Pulmonary Hypertension Clinic

The symptoms of pulmonary hypertension develop gradually and the patient may not even notice them for years. As the disease progresses, the symptoms of this disease tend to worsen.

The signs and symptoms of pulmonary hypertension include:

• Chest pain or pressure
• Blue skin and lips
• Dizziness or fainting spells
• Fatigue
• Pounding heartbeat or fast pulse
• Swelling in the legs, ankles, and eventually the abdomen
• Shortness of breath, initially while performing any kind of exercise and eventually even at rest

A normal heart contains two lower chambers called ventricles and two upper chambers called atria. Every time blood pumps across the heart, it is pumped to the lungs by the lower right chambers through a large artery called the pulmonary artery.

The blood picks up oxygen and releases carbon dioxide in the lungs. Usually, the blood flows through the blood vessels of the lungs to the left side of the heart very easily. However, alterations in the cells lining the pulmonary arteries can cause stiffening, swelling, and thickening of the arterial walls. Due to these changes, blood flow through the lungs may be blocked or slow down, resulting in pulmonary hypertension.

Depending on the cause, pulmonary hypertension is divided into five groups:

Group 1: Pulmonary arterial hypertension (PAH)

Following are some the common causes:

• Idiopathic pulmonary arterial hypertension
• Use of illegal substances such as certain drugs
• Heritable pulmonary arterial hypertension
• Congenital heart diseases
• Other conditions like chronic liver disease, HIV infections, and connective tissue disorders

Group 2: Pulmonary hypertension caused by left-sided heart disease

Following are some of the common causes:

• Failure of left ventricle— the left lower chamber of the heart
• Left-sided heart valve disease like aortic valve disease or mitral valve disease

Group 3: Pulmonary hypertension caused by lung disease

Following are some of the common causes:

• Scarring of the tissues that lie between the air sacs of the lungs (pulmonary fibrosis)
• Chronic obstructive pulmonary disease (COPD)
• Long-term high altitude exposure in people at a higher risk of pulmonary hypertension
• Obstructive sleep apnea

Group 4: Pulmonary hypertension caused by chronic blood clots

Following are some of the common causes:

• Pulmonary emboli or blood clots in the lungs
• Other clotting disorders

Group 5: Pulmonary hypertension triggered by other health conditions

Following are some of the common causes:

• Inflammatory disorders like vasculitis and sarcoidosis
• Blood disorders including essential thrombocytopenia and polycythemia vera
• Metabolic disorders like glycogen storage disease
• Tumours pressing against pulmonary arteries
• Kidney disease

Following are some of the potential complications of pulmonary hypertension:

• Cor pulmonale
  In this condition, the right lower chamber of the heart called the right ventricle becomes enlarged. It needs to put in more effort than usual to pump blood through the narrowed and blocked pulmonary arteries. Therefore, the walls of the heart thicken eventually and the right ventricle expands to accommodate more blood. These changes however create more pressure on the heart which results in right ventricular failure.
• Blood clots
  The risk of developing blood clots in the small pulmonary arteries increases if you have pulmonary hypertension.
• Arrhythmias or irregular heartbeats
  Pulmonary hypertension can cause certain life-threatening arrhythmias.
• Lung bleeding
  Life-threatening bleeding inside the lungs can also occur as a result of pulmonary hypertension which can present in the form of coughing up of blood or haemoptysis.
• Pregnancy complications
  Pulmonary hypertension can present with life-threatening complications for a developing baby.

Early diagnosis of pulmonary hypertension is quite hard and it is often diagnosed coincidentally or during a routine medical examination. Even when the condition is advanced, its symptoms mimic those of other related diseases such as heart and lung conditions.

The doctor will review your symptoms and perform a complete physical examination to diagnose pulmonary hypertension. He will also ask you a number of questions regarding your personal and family history.

Following are some of the blood and imaging tests done to diagnose pulmonary hypertension:

• Blood tests
  Blood tests can help detect signs of complications of pulmonary hypertension or determine its cause.
• Chest X-ray
  Images of the chest, lungs, and heart are produced in a chest X-ray, which can help detect conditions causing pulmonary hypertension.
• Electrocardiogram (ECG)
  This test records the heart’s electrical activity and detects any changes in the heartbeat. ECG patterns may reveal signs of strains on the heart or that of right ventricular enlargement.
• Echocardiogram
  Images of the beating heart are created by sound waves by an echocardiogram, which reveals the blood flow through the heart. This test is performed to help determine how well the treatments for pulmonary hypertension are working or how the condition is diagnosed.
  
  Sometimes, an echocardiogram is performed when the patient is working out on a treatment or a stationary bike to determine how activities affect the heart. During this test, you may also be directed to wear a mask that determines how well the lungs and the heart utilise oxygen and carbon dioxide.
• Right heart catheterization
  If you are found to have pulmonary hypertension on an echocardiogram, the diagnosis is usually confirmed by right heart catheterisation. This procedure involves placement of a catheter into a blood vessel of the groin which is then gently guided into the right ventricle— the lower right chamber of the heart and the pulmonary artery. The blood pressure in the right ventricle and main pulmonary arteries is then measured by a cardiologist.

Some other tests may also be recommended to determine the working condition of the lungs and their arteries so that further causes of pulmonary hypertension can be determined. Those tests may include:

• Computerised tomography (CT)
  This test involves taking a series of X-ray images to produce cross-sectional images of the blood vessels, bones, and the soft tissues. A contrast dye may be introduced into a vein during the process so that it is seen more distinctly on the images. A CT scan of the heart can reveal its size apart from any obstructions in the pulmonary arteries. It can also help diagnose pulmonary conditions that might contribute towards pulmonary hypertension such as pulmonary fibrosis and COPD.
• Magnetic resonance imaging (MRI)
  During this test, radio waves and magnetic fields are used to produce detailed images of the heart. It can reveal the flow of blood in the pulmonary arteries and determine the working condition of the right ventricle— lower right chamber of the heart.
• Lung (pulmonary) function tests
  These consist of a series of non-invasive tests that help decrease how much air the lungs can accommodate in any given time and how well the air flows in and out of the lungs. During the test, the patient is directed to blow into an instrument called a spirometer.
• Polysomnography
  It is a sleep study measuring the heart rate, brain activity, oxygen levels, blood pressure, and other factors when the person is sleeping. This test mainly helps diagnose a condition called obstructive sleep apnea, which can be a potential cause of pulmonary hypertension.
• Ventilation/perfusion (V/Q) scan
  A radioactive trace is introduced intravenously during this test, which helps reveal the airflow and blood flow in and out of the lungs. This test can help determine whether pulmonary hypertension has occurred due to any blood clots.

Although pulmonary hypertension can’t be completely cured, several treatment options are available to slow the progression of the disease and control its signs and symptoms. Finding the most appropriate treatment for pulmonary hypertension takes some time as the condition is often complex and demands proper follow-up care. If the pulmonary hypertension has happened secondary to some other condition, treatment is directed towards the underlying cause so that the condition is effectively controlled.

The Pulmonary Hypertension Clinic at Kokilaben Dhirubhai Ambani Hospital, Indore offers comprehensive medical care and treatment for those suffering from Pulmonary Hypertension. Our clinic houses highly qualified and experienced medical staff who deliver tailored treatment plans suited to the needs of the individual patient.