmonary hypertension signs and symptoms include:

  • New or increased shortness of breath 

  • Exercise intolerance 

  • Dizziness/syncope, especially after any physical exertion 

  • Chest pain 

  • Palpitations 

  • Swelling of your feet, legs, or abdomen

  • Bluishness of lips and/or fingers 

  • Cold extremities  

What are Causes of Pulmonary Hypertension?

Common causes of pulmonary hypertension(PH) are-

  • Congenital heart defects (Untreated hole in the heart: Eisenmenger syndrome)

  • Connective tissue disorders (Systemic sclerosis, myositis, SLE etc.)

  • Lung diseases (chronic obstructive pulmonary disease (COPD), Interstitial lung disease etc.)

  • A clot in the lung arteries

  • Left heart disease exerts back pressure on the lung arteries

  • Liver disease (cirrhosis)

  • Use of certain drugs, including some weight-loss medications and illegal drugs such as cocaine or methamphetamine

  • Infection: chronic lung infection, HIV, Schistosomiasis

  • Genetics (hereditary) pulmonary hypertension

  • Idiopathic pulmonary hypertension: When we could not find any other cause

What are the Risk Factors of Pulmonary Hypertension?

  • Pulmonary hypertension can affect any age group, including children, with increasing incidence with age

  • It is more common among women (around two times higher incidence in females)

  • A family history of pulmonary hypertension

Can Pulmonary hypertension be prevented?

  • Pulmonary hypertension is secondary to congenital cardiac disease, and valvular heart disease can be prevented by timely correction of the defect and timely intervention for valvular lesions. If there is a need for cardiac medication, that, too, needs to be adjusted as per the patient's cardiac status. 

  • Systemic diseases causing PH should be evaluated at various intervals, optimal treatment of primary disease to prevent the development of complications like PH, and if PH develops, PH medications should be used as per the specialist.

  • Drugs causing PH should be avoided. 

  • Healthy lifestyle changes, any infection like lung infections or HIV, should be aggressively treated.

How is Pulmonary Hypertension Treated?

All efforts should be made to look for treatable causes of pulmonary hypertension, as listed above. There are many drugs targeted for PH treatment:

  • Phosphodiesterase type 5 (PDE5) inhibitor: Sildenafil, Tadalafil

  • Endothelin receptor antagonists: Bosentan, Ambrisentan, Macitentan 

  • Prostacyclin receptor agonist, Selexipeg 

  • Soluble guanylate cyclase (sGC) stimulator -Riociguat: Mainly for patients with chronic thromboembolic PH 

  • Calcium channel blocker

  • Diuretics in patients with swelling 

  • Oxygen therapy

  • In the advanced stage of the disease:

    • Pott's shunt - Pott's shunt, also known as Pott's anastomosis or Pott's operation, is a surgical procedure used to treat certain types of pulmonary hypertension (PH). The procedure involves creating a direct connection between the pulmonary artery and the aorta, thus bypassing the pulmonary capillaries. It helps in reducing the load on the right heart and improving cardiac output.

    • Lung transplant - It is a surgical procedure in which a diseased or damaged lung is replaced with a healthy lung from a deceased donor or, less commonly, from a living donor.

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