Classifying Pulmonary Hypertension in Hereditary Hemorrhagic Telangiectasia. Hemodynamics Matter

Roman, Beth L.; Cuttica, Michael J.

doi:10.1164/rccm.201704-0765ed

Cited by 1 publication

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“…Interestingly, one study reported a significant correlation between systolic pulmonary artery pressure and Vc/Dm ratio in patients with chronic infiltrative lung disease and controls [13]. Pulmonary hypertension may develop in HHT patients as a consequence of high output cardiac failure associated with LVAMs [14]. Although right heart catheterization measurements taken as a gold standard were not completed in our study population, transthoracic echocardiography Doppler allows for accurate measurements of the pulmonary circulation [9].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary gas exchange in hereditary hemorrhagic telangiectasia patients with liver arteriovenous malformations

et al. 2019

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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 LCO ) and nitric oxide (D LNO ) 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. 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 LCO /D LNO , arterial blood gas at rest, contrast echocardiography and enhanced computed tomography scan of the liver and chest the day of pulmonary function testing. 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. 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.

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Section: Discussionmentioning

confidence: 99%