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primary pulmonary hypertension

MIM.178600

Sunday 15 January 2006

Types

- autosomal recessive form of PPH (MIM.265400)

Synopsis

- numerous plexiform vascular lesions
- plexogenic pulmonary arteriopathy
- medial hypertrophy (41.0%)
- intimal cellular proliferation (2.5%)
- intimal fibroelastic proliferation (36.9%)
- medial destructive changes (19.6%)

  • dilatation lesions
  • plexiform lesions
  • arteritis
  • fibrinoid necrosis

- index of pulmonary vascular disease (IPVD) : 1.6 to 3.4 (mean 2.5).

Etiology

- Locus PPH1 at 2q33: germline mutations in the BMPR2 gene (MIM.600799)

Associations

- hereditary hemorrhagic telangiectasia type 2 (HHT2) (MIM.600376)

  • germline mutation in the ACVRL1 (ALK1) gene (MIM.601284)

References

- Knight JA, Wilson JF. Primary pulmonary hypertension in childhood: a report of two brothers. Pediatr Pathol. 1985;4(1-2):13-23. PMID: 2937032

Pathology references

- Histopathologic findings of lung vessels in five children with primary pulmonary hypertension. Sato T, Akiba T, Suzuki H, Nakasato M, Sato S, Yoshikawa M, Yamaki S. Tohoku J Exp Med. 1994 Jan;172(1):9-15. PMID: 8036624 (Free article at journal site)

- 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.