Treatment Options For Pulmonary Hypertension

An Overview Of Pulmonary Hypertension And Its Symptoms

Pulmonary hypertension (PH) is a life-threatening medical condition that occurs when the arteries located on the right side of the heart that carry blood to the lungs are narrow. This restricts blood flow and as a result, impacts the air-exchange that takes place. Consequently, the supply of oxygen-carrying blood to various parts of the body is reduced.

This is a rare form of high blood pressure in the pulmonary arteries and capillaries suffer damage. Due to the damage to these blood vessels, the heart puts in excessive effort when pumping blood. This in turn injures and weakens the heart’s muscles, thereby, increasing the risk of heart failure and death.

Causes

• Idiopathic (no clear causative factors): Usually found in young adults
• Obesity
• Genetic factors and family history of PH
• Living in high altitude locations with a family history of PH
• Use of illegal drugs such as cocaine
• Consumption of appetite suppressant drugs
• Certain medical conditions such as liver or lung disease, infections like HIV, auto-immune conditions such as rheumatoid arthritis, lupus, and so on.

Symptoms

PH is almost impossible to detect during the early stages, but the diagnosis becomes clearer as the condition gets worse. Some of the warning signs include:

• Frequent shortness of breath, initially during exertion/exercise and consequently during resting periods
• Fatigue with no apparent cause
• Dizziness and fainting
• Chest pain/pressure
• Swelling in legs/ankles in early stages, in later stages in the abdomen due to fluid retention
• Cyanosis or blue tinge on lips and skin
• Rapid pulse rate and heart palpitations

Types of PH

PH is classified into 5 major types:

• Group 1 Pulmonary Arterial Hypertension (GP1 PAH): Idiopathic, may be caused by a genetic mutation, heritable family condition. It may also result from congenital heart defects or use of certain prescription/illegal drugs, or other conditions such as HIV, lupus, chronic liver disease, etc.
• Group 2 PAH caused by Left-side Heart Disease (GP2 PAH): also known as Mitral/Aortic Valve Disease, it results in failure of the heart’s left ventricle (lower chamber)
• Group 3 PAH caused by Lung Disease (GP3 PAH): caused by chronic lung diseases such as emphysema or pulmonary fibrosis, sleep apnea or by long-term exposure to high altitude.
• Group 4 PAH caused by blood clots (GP4 PAH): caused by pulmonary embolism
• Group 5 PAH due to associated conditions (GP5 PAH): caused by blood disorders, organ disorders, tumors, and metabolic disorders
• Congenital Heart Disease: Eisenmenger Syndrome where the patient is born with a “hole in the heart”: a ventricular septal defect. This also results in PH.

Different Tests to Diagnose Pulmonary Hypertension

Although PH is rarely detected in the early stages, based on the symptoms, family history, and other factors, doctors may suspect the presence of this condition. There are several tests that can confirm a diagnosis. These include:

• ECG: Electrocardiogram is a non-invasive test that reveals electrical patterns in the heart. PH may be diagnosed if abnormal rhythms, right ventricle enlargement, and/or strain are detected.
• Chest X-ray: This shows enlargement of the right ventricle, deformities in blood-vessels, etc.
• Echocardiogram: This test reveals the size and functioning of the right ventricle, valves, the thickness of the wall, etc. It can also be used to measure blood pressure in the pulmonary arteries. Exercise echocardiogram is another tool that can be used to measure the heart’s functioning during exertion, along with an oxygen consumption test to show the functioning of the air exchange pattern.
• Right heart catheterization: Allows the doctor to directly measure pulmonary artery pressure by inserting a catheter into the right ventricle and pulmonary artery. It is also used to measure the effect of different medications during treatment.
• CT/MRI Scan: Are used to check the appearance and function of the heart and pulmonary arteries.
• Open-Lung Biopsy: Can identify secondary causes of PH.
• Pulmonary Function Test: Checks airflow in the lungs
• Polysomnogram: Helps to identify sleep apnea as a cause of PH
• Ventilation-Perfusion VQ Test: Tracer dye is injected into the bloodstream to map the passage of blood and detect the presence of clots.

Treatment Options

Though PH is incurable, the pulmonary hypertension treatment helps to alleviate the symptoms, manage the condition and slow down the disease’s progress. Options include:

• Medication: Vasodilators, Endothelin-receptor antagonists, Calcium-channel blockers, anticoagulants, diuretics, oxygen, etc. can reduce discomfort and distress.
• Surgery and Transplantation: This is usually taken into consideration as a last resort for younger patients.

Road To Recovery

It’s important to:

• Make major lifestyle and diet changes
• Maintain a symptom diary
• Get more awareness of your condition
• Stay in touch with your physicians
• Avoid aggravating factors like too much exertion, traveling to high-altitudes, taking OTC medications
• Get emotional support and/or psychological counseling