Pulmonary Capillary Hemangiomatosis: The Role of Invasive Cardiopulmonary Exercise Testing

DuBrock, Hilary M.; Kradin, Richard L.; Rodriguez-Lopez, Josanna; Channick, Richard N.

doi:10.1086/682227

Cited by 6 publications

(6 citation statements)

References 29 publications

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“…Because of these constraints, pulmonary capillary dysfunction is inferred using indirect means, such as tissue density via computed tomography (CT) or invasive left heart catheterization (7,14). Imaging modalities such as CT and MRI can be used to quantify dynamics in the larger pulmonary vasculature (14) and are used extensively in subjects with pulmonary hypertension (10,12) and interstitial lung disease (49).…”

Section: Introductionmentioning

confidence: 99%

Mapping cardiopulmonary dynamics within the microvasculature of the lungs using dissolved ¹²⁹Xe MRI

Niedbalski

Bier

Wang

et al. 2020

Journal of Applied Physiology

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Magnetic Resonance (MR) imaging and spectroscopy using dissolved hyperpolarized (HP) 129Xe have expanded the ability to probe lung function regionally and non-invasively. In particular, HP 129Xe imaging has been used to quantify impaired gas uptake by the pulmonary tissues. Whole-lung spectroscopy has also been used to assess global cardiogenic oscillations in the MR signal intensity originating from 129Xe dissolved in the red blood cells of pulmonary capillaries. Herein, we show that the magnitude of these cardiogenic dynamics can be mapped 3-dimensionally using radial MRI, because dissolved 129Xe dynamics are encoded directly in the raw imaging data. Specifically, 1-point Dixon imaging is combined with post-acquisition, keyhole image reconstruction to assess regional blood volume fluctuations within the pulmonary microvasculature throughout the cardiac cycle. This "oscillation mapping" was applied in healthy subjects (mean amplitude 9% of total RBC signal) and patients with pulmonary arterial hypertension (PAH, mean 4%) and idiopathic pulmonary fibrosis (IPF, mean 14%). Whole-lung mean values from these oscillation maps correlated strongly with spectroscopy and clinical pulmonary function testing, but exhibited significant regional heterogeneity, including gravitationally dependent gradients in healthy subjects. Moreover, regional oscillations were found to be sensitive to disease state. Greater percentages of the lungs exhibit low-amplitude oscillations in PAH patients, and longitudinal imaging shows high-amplitude oscillations increase significantly over time (4-14 months, p = 0.02) in IPF patients. This technique enables regional dynamics within the pulmonary capillary bed to be measured, and in doing so, provides insights into the origin and progression of pathophysiology within the lung microvasculature.

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Section: Introductionmentioning

confidence: 99%

Mapping cardiopulmonary dynamics within the microvasculature of the lungs using dissolved ¹²⁹Xe MRI

Niedbalski

Bier

Wang

et al. 2020

Journal of Applied Physiology

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“…The lack of normal capillary structure would also affect both pulmonary hemodynamics and gas exchange. These abnormal capillaries have been reported in several conditions with a pathologic picture reminiscent of the PCH‐PVOD spectrum of disease and are often associated with clinically progressive PH and hypoxemia .…”

Section: Discussionmentioning

confidence: 85%

Capillary Proliferation in Systemic‐Sclerosis‐Related Pulmonary Fibrosis: Association with Pulmonary Hypertension

Seki

Anklesaria

Saggar

et al. 2019

ACR Open Rheumatology

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ObjectiveWe sought to determine if any histopathologic component of the pulmonary microcirculation can distinguish systemic sclerosis (SSc)‐related pulmonary fibrosis (PF) with and without pulmonary hypertension (PH).MethodsTwo pulmonary pathologists blindly evaluated 360 histologic slides from lungs of 31 SSc‐PF explants or autopsies with (n = 22) and without (n = 9) PH. The presence of abnormal small arteries, veins, and capillaries (pulmonary microcirculation) was semiquantitatively assessed in areas of preserved lung architecture. Capillary proliferation (CP) within the alveolar walls was measured by its distribution, extent (CP % involvement), and maximum number of layers (maximum CP). These measures were then evaluated to determine the strength of their association with right heart catheterization–proven PH.ResultsUsing consensus measures, all measures of CP were significantly associated with PH. Maximum CP had the strongest association with PH (P = 0.013; C statistic 0.869). Maximum CP 2 or more layers and CP % involvement 10% or greater were the optimal thresholds that predicted PH, both with a sensitivity of 56% and specificity of 91%. The CP was typically multifocal rather than focal or diffuse and was associated with a background pattern of usual interstitial pneumonia. There was a significant but weaker relationship between the presence of abnormal small arteries and veins and PH.ConclusionIn the setting of advanced SSc‐PF, the histopathologic feature of the pulmonary microcirculation best associated with PH was capillary proliferation in architecturally preserved lung areas.

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“… 1 This hemodynamics clearly shows progression of disease over the last three years. Dubrock et al 16 reported three cases of unexplained dyspnea where cardiopulmonary exercise testing suggested pulmonary vascular disease. Subsequent lung biopsy confirmed PCH.…”

Section: Discussionmentioning

confidence: 99%

Progression of pulmonary veno‐occlusive disease without pulmonary hypertension

et al. 2022

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Pulmonary veno‐occlusive disease (PVOD) is a progressively fatal disease with no definitive treatment options. PVOD can be a result of genetic mutation but can also be due secondary to exposure to solvents or chemotherapeutic agents. Generally, at the time of diagnosis PVOD is associated with hemodynamically confirmed pulmonary hypertension (PH). In this study, we describe a patient who was diagnosed with PVOD early in the disease without hemodynamically confirmed PH. She had histologically confirmed PVOD. Her clinical presentation posed management challenges and prednisone therapy was used to stabilize her disease. This case and some recently published reports highlight possible immune dysregulation in PVOD and role for immuno‐suppressive therapy in these patients.

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Pulmonary Capillary Hemangiomatosis: The Role of Invasive Cardiopulmonary Exercise Testing

Cited by 6 publications

References 29 publications

Mapping cardiopulmonary dynamics within the microvasculature of the lungs using dissolved ¹²⁹Xe MRI

Mapping cardiopulmonary dynamics within the microvasculature of the lungs using dissolved ¹²⁹Xe MRI

Capillary Proliferation in Systemic‐Sclerosis‐Related Pulmonary Fibrosis: Association with Pulmonary Hypertension

Progression of pulmonary veno‐occlusive disease without pulmonary hypertension

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Pulmonary Capillary Hemangiomatosis: The Role of Invasive Cardiopulmonary Exercise Testing

Cited by 6 publications

References 29 publications

Mapping cardiopulmonary dynamics within the microvasculature of the lungs using dissolved 129Xe MRI

Mapping cardiopulmonary dynamics within the microvasculature of the lungs using dissolved 129Xe MRI

Capillary Proliferation in Systemic‐Sclerosis‐Related Pulmonary Fibrosis: Association with Pulmonary Hypertension

Progression of pulmonary veno‐occlusive disease without pulmonary hypertension

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Product

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Mapping cardiopulmonary dynamics within the microvasculature of the lungs using dissolved ¹²⁹Xe MRI

Mapping cardiopulmonary dynamics within the microvasculature of the lungs using dissolved ¹²⁹Xe MRI