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.     

Rupture of a solitary,erosive peripheral pulmonary artery aneurysm

Aneurysms of the pulmonary arteries and their branches are rare. Although they may occur as isolated congenital anomalies, they more frequently are associated with congenital heart disease and pulmonary arterial hypertension. Less common predisposing factors include primary pulmonary hypertension, collagen vascular disease, trauma, and pulmonary banding. Inflammatory or infectious contributing factors include chronic granulomatous infection, infective endocarditis, pneumonia, and the Hughes-Stovin and Behçet's syndromes. Chronic pulmonary arterial hypertension, due to an underlying left-to-right shunt, as well as a superimposed focal bacterial pneumonia were the etiologic factors in the development of the segmental pulmonary artery aneurysm in our patient. The rarity of this constellation of predisposing factors and the diagnostic dilemma posed by the findings in our patient led us to report our case and review the pertinent literature.     

Lung neovascularity in pulmonary arterial hypertension associated with congenital heart defects and idiopathic pulmonary arterial hypertension: study of 198 patients

Objectives

To correlate the severity of lung neovascularity (Sheehan vessels) with the cause and haemodynamic severity of pulmonary arterial hypertension (PAH), pulmonary artery (PA) size and heart disease type in patients with PH associated with congenital heart diseases (PAH-CHD) and idiopathic PH (IPAH).

Methods

We reviewed the HRCT and CT pulmonary angiography studies of 87 patients with PAH-CHD and 111 with IPAH; all had undergone right heart catheterisation. We evaluated the PA size and severity of neovascularity on CT.

Results

Neovascularity, which was found in 72% of PAH-CHD (56% with Eisenmenger’s syndrome) and in 22% of IPAH patients, is significantly related to the severity of PH and all patients with severe neovascularity had intermediate or high PH. All PAH-CHD patients had a dilated PA with a greater risk of developing severe dilatation (diameter >5?cm). The neovascularity correlated with the PA size only in IPAH.

Conclusions

Neovascularity even if not pathognomonic for PAH-CHD, is significantly more common in these patients, especially in Eisenmenger’s syndrome. It is often the first CT sign to indicate the severity of PH in PAH-CHD and IPAH. A neovascularity ≥5 on CT indicates a intermediate or high PH.

Key Points

? Large retrospective studying specific lung disorders in patients with pulmonary arterial hypertension. ? Neovascularity is often the first CT sign indicating the severity of pulmonary hypertension Alterations of lung parenchyma on CT in pulmonary hypertension are described ? The first study to assess the severity of pulmonary hypertension by CT ? If substantiated, CT might eventually replace some cardiac catheterisation for evaluating PH     

血管钠肽对低氧性肺动脉高压和肺动脉平滑肌细胞增殖的影响

目的：研究血管钠肽（VNP）对慢性低氧性肺动脉高压（HPH）大鼠的治疗作用和肺动脉平滑肌细胞（PASMC）增殖的影响,并与C型钠尿肽（CNP）和心房钠尿肽（ANP）作比较。方法：采用整体静脉给药、离体血管灌流和PASMC培养等方法,测定肺血流动力学参数、离体肺动脉的等长张力和PASMC增殖的变化。结果：（1）VNP、CNP和ANP都能有效降低HPH大鼠的平均肺动脉压（mPAP),P＜0．05－0．01,除CNP外,VNP和ANP并能明显降低肺血管阻力（PVR）,P＜0．05－0．01,其中VNP降低mPAP和PVR的作用明显大于CNP和ANP。（2）VNP对离体肺支 浓度依赖性舒张作用,该作用不受内皮细胞的影响;但在浴槽内预先加入格列苯脲或普萘洛尔可显著降低肺动脉对VNP和CNP的最大舒张反应（Rmax),P＜0．05－0．01,而对ANP却无明显影响 。（3）VNP能明显抑制低氧介导的PASMC的增殖及DNA合成,其作用明显大于CNP和ANP。结论：VNP对HPH具有显著的治疗作用,它是一个新型、强效、非内皮依赖性的血管松弛多肽和细胞增殖负调控因子。     

Value of high spatial and high temporal resolution magnetic resonance angiography for differentiation between idiopathic and thromboembolic pulmonary hypertension: initial results

Differentiation between different forms of pulmonary hypertension (PH) is essential for correct disease management. The goal of this study was to elucidate the clinical impact of high spatial resolution MR angiography (SR-MRA) and time-resolved MRA (TR-MRA) to differentiate between patients with chronic thromboembolic PH (CTEPH) and idiopathic pulmonary arterial hypertension (IPAH). Ten PH patients and five volunteers were examined. Twenty TR-MRA data sets (TA 1.5 s) and SR-MRA (TA 23 s) were acquired. TR-MRA data sets were subtracted as angiography and perfusion images. Evaluation comprised analysis of vascular pathologies on a segmental basis, detection of perfusion defects, and bronchial arteries by two readers in consensus. Technical evaluation comprised evaluation of image quality, signal-to-noise ratio (SNR) measurements, and contrast-media passage time. Visualization of the pulmonary arteries was possible down to a subsegmental (SR-MRA) and to a segmental (TR-MRA) level. SR-MRA outperformed TR-MRA in direct visualization of intravascular changes. Patients with IPAH predominantly showed tortuous pulmonary arteries while in CTEPH wall irregularities and abnormal proximal-to-distal tapering was found. Perfusion images showed a diffuse pattern in IPAH and focal defects in CTEPH. TR-MRA and SR-MRA resulted in the same final diagnosis. Both MRA techniques allowed for differentiation between IPAH and CTEPH. Therefore, TR-MRA can be used in the clinical setting, especially in dyspneic patients.     

MRI和心导管检查对肺动脉血流动力学的对比性研究

目的　评价磁共振血流分析技术无创性检测肺动脉血流动力学的价值。材料与方法　应用MR相位速度图测量 45例患者的主肺动脉直径、流速及其最大返流速度并与右心导管检查进行比较。通过直线相关分析评判MRI测量结果的敏感性和特异性。结果　 45例患者中 40例存在返流 ,占 88.89%。 2 5例肺动脉高压 (PAH)组主肺动脉的流速及其肺血管的顺应性均低于非PAH组 ,而返流的百分比也显著增高 (P <0 .0 5 ) ,且返流量与肺动脉平均压呈正相关 (r=0 .89,P <0 .0 1) ,与肺动脉峰值流速呈负相关 (r =-0 .82 ,P <0 .0 1)。结论　MR相位速度图是评价肺动脉血流动力学变化一种新的可靠技术     

The prevalence and clinical predictors of incidental atherosclerotic renal artery stenosis

Objective

To evaluate the prevalence of incidental renal artery stenosis due to atherosclerosis and associated risk factors in patients with peripheral arterial disease (PAD).

Materials and methods

To determine renal artery stenosis, aortofemoropopliteal digital substraction angiographies (DSA) of 629 consecutive patients with PAD were prospectively reviewed. Angiographies were performed as catheter angiography with automated pump injection. Of the patients, 540 were male (86%) and 89 female (14%) (mean age ± S.D.: 61.5 ± 11.1 years). Statistical analysis was performed to determine the association of significant renal artery stenosis (≥60% diameter stenosis) with patient demographics (age, sex, reason for angiography and smoking status), medical history (diabetes mellitus, hypertension and coronary artery disease), laboratory values (blood creatinine, fasting glucose, triglycerides, LDL, HDL and total cholesterol) and distribution of PAD (aortoiliac, femoropopliteal and crural diseases and multisegment involvement).

Results

Renal artery disease was found in 33% (207 of 629) of all patients with peripheral arterial disease, and 9.6% of patients (n = 60) had significant (≥60%) renal artery stenosis. Only age and hypertension (blood pressure systolic >140 mmHg or diastolic >90 mmHg) were independent risk factors for significant renal artery stenosis on multivariate analysis. Mean age of patients with RAS was 66.5 ± 8.9 years compared with 61 ± 11.2 years for patients without RAS (p < 0.001). Hypertension was found in 41% of the patients in control group and in 63% of the patients in RAS group (p = 0.01).

Conclusion

Incidental renal artery stenosis which can be mild or significant is a relatively common finding among patients with peripheral arterial disease. Advance age and hypertension are closely associated with significant renal artery stenosis.     

Embolization for Hemoptysis in Chronic Thromboembolic Pulmonary Hypertension: Report of Two Cases and a Review of the Literature

Hemoptysis is a known complication in patients with bronchial artery hypertrophy due to a variety of chronic pulmonary disorders. Bronchial artery hypertrophy is observed in most patients with chronic thromboembolic pulmonary hypertension (CTEPH), but surprisingly little is known about the incidence of hemoptysis in these patients. In this paper, we report on 2 patients with CTEPH and recurrent severe hemoptysis, who were treated by bronchial artery embolization. One patient recovered and 1 patient died as a consequence of the bleeding. A systematic review revealed 21 studies on the underlying pathology in 1,844 patients with moderate to severe hemoptysis. CTEPH was reported to be the cause of bleeding in 0.1% (n = 2), pulmonary arterial hypertension without chronic thromboembolic disease in 0.2% (n = 4), and acute pulmonary embolism in 0.7% (n = 12) of the patients. In contrast to this, 5 patients (6%) in our own series of 79 CTEPH patients suffered from moderate to severe hemoptysis requiring medical intervention. Severe hemoptysis appears to be an uncommon, but possibly underreported, life-threatening complication in CTEPH patients. As most CTEPH patients require life-long anticoagulants a therapeutic dilemma may ensue. Therefore, we propose that even mild hemoptysis in CTEPH patients warrants prompt evaluation, and treatment by embolization should be offered as first choice in CTEPH patients.     

Performance of computed tomography-derived pulmonary vasculature metrics in the diagnosis and haemodynamic assessment of pulmonary arterial hypertension

BackgroundFew studies have addressed the value of combining computed tomography-derived pulmonary vasculature metrics for the diagnosis and haemodynamic evaluation of pulmonary arterial hypertension (PAH).Materials and methodsWe measured three computed tomography parameters for the pulmonary artery, peripheral vessels, and pulmonary veins: the ratio of the diameter of the pulmonary artery to the aorta (PA/Ao), the cross-sectional area of small pulmonary vessels <5 mm² as a percentage of total lung area (%CSA_<5), and the diameter of the right inferior pulmonary vein (PVD). The measured quantities were compared between patients with PAH (n = 45) and control subjects (n = 56), and their diagnostic performance and associations with PAH-related clinical indices, including right heart catheterization measurements, were examined.ResultsPA/Ao and %CSA_<5 were significantly higher in patients with PAH than in controls. Receiver-operating characteristic curve analysis for ability to diagnose PAH showed a high area under the curve (AUC) for PA/Ao (0.95) and modest AUCs for %CSA_<5 (0.75) and PVD (0.56). PA/Ao correlated positively with mean pulmonary arterial pressure and PVD correlated negatively with pulmonary vascular resistance. The %CSA_<5 correlated negatively with mean pulmonary arterial pressure and pulmonary vascular resistance and positively with cardiac index. Notably, the PA/Ao and PVD values divided by %CSA_<5 correlated better with right heart catheterization indices than the non-divided values.ConclusionPA/Ao, %CSA_<5, and PVD are useful non-invasive pulmonary vasculature metrics, both alone and in combination, for diagnosis and haemodynamic assessment of PAH.     

Endovascular Treatment of a Coronary Artery Bypass Graft to Pulmonary Artery Fistula with Coil Embolization

Fistula formation between a coronary artery bypass graft (CABG)and the pulmonary arterial circulation represents a rare cause of recurrent angina in patients following bypass grafting. Therapy has traditionally involved surgical ligation by open thoracotomy. We describe a case of left internal mammary artery–left upper lobe pulmonary artery fistula presenting as early recurrent angina following CABG. The fistula was embolized using platinum coils, resulting in symptomatic relief and improvement in myocardial perfusion on cardiac perfusion scintigraphy. Coil embolization should be considered a therapeutic option in patients with coronary–pulmonary steal syndrome.     

健康成人主肺动脉-主动脉直径比的多层螺旋CT测量

目的 确定多层螺旋CT(MSCT)上健康成人主肺动脉-升主动脉直径比(rPA)的正常值.资料与方法 140名健康成人作了胸部CT检查,根据性别、年龄分为4组,每组35名.在CT同一层面上,两名放射科医师独立测量主肺动脉和升主动脉的直径.结果 rPA的大小与性别无明显相关性(P＞0.05).＜50岁者,rPA范围0.684～0.988,平均值为0.833;≥50岁者,rPA范围0.555～0.965,平均值为0.762,两者差异有统计学意义(P＜0.001).结论 在MSCT横断面图像上可很容易获得rPA,健康成人rPA＜1.     

The dilatation of main pulmonary artery and right ventricle observed by enhanced chest computed tomography predict poor outcome in inoperable chronic thromboembolic pulmonary hypertension

BackgroundDilatation of the pulmonary artery and right ventricle on chest computed tomography images is often observed in patients with pulmonary hypertension. The clinical significance of these image findings has not been defined in chronic thromboembolic pulmonary hypertension. We investigated whether the pulmonary arterial and right ventricle dilatation was associated with poor outcome in chronic thromboembolic pulmonary hypertension.MethodsThis was a retrospective cohort investigation in 60 subjects with inoperable chronic thromboembolic pulmonary hypertension diagnosed consecutively between 1997 and 2010 at Chiba University Hospital. Digital scout multi-detector chest computed tomography images were obtained. The main pulmonary arterial to ascending aortic diameter ratio and the right ventricular to left ventricular diameter ratio were calculated.ResultsMain pulmonary arterial to ascending aortic diameter ratio ranged from 0.85 to 1.84, and right ventricular to left ventricular diameter ratio ranged from 0.71 to 2.88. During the observation period of 1284.5 days (range, 21–4550 days), 13 patients required hospitalization due to worsening; 6 of them died. Kaplan–Meier analysis showed significant differences in hospitalization between the patients with main pulmonary arterial to ascending aortic diameter ratio of ≥1.1 and <1.1 (log-rank test, p = 0.014) and between the patients with right ventricular to left ventricular diameter ratio of ≥1.2 and <1.2 (log-rank test, p = 0.013). There was a significant difference in the prognosis between the patients with RV/LV ratio ≥ 1.2 and those with RV/LV ratio < 1.2 (log-rank test, p = 0.033).ConclusionsMain pulmonary arterial to ascending aortic diameter ratio measured using enhanced CT images was associated with the risk for first clinical exacerbation, and right ventricular to left ventricular diameter ratio was associated with the risk for poor prognosis in inoperable chronic thromboembolic pulmonary hypertension.     

先天性心脏病合并肺动脉高压分级及性质的评估

鉴别先天性心脏病合并肺动脉高压是动力型还是阻力型对治疗方案的选择和预后的评估均具重要意义。本文综述先天性心脏病合并肺动脉高压的临床分级、分期及肺动脉高压性质的判断,旨在为临床诊疗提供参考。     

Pulmonary artery involvement in aortoarteritis: An angiographic study

Angiographic findings of 47 patients with pulmonary artery involvement (PAI) in aortoarteritis are described together with the diagnostic evaluation of PAI and its clinical implications. Pulmonary arteriography was obtained by intravenous digital subtraction angiography in 133 patients with aortoarteritis; 45 (33.8%) were found to have PAI. Two patients with PAI, studied by conventional arteriography, are also included. Stenosis and/or occlusion of segmental and/or lobar pulmonary arteries, and subsegmental branches, were the basic angiographic findings in PAI. Pulmonary artery branches in the upper lobes were more commonly affected than those in the lower and middle (lingula) lobes. Bilateral lesions were more common than unilateral ones. Single lobar and segmental lesions were quite rare. Dilatation was uncommon. No main pulmonary artery involvement was detected. Pulmonary artery hypertension, a late complication of patients with PAI, was revealed in 9 of the 46 patients of the present series. If it occurs, PAI becomes a factor effecting the patient's clinical course and prognosis.     

Pulmonary hypertension in patients with chronic pulmonary thromboembolism: chest radiograph and CT evaluation before and after surgery

The purpose of this study was to assess the value of morphometric data on conventional radiography and CT predicting the presence and degree of pulmonary hypertension and to assess the reversibility after surgery. On preoperative X-ray films and CT scans of 50 patients with pulmonary hypertension secondary to chronic thromboembolism, we measured the cardiothoracic ratio, basal diameter, length of cardiac contact to sternum, pulmonary trunk, right and left descending pulmonary artery, and the septum angle. These data were correlated with pulmonary arterial pressure. In 14 X-ray patients and 18 CT patients, with follow-up after surgical thromboendarterectomy the reversibility of these changes was assessed. A dilated pulmonary trunk was the most common abnormality (96% each on X-ray and CT). Pulmonary arteries were dilated on X-ray in 40% (right) and 14% (left), and on CT in 92% (right) and 96% (left). The best correlation with mean arterial pressure was found measuring the pulmonary trunk on CT (r = 0.43, p < 0.01). After surgery, reversibility was most significant for the pulmonary trunk on CT (p < 0.0001). In patients with chronic pulmonary embolism, pulmonary hypertension can best be predicted by assessing the diameter of the pulmonary trunk both on X-ray and CT. No close correlation is present between the extent of any parameter and the level of the pulmonary pressure.Correspondence to: H. C. Schmidt     

PVOD suggested by MDCT and clinical findings in a pregnant woman

Pulmonary hypertension secondary to pulmonary venoocclusive disease (PVOD) is increasingly recognized (Wagenvoort, Chest 69:82–86, [20]; Scully et al., N Engl J Med 308:823–834, [21]). The clinical presentation is usually progressive pulmonary hypertension. It should be kept in mind when there is pulmonary arterial hypertension, pulmonary edema, and a normal pulmonary artery wedge pressure. Importance of diagnosing this condition is to protect patient from fatal pulmonary edema when using prostacyclins that are effective for treatment of primary pulmonary hypertension. Herein, we present multidetector computed tomography findings of PVOD in a pregnant woman that presented with pulmonary hypertension.     

Management of dyslipidemia with statins in the patient with peripheral arterial disease

The relationship between peripheral arterial disease (PAD), cardiovascular disease (CAD), and underlying atherosclerotic risk factors such as dyslipidemia, smoking, diabetes, and hypertension is well established. Patients with PAD have dramatically increased risks of cardiovascular mortality due to underlying atherosclerosis. As interventionalists, we focus primarily on improving the functional status of our patients by "fixing" their peripheral arterial occlusions, often ignoring the more serious concerns, as well as the root cause of the problem. In addition to interventional strategies, comprehensive clinical management of PAD should include attention to risk factor modification, lifestyle changes, exercise therapy, and pharmacologic interventions. Conventional training in interventional radiology imparts very little familiarity with these management issues. The following article addresses one critical modifiable risk factor, dyslipidemia, and treatment with the powerful class of drugs, the statins, reviewing the rationale for as well as the practical considerations in the use of these agents.     

Sudden,unexpected infant death due to pulmonary arterial hypertension

A 3-year-old girl with no particular medical history complained of a stomachache and died on the way to the hospital. The autopsy revealed marked right ventricular hypertrophy and dilation with no other cardiac abnormalities. Microscopically, the pulmonary small arteries showed marked medial hypertrophy and varying degrees of intimal and adventitial thickening. We supposed that the cause of death was attributable to pulmonary arterial hypertension (PAH). PAH is a rare disease that can cause sudden, unexpected death at any age. Forensic pathologists should consider PAH in the differential diagnosis of sudden death.     

特发性肺动脉高压与先天性心脏病相关肺动脉高压患者右心室心肌葡萄糖代谢的比较研究

目的:比较分析特发性肺动脉高压（IPAH）与先天性心脏病相关肺动脉高压（PAH-CHD）患者右心室心肌葡萄糖代谢的差异。方法:选取2016年11月至2018年12月在中国医学科学院北京协和医学院阜外医院确诊的26例IPAH患者（IPAH组）[女性17例、男性9例,年龄（28.23±8.92）岁]和16例PAH-CHD患...     

In situ central pulmonary artery thrombosis in primary pulmonary hypertension

A rare case of extensive in situ central pulmonary artery thrombosis in primary pulmonary hypertension (PPH) is presented. The differentiation from chronic thromboembolic pulmonary arterial hypertension (CTEPH) is of paramount importance because of different therapeutic strategies. In this case, the presence of mural thrombus in the central pulmonary arteries on computed tomography made the distinction difficult. However, the possibility of in situ thrombosis was suggested on the basis of absence of other findings of CTEPH (abrupt narrowing/truncation of segmental arteries, variation in size of segmental vessels, arterial webs, mosaic attenuation, pulmonary infarcts, and dilated bronchial arteries), and this was confirmed on final pathology.