Right ventricular regional function using MR tagging: Normals versus chronic pulmonary hypertension

Right ventricular (RV) regional function, in both normal and diseased states, is not well characterized. Using 1D MR myocardial tagging, RV and septal intramyocardial segmental shortening was noninvasively measured in ten healthy subjects and in seven patients with chronic pulmonary hypertension. The normal RV free wall regional shortening was not uniform. A pattern of increasing RV free wall short-axis shortening was found from the RV outflow tract to the RV apex, and a more complex pattern of RV free wall long-axis shortening was observed. Both regional short- and long-axis shortening were globally reduced in pulmonary hypertension patients, with the greatest decreases in the RV outflow tract and in the basal septal wall region. Regional RV function can be quantitatively evaluated using MR tagging to determine the impact of chronic pulmonary hypertension on RV performance.     

CT angiography findings of the left atrium and right ventricle in patients with massive pulmonary embolism

OBJECTIVE: The purpose of this study was to show the imaging findings of the left atrium and right ventricle on CT angiography in patients with massive pulmonary embolism. CONCLUSION: Massive pulmonary embolism can cause abrupt acute pulmonary arterial hypertension, right ventricular dysfunction, and decrease in left ventricular preload. Patients with these findings on CT angiography can have a poorer prognosis than those without these imaging findings. Consequently, recognizing anatomic changes such as right ventricular dilation or septal bowing, decrease in size of left atrium and pulmonary veins (a manifestation of decreased pulmonary venous return) would be useful for risk stratification at the time of massive pulmonary embolism.     

Patients with pulmonary fibrosis: cardiac function assessed with MR imaging

PURPOSE: To detect abnormalities in cardiac function by using magnetic resonance (MR) imaging in patients with mild to moderate pulmonary fibrosis and to evaluate the relationship between pulmonary function and cardiac function. MATERIALS AND METHODS: Sixteen patients were compared with 16 sex- and age-matched healthy control subjects. Systolic function was assessed by using multisection multiphase cine MR imaging. Diastolic function was assessed with flow-sensitive MR imaging across the mitral and tricuspid valves. MR imaging results were compared with the severity of impairment in pulmonary function. RESULTS: Biventricular systolic function and left ventricular diastolic function were normal in patients, but right ventricular diastolic function was significantly impaired versus that of control subjects, with a ratio of peak flow during early diastolic (E) filling to peak flow during atrial contraction (A) of 0.85 +/- 0.40 versus 1.28 +/- 0.50 (P =.035). Biventricular E/A ratios were strongly correlated to age in patients and control subjects. The right ventricular E/A ratio in patients corresponded with values that are normally expected in people 20 years older. Diastolic left and right ventricular functions were significantly correlated with each other. There was no relationship between pulmonary function and cardiac function. CONCLUSION: Impairment of right ventricular diastolic function was found by using MR imaging in patients with mild to moderate pulmonary fibrosis, whereas left ventricular diastolic function and biventricular systolic function were preserved.     

Multistage ethanol sclerotherapy of soft-tissue arteriovenous malformations: effect on pulmonary arterial pressure

PURPOSE: To retrospectively investigate how repeat injections of absolute ethanol in therapeutic doses, required for multisession sclerotherapy of large high-flow soft-tissue arteriovenous malformations (AVMs) in patients with normal cardiopulmonary function, affect pulmonary arterial pressure (PAP). MATERIALS AND METHODS: Study received approval and waiver of informed consent by institutional review board and was conducted in 16 male and 16 female patients with AVMs who underwent repeat sclerotherapy (142 sessions total) with absolute ethanol from July 1997 to December 2003. PAPs were monitored during first session in all patients. In subsequent sessions, PAP was monitored with pulmonary catheter when predicted single dose of ethanol exceeded 3 mL and total amount exceeded 0.25 mL/kg. PAP was measured in 104 sessions. Serum ethanol levels from blood samples obtained at end of each session were reviewed. PAP parameters were analyzed at beginning and end of each session, and highest value was recorded to assess any increase after repeat therapy. Difference between initial and highest PAP values recorded in a session (Delta(max)) was noted to determine any increase during repeat sessions. Possible relationship was reviewed between this value and amount of ethanol used. For sessions without PAP monitoring, mixed model was used for statistical analysis. RESULTS: Total ethanol used was variable. In 43 sessions, highest mean PAP exceeded 25 mm Hg. Incidence of sustained pulmonary hypertension (mean PAP > 25 mm Hg) at end of each session was 30.8% (32 of 104 sessions). Initial PAP parameters did not increase or decrease during repeat sessions. No significant changes in Delta(max) of systolic and mean PAP were observed with increasing number of sessions. Rather, Delta(max) of diastolic PAP was reduced after repeat sessions (P = .03). There was no significant correlation between serum ethanol level and PAP parameters at end of sessions. Relationships between Delta(max) values of systolic, mean, and diastolic PAP and total ethanol used were not significant. CONCLUSION: High incidence of acute pulmonary hypertension was observed in each sclerotherapy session without lasting effect on PAP.     

Quantification of the curvature and shape of the interventricular septum.

The interventricular septum (IVS) occupies a unique position within the heart, lying between the left (LV) and right (RV) ventricular cavities. Changes in its normal geometry may signify not only abnormalities of the septal myocardium, but also abnormal pressure differences between the LV and RV. Flattening of the IVS has been noted with cross-sectional imaging in association with pulmonary hypertension, but the septal curvature and shape have not previously been measured in three dimensions. This paper describes a method to model the RV surface of the IVS from spatially registered cross-sectional images for measurements of curvature. A smoothing 2D spline surface is constructed through the RV septal surface at regular times during the cardiac cycle, and the principal curvatures, as well as the Gaussian and mean curvatures, shape index, and curvedness, are calculated. Vector and color surface maps and graphs of average curvature and shape indices are constructed. Consistent curvature patterns were observed in four normal subjects. This method of measuring septal geometry can provide potentially useful new information on the effects of RV disease. We examine the problem of describing septal motion, and describe a simple measure of septal curvature that may be of clinical value.     

A comparison of noninvasive MRI-based methods of estimating pulmonary artery pressure in pulmonary hypertension

PURPOSE: To assess the accuracy of several noninvasive MRI-based estimators of pulmonary artery pressure by comparing them with invasive pressure measurement. MATERIALS AND METHODS: We compared five MRI methods with invasive pressure measurement by catheterization, in one group of pulmonary hypertension (PH) patients. Doppler echocardiography was included as a reference method. Main inclusion criterion was a mean pulmonary artery pressure above 25 mmHg at catheterization. MRI velocity quantification was used to obtain pulmonary flow acceleration and ejection times, and pulse wave velocity. The ventricular mass index was also assessed on MRI. Two commercially available 1.5-T systems were used for this study. RESULTS: Data from 44 patients were analyzed. Correlation of acceleration time with mean pressure was: r = -0.21, P = 0.21, correlation of the acceleration/ejection time ratio with systolic pressure was: r = -0.26, P = 0.01. The ventricular mass index showed the best correlation with mean pressure, with r = 0.56, P < 0.001. Using the pulse wave velocity and the cross-sectional area of the pulmonary artery, the mean pressure could not be estimated accurately. CONCLUSION: Accurate estimation of pulmonary artery pressure in PH patients was not feasible by the MRI estimators studied. These noninvasive methods cannot replace right heart catheterization at this moment.     

Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease

OBJECTIVE: Pulmonary venoocclusive disease is a rare cause of pulmonary hypertension that is often difficult to distinguish from severe primary pulmonary hypertension. Unfortunately, medical treatment of primary pulmonary hypertension with prostacyclin can be fatal in patients with venoocclusive disease, and an early pretreatment diagnosis of this uncommon condition is critical. The aim of our study was to evaluate this disease noninvasively using CT of the chest. MATERIALS AND METHODS: We reviewed cross-referenced records from 1996 to 2001 in our departments of radiology and pathology and identified 15 patients with initial pretreatment CT scans who had pathologically confirmed pulmonary venoocclusive disease. Their CT scans were compared with the CT scans of 15 consecutive patients with pathologically confirmed primary pulmonary hypertension. All patients had undergone a postmortem or posttransplantation examination. RESULTS: Ground-glass opacities were significantly more frequent in pulmonary venoocclusive disease (p = 0.003); the opacities were abundant with random zonal predominance and preferentially centrilobular distribution (p = 0.03). Subpleural septal lines and adenopathy were also significantly more frequent (p < 0.0001). CONCLUSION: On the initial pretreatment chest CT scan, the presence of ground-glass opacities (particularly with a centrilobular distribution), septal lines, and adenopathy are indicative of pulmonary venoocclusive disease in patients displaying pulmonary hypertension. Caution should be exercised before vasodilator therapy is initiated in the patients whose scans show such radiologic abnormalities.     

Early onset of retrograde flow in the main pulmonary artery is a characteristic of pulmonary arterial hypertension

Purpose:

To evaluate if early onset of retrograde flow in the main pulmonary artery is a characteristic of pulmonary arterial hypertension (PAH).

Materials and Methods:

Fifty‐five patients with suspected pulmonary hypertension (PH) underwent right‐sided heart catheterization and retrospectively ECG‐gated MR phase‐contrast velocity quantification in the main pulmonary artery. Pulmonary hypertension was defined by a mean pulmonary artery pressure being larger than 25 mmHg. The onset time of the retrograde flow relative to the cardiac cycle duration (Relative Onset Time = ROT) was compared with mean pulmonary artery pressure.

Results:

By the catheterization, 38 patients were identified as having PAH. The ROT for these PAH patients was significantly different from those found in the 17 non‐PH subjects (0.14 ± 0.06 versus 0.37 ± 0.06, P < 0.001). The mean pulmonary artery pressure was related to the ROT (r² = 0.62, P < 0.001) and could be estimated from the ROT with a standard deviation of 11.7 mmHg. With a cutoff value of 0.25, the ROT distinguished PAH patients from non‐PH subjects.

Conclusion:

Early onset of retrograde flow in the main pulmonary artery is a characteristic of pulmonary arterial hypertension and is visible by standard MR phase‐contrast velocity quantification. J. Magn. Reson. Imaging 2011;33:1362–1368. © 2011 Wiley‐Liss, Inc.     

Pre-operative evaluation with MR in tetralogy of fallot and pulmonary atresia with ventricular septal defect

Purpose: To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule.Material and Methods: Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5±4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction.Results: There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supravalvular stenosis, but the agreement was somewhat lower for the subvalvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the subvalvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts.Conclusion: Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD.     

Impaired systemic oxygen extraction at maximum exercise in pulmonary hypertension

PURPOSE: To determine the relative contributions of the Fick principle variables to impaired exercise tolerance in pulmonary arterial hypertension compared with pulmonary venous hypertension. METHODS: One hundred forty-seven consecutive, complete, clinically indicated cardiopulmonary exercise tests done with radial and pulmonary arterial catheters and radionuclide ventriculographic scanning were screened for an exercise limit attributable to 1) pulmonary arterial hypertension (N = 34), 2) left ventricular systolic (N = 23), or 3) diastolic dysfunction (N = 36), defined by mean pulmonary artery pressure, pulmonary capillary wedge pressure, and left ventricular ejection fraction. Systolic and diastolic dysfunction are together referred to as pulmonary venous hypertension. Patients with other limits were excluded, including those with a pulmonary mechanical limit. For the resulting 93 exercise tests, the Fick principle variables' contributions to a depressed maximum oxygen consumption were compared by ANOVA and ANCOVA. RESULTS: Maximum oxygen consumption (54.5 +/- 15.5 vs 73.2 +/- 20.1 vs 66.0 +/- 15.7% predicted) and oxygen delivery (1457 +/- 456 vs 2161 +/- 824 vs 2007 +/- 665 mL x min(-1)) were reduced in systolic dysfunction versus both diastolic dysfunction and pulmonary arterial hypertension, respectively (P < 0.05 by ANOVA). Maximum systemic oxygen extraction ratio was highest in systolic dysfunction, intermediate in diastolic dysfunction, and lowest in pulmonary arterial hypertension (0.744 +/- 0.091 vs 0.680 +/- 0.091 vs 0.619 +/- 0.113, respectively, P < 0.05 among all groups). Systemic oxygen extraction at peak exercise was inversely related to maximum cardiac output in pulmonary arterial hypertension, but it was blunted versus systolic dysfunction throughout the range of peak cardiac outputs (P < 0.05 by ANCOVA). CONCLUSIONS: Maximum systemic oxygen extraction is impaired in pulmonary arterial versus pulmonary venous hypertension and contributes to the exercise limit.     

Quantitative analysis of lung perfusion in patients with primary pulmonary hypertension.

The aim of this study was to evaluate quantitatively the heterogeneity of lung perfusion scans in patients with primary pulmonary hypertension (PPH) and to compare it with the severity of disease. METHODS: Lung perfusion scans were obtained on 22 patients with PPH and 12 age-matched control subjects. The perfused area rates (PARs) were calculated by dividing the lung area in each 10% threshold width from 10% to 100% of maximal counts by total lung area. The total absolute difference in the PAR between each patient and the mean control value was assumed as the perfusion index of the lung (P index). The P index was compared with hemodynamic parameters and the right ventricular ejection fraction (RVEF), including 7 patients who received long-term vasodilator therapy. RESULTS: The P index correlated significantly with mean pulmonary arterial pressure (P < 0.001) and RVEF (P < 0.05). In patients with vasodilator therapy, the P index was improved significantly after therapy (P < 0.05) and was associated with a reduction in pulmonary arterial pressure. CONCLUSION: Quantitative assessment of lung perfusion irregularity might provide useful information about the severity of disease and the effect of therapy in addition to the routine visual representation.     

Chronic thromboembolic pulmonary hypertension: pre- and postoperative assessment with breath-hold MR imaging techniques

PURPOSE: To evaluate the potential of breath-hold magnetic resonance (MR) imaging techniques in morphologic and functional assessment of patients with chronic thromboembolic pulmonary hypertension (CTEPH) before and after surgery. MATERIALS AND METHODS: Thirty-four patients with CTEPH were examined before and after pulmonary thromboendarterectomy (PTE). For morphologic assessment, contrast material-enhanced MR angiography was used; for assessment of hemodynamics, velocity-encoded gradient-echo sequences and cine gradient-echo sequences along the short axis of the heart were performed. Contrast-enhanced MR angiography was compared with selective digital subtraction angiography (DSA) for depiction of central thromboembolic material and visualization of the pulmonary arterial tree. Functional analysis included calculation of left and right ventricular ejection fractions and peak velocities, net forward volumes per heartbeat, and blood volume per minute in the left and right pulmonary arteries and ascending aorta. Flow measurements were compared with invasively measured mean pulmonary arterial pressure (MPAP) and pulmonary vascular resistance (PVR) measurements. Nonparametric Wilcoxon and sign tests were used for statistical analysis. RESULTS: MR angiography revealed typical findings of CTEPH (intraluminal webs and bands, vessel cutoffs, and organized central thromboemboli) in all patients. It depicted pulmonary vessels up to the segmental level in all cases. For subsegmental arteries, DSA revealed significantly more patent vessel segments than did MR angiography (733 versus 681 segments, P <.001). MR angiography revealed technical success of surgery in 33 of 34 patients. Patients had reduced right ventricular ejection fractions and pulmonary peak velocities that significantly increased after PTE (P <.001 for both). Right ventricular ejection fraction had good correlation with PVR (r = 0.6) and MPAP (r = 0.7). The postoperative decrease in MPAP correlated well with the increase in right ventricular ejection fraction (r = 0.8). Postoperatively, there was complete reduction of a preoperatively existing bronchosystemic shunt volume in 33 of 34 patients. CONCLUSION: Breath-hold MR imaging techniques enable morphologic and semiquantitative functional assessment of patients with CTEPH.     

右室组织多普勒Tei指数评价室间隔缺损右室功能的研究

目的探讨组织多普勒（TDI）Tei指数评价室间隔缺损患者右心室功能的价值。方法根据患者肺动脉收缩压（PASP）水平,将56例室间隔缺损患者分为合并肺动脉高压[PH（＋）]组（33例,PASPI〉40mmHg）和未合并肺动脉高压[PH（-）]组（23例,PASP〈40mmHg）;选取25例健康体检者为正常对照组。利用超声TDI技术获得三尖瓣环组织运动频谱,计算研究对象的右室Tei指数。结果PH（＋）组Tei指数明显高于对照组及PH（-）组（P〈0．05）;PH（＋）组IVRT明显大于PH（-）组（P〈0．05）;与对照组比较,PH（-）组IVRT、IVCT、ET及右室Tei指数均无显著差异（P〉0．05）。TDI测得右室Tei指数与PASP呈正相关（P〈O．01）。结论室间隔缺损合并肺高压患者右室整体功能受损,TDI．Tei指数可较准确反映室间隔缺损患者右室功能。     

Tei指数评价高海拔地区肺动脉高压患者右心功能的临床研究

目的：探讨Tei指数评价高海拔地区肺动脉高压患者右心功能的临床价值。方法：对西宁地区48例肺动脉高压患者和33例正常人,行超声检查,测量右房横径、右室横径、右室前壁厚度、肺动脉主干内径根据三尖瓣返流和肺动脉瓣返流估测肺动脉收缩压、肺动脉舒张压、肺动脉平均压,并计算右心Tei指数。结果：肺动脉高压患者与对照组右心Tei指数有显著统计学差异,不同肺动脉压力组之间右心Tei也有统计学差异。结论：右心Tei指数是反映右心整体功能较敏感的指标。     

Non-invasive measurement of pulmonary artery pressure in humans with simulated altitude-induced venous gas emboli

BACKGROUND: Decompression to simulated altitude causes super-saturation of nitrogen desolved in body tissues and can result in venous gas emboli (VGE), which are usually "cleared" in the lung. Large intravenous boli of air administered to animals increase pulmonary artery pressure (PAP), and may induce cross-over of gas to the left side of the heart (creating dangerous arterial gas emboli). This study was conducted to determine whether high VGE grades induced at simulated altitude elevate PAP in humans. METHODS: Eight human subjects with subclinical tricuspid regurgitation were exposed to simulated altitude of > or = 24,000 ft (7315 m) for up to 4 h. Systolic PAP was derived from Doppler ultrasound echo imaging measurements of peak flow velocity of the regurgitant jet. VGE was rated using the Spencer scale. A technique of "bubble titration" was employed with changes in exercise and altitude to maintain sufficient bubbles without decompression sickness. RESULTS: All subjects developed grade III-IV VGE, but 3 developed decompression sickness, resulting in earlier termination. Pre-exposure systolic PAP averaged 24.4 +/- 1.3 (SE) mm Hg. After 1, 2, 3 and 4 h, systolic PAP was 23.7 +/- 1.2 (n = 8), 23.4 +/- 1.1 (n = 8), 23.3 +/- 1.0 (n = 6), and 25.9 +/- 0.6 (n = 5) mm Hg, respectively. Systolic PAP remained unchanged, in spite of bubble grades III-IV for up to 4 h. CONCLUSION: As systolic PAP did not increase with hypobaric exposures that created substantial VGE, the bubble loading was not sufficient to overwhelm the lung clearing capacity. The risk of high PAP resulting in VGE cross-over is low during typical operational altitude exposures.     

Pulmonary hypertension: pulmonary flow quantification and flow profile analysis with velocity-encoded cine MR imaging.

Velocity-encoded cine magnetic resonance (MR) imaging provides two-dimensional velocity maps of a cross-sectional area of a vessel. Pulmonary flow and flow patterns in the main pulmonary artery were analyzed with velocity-encoded cine MR imaging and Doppler echocardiography in 10 patients with pulmonary hypertension (PH), one patient with a dilated main pulmonary artery, and 10 healthy subjects, and these findings were compared. Peak systolic velocity measured with velocity-encoded cine MR imaging was similar to that measured with Doppler echocardiography in healthy subjects and in patients with PH. Velocity-encoded cine MR imaging demonstrated substantial differences in velocity across the vascular lumen in PH. The flow pattern in healthy subjects was different than that in patients with PH; the latter had lower peak systolic velocity and greater retrograde flow after middle to late systole. The retrograde flow observed in patients with PH reflected hemodynamic events, since it was inversely proportional to pulmonary flow volume and directly proportional to pulmonary resistance and cross-sectional area of the vessel. Velocity-encoded cine MR imaging demonstrates an inhomogeneous flow profile in PH and may serve as a noninvasive method to estimate pulmonary vascular resistance.     

Perfusion lung scintigraphy in primary pulmonary hypertension

Fifteen cases with primary pulmonary hypertension (PPH) were classified into two groups by using the perfusion lung scan pattern. Eight cases had multiple, small, ill-defined defects (mottled pattern), and remaining seven cases had no mottled pattern. These two groups were compared with mean pulmonary arterial pressure (mean PAP), right ventricular ejection fraction (RVEF), blood gas at room air (PaO2), and alveolar-arterial O2 difference (A-aDo2). The cases with mottled pattern showed a significant increase in mean PAP. There were no significant differences in RVEF, PaO2, and A-aDo2, between the groups. The survival rate of the patients with mottled pattern was significantly lower than that without mottled pattern (p less than 0.05). We concluded that perfusion lung scan is very useful for evaluation of the prognosis in primary pulmonary hypertension.     

MR imaging of pulmonary arterial hypertension and pulmonary emboli

Intraluminal signal in the pulmonary arteries on spin-echo, ECG-gated MR images is limited to the diastolic phase of the cardiac cycle in normal subjects. Initial experience has indicated that signal persisting during systole may be characteristic of slow blood flow associated with pulmonary arterial hypertension (PAH) or of thrombotic material secondary to pulmonary embolism. This study analyzes our cumulative experience (31 patients) with multiphasic, double spin-echo MR for assessing PAH and/or suspected pulmonary embolism. In PAH, the abnormal systolic signal showed an intensity increase from first to second echo. This pattern was observed in 92% of PAH patients, including 100% of patients with pulmonary systolic pressures greater than or equal to 80 mm Hg and 60% of patients with pressures less than 80 mm Hg. At any focus in the pulmonary arteries, such signal disappeared at some phase of the cardiac cycle. In patients with pulmonary embolism, signal from thrombus was fixed throughout the cardiac cycle and showed little or no increase in relative intensity change from first- to second-echo image. Using this guideline, MR made six confirmed positive and four confirmed negative diagnoses of proximal pulmonary embolism, while it failed to identify thrombus in the one patient with a peripheral pulmonary embolism. Intraluminal signal in the pulmonary arteries caused by PAH or pulmonary embolism can be differentiated in most instances using multiphasic, double spin-echo, ECG-gated MR. However, at its current stage of development, the procedure does not appear to be useful for the evaluation of peripheral pulmonary embolism.     

斑点追踪技术评价肺动脉高压患者右心室舒张功能的研究

目的：应用斑点追踪成像技术评价肺动脉高压患者右心室舒张功能,并与常规多普勒超声心动对比研究。方法：肺动脉高压患者31例,正常对照组33例,二维斑点追踪成像技术获得右心室各个节段舒张期纵向应变率及运动速度。结果：右心室游离壁及室间隔各段舒张早期应变率（SRe）有显著性差异（P〈0．05）;右室游离壁及室间隔舒张早期应变率（SRe）平均值与右室游离壁厚度、肺动脉收缩压相关性较好。结论：舒张早期应变率与右室游离壁厚度及肺动脉收缩压相关性较好,二维斑点追踪成像技术为评价肺动脉高压右室舒张功能提供了一种可行的方法。     

肺泡蛋白沉着症X线胸片及高分辨率CT表现与肺功能的关系

目的 探讨肺泡蛋白沉着症X线胸片及胸部高分辨率CT(HRCT)影像表现及其与肺功能检查的相关性.方法 回顾性分析19例肺泡蛋白沉着症患者的X线胸片、胸部HRCT及肺功能检查[肺总量、一氧化碳弥散量(DLCO)、第一秒用力肺活量(FEV1)及一秒率(FEV1/FVC)],根据肺部阴影的程度、范围、严重程度及网格阴影范围进行影像学评分,将X线胸片及HRCT的影像评分与肺功能指标进行相关性分析.结果 X线胸片以双侧对称的自肺门向外放射的"蝶翼征"表现为主,胸部HRCT特征性的表现为两肺弥漫性分布的斑片状磨玻璃样气腔实变影,伴小叶间隔增厚及网格影,这种"铺路石"样病变呈地图样分布.19例患者的肺功能结果 为DLCO占预计值百分比:(54.49±16.78)%;FEV1/FVC:(86.00±6.70)%.X线胸片的范围评分及严重度评分与DLCO占预计值百分比呈负相关(r=-0.661,P＜0.01;r=-0.475,P＜0.05).HRCT严重度评分为(16.47±5.26)分,在HRCT的各项评分中严重度评分与限制性通气功能障碍(FEV1/FVC升高)及弥散功能障碍(DLCO占预计值百分比下降)的相关性最好(DLCO占预计值百分比r=-0.809,P＜0.01;FEV1/FVC r=0.573,P＜0.05);HRCT的范围评分比程度评分与肺功能的相关性更好,而HRCT的网格范围评分只与弥散功能障碍相关.结论 肺泡蛋白沉着症X线胸片、胸部HRCT与肺功能相关性良好,HRCT比X线胸片更能准确反映肺功能受损情况.