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Pulmonary gas exchange in hereditary hemorrhagic telangiectasia patients with liver arteriovenous malformations.
- Source :
-
Respiratory research [Respir Res] 2019 Jul 04; Vol. 20 (1), pp. 137. Date of Electronic Publication: 2019 Jul 04. - Publication Year :
- 2019
-
Abstract
- Background: The severity of Hereditary Hemorrhagic Telangiectasia (HHT) disease is generally related to vascular visceral involvement represented by arteriovenous malformations (AVMs). Pulmonary function tests (PFTs) remain normal in HHT patients without Pulmonary AVMs (PAVMs) and respiratory comorbidity. The aim of our study was to compare the diffusing capacity of the lung for carbon monoxide (D <subscript>LCO</subscript> ) and nitric oxide (D <subscript>LNO</subscript> ) and its 2 components: the pulmonary capillary blood volume (Vc) and the alveolar-capillary membrane conductance (Dm), in HHT patients with PAVMs, PAVMs and liver AVMs (LAVMs), LAVMs without PAVM, no PAVM and LAVM, and controls.<br />Methods: Sixty one consecutive adult patients (HHT without PAVM and LAVM: n = 7; HHT with PAVMs: n = 8; HHT with PAVMs and LAVMs: n = 25; HHT with LAVMs: n = 21) and controls matched for age and sex ratio without respiratory, heart and liver pathology (n = 15) were non-invasively evaluated using PFTs, combined D <subscript>LCO</subscript> /D <subscript>LNO</subscript> , arterial blood gas at rest, contrast echocardiography and enhanced computed tomography scan of the liver and chest the day of pulmonary function testing.<br />Results: We found that patients with LAVMs but without PAVMs exhibited increased Vc/Dm ratio. Interestingly, HHT patients with hepatic artery enlargement showed higher Vc/Dm ratio than HHT patients with normal hepatic artery diameter.<br />Conclusion: Vc/Dm ratio may have practical impact in HHT patients' management to detect precociously the occurrence of LVAMs. However, further studies are needed to assess the accuracy and potential prognostic value of pulmonary gas exchange measurements in HHT patients with LVAMs.
- Subjects :
- Adolescent
Adult
Aged
Arteriovenous Malformations diagnosis
Arteriovenous Malformations epidemiology
Female
Humans
Liver Diseases diagnosis
Liver Diseases epidemiology
Male
Middle Aged
Telangiectasia, Hereditary Hemorrhagic diagnosis
Telangiectasia, Hereditary Hemorrhagic epidemiology
Young Adult
Arteriovenous Malformations physiopathology
Blood Volume physiology
Liver Diseases physiopathology
Pulmonary Gas Exchange physiology
Telangiectasia, Hereditary Hemorrhagic physiopathology
Subjects
Details
- Language :
- English
- ISSN :
- 1465-993X
- Volume :
- 20
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Respiratory research
- Publication Type :
- Editorial & Opinion
- Accession number :
- 31272464
- Full Text :
- https://doi.org/10.1186/s12931-019-1106-y