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Annual Congress of Malaysian Thoracic Society
Symposium 5B
Orphan Lung Diseases
Pulmonary Arterial Hypertension – Early Diagnosis and Treatment
Ashari Yunus
(Lung Transplant & Sleep Medicine), Institut Perubatan Respiratori, Hospital Kuala Lumpur, Malaysia
Pulmonary Arterial Hypertension (PAH) is defined as a group of diseases characterised by a progressive
increase of pulmonary vascular resistance (PVR) leading to right ventricular failure and premature death.
PAH can be idiopathic, heritable, or associated with connective tissue disease (CTD), Congenital Heart
Disease (CHD) , HIV infection, and exposure to toxins and drugs. Mortality rates in patients with PAH are high,
historically the median life expectancy of idiopathic PAH (iPAH) without specific therapy is 2.8 years from
diagnosis, with 1-year, 3-year, and 5-year survival rates of 68%, 48% and 34% respectively.¹
The diagnosis of PAH is in part through the exclusion of other diseases. It requires a series of investigations
that are intended to make the diagnosis, clarify the clinical class of PAH, the type of PAH and to evaluate the
degree of functional and haemodynamic impairment. Formal guidelines and consensus documents have been
published by the European Society of Cardiology (ESC)², the National Pulmonary Hypertension Centres of the
UK and Ireland³, the ACCP, and the American College of Cardiology Foundation Task Force (ACCF)/American
Heart Association. For the practical purposes the approach should includes four stages : 1. Clinical suspicion
of PAH 2. Detection of PAH 3. PAH clinical class identification 4. PAH evaluation.
The aims of treatment in patients with PAH are to improve survival, disease-related symptoms and Quality of Life.
The treatments can be classified as conventional therapy and targeted PAH-specific therapy. In conventional
therapy, the options include oxygen supply in cases of hypoxemia, anticoagulants such as warfarin, and
digoxin with diuretics in cases of right-sided heart failure. For targeted PAH-specific therapy, few drugs are
available in Malaysia and these include bosentan, ambrisentan, iloprost and sildenafil. Generally, we initiated
targeted PAH-specific drug with monotheraphy and consider sequential combination therapy if patients have
shown inadequate response to monotherapy. Atrial septostomy and lung transplantation are reserved for
selected patients who have refractory symptoms.
References
1. D’Alonzo DE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary
hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115:343-9
2. Galie N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottom T, et al. Guidelines on diagnosis and treatment of pulmonary
arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of
Cardiology. Eur Heart J.2004;25:2243-78
3. Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. Heart. 2008;94
Suppl 1:11-41
4. Rubin LJ. Diagnosis and management of pulmonary arterial hypertension. ACCP evidence –based clinical practice guidelines.
Chest. 2004;126:7S-10S
5. McLaughlin VV, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College
of Cardiology Foundation Task Force on Expert Consensus Documents and the American College of Chest Physicians: American
Thoracic Society, Inc; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53:1573-619
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