Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Mache, CJ; Gamillscheg, A; Popper, HH; Haworth, SG.
Early-life pulmonary arterial hypertension with subsequent development of diffuse pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia type 1.
THORAX. 2008; 63(1): 85-86.
Doi: 10.1136/thx.2007.076109
(- Case Report)
[OPEN ACCESS]
Web of Science
PubMed
FullText
FullText_MUG
Google Scholar
- Führende Autor*innen der Med Uni Graz
- Mache Christoph
- Co-Autor*innen der Med Uni Graz
- Gamillscheg Andreas
- Popper Helmuth
- Altmetrics:
- Dimensions Citations:
- Plum Analytics:
- Scite (citation analytics):
- Abstract:
- The case history is presented of a male infant who was thought to have idiopathic pulmonary arterial hypertension (PAH) at 3 months of age. Subsequently the PAH decreased unexpectedly and diffuse pulmonary arteriovenous malformations (PAVMs) were seen at 6.9 years of age for the first time. Hereditary haemorrhagic telangiectasia type 1 (HHT1) related to an endoglin mutation was diagnosed. At 10.3 years of age a lung biopsy showed diffuse PAVMs as well as pulmonary arteriopathy with medial hypertrophy. This is the first case of HHT1 presenting with PAH at such a young age. The subsequent decrease in pulmonary arterial pressure (PAP) was probably caused by the development of PAVMs. In the presence of PAVMs, measurement of the PAP may underestimate the extent of PAH-related vasculopathy.
- Find related publications in this database (using NLM MeSH Indexing)
- Arteriovenous Malformations - pathology
- Child -
- Humans -
- Hypertension, Pulmonary - pathology
- Infant -
- Male -
- Pulmonary Artery - abnormalities
- Pulmonary Veins - abnormalities
- Telangiectasia, Hereditary Hemorrhagic - pathology