Pulmonary hypertension is a general term used to describe high blood pressure in the lungs, it is different from the regular hypertension also known as systemic blood pressure.
In regular hypertension, the arteries throughout the body are constricted. In pulmonary hypertension, the pulmonary arteries, which are the blood vessels responsible for transporting blood from the heart to the lungs are affected.
Pulmonary hypertension is a rare chronic disease in which the pulmonary arteries become narrowed and thickened, making it difficult for blood to flow through and as a result causing the heart to work harder. Over time, the hearts become enlarge, weakens and it can lead to the development of right heart failure.
Pulmonary hypertension affects people of all ages, races and ethnic background.
Types of pulmonary hypertension
According to the World Health Organisation,there are five different group of pulmonary hypertension based on their different causes.
Group 1 Pulmonary Arterial Hypertension (PAH)
PAH can occur with no underlying cause, it is called idiopathic PAH (IPAH)
Heritable PAH is linked to genes that are inherited from family members
PAH can also develop as a complication of other medical conditions including congenital heart disease, liver disease, HIV and connective tissue diseases such as scleroderma and lupus.
Also it can be caused by certain drug use like methamphetamine,cocaine or certain diet pills.
Group 2 Pulmonary hypertension due to left Heart disease
Pulmonary hypertension can be caused due to left heart disorders such as left ventricular diastolic dysfuction or valvular disease
Group 3 pulmonary hypertension due to lung diseases or hypoxemia (low blood oxygen conditions)
Caused by COPD (chronic obstructive pulmonary disease, pulmonary fibrosis, obstructive sleep apnea, high altitude amongst others.
group 4 pulmonary hypertension due to chronic blood clots in the lungs CCTEPH)
chronic thromboembolic pulmonary hypertension can occur when the body is not able to dissolve a blood clot in the lungs.
group 5 pulmonary hypertension of unknown cause
The associated conditions includes disorders like sickle cell disease, metabolic disorders,thyroid disease and chronic kidney disease
Symptoms of pulmonary hypertension includes:
fatigue
shortness of breath, in the early phase of the illness, this may occur during exercise orexertion.But later will progress and become apparent during rest
cyanosis (bluish tinted lips, nails or skin)
oedema, swelling in legs, ankles or feet.
palpitations
chest pains
dizziness (syncope)
coughing up blood.
Oedema
Diagnosing pulmonary hypertension
Pulmonary hypertension is usually difficult to diagnose and mostly diagnosed at the advanced stage. To diagnose pulmonary hypertension, physical examinations needs to be carried out, family history reviewed. several Tests need to be carried out, which includes:
* Echocardiogrm
* Chest X-ray
* Electrocardigram (ECG)
* Right heart catheterization which is the gold standard test for pulmonary hypertension diagnosis. The Right heart catheterisation is one of the most acurate and useful tests to get a definitive diagnosis for pulmonary hypertension. The test directly measures the pressure inside the pulmonary arteries. The test isdone by inserting a catheter (thin rubber tube) through a large vein in the patients groin or neck. They then pass the catheter up into the patientsheart to measure the blood pressure in the right side of the heart and lungs.
After pulmonary hypertension diagnosis, tests needs to be carried to determine what group of pulmonary hypertension in order to treat it accordyling. Test carried out includes:
*Computerized tomography (CT) scan produces detailed images of the structures of the heart and lungs.
*Chest magnetic resonance imaging (MRI) provides information on the blood flow in the lungs and how the heart is functioning
*Pulmonary function tests
*Polysomnogram
*Ventilation/perfussion (V/Q) scan.
*Blood tests to check for HIV, thyroid, liver and auto immune diseases.
*Genetic tests
To diagnose the severity of pulmonary hypertension, a cardiopulmonary test which measures how well the lungs and heart work while a patient is on a treadmill or bicycle. Six minute walk test which measures how far a patient can walk in six minutes. These test helps rate the severity of the disease from class 1 to 4
*class one: Patients experience no symptoms with normal activity
*class two: patients experience symptoms with regular physical activity
*class three: Patients experience symptoms with slight physical activity
*class four :Patients experience symmptomswhile resting,and severe symptoms with physical activity.
Treatment for pulmonary hypertension
There is no known cure for pulmonary hypertension,treatments help controls and reduce symptoms and slow progression of disease.They can be taken as pills, inhaled or through an IV, Treatment varies on the different underlying cause,but it generally includes
Medications
*Blood vessel dilators (vasodilators)
Vasodilators open narrowed blood vessels
*Endothelin receptor antagonists
These medication reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow.
*Sildenafil and tadalafil
These drugs work by opening the blood vessels in the lungs to allow blood to flow through more easily.
*Calcium channel blockers
These drugs help relax the muscles in the walls of your blood vessels.
*Soluble guanylate cyclase (SGC) stimulators
*Anticoagulant
These drugs help prevent the formation of blood clots with in the small pulmonary arteries.
*Digoxin
Assists the pumping of the heart.
*Diuretics
known as water pills,these medication help eliminate excess fluid from the body that puts pressure on the heart.
*Oxygen
Oxygen therapy helps increase the level of oxygen in the blood
Surgery
Pulmonary thromboendarterectomy (PTE) is a surgical procedure that is performed to remove blood clots in the lungs. This procedure is for Group 4 (CTEPH) pulmonary hypertension
When a patients is not responding to treatment, a double lungs or in some cases both heart-lungs transplant will be needed.
In Nigeria, the medications available for treating pulmonary hypertension are sildenafil, diurectics and anticoagulant.