• Journal of Chest Surgery
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• v.33 no.10
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• pp.823-826
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• 2000

Primary intimal sarcoma of the pulmonary artery is a rare disease and there has been no report of any case originating from the pulmonary valve. Recently we experienced a 62 year-old female patient who had a primary intimal sarcoma of the pulmonary valve with distal metastasis. She was brought to medical attention due to exertional dyspnea facial edema productive coughing and general weakness for 1 month. Chest CT and echocardi-ography suggest an acute pulmonary thromboembolism or tumor. Exploration showed a large polypoid mass arising from the pulmonary leaflets and multiple masses on distal pulmonary arteries. We replaced the pulmonary valve and reconstructed the pulmonary artery. She received radiotherapy 1 month postoperatively and now 4 months after surgery she has begun receiving chemotherapy.

• Tuberculosis and Respiratory Diseases
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• v.45 no.3
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• pp.649-653
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• 1998

Pulmonary arteriovenous fistula is a congenital vascular malformation in lung resulting from abnormal capillary development and the exact etiology is not well known but it may occur with or without hereditary hemorrhagic telangiectasia. Hereditary hemorrhagic telangiectasia (HHT) or Rendu-osler-Weber disease is an autosomal dominant vascular disorder which associates epistaxis, mucocutaneous and visceral telangicetases, and recurrent hemorrhage with chronic anemia and visceral shuntings. Recently we experienced a case of the pulmonary arteriovenous fistula in a 23 year-old woman with a family history of this disease, which was confirmed by pulmonary angiography.

• Tuberculosis and Respiratory Diseases
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• v.60 no.2
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• pp.142-150
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• 2006

Pulmonary arterial hypertension (PAH) is often difficult to diagnose and challenging to treat. Untreated, it is characterized by a progressive increase in pulmonary vascular resistance leading to right ventricular failure and death. The past decade has seen remarkable improvements in therapy, driven largely by the conduct of randomized controlled trials. Still, the selection of most appropriate therapy is complex, and requires familiarity with the disease process, evidence from treatment trials, complicated drug delivery systems, dosing regimens, side effects, and complications. We tried to provide evidence-based treatment recommendations for physicians involved in the care of these complex patients. Due to the complexity of the diagnostic evaluation required, and the treatment options available, it is strongly recommended that consideration be given to referral of patients with PAH to a specialized center.

• Asian Journal of Atmospheric Environment
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• v.1 no.1
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• pp.28-35
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• 2007

Accumulated epidemiological and animal studies have suggested that prolonged exposure to ambient particulate matter (PM) is associated with an increased risk of cardiovascular disease and pulmonary dysfunction. While diesel exhaust particles (DEP) contain large variety of compounds, polycyclic aromatic hydrocarbons (PAHs) are a dominant component contaminated in DEP. This article reviews effects of two PAH quinones, 9,10-phenanthraquinone (9,10-PQ) and l,2-naphthoquinone (l,2-NQ), on vascular and respiratory systems.

• Korean Circulation Journal
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• v.54 no.7
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• pp.409-421
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• 2024

Background and Objectives: The recent developments in chronic thromboembolic pulmonary hypertension (CTEPH) are emphasizing the multidisciplinary team. We report on the changes in clinical practice following the development of a multidisciplinary team, based on our 7 years of experience. Methods: Multidisciplinary team was established in 2015 offering both balloon pulmonary angioplasty (BPA) and pulmonary endarterectomy (PEA) with technical upgrades by internal and external expertise. For operable cases, PEA was recommended as the primary treatment modality, followed by pulmonary angiography and right heart catheterization after 6 months to evaluate treatment effect and identify patients requiring further BPA. For patients with inoperable anatomy or high surgical risk, BPA was recommended as the initial treatment modality. Patient data and clinical outcomes were closely monitored. Results: The number of CTEPH treatments rapidly increased and postoperative survival improved after team development. Before the team, 38 patients were treated by PEA for 18 years; however, 125 patients were treated by PEA or BPA after the team for 7 years. The number of PEA performed was 64 and that of BPA 342 sessions. World Health Organization functional class I or II was achieved in 93% of patients. The patients treated with PEA was younger, male dominant, higher pulmonary artery pressure, and smaller cardiac index, than BPA-only patients. In-hospital death after PEA was only 1 case and none after BPA. Conclusions: The balanced development of BPA and PEA through a multidisciplinary team approach proved synergistic in increasing the number of actively treated CTEPH patients and improving clinical outcomes.

• The Korean Journal of Physiology and Pharmacology
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• v.17 no.1
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• pp.1-8
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• 2013

Understanding the cellular and molecular mechanisms involved in the development and progression of pulmonary hypertension (PH) remains imperative if we are to successfully improve the quality of life and life span of patients with the disease. A whole plethora of mechanisms are associated with the development and progression of PH. Such complexity makes it difficult to isolate one particular pathway to target clinically. Changes in intracellular free calcium concentration, the most common intracellular second messenger, can have significant impact in defining the pathogenic mechanisms leading to its development and persistence. Signaling pathways leading to the elevation of $[Ca^{2+}]_{cyt}$ contribute to pulmonary vasoconstriction, excessive proliferation of smooth muscle cells and ultimately pulmonary vascular remodeling. This current review serves to summarize the some of the most recent advances in the regulation of calcium during pulmonary hypertension.

• Korean Circulation Journal
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• v.53 no.7
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• pp.425-451
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• 2023

Most patients with heart failure (HF) have multiple comorbidities, which impact their quality of life, aggravate HF, and increase mortality. Cardiovascular comorbidities include systemic and pulmonary hypertension, ischemic and valvular heart diseases, and atrial fibrillation. Non-cardiovascular comorbidities include diabetes mellitus (DM), chronic kidney and pulmonary diseases, iron deficiency and anemia, and sleep apnea. In patients with HF with hypertension and left ventricular hypertrophy, renin-angiotensin system inhibitors combined with calcium channel blockers and/or diuretics is an effective treatment regimen. Measurement of pulmonary vascular resistance via right heart catheterization is recommended for patients with HF considered suitable for implantation of mechanical circulatory support devices or as heart transplantation candidates. Coronary angiography remains the gold standard for the diagnosis and reperfusion in patients with HF and angina pectoris refractory to antianginal medications. In patients with HF and atrial fibrillation, longterm anticoagulants are recommended according to the CHA₂DS₂-VASc scores. Valvular heart diseases should be treated medically and/or surgically. In patients with HF and DM, metformin is relatively safer; thiazolidinediones cause fluid retention and should be avoided in patients with HF and dyspnea. In renal insufficiency, both volume status and cardiac performance are important for therapy guidance. In patients with HF and pulmonary disease, beta-blockers are underused, which may be related to increased mortality. In patients with HF and anemia, iron supplementation can help improve symptoms. In obstructive sleep apnea, continuous positive airway pressure therapy helps avoid severe nocturnal hypoxia. Appropriate management of comorbidities is important for improving clinical outcomes in patients with HF.

• Tuberculosis and Respiratory Diseases
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• v.62 no.3
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• pp.232-236
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• 2007

Primary pulmonary artery sarcoma is a rare malignant tumor arising from the pulmonary artery. Diagnosis of primary pulmonary artery sarcoma is quite difficult and the conditon is often misdiagnosed as a more common disease, such as a pulmonary embolism. PET can help in diagnosing a pulmonary artery sarcoma due to the increased uptake of $^{18}F-FDG$ in the area of the tumor. However, the poor anatomic resolution of PET has limited its clinical applications in pulmonary vascular disease. The recently developed PET/CT is the fusion of PET and CT that improves the anatomical resolution of PET. We report a case of a primary pulmonary artery sarcoma mimicking a pulmonary embolism that was diagnosed with PET/CT and confirmed with a surgical resection.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.86-90
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• 2003

Alagille syndrome is a autosomal dominant disorder characterized by intrahepatic bile duct paucity and resultant chronic cholestasis in combination with cardiac(mainly peripheral pulmonary stenosis), skeletal, ocular, and facial abnormalities. In addition to the pulmonary stenosis, in large series, anecdotal reports of vascular lesions have concerned the renal artery, aorta, hepatic artery, carotid artery, celiac artery or subclavian artery. Theses diffuse vascular abnormalities, which appear to be a feature of Alagille syndrome, suggest Notch signaling pathway defects affect angiogenesis. The associations of Alagille syndrome with moyamoya disease, the chronic cerebrovascular occlusive disease, were reported and suggested as additional evidence of vasculopathy of Alagille syndrome. We report another 25 month-old Alagille syndrome girl who presented with acute left hemiparesis and was diagnosed with moyamoya disease through the cerebral angiographic study.

• Journal of Chest Surgery
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• v.25 no.6
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• pp.650-660
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• 1992

To elucidate a potential contribution of endotheline-1[ET-1] to the genesis of pulmonary hypertension and postoperative pulmonary hypertensive crisis in the patients with heart disease, we measured plasma levels of the ET-1 during perioperative period of open heart surgery. In addition, we examined changes of ET-1 during perioperative period and correlations between ET-1 levels and hemodynamic variables. 12 patients including 5 acquired heart disease and 7 congenital heart disease patients were selected randomly as a study group, Group A and B, respectively. 6 patients proved not having heart or hemodynamic problem were selected as a control, Group C. 110 blood samples from pulmonary artery[ET-P] and radial artery[ET-S] were taken and assayed by Sep-pak extraction and RIA. ET-1 levels of Group A were ET-P, 3.94$\pm$5.31pg /ml, ET-S, 3.10$\pm$2.90pg/ml[p>0.05], Group B were ET-P, 1.63$\pm$0.62pg/ml, ET-S, 1.99$\pm$2.45pg/ml[p>0.05], Group C were ET-P, 1.97$\pm$2.02pg/ml, ET-S, 1.72$\pm$0.77pg/ml[p>0.05]. There were no statistically significant differences of ET-1 levels among the Group A, B, C[p>0.05]. There was no correlation between pulmonary artery pressure[PAP] and ET-1 level[p>0.05], and ET-1 levels were not increased even in the cases of pulmonary hypertensive criwis or low cardiac output syndrome, whereas significant correlation between ET-S and pulmonary vascular res-istance[Rp] [r=0.36, p<0.05], and negative correlation between ET-S and OS saturation of pulmonary artery[OS-P][r= -0.49, p<0.01] were identified. Another significant finding was peak increase of ET-1 levels in the postoperative period 1 hour[p<0.05] and then gra-dualy decrease through the postoperative period. In conclusion, ET-1 has no correlation with PAP, whereas correlation with Rp, and inverse correlation with OS-P. It is suggested that ET-1 is neither the direct causative substance of pulmonary hypertension nor pulmonary vasospasm but there must be increased production of ET-1 in chronic pulmonary hypertensive state. Counter-regulatory mechanism to ET-1 is speculated during the pulmonary vasospasm.