• Tuberculosis and Respiratory Diseases
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• 제69권4호
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• pp.237-242
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• 2010

Pulmonary vascular disease is a category of disorders, including pulmonary hypertension, pulmonary embolism or chronic thromboembolic pulmonary hypertension, pulmonary vasculitis, pulmonary vascular disease secondary to chronic respiratory disease, and pulmonary vascular tumor and malformations. This article reviews the recent advances in this wide spectrum of pulmonary vascular diseases.

• Journal of Chest Surgery
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• 제14권1호
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• pp.9-16
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• 1981

Twenty eight patients with conpnital heart disuse underwent lung biopsy to assets pulmonary obstructive vascular disease at cardiac surpry. Thirteen patients had patent ductus arteriosus, 10, ventricular septal defects and S, atrial septal defects. The aaes were between 2 and 30 years. In patients with patent ductus arteriosus lung biopsy was performed from the IIngular Hlment. The anterior seament of the right upper lobe was blopsled in cases with ventricular septal defect and atrial septal defect. Grading of pulmonary obstructive vascular disease could not be assessed In 9 cases. In 2 cases poor quality of the slides made us impossible to evaluate and In 7 cases there were no suitable small muscular arteries to evaluate in the slides of lung tissue especially taken from the IIngular seament. Nineteen cases were evaluated pulmonary obstructive vascular disease. Among them 17 cases had Heath-Edwards changes of grade 1 and 2 patients had that of grade 3. The thickness of media was measured. It was expressed as percentage of medial thickness to outer diameter of artery. The medial thickness was correlated proportionally with elevation of pulmonary arterial pressure and pulmonary vascular resistance to systemic vascular resistance ratio. There were no complications related to the procedure of lung biopsy.

• Korean Journal of Radiology
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• 제21권6호
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• pp.756-763
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• 2020

Objective: To evaluate the effects of attenuation threshold on CT pulmonary vascular volume ratios in children and young adults with congenital heart disease, and to suggest an optimal attenuation threshold. Materials and Methods: CT percentages of right pulmonary vascular volume were compared and correlated with percentages calculated from nuclear medicine right lung perfusion in 52 patients with congenital heart disease. The selected patients had undergone electrocardiography-synchronized cardiothoracic CT and lung perfusion scintigraphy within a 1-year interval, but not interim surgical or transcatheter intervention. The percentages of CT right pulmonary vascular volumes were calculated with fixed (80-600 Hounsfield units [HU]) and adaptive thresholds (average pulmonary artery enhancement [PA_avg] divided by 2.50, 2.00, 1.75, 1.63, 1.50, and 1.25). The optimal threshold exhibited the smallest mean difference, the lowest p-value in statistically significant paired comparisons, and the highest Pearson correlation coefficient. Results: The PA_avg value was 529.5 ± 164.8 HU (range, 250.1-956.6 HU). Results showed that fixed thresholds in the range of 320-400 HU, and adaptive thresholds of PA_avg/1.75-1.50 were optimal for quantifying CT pulmonary vascular volume ratios. The optimal thresholds demonstrated a small mean difference of ≤ 5%, no significant difference (> 0.2 for fixed thresholds, and > 0.5 for adaptive thresholds), and a high correlation coefficient (0.93 for fixed thresholds, and 0.91 for adaptive thresholds). Conclusion: The optimal fixed and adaptive thresholds for quantifying CT pulmonary vascular volume ratios appeared equally useful. However, when considering a wide range of PAavg, application of optimal adaptive thresholds may be more suitable than fixed thresholds in actual clinical practice.

• The Korean Journal of Physiology and Pharmacology
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• 제20권6호
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• pp.641-647
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• 2016

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by vascular remodeling of pulmonary arteries (PAs) and increased vascular resistance in the lung. Monocrotaline (MCT), a toxic alkaloid, is widely used for developing rat models of PAH caused by injury to pulmonary endothelial cells; however, characteristics of vascular functions in MCT-induced PAH vary and are not fully understood. Here, we investigated hypoxic pulmonary vasoconstriction (HPV) responses and effects of various vasoconstrictors with isolated/perfused lungs of MCT-induced PAH (PAH-MCT) rats. Using hematoxylin and eosin staining, we confirmed vascular remodeling (i.e., medial thickening of PA) and right ventricle hypertrophy in PAH-MCT rats. The basal pulmonary arterial pressure (PAP) and PAP increase by a raised flow rate (40 mL/min) were higher in the PAH-MCT than in the control rats. In addition, both high $K^+$ (40 mM KCl)- and angiotensin II-induced PAP increases were higher in the PAH-MCT than in the control rats. Surprisingly, application of a nitric oxide synthase inhibitor, L-$N^G$-Nitroarginine methyl ester (L-NAME), induced a marked PAP increase in the PAH-MCT rats, suggesting that endothelial functions were recovered in the three-week PAH-MCT rats. In addition, the medial thickening of the PA was similar to that in chronic hypoxia-induced PAH (PAH-CH) rats. However, the HPV response (i.e., PAP increased by acute hypoxia) was not affected in the MCT rats, whereas HPV disappeared in the PAH-CH rats. These results showed that vascular contractility and HPV remain robust in the MCT-induced PAH rat model with vascular remodeling.

• Korean Journal of Radiology
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• 제23권2호
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• pp.202-217
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• 2022

Pulmonary arteriovenous malformation (AVM) is a congenital vascular disease in which interventional radiologists can play both diagnostic and therapeutic roles in patient management. The diagnosis of pulmonary AVM is simple and can usually be made based on CT images. Endovascular treatment, that is, selective embolization of the pulmonary artery feeding the nidus of the pulmonary AVM, and/or selectively either the nidus or draining vein, has become a first-line treatment with advances in interventional devices. However, some vascular diseases can simulate pulmonary AVMs on CT and pulmonary angiography. This subset can confuse interventional radiologists and referring physicians. Vascular mimickers of pulmonary AVM have not been widely known and described in detail in the literature, although some of these require surgical correction, while others require regular follow-up. This article reviews the clinical and radiologic features of pulmonary AVMs and their mimickers.

• Journal of Chest Surgery
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• 제17권3호
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• pp.339-345
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• 1984

Thirty four patients with a ventricular septal defect, aged 2 months to 24 years [median 2.33 years], had lung biopsies during open heart procedures to assess the degree of pulmonary vascular disease. The patients were divided into two groups according to their age; group 1 - under 2 years of age [15 patients], and group 2- over 2 years of age [19 patients]. The, pathologic change of the pulmonary arterioles was correlated with pre-operative hemodynamic data in 34 patients. There was no significant correlation between the severity of pulmonary vascular disease according to Heath-Edwards classification and the patients age [X2=1.8381, P=0.1751 ]. There was a significant correlation between degree of medial wall thickness and arteriolar diameter and the level of preoperative peak pulmonary artery pressure and peak systemic artery pressure [Pp/Ps]. Also, there was a good correlation between the preoperative pulmonary vascular resistance and systemic vascular resistance [Rp/Rs]. There was no significant correlation between wall thickness and Pp/Ps, and Rp/Rs below the age of 2 years. We conclude that, in patients over 2 years of age, there was a significant correlation between the medial wall thickness of the pulmonary arteriole and elevation of Pp/Ps and Rp/Rs. This is not true in patients under 2 years of age.

• Journal of Chest Surgery
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• 제23권4호
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• pp.659-666
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• 1990

We evaluated 68 patients with mitral valve disease who underwent mitral valve replacement from April, 1986, to December, 1988. Among them 20 patients showed increased systolic pulmonary arterial pressure greater than 60 mmHg and postoperative hemodynamic data were obtained in 19 patients average 13 months after mitral valve replacement. The results were as followings. 1. Average pulmonary systolic pressure decreased from 84.8$\pm$4.5 preoperatively to 33. 0$\pm$1.9mmHg postoperatively[P<0.001]. 2. Average pulmonary vascular resistance index decreased from 1425$\pm$148 preoperatively to 287+35.8 dyne * sec * cm2 * m postoperatively[P<0.001]. 3. Average cardiac index rose from 1.927$\pm$0.169 preoperatively to 2.625$\pm$0.159 L/min/m2 postoperatively [P <0.005]. This study shows that pulmonary hypertension and the increased pulmonary vascular resistance index due to mitral valve disease can regress significantly after mitral valve replacement.

• Molecules and Cells
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• 제37권3호
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• pp.196-201
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• 2014

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by the vascular remodeling of the pulmonary arterioles, including formation of plexiform and concentric lesions comprised of proliferative vascular cells. Clinically, PAH leads to increased pulmonary arterial pressure and subsequent right ventricular failure. Existing therapies have improved the outcome but mortality still remains exceedingly high. There is emerging evidence that the seven-transmembrane G-protein coupled receptor APJ and its cognate endogenous ligand apelin are important in the maintenance of pulmonary vascular homeostasis through the targeting of critical mediators, such as Kr$\ddot{u}$ppel-like factor 2 (KLF2), endothelial nitric oxide synthase (eNOS), and microRNAs (miRNAs). Disruption of this pathway plays a major part in the pathogenesis of PAH. Given its role in the maintenance of pulmonary vascular homeostasis, the apelin-APJ pathway is a potential target for PAH therapy. This review highlights the current state in the understanding of the apelin-APJ axis related to PAH and discusses the therapeutic potential of this signaling pathway as a novel paradigm of PAH therapy.

• Korean Journal of Radiology
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• 제2권3호
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• pp.121-131
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• 2001

Echocardiography and catheterization angiography suffer certain limitations in the evaluation of congenital heart diseases in adults, though these are overcome by MRI, in which a wide field-of view, unlimited multiplanar imaging capability and three-dimensional contrast-enhanced MR angiography techniques are used. In adults, recently introduced fast imaging techniques provide cardiac MR images of sufficient quality and with less artifacts. Ventricular volume, ejection fraction, and vascular flow measurements, including pressure gradients and pulmonary-to-systemic flow ratio, can be calculated or obtained using fast cine MRI, phase-contrast MR flow-velocity mapping, and semiautomatic analysis software. MRI is superior to echocardiography in diagnosing partial anomalous pulmonary venous connection, unroofed coronary sinus, anomalies of the pulmonary arteries, aorta and systemic veins, complex heart diseases, and postsurgical sequelae. Biventricular function is reliably evaluated with cine MRI after repair of tetralogy of Fallot, and Senning's and Mustard's operations. MRI has an important and growing role in the morphologic and functional assessment of congenital heart diseases in adolescents and adults.

• Journal of Chest Surgery
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• 제49권2호
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• pp.115-118
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• 2016

Williams syndrome (WS) is a developmental disorder characterized by vascular abnormalities such as thickening of the vascular media layer in medium- and large-sized arteries. Supravalvular aortic stenosis (SVAS) and peripheral pulmonary artery stenosis (PPAS) are common vascular abnormalities in WS. The natural course of SVAS and PPAS is variable, and the timing of surgery or intervention is determined according to the progression of vascular stenosis. In our patient, SVAS and PPAS showed rapid concurrent progression within two weeks after birth. We report the early manifestation of SVAS and PPAS in the neonatal period and describe the surgical treatment for stenosis relief.