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Dr. Tamer Sayin
Prof. Cetin Erol,
Pulmonary arterial hypertension is a cardiopulmonary disease that is a form of pulmonary hypertension which has its own diagnosis and specific medications, distinct from the four other forms of pulmonary hypertension that are pulmonary hypertension with left heart disease, chronic lung disease, chronic thromboembolism or with unclear mechanisms. Review the latest considerations and therapy specific to pulmonary arterial hypertension in this article.
Pulmonary hypertension is defined as a mean pulmonary artery pressure of ≥25 mmHg at right heart catherisation. The continuous update provided by five world symposia on pulmonary hypertension since 1973 up until 2013 have classified pulmonary hypertension into 5 clinical groups:
Pulmonary arterial hypertension is defined as mean pulmonary artery pressure of ≥ 25 mmHg at right heart catheterisation, pulmonary artery wedge pressure ≤ 15 mmHg and pulmonary vascular resistance of > 3 Wood units or mmHg·min/l. Pulmonary arterial hypertension is a rare disease that can be fatal. The other four groups are pulmonary hypertension with:
Groups 1,3,4 and 5 can be labelled as “precapillary pulmonary hypertension” (1-5).
Idiopathic pulmonary hypertension accounts for half of all cases of pulmonary arterial hypertension in our centers. The other half is due to conditions associated, of which connective tissue disease, HIV infection, portal hypertension, congenital heart disease or schistosomiasis. Pulmonary arterial hypertension is a rare disease - reported at 15 per million in a French registry. Distal pulmonary arterioles have an imbalanced vasoconstrictor/vasodilator and proliferation/apoptosis (proliferation dominant) milieu leading to increased pulmonary vascular resistance and right heart failure. Prognosis is poor with a 15% mortality within 1 year on modern therapy (6,7).
It might be difficult to differentiate pulmonary arterial hypertension from pulmonary hypertension in case of heart failure with preserved ejection fraction. Means to do so are:
Whereas pulmonary hypertension with left heart disease or with lung disease have no specific medication pulmonary arterial hypertension does. We gain to be aware of the latest clinical classification of pulmonary hypertension brought to you and best performed in expert centers. Pulmonary arterial hypertension is a distinguishable form treatable with its own dedicated medication.
1 - Updated clinical classification of pulmonary hypertension Simonneau G, Gatzoulis MA, Adatia I et al. J Am Coll Cardiol 2013;62:D34-41
2 - Updated treatment algorithm of pulmonary arterial hypertension.Galie N, Corris PA, Frost A et al J Am Coll Cardiol 2013;62:D60-72
Ghofrani HA, D’armini AM, Grimminger F et al. N Engl J Med 2013;369:319-29.
4 - Definitions and diagnosis of pulmonary hypertension Hoeper MM, Bogaard HJ, Condliffe R et al. J Am Coll Cardiol 2013;62: D42-50.
5 - A USA-based registry for pulmonary arterial hypertension:1982-2006. Thenappan T, Shah SJ, Rich S et al. Eur Respir J. 2007;30:1103-10.
6 - Pulmonary arterial hypertension in France: results from a national registry Humbert M, Sitbon O, Chaouat A et al. Am J Respir Crit Care Med. 2006;173:1023-30.
7 - ACCF/AHA expert consensus document on pulmonary hypertension. McLaughlin VV, Archer SL, Badesch DB et al. J Am Coll Cardiol 2009;53:1573-619.
8 - Guidelines for the diagnosis and treatment of pulmonary hypertension Galie N, Hoeper MM, Humbert M et al. Eur Heart J. 2009;30:2493-537.
9 - Pulmonary hypertension due to left heart diseases. Vachiery JL, Adir Y, Barbera JA et al. J Am Coll Cardiol 2013;62:D100-8.