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Primary pulmonary hypertension
Diagnosis and treatment of pulmonary hypertension
  1. Marlieke L A Haeck,
  2. Hubert W Vliegen
  1. Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands
  1. Correspondence to Dr Hubert W Vliegen, Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, The Netherlands; h.w.vliegen{at}

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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.

Curriculum topic: Primary pulmonary 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 hypertension5

1 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

  •  Schistosomiasis

1′ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis

1″ Persistent pulmonary hypertension of the newborn

2 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 cardiomyopathies

3 PH …

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  • Contributors Both authors contributed equally to the manuscript.

  • Competing interests In compliance with EBAC/EACCME guidelines, all authors participating in Education in Heart have disclosed potential conflicts of interest that might cause a bias in the article. The authors have no competing interests.

  • Provenance and peer review Commissioned; externally peer reviewed.

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