Pulmonary hypertension

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Pulmonary hypertension is bad stuff that arises from heart problems and an assortment of weird stuff.

General classification

  • Primary, i.e. primary pulmonary hypertension, or
  • Secondary, e.g. due to congenital heart disease (like ventricular septal defect), interstitial pulmonary fibrosis.

Non-secondary pulmonary hypertension

Causes:[1]

  • Primary pulmonary hypertension.
  • Pulmonary embolic disease (thromboembolism, and non-thrombotic embolism).
  • Pulmonary capillary haemangiomatosis (PCH).
  • Pulmonary veno-occlusive disease (PVOD).

Notes:

  • Some people consider PCH and PVOD to the be same thing.[2]
    • Both have a poor prognosis.
    • Clinically they present the same way.
  • PVOD is based on case reports - it is extremely rare.[3]

Primary pulmonary hypertension

  • AKA pulmonary plexogenic arteriopathy.[4]
  • Like chronic pulmonary hypertension due to congenital heart disease but without the congenital heart disease.[4]
    • Classified by Heath-Edwards classification (see below) into six grades.

Pulmonary veno-occlusive disease (PVOD)

Features:[5]

  • Clinical - gradual dyspnea +/- non-productive cough, +/- clubbing.
  • Thrombosis - small veins & venules, particularily at the interlobular septae.
  • Associated with mild homogenous peripheral interstitial fibrosis.

DDx: chronic interstitial pneumonia.

Pulmonary capillary hemangiomatosis (PCH)

General:

  • First reported in 1978 by Wagenvoort et al..[6]

Features:

  • Proliferating and invasive capillaries.[7]
  • Demonstrated by CD34 immunostaining.[2]
  • Dilated capillaries[8][9] - key feature.

DDx:

  • Passive congestion (PC).
    • Differentiated by fact that PCH has multiple channels in alveolar wall (PC has only one).

Chronic pulmonary hypertension due to congenital heart disease

  • Graded using the Heath-Edwards system.[10]
  • A reason for open lung biopsy in children.[11]

Heath-Edwards classification

Definition:[10]

  • Six grades - based on intimal reaction and media of arteries and arterioles:
    • Grade 1:
      • Intima: no intimal reaction.
      • Media: hypertrophied.
    • Grade 2:
      • Intima: cellular intimal reaction.
      • Media: hypertrophied.
    • Grade 3:
      • Intima: fibrous & fibroelastic reaction + cellular intimal reaction.
      • Media: hypertrophy +/- generalized dilation.
    • Grade 4:
      • Intima: "plexiform lesions" + fibrous & fibroelastic reaction, + cellular intimal reaction.
          • Plexiform lesions = multiple channels that are dilated, assoc. with loss of elastic laminae; thought to arise at branch points due to aberant WSS.[12]
      • Media: generalized dilation +/- local "dilation lesions".
      • Micrographs: Plexiform lesions (ucsf.edu), Plexiform lesions (pvrireview.org).
    • Grade 5:
      • Intima: as in Grade 4.
      • Media: generalized dilation + local "dilation lesions" + pulmonary hemosiderosis.
    • Grade 6:
      • Intima: as in Grade 4.
      • Media: generalized dilation + local "dilation lesions" + pulmonary hemosiderosis + necrotizing arteritis.

Notes:

See also

References

  1. ↑ Bush A (December 2000). "Pulmonary hypertensive diseases". Paediatr Respir Rev 1 (4): 361-7. doi:10.1053/prrv.2000.0077. PMID 16263465.
  2. ↑ 2.0 2.1 Lantuéjoul S, Sheppard MN, Corrin B, Burke MM, Nicholson AG (July 2006). "Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: a clinicopathologic study of 35 cases". Am. J. Surg. Pathol. 30 (7): 850-7. doi:10.1097/01.pas.0000209834.69972.e5. PMID 16819327.
  3. ↑ Vevaina JR, Mark EJ (March 1988). "Thoracic hemangiomatosis masquerading as interstitial lung disease". Chest 93 (3): 657-9. PMID 3342678.
  4. ↑ 4.0 4.1 Lie JT, Silver MD. Diagnostic criteria of cardiovascular pathology: acquired diseases. ISBN 0-397-51630-4. PP.208-9.
  5. ↑ PPP PP.393-6.
  6. ↑ Wagenvoort CA, Beetstra A, Spijker J (November 1978). "Capillary haemangiomatosis of the lungs". Histopathology 2 (6): 401-6. PMID 730121.
  7. ↑ Tron V, Magee F, Wright JL, Colby T, Churg A (November 1986). "Pulmonary capillary hemangiomatosis". Hum. Pathol. 17 (11): 1144-50. PMID 3770733.
  8. ↑ MC August 2009.
  9. ↑ PPP PP.396-7.
  10. ↑ 10.0 10.1 HEATH D, EDWARDS JE (October 1958). "The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects". Circulation 18 (4 Part 1): 533-47. PMID 13573570.
  11. ↑ Jaklitsch MT, Linden BC, Braunlin EA, Bolman RM, Foker JE (June 2001). "Open-lung biopsy guides therapy in children". Ann. Thorac. Surg. 71 (6): 1779-85. PMID 11426747.
  12. ↑ http://pathhsw5m54.ucsf.edu/overview/vessels.html
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