Abstract
BackgroundThe 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 (DLCO) and nitric oxide (DLNO) 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.MethodsSixty 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 DLCO/DLNO, arterial blood gas at rest, contrast echocardiography and enhanced computed tomography scan of the liver and chest the day of pulmonary function testing.ResultsWe 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.ConclusionVc/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.
Highlights
The severity of Hereditary Hemorrhagic Telangiectasia (HHT) or Rendu-Osler-Weber disease is generally related to vascular visceral involvement represented by arteriovenous malformations (AVMs)
Assuming that DLNO represents the alveolar membrane component of gas transfer, and that DLCO depends on Alveolo-capillary membrane conductance (Dm), Pulmonary capillary blood volume (Vc), hemoglobin concentration and pulmonary capillary oxygen tension, the DLNO/DLCO ratio might differ between patients with alveolocapillary membrane modifications and patients with microvascular disease
DLNO/DLCO ratio was increased in HHT patients (p = 0.01) and in P + liver AVMs (LAVMs) patients (p = 0.01) versus controls but did not differ between patients groups (Table 1)
Summary
The severity of Hereditary Hemorrhagic Telangiectasia (HHT) or Rendu-Osler-Weber disease is generally related to vascular visceral involvement represented by arteriovenous malformations (AVMs). The aim of our study was to compare diffusing capacity of the lung for carbon monoxide (DLCO) and nitric oxide (DLNO) and its 2 components Dm and Vc, in HHT patients with PAVMs, PAVMs and liver AVMs (LAVMs), LAVMs without PAVM, no PAVM and LAVM, and controls. The severity of Hereditary Hemorrhagic Telangiectasia (HHT) disease is generally related to vascular visceral involvement represented by arteriovenous malformations (AVMs). The aim of our study was to compare the diffusing capacity of the lung for carbon monoxide (DLCO) and nitric oxide (DLNO) 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
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