Abstract
BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) is generally recognized to be caused by persistent organized thrombi that occlude the pulmonary arteries. The aim of this study was to investigate the characteristics of small vessel remodeling and its impact on the hemodynamics in CTEPH patients.Methods and ResultsHemodynamic data were obtained from right heart catheterization in 17 CTEPH patients before pulmonary endarterectomy (PEA). Lung tissue specimens were obtained at the time of PEA. Pathological observations and evaluation of quantitative changes in pulmonary muscular arteries and veins were performed using light microscopy on 423 slides in 17 patients. The relationship between the results and the hemodynamics of CTEPH was investigated. Pulmonary arteriopathy and venopathy were recognized in most cases, although no plexiform lesions and no capillary-hemangiomatosis-like lesions were detected in any of the specimens. The severity of pulmonary arteriopathy was correlated with pulmonary vascular resistance (PVR) in the postoperative and follow-up periods. The PVR and mean pulmonary arterial pressure were significantly higher in the high-obstruction group than in the low-obstruction group. The findings in pulmonary venopathy were similar to the findings seen in pulmonary veno-occlusive disease in some cases, although severe venopathy was only observed in a portion of the pulmonary veins. There was a significant correlation between the extent of pulmonary arteriopathy and venopathy, although an effect of pulmonary venopathy to hemodynamics, including pulmonary arterial wedged pressure (PAWP), could not be identified.ConclusionThe vascular remodeling of the pulmonary muscular arteries was closely associated with the hemodynamics of CTEPH. Severe pulmonary arteriopathy might be related to residual pulmonary hypertension after PEA. Those altered pulmonary arteries might be a new target for the persistent PH after the operation.
Highlights
Chronic thromboembolic pulmonary hypertension (CTEPH) is generally characterized by the presence of persistent intravascular organized thrombi that partially or completely occlude the pulmonary arteries, leading to an elevated pulmonary arterial pressure (Ppa)[1,2,3]
Hemodynamic data were obtained from right heart catheterization in 17 CTEPH patients before pulmonary endarterectomy (PEA)
Pulmonary arteriopathy and venopathy were recognized in most cases, no plexiform lesions and no capillary-hemangiomatosis-like lesions were detected in any of the specimens
Summary
Chronic thromboembolic pulmonary hypertension (CTEPH) is generally characterized by the presence of persistent intravascular organized thrombi that partially or completely occlude the pulmonary arteries, leading to an elevated pulmonary arterial pressure (Ppa)[1,2,3]. The vascular morphologic changes have recently been recognized in areas both with and without proximal thrombi in animal models [13] and clinical investigations [11,12].The small vessel remodeling distal to both obstructed and non-obstructed elastic pulmonary arteries has been demonstrated to play a potential role in the long-term progression of PH in patients with CTEPH, which is the so-called “small vessel disease hypothesis” [1,6]. Chronic thromboembolic pulmonary hypertension (CTEPH) is generally recognized to be caused by persistent organized thrombi that occlude the pulmonary arteries. The aim of this study was to investigate the characteristics of small vessel remodeling and its impact on the hemodynamics in CTEPH patients
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