Skip Ribbon Commands
Skip to main content

Pulmonary Hypertension

Pulmonary Hypertension - How to prevent?

Pulmonary Hypertension - Diagnosis

In the early stages of the disease, a patient’s physical examination results may appear almost normal. To establish a diagnosis, doctors may order a series of tests to measure the lung pressure and the function of the patient’s heart and lungs. These tests are done at the same time to accurately narrow down the heart condition(s) causing the symptoms.

nhcs cardiac lab test echo

  • ​Chest X-Ray (of the heart and lungs)
  • Electrocardiogram
  • Echocardiogram (an ultrasound of the heart to help determine its structure and how well it functions)
  • Right-heart catheterisation (the cardiologist inserts a special tube through a vein into the heart and lung vessels to measure the lung pressure and test how well the heart is pumping)
  • Lung/pulmonary function tests (to measure lung function)
  • Perfusion lung scan (an imaging of blood flow through the lungs)
  • Six-minute walk test (a measurement of how far a patient can walk within six minutes)

How serious is pulmonary hypertension?


Even though there is no known cure for or prevention against pulmonary hypertension, the condition is still treatable.

According to the American Lung Association, approximately 50 per cent of people diagnosed with pulmonary hypertension die within five years. For those whose condition is left untreated, the average survival is shortened to about three years.

Even with treatment, the high blood pressure in the lung arteries will continue to worsen and make daily activities more difficult. Adopting lifestyle modifications, however, may help patients with pulmonary hypertension get through their daily routine.

Cardiologists refer to the World Health Organization (WHO) Functional Classification to identify the severity of pulmonary hypertension in patients and decide on suitable treatments.

Functional status is a person’s ability to perform daily activities and is ranked from Class I (no limitations) to Class IV (unable to perform any physical activity). Below are the brief descriptions of the four NYHA functional classes.

  • WHO Class I: No limitation of usual physical activity; ordinary physical activity does not cause increased breathing problems, fatigue, chest pain or lightheadedness.
  • WHO Class II: Mild limitation of physical activity; there is no discomfort when at rest, but normal physical activity causes increased breathing problems, fatigue, chest pain or lightheadedness.
  • WHO Class III: Marked limitation of physical activity; there is no discomfort when the person is at rest, but less than ordinary physical activity causes increased breathing problems, fatigue, chest pain or lightheadedness.
  • WHO Class IV: Unable to perform any physical activity and possible signs of heart failure in the right ventricle (the heart chamber that pumps blood to the lungs); at rest, patients have difficulty breathing and/or fatigue, and symptoms get worse with almost any physical activity. History of fainting will place patients in NYHA Class IV even if their limitations in activity is categorised under NYHA Classes II or III.

Clinical classification of pulmonary hypertension

1.Pulmonary arterial hypertension

1. Idiopathic
2. Heritable
  • BPMR2 mutation
  • Other mutations
  • Drugs and toxins induced
3. Drugs and toxins induced
4. Associated with:
  • Connective tissue disease
  • Human immunodeficiency virus (HIV) infection
  • Portal Hypertension
  • Congenital heart disease
  • Schistosomiasis
1’ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis

1. Idiopathic
2. Heritable
  • EIF2AK4 mutation
  • Other mutations
3. Drugs, toxins and radiation induced
4. Associated with:
  • Connective tissue disease
  • (HIV) infection

1” Persistent pulmonary hypertension of the newborn

2. Pulmonary hypertension due to left heart disease

1. Left ventricular systolic dysfunction
2. Left ventricular diastolic dysfunction
3. Valvular disease
4. Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
5. Congenital/acquired pulmonary veins stenosis

3.Pulmonary hypertension due to lung diseases and/or hypoxia

1. Chronic obstruction pulmonary disease
2. Interstitial lung disease
3. Other pulmonary diseases with mixed restrictive and obstructive pattern
4. Sleep-disordered breathing
5. Alveolar hypoventilation disorders
6. Chronic exposure to high altitude
7. Developmental lung diseases

4. Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions

1. Chronic thromboembolic pulmonary hypertension
2. Other pulmonary artery obstructions
  • Angiosarcoma
  • Other intravascular tumours
  • Arteritis
  • Congenital pulmonary arteries stenosis
  • Parasites (hydatidosis)

5. Pulmonary hypertension with unclear and/or multifactorial mechanisms
1. Haematological disorders: chronic haemolytic anaemia, myeloproliferative disorders, splenectomy
2. Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis neurofibromatosis
3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
4. Others: pulmonary tumoral thrombothic microangiopathy, fibrosing mediastinitis, chronic renal failure (with/without dialysis), segmental pulmonary hypertension

Extracted from the ESC/ERS Guidelines published on 15 September 2015

Pulmonary Hypertension - Preparing for surgery

Pulmonary Hypertension - Post-surgery care

Discover articles,videos, and guides afrom Singhealth's resources across the web. These information are collated, making healthy living much easier for everyone.