Pathology of Pulmonary Hypertension
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Pathology of Pulmonary Hypertension. Wolter J. Mooi Department of Pathology, VUmc Amsterdam [email protected] Pulmonary arterial hypertension (WHO definition). Sustained elevation of pulmonary arterial pressure to more than 25 mm Hg at rest, or to more than 30 mm Hg with exercise

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Pathology of Pulmonary Hypertension

Wolter J. Mooi

Department of Pathology, VUmc


[email protected]

Pulmonary arterial hypertension who definition l.jpg
Pulmonary arterial hypertension(WHO definition)

  • Sustained elevation of pulmonary arterial pressure to more than 25 mm Hg at rest, or to more than 30 mm Hg with exercise

  • Mean pulmonary wedge pressure and left ventricular end-diastolic pressure less than 15 mm Hg

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WHO Classification of Pulmonary Hypertension

  • Group I: Pulmonary arterial hypertension

    • Idiopathic (primary)

    • Familial

    • Associated with significant venous or capillary involvement

    • Persistent pulmonary hypertension of the newborn

  • Group II: Pulmonary venous hypertension

    • Left-sided artial or ventricular heart disease

    • Left-sided valvular heart disease

  • Group III: Pulmonary hypertension associated with hypoxemia

  • Group IV: Pulmonary hypertension due to chronic thrombotic disease, embolic disease, or both

  • Group V: Miscellaneous

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WHO Classification of Pulmonary Hypertension: criticisms

  • Pulmonary arterial hypertension is not limited to Group I (this is inappropriately suggested by its designation as ‘pulmonary arterial hypertension’)

    • It may be associated with pulmonary venous hypertension

    • It may be posttrombotic (Group IV) or hypoxic (Group III)

  • The group ‘miscellaneous’ includes: compression of pulmonary veins, which should be in Group II (pulmonary venous hypertension)

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Main histological patterns of pulmonary hypertensive vascular disease

  • Congestive vasculopathy

    • Backward failure of left side of heart

    • Obstruction of pulopmary large veins

  • Hypoxic pulmonary vasculopathy

    • COPD, high altitude dwellers, alevoar hypoventialtion disorders, &c

  • Postthrombotic pulmonary vasculopathy

    • Chronic recurrent thromboembolism, primary thrombosis

  • Plexogenic pulmonary arteriopathy

    • Posttricuspid LR shunts; associated with anorexigens, HIV, portal hypertension, idiopathic

  • Pulmonary vascular occlusive disease

  • Miscellaneous rare disorders

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Congestive pulmonary vasculopathy vascular disease

  • Veins:

    • Intimal fibrosis

    • Medial hypertrophy, ‘arterialization’

  • Interstitial fibrosis

  • Haemosiderosis

  • Arteries:

    • Intimal fibrosis

    • Medial hypertrophy

    • Adventitial thickening

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Hypoxic pulmonary vasculopathy vascular disease

  • Arteries and arterioles:

    • Medial hypertrophy (accentuated in smaller branches)

    • Muscularization of arterioles

    • Longitudinal smooth muscle bundles

    • Mild intimal thickening

  • Veins:

    • Mild thickening of venule walls (mild smooth medial hypertrophy, mild fibrosis)

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Postthrombotic pulmonary arteriopathy vascular disease

  • Arteries:

    • Eccentric intimal fibrosis

    • Concentric intimal fibrosis (not concentric laminar)

    • Bands and webs

    • Medial atrophy, fibrosis

    • Arterial obliteration; fibroelastic scars

      NB-1: abnormalities are focal, so that many arteries may appear normal or near normal, even in the presence of severe obstruction

      NB-2: arterial changes in fibrosing lung disease are very similar if not identical to postthrombotic arteriopathy

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Plexogenic pulmonary arteriopathy vascular disease

  • Early phase:

    • Medial thickening of elastic and muscular pulmonary arteries

    • Muscularization of pulmonary arterioles

    • Mild intimal fibrosis

  • Late phase:

    • Concentric laminar intimal fibrosis

    • Fibrinoid necrosis, arteritis

    • Dilatation lesions

    • Plexifom lesions

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BMPR2 germline mutations and familial pulmonary plexogenic arteriopathy

  • Deng et al., Am J Hum Genet 2000; 67: 737-44; Lane et al., Nat Genet 2000; 26: 81-84

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Heath Edwards grading system arteriopathyof hypertensive pulmonary vasular disease in congenital cardiac septal defects (1958)

  • Grade 1: retention of foetal-type pulmonary arteries

  • Grade 2: medial hypertrophy with cellular intimal proliferation

  • Grade 3: progressive fibrous vascular occlusion

  • Grade 4: progressive generalized arterial dilatation with formation of dilatation lesions

  • Grade 5: chronic dilatation with numerous dilatation lesions and pulmonary haemosiderosis

  • Grade 6: necrotizing arteritis

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Grade arteriopathy







Intimal reaction


Cellular proliferation

Fibrous and fibroelastic changes

Plexiform lesion

Medial changes


Some generalized dilatation

Local dilatation lesions


Necrotizing arteritis

Heath Edwards grading system

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Miscellaneous pulmonary hypertensive vasculopathies arteriopathy

  • Sarcoidosis

  • Langerhans cell histiocytosis

  • Lymphangioleiomyomatosis

  • Pulmonary vascular misalignment

  • Pulmonary capillary hemangiomatosis

  • Developmental abnormalities

  • &c &c