Pulmonary hypertension

Respiratory (12%) Core Clinical Conditions

2B The Physician Associate is able to undertake the day to day management of the patient and condition once the diagnosis and strategic management decisions have been made by another.

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Definition Aetiology Pathophysiology Risk factor Sign and Symptoms Investigations Management

Definition

Pulmonary hypertension (PH) is a medical condition marked by high blood pressure in the pulmonary arteries.

Aetiology

Connective Tissue Disorders: scleroderma, systemic lupus erythematosus, and rheumatoid arthritis (RA).
Congenital Heart Diseases: such as atrial septal defect (ASD), ventricular septal defect (VSD) and patent ductus arteriosus (PDA).
Chronic obstructive pulmonary disease (COPD).
Heart Disease.
Exposure to drugs and Toxins.
Hereditary Factors: mutations in the bone morphogenetic protein receptor type 2 (BMPR2) gene.
Idiopathic disease is caused by a combination of genetic factors, environmental triggers, and abnormalities in the blood vessels of the lungs.

Pathophysiology

As a consequence of constriction, obstruction, or damage to the pulmonary blood vessels, blood flow resistance increases. This elevated pressure causes the right side of the heart to work harder to circulate blood through the airways, which can lead to right-sided heart failure in the long run.
Idiopathic pulmonary arterial hypertension (IPAH) is the most common form of pulmonary hypertension.

Risk factors

Family History
Genetics: genetic mutations of the BMPR2 gene.
Age: young adults and elderly have increased risk.
Gender - females are more at risk of PH.
Underlying Medical Conditions: scleroderma, systemic lupus erythematosus (SLE), and rheumatoid arthritis (RA).
Congenital Heart Diseases: such as atrial septal defect (ASD), ventricular septal defect (VSD), or patent ductus arteriosus (PDA).
Chronic Lung Diseases: Chronic obstructive pulmonary disease (COPD), interstitial lung disease and sleep apnea.
Heart Diseases.
History of pulmonary embolism (PE).
HIV Infection.
Certain drugs and Toxins.
Obesity.
Pregnancy.

Sign and symptoms

Shortness of Breath (Dyspnea).

Fatigue.
Chest Pain.
Dizziness.
Rapid Breathing (Tachypnea).
Palpitations.
Ankle / leg swelling (Oedema).
Bluish Lips and Skin (Cyanosis).

Diagnosis and investigations

Echocardiogram (Echo).
Electrocardiogram (ECG).
Chest X-ray.
Lung Function Tests.
Blood Tests: full blood count (FBC), liver and kidney function (LFT), thyroid function tests (TSH).
Exercise Testing.
Cardiac Catheterization: Right side.
Ventilation - perfusion Scan..
Computed Tomography (CT)/Magnetic Resonance Imaging (MRI) Scans.