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Pulmonary Hypertension

2024/07/01
Introduction

Pulmonary hypertension (PH) develops when the pressures in the arteries of the lung are elevated. This condition may lead to right-sided heart failure - the right ventricle of the heart swells, weakens and functions poorly as a pump.

Symptoms of PH may include fatigue, shortness of breath on exertion, abdominal swelling, leg swelling, dry cough, palpitations and rhythm disorders, giddiness and fainting. The symptoms of PH can be mistaken for other disease. Therefore, diagnosis PH can be difficult and requires a specialist. Patients can deteriorate rapidly with this condition, but early diagnosis as well as aggressive treatment can improve prognosis tremendously.

We are a tertiary referral centre for pulmonary hypertension, dedicated to providing accurate comprehensive diagnosis, full range of treatment modalities and multi-disciplinary support for patients with pulmonary hypertension. As a well-recognised expert centre in the region, we also have access to latest pulmonary hypertension drug therapy though global clinical trials participation.

Conditions Treated

PH affects people of all ages and races. Although anyone can get PH, there are risk factors that make some people more likely to develop the disease. Not all PH is the same. PH is classified by the World Health Organisation (WHO) into 5 different groups, based on aetiology and treatment trend.

WHO GroupAssociated causes

WHO Group I

Pulmonary arterial hypertension

Unknown (idiopathic)

Hereditary or genetics

Associated disease including

  • connective tissue disease
  • congenital heart disease
  • liver disease with portal hypertension
  • HIV infection and drugs/toxins

WHO Group II

Pulmonary hypertension

due to left heart disease

This is often a result of coronary artery disease, heart valve disease, heart muscle damage that lead to systolic or diastolic dysfunction of the left ventricle of the heart

WHO Group III

Pulmonary hypertension

due to lung disease

Chronic obstructive lung disease Interstitial lung disease Sleep ventilation disorder

WHO Group IV

Chronic thromboembolic

pulmonary hypertension

This is related to a history of pulmonary embolism (blood clots formed in lung arteries

WHO Group V

Pulmonary hypertension

with multifactorial mechanisms

This can be related to certain blood cancers, thyroid disease, systemic disorder, renal failure on dialysis

A thorough assessment is required to confirm the diagnosis and look for the underlying cause of pulmonary hypertension in patients. The following investigations are performed in our centre:

  • Right heart catheterisation as a standard for determination of pressures in the lung arteries
  • Echocardiogram (ultrasound imaging of the heart)
  • Exercise tolerance test, i.e. 6 minute walk test and cardiopulmonary stress test
  • Blood investigations including cardiac and autoimmune markers, liver function tests etc.
  • High resolution lung scans and lung function testing
  • Specialised scans to look for blood clots in the lungs, i.e. scintigraphy ventilation-perfusion scans, computed tomography perfusion scan
Treatments Offered

Appropriate treatment for pulmonary hypertension must be instituted early to improve symptoms, exercise capacity, and longevity of patients. Therapeutic options can vary based on the underlying cause. Some treatments offered include:

  • Drugs that target pulmonary hypertension specifically by acting on pathways to relax and dilate lung arteries. Patients are often prescribed combinations of the following therapy best outcomes.
    • Phosphodiesterase V inhibitors - Sildenafil (Viagra), Tadalafil (Cialis)
    • Endothelin Receptor Antagonists - Bosentan (Tracleer), Macitentan (Opsumit), Ambrisentan (Volibris)
    • Prostacyclin analogues - inhaled Iloprost (Ventavis), intravenous Epoprostenol (Veletri)
    • Soluble Guanylate Cyclase stimulators – Riociguat (Adempas)
  • Other medication that may be prescribed to improve function.
    • Diuretics – medication that help to remove excess water from the body
    • Anticoagulation – medication that thins the blood and improve lung perfusion
    • Long term oxygen therapy
  • Specialised procedures for Group IV PH.

Patients with chronic thromboembolic pulmonary hypertension may benefit from pulmonary endarterectomy and/or balloon pulmonary angioplasty, which is a procedure involving using small medical balloons are used to open up occluded lung arteries.

Care Team

A multi-disciplinary care team is important as patients often have complex systemic problems that accompany their disease. The pulmonary hypertension service is strongly supported by physicians from respiratory medicine, rheumatology, as well as the intensive care unit.

Additionally, our case managers and nurses play a critical role in guiding our patients through the disease process including provision of support, monitoring as well as advice.

Clinicians:

Case Managers and Nurses

  • Ms Li Geying
  • Ms Then Ai Houng
  • Ms Christine Tioh
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