| Pulmonary capillary haemangiomatosis coexistence with sinus venosus ASD: morphometric analysis and literature review. | |
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MedLine Citation:
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PMID: 9701445 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A 24 yr old white female presented with dyspnoea, orthopnoea, paroxysmal nocturnal dyspnoea, cough and fatigue. Transthoracic echocardiography revealed a sinus venosus atrial septal defect (ASD). Right heart catheterization confirmed severe pulmonary hypertension (80/37 mmHg). A chest radiograph showed enlarged pulmonary arteries with peripheral pruning. Surgical repair of the ASD and lung biopsy were performed. Two days later, she developed right heart failure and was treated with inhaled nitric oxide and then a calcium channel blocker. She failed to improve and was readmitted three months later with severe right heart failure and progressive dyspnoea. While waiting for lung transplantation, she developed haematochezia and died. Light microscopy of lung biopsy and autopsy tissue revealed the structural changes of pulmonary hypertension and focal increases in congested pulmonary capillaries consistent with the diagnosis of pulmonary capillary haemangiomatosis. Quantitative analysis demonstrated that the pathological changes were rapidly progressive. |
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Authors:
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B S Slovis; I Chazova; J E Loyd; B O Meyrick |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.; Review |
Journal Detail:
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Title: The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology Volume: 12 ISSN: 0903-1936 ISO Abbreviation: Eur. Respir. J. Publication Date: 1998 Jul |
Date Detail:
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Created Date: 1998-11-06 Completed Date: 1998-11-06 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 8803460 Medline TA: Eur Respir J Country: DENMARK |
Other Details:
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Languages: eng Pagination: 240-4 Citation Subset: IM |
Affiliation:
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Division of Pulmonary and Critical Care Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Capillaries / pathology Fatal Outcome Female Heart Septal Defects, Atrial / complications*, pathology Hemangioma, Capillary / complications*, pathology Humans Hypertension, Pulmonary / complications, pathology Lung / pathology Lung Neoplasms / complications*, pathology Pulmonary Artery / pathology |
| Grant Support | |
ID/Acronym/Agency:
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HL 07123/HL/NHLBI NIH HHS; HL 48164/HL/NHLBI NIH HHS; HL 48536/HL/NHLBI NIH HHS |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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