pulmonary hypertension

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
  • Pulmonary veno-occlusive disease
  • Pulmonary capillary hemangiomatosis

• 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)

Types

 acute pulmonary hypertension
 chronic pulmonary hypertension

Grading system

 Heath-Edwards system

Etiology

 primary pulmonary vascular diseases

• primary pulmonary hypertension (PPH)
• persistent pulmonary hypertension of the newborn (PPHN) or persistent fetal circulation (PFC)

 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

• premature closure of ductus arteriosus
• premature closure of foramen ovale
• alveolar capillary dysplasia with misalignement of pulmonary vessels
• pulmonary veno-occlusive disease
• invasive pulmonary capillary haemangiomatosis
• pulmonary embolic disease
  • thromboembolism
  • non-thrombotic embolism

• congenital and acquired heart diseases
  • with pulmonary venous hypertension
    • obstruction to left ventricular outflow
      • aortic atresia
      • aortic stenosis
      • hypoplastic left heart
      • aortic coarctation with VSD
      • interrupted aortic arch

• • • 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

• sleep disordered breathing due to obstructive sleep apnea
• neuromuscular disease
• portocaval shunt (12447666, 6886359)
• portal hypertension (159649)
• cirrhosis of the liver with portacaval shunts (5634734)

Associations

 portal hypertension (1409142)

Grading system

 Heath-Edwards grading system of pulmonary hypertension

 The Evolving Classification of Pulmonary Hypertension. http://www.archivesofpathology.org/doi/pdf/10.5858/arpa.2016-0035-RA (Fre paper)

See also

 pulmonary plexogenic arteriopathy

Open references

 The Evolving Classification of Pulmonary Hypertension. 2017. http://www.archivesofpathology.org/doi/pdf/10.5858/arpa.2016-0035-RA (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.