TY - JOUR T1 - Diagnosis and treatment of pulmonary hypertension JF - Heart JO - Heart SP - 311 LP - 319 DO - 10.1136/heartjnl-2011-301386 VL - 101 IS - 4 AU - Marlieke L A Haeck AU - Hubert W Vliegen Y1 - 2015/02/15 UR - http://heart.bmj.com/content/101/4/311.abstract N2 - Learning objectives Learn the current definition and classification of pulmonary hypertension. Learn the role of different diagnostic techniques in pulmonary hypertension. Learn about current medical and surgical treatment of pulmonary (arterial) hypertension. Pulmonary hypertension (PH) is a descriptive name for abnormally elevated pressures in the pulmonary vasculature. PH has been defined as a mean pulmonary arterial pressure (mPAP) ≥25 mm Hg at rest, measured by right heart catheterisation (RHC).1 It can be caused by an increase in pulmonary vascular resistance (PVR), pulmonary blood flow, pulmonary venous pressure or a combination of these factors. PH leads to right ventricular (RV) overload and finally RV failure and death.2 Commonly, PH is diagnosed at a late stage of the disease, and is associated with poor survival.3 This underscores the importance of early recognition and treatment of PH in order to improve the outcome in this patient population.4 PH is characterised by different pathological lesions in the pulmonary vasculature, depending on the underlying cause. The 5th World Symposium on Pulmonary Hypertension in Nice in 2013 classified PH according to these underlying aetiologies, creating five groups: (1) pulmonary arterial hypertension (PAH); (2) PH due to left sided disease; (3) PH due to lung disease; (4) chronic thromboembolic PH (CTEPH); (5) PH with unclear or multifactorial mechanisms (box 1).5 Box 1 Clinical classification of pulmonary hypertension51 Pulmonary arterial hypertension (PAH)  1.1 Idiopathic   1.2.1 Heritable: BMPR2   1.2.2 Heritable: ALK1, endoglin, SMAD9, CAV1, KCNK3   1.2.3 Heritable: unknown  1.3 Drugs and toxins induced  1.4 Associated with (APAH)  Connective tissue disease  HIV infection  Portal hypertension  Congenital heart disease  Schistosomiasis1′ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis1″ Persistent pulmonary hypertension of the newborn2 Pulmonary hypertension (PH) due to left heart disease  2.1 Systolic dysfunction  2.2 Diastolic dysfunction  2.3 Valvular disease  2.4 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies3 PH … ER -