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. 1989 Jul;62(1):74–77. doi: 10.1136/hrt.62.1.74

Severe pulmonary hypertension in Ullrich-Noonan syndrome.

A Tinker 1, N Uren 1, J Schofield 1
PMCID: PMC1216735  PMID: 2757877

Abstract

A 19 year old woman with Ullrich-Noonan syndrome presented with a six month history of progressive dyspnoea and cyanosis. Clinical features were suggestive of severe pulmonary hypertension. Cross sectional echocardiography showed pronounced right ventricular pressure overload with right ventricular hypertrophy. Cardiac catheterisation confirmed severe pulmonary hypertension. Attempts to reduce pulmonary artery pressure with calcium channel blockers were unsuccessful. The patient died shortly after investigation. Necropsy confirmed primary pulmonary hypertension.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Craig R. J., Selzer A. Natural history and prognosis of atrial septal defect. Circulation. 1968 May;37(5):805–815. doi: 10.1161/01.cir.37.5.805. [DOI] [PubMed] [Google Scholar]
  2. EVANS J. R., ROWE R. D., KEITH J. D. The clinical diagnosis of atrial sefect defect in children. Am J Med. 1961 Mar;30:345–356. doi: 10.1016/0002-9343(61)90045-6. [DOI] [PubMed] [Google Scholar]
  3. Fuster V., Steele P. M., Edwards W. D., Gersh B. J., McGoon M. D., Frye R. L. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation. 1984 Oct;70(4):580–587. doi: 10.1161/01.cir.70.4.580. [DOI] [PubMed] [Google Scholar]
  4. HEATH D., EDWARDS J. E. 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. 1958 Oct;18(4 Pt 1):533–547. doi: 10.1161/01.cir.18.4.533. [DOI] [PubMed] [Google Scholar]
  5. Haworth S. G. Primary pulmonary hypertension. Br Heart J. 1983 Jun;49(6):517–521. doi: 10.1136/hrt.49.6.517. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Haworth S. G. Pulmonary vascular disease in secundum atrial septal defect in childhood. Am J Cardiol. 1983 Jan 15;51(2):265–272. doi: 10.1016/s0002-9149(83)80047-2. [DOI] [PubMed] [Google Scholar]
  7. Markman P., Howitt G., Wade E. G. Atrial septal defect in the middle-aged and elderly. Q J Med. 1965 Oct;34(136):409–426. [PubMed] [Google Scholar]
  8. Nora J. J., Nora A. H., Sinha A. K., Spangler R. D., Lubs H. A. The Ullrich-Noonan syndrome (Turner phenotype). Am J Dis Child. 1974 Jan;127(1):48–55. doi: 10.1001/archpedi.1974.02110200050007. [DOI] [PubMed] [Google Scholar]
  9. Nora J. J., Torres F. G., Sinha A. K., McNamara D. G. Characteristic cardiovascular anomalies of XO Turner syndrome, XX and XY phenotype and XO-XX Turner mosaic. Am J Cardiol. 1970 Jun;25(6):639–641. doi: 10.1016/0002-9149(70)90612-0. [DOI] [PubMed] [Google Scholar]
  10. Packer M. Vasodilator therapy for primary pulmonary hypertension. Limitations and hazards. Ann Intern Med. 1985 Aug;103(2):258–270. doi: 10.7326/0003-4819-103-2-258. [DOI] [PubMed] [Google Scholar]
  11. Rich S., Brundage B. H. High-dose calcium channel-blocking therapy for primary pulmonary hypertension: evidence for long-term reduction in pulmonary arterial pressure and regression of right ventricular hypertrophy. Circulation. 1987 Jul;76(1):135–141. doi: 10.1161/01.cir.76.1.135. [DOI] [PubMed] [Google Scholar]
  12. Rich S., Brundage B. H., Levy P. S. The effect of vasodilator therapy on the clinical outcome of patients with primary pulmonary hypertension. Circulation. 1985 Jun;71(6):1191–1196. doi: 10.1161/01.cir.71.6.1191. [DOI] [PubMed] [Google Scholar]

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