Early-life pulmonary arterial hypertension with subsequent development of diffuse pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia type 1

Thorax

Volumn 63, Issue 1, 2008, Pages 85-86

  Mache, C J ^a   Gamillscheg, A ^a   Popper, H H ^a   Haworth, S G ^b  

^a MEDICAL UNIVERSITY OF GRAZ   (Austria)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

BOSENTAN; NIFEDIPINE;

ARTICLE; CASE REPORT; CHILD; CLINICAL FEATURE; CONTRAST ECHOCARDIOGRAPHY; DISEASE COURSE; EPISTAXIS; EXERCISE TOLERANCE; FOLLOW UP; GENE MUTATION; HEART CATHETERIZATION; HOME OXYGEN THERAPY; HUMAN; LUNG BIOPSY; MALE; PRIORITY JOURNAL; PULMONARY ARTERIOVENOUS FISTULA; PULMONARY HYPERTENSION; RENDU OSLER WEBER DISEASE; SPIRAL COMPUTER ASSISTED TOMOGRAPHY; VASCULAR DISEASE; ARTERIOVENOUS MALFORMATION; CONGENITAL MALFORMATION; INFANT; PATHOLOGY; PULMONARY ARTERY; PULMONARY VEIN;

ARTERIOVENOUS MALFORMATIONS; CHILD; HUMANS; HYPERTENSION, PULMONARY; INFANT; MALE; PULMONARY ARTERY; PULMONARY VEINS; TELANGIECTASIA, HEREDITARY HEMORRHAGIC;

EID: 38349175012     PISSN: 00406376     EISSN: None     Source Type: Journal    
DOI: 10.1136/thx.2007.076109     Document Type: Article

References (10)

1. Simmoneau G, Galiè N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004;43(Suppl S):5-12.
2. Barst RJ. Recent advances in the treatment of pediatric pulmonary artery hypertension. Pediatr Clin North Am 1999;46:331-45.
3. Harrison RE, Berger R, Haworth SG, et al. Transforming growth factor-β receptor mutations and pulmonary arterial hypertension in childhood. Circulation 2005;111:435-41.
4. Pietra GG, Capron F, Stewart S, et al. Pathologic assessment of vasculopathies in pulmonary hypertension. J Am Coll Cardiol 2004;43(Suppl S):25-32.
5. Rosenzweig EB, Ivy DD, Widlitz A, et al. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol 2005;46:697-704.
6. Bonderman D, Nowotny R, Skoro-Sajer N, et al. Bosentan therapy for pulmonary arterial hypertension associated with hereditary haemorrhagic telangiectasia. Eur J Clin Invest 2006;36(Suppl 3):71-2.
7. Rosenzweig EB, Widlitz AC, Barst RJ. Pulmonary arterial hypertension in children. Pediatr Pulmonol 2004;38:2-22.
8. Shovlin CL, Letarte M. Hereditary haemorrhagic telangiectasia and pulmonary arteriovenous malformations: issues in clinical management and review of pathogenic mechanisms. Thorax 1999;54:714-29.
9. Harrison RE, Flanagan JA, Sankelo M, et al. Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia. J Med Genet 2003;40:865-71.
10. Chaouat A, Coulet F, Favre C, et al. Endoglin germline mutation in a patient with hereditary haemorrhagic telangiectasia and dexfenfluramine associated pulmonary arterial hypertension. Thorax 2004;59:446-8.