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.
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 Group | Associated 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
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.
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
