Key Points
ÎThe proposed new definition of pulmonary hypertension (PH) is a resting mPAP ≥ 25 mm Hg.
ÎThe definition of PAH also requires that the PCWP/ LVEDP must be ≤ 15 mm Hg.
ÎDue to the complexity of the diagnostic evaluation required and the treatment options available, referral of patients with PAH to a specialized center should be considered.
ÎPAH is a serious disease with a poor prognosis in many cases.
ÎA variety of agents are available for the medical treatment of PAH.
ÎClassification attempts to:
> Distinguish conditions affecting the pulmonary arteries (PAH) from causes of pulmonary venous hypertension such as left-sided cardiac disease) (Table 2).
> Categorize pulmonary hypertension due to other underlying conditions such as intrinsic lung disease, chronic recurrent thromboembolic disease, etc.
Table 1. Agents That Can Cause Drug and Toxin Induced PAH Definite
Possible
Aminorex Fenfluramine Dexfenfluramine Toxic rapeseed oil
Likely
Amphetamines L-tryptophan Methamphetamines
Cocaine Phenylpropanolamine St. John's Wort Chemotherapeutic agents, SSRI
Unlikely
Oral contraceptives Estrogen Cigarette smoking
SSRI - selective serotonin reuptake inhibitor. Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classication of pulmonary hypertension. J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl):S45.
