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pulmonary hypertension

Sunday 15 January 2006

pulmonary hypertensive diseases, pulmonary arterial hypertension (PAHT)

Clinical Classification of Pulmonary Hypertension

Primary pulmonary hypertension

- Category 1: PAH

  • idiopathic primary pulmonary hypertension (IPPH)
  • Familial
  • Pulmonary hypertension associated with:
    • Collagen vascular disease
    • Congenital systemic-to-pulmonary shunts
    • Portal hypertension
    • Drugs/toxins
    • HIV infection
    • Other (Gaucher’s, hereditary hemorrhagic telangiectasia, hemoglobinopathies, splenectomy)
  • Associated with significant venous or capillary involvement
  • Persistent pulmonary hypertension of the newborn

Secondary pulmonary hypertension (Non–Category 1)

- Category 2: Pulmonary venous hypertension

- Category 3: Pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia

- Category 4: Pulmonary hypertension due to chronic thrombotic and/or embolic disease

- Category 5: Pulmonary hypertension due to miscellaneous disorders directly affecting the pulmonary vasculature

  • sarcoidosis
  • histocytosis X
  • lymphangiomatosis
  • compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis)


- acute pulmonary hypertension
- chronic pulmonary hypertension

Grading system

- Heath-Edwards system


- primary pulmonary vascular diseases

- secondary pulmonary hypertension

  • meconium aspiration
  • pulmonary parenchymal diseases
    • bacterial pneumonia
    • postviral obliterative bronchiolitis
    • bronchopulmonary dysplasia
    • acute respiratory distress syndrome (ARDS)
    • pulmonary interstitial fibrosis
    • airways diseases
    • persistent upper airway obstruction
    • chronic airway obstruction (asthma)
    • chronic lower airway obstruction (cystic fibrosis)
    • endstage pulmonary disease
      • obstruction to pulmonary venous return
        • critical mitral stenosis
        • cor triatrium
        • pulmonary venous stenosis
        • intradiaphragmatic TAPVR
      • decreased left ventricular contractility
        • transient left ventricular dysfuntion
        • endocardial fibroelastosis
        • glycogen storage disease type II (GSD2) (Pompe disease)
    • with increased pulmonary blood flow
      • transposition of great arteries
      • supradiaphragmatic TAPVR
      • endocardial cushion defect
      • arteriovenous malformations
    • with isolated VSD
  • decreased pulmonary vascular bed
    • pulmonary hypoplasia
    • primary pulmonary hypoplasia
    • secondary pulmonary hypoplasia
      • congenital diaphragmatic hernia
      • oligohydramnios sequence
      • asphyxiating thoracic dystrophy (Jeune syndrome)
      • fetal pleural effusions
  • generalized and local vascular disorders
    • portocaval shunt
    • collagen vascular diseases (dysimmune diseases)
    • intravenous drug abuse
    • vascular toxins
    • drugs
  • mechanical disorders
    • chest wall restriction (kyphoscoliosis)
    • muscle weakness (myasthenia gravis)
  • central nervous system disorders
    • acquired (head injury, ence^phalitis)
    • congenital (Arnold-Chiari malformations)
    • ventilatory control diorders
  • blood hyperviscosity
    • polycythemia
    • fetal-maternal transfusion
    • fetal-fetal transfusion
    • sickle-cell disease
    • high-altitude residence
    • antiphospholipid antibody syndrome


- portal hypertension (1409142)

Grading system

- Heath-Edwards grading system of pulmonary hypertension

- The Evolving Classification of Pulmonary Hypertension. (Fre paper)

See also

- pulmonary plexogenic arteriopathy

Open references

- The Evolving Classification of Pulmonary Hypertension. 2017. (Fre paper)

Paywall References

- Tulloh RM. Congenital heart disease in relation to pulmonary hypertension in paediatric practice. Paediatr Respir Rev. 2005 Sep;6(3):174-80. PMID: 16153566

- Bush A. Pulmonary hypertensive diseases. Paediatr Respir Rev. 2000 Dec;1(4):361-7. PMID: 16263465

- Alt B, Shikes RH. Pulmonary hypertension in congenital heart disease: irreversible vascular changes in young infants. Pediatr Pathol. 1983 Oct-Dec;1(4):423-34. PMID: 6687292

- Wagenvoort CA. Grading of pulmonary vascular lesions—a reappraisal. Histopathology. 1981 Nov;5(6):595-8. PMID: 7319479

- Yi ES, Kim H, Ahn H, Strother J, Morris T, Masliah E, Hansen LA, Park K, Friedman PJ. Distribution of obstructive intimal lesions and their cellular phenotypes in chronic pulmonary hypertension: a morphometric and immunohistochemical study. Am J Respir Crit Care Med 2000;162:1577–1586

- Smith P, Heath D, Yacoub M, Madden B, Caslin A, Gosney J. The ultrastructure of plexogenic pulmonary arteriopathy. J Pathol 1990;160:111–121.

- Heath D, Smith P, Gosney J, Mulcahy D, Fox K, Yacoub M, Harris P. The pathology of the early and late stages of primary pulmonary hypertension. Br Heart J 1987;58:204–213.

- Wagenvoort CA. Lung biopsies and pulmonary vascular disease. In: Weir EK, Reeves JT, editors. Pulmonary hypertension. Mount Kisco, NY: Futura Publishing Co; 1984. pp. 393–438.

- Reid LM. Structure and function in pulmonary hypertension: new perceptions. Chest 1986;89:279–288.