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The Clinical Efficacy of Pulmonary Hypertension-Specific Agents in Idiopathic Pulmonary Fibrosis: Systematic Review and Meta-Analysis of Randomized Controlled Clinical Trials

Lee J, Song JU

BACKGROUND: Pulmonary hypertension (PH) is common in patients with idiopathic pulmonary fibrosis (IPF) and is associated with poor outcomes. This study was performed to determine the clinical efficacy of PH-specific...
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Canonical Transient Receptor Potential Channels and Vascular Smooth Muscle Cell Plasticity

Nishida M, Tanaka T, Mangmool S, Nishiyama , Nishimura

Vascular smooth muscle cells (VSMCs) play a pivotal role in the stability and tonic regulation of vascular homeostasis. VSMCs can switch back and forth between highly proliferative (synthetic) and fully...
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Echocardiographic Screening Methods for Pulmonary Hypertension: A Practical Review

Jang AY, Shin MS

Pulmonary hypertension (PH) is a debilitating condition defined as mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg. The importance of impaired right ventricular (RV) hemodynamics is increasingly being recognized in...
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Echocardiographic features of indirect Gerbode defect in a cat

This report describes the echocardiographic features of an indirect Gerbode defect in a young cat. Echocardiography revealed high-velocity, turbulent systolic flow directed from left ventricle to right ventricle through a...
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Effect of Ambrisentan Therapy on the Expression of Endothelin Receptor, Endothelial Nitric Oxide Synthase and NADPH Oxidase 4 in Monocrotaline-induced Pulmonary Arterial Hypertension Rat Model

Lee H, Yeom A, Kim KC, Hong YM

BACKGROUND AND OBJECTIVES: Elevated endothelin (ET)-1 level is strongly correlated with the pathogenesis of pulmonary arterial hypertension (PAH). Expression level of nicotinamide adenine dinucleotide phosphate oxidase (NOX) 4 is increased...
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Metabolic Syndrome in Adult Congenital Heart Disease

Niwa K

In adult congenital heart disease (ACHD), residua and sequellae after initial repair develop late complications such as cardiac failure, arrhythmias, thrombosis, aortopathy, pulmonary hypertension and others. Acquired lesions with aging...
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Emergency Pulmonary Artery–to-Systemic Artery Shunt to Break the Positive Feedback Loop of a Pulmonary Hypertensive Crisis after Neonatal Coarctation Repair

Shin YR, Yang YH, Park YH, Park HK

A 2.5-kg neonate with coarctation of the aorta and a small left ventricle experienced a severe pulmonary hypertensive crisis. An emergency pulmonary artery-to-systemic artery shunt was placed to break the...
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A case of FLNA gene mutation with respiratory insufficiency and periventricular heterotopia

Park H, Park MS, Ki CS, Cho J, Lee J, Kim J, Ahn K

Filamin A is an actin-binding protein and, in humans, is encoded by FLNA gene in the long arm of X chromosome. Filamin A plays a role in the formation of...
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Assessment of Right Ventricular Function by Tissue Doppler Imaging in Pulmonary Arterial Hypertensive Rat

Hong JE, Kim KC, Hong YM

OBJECTIVES: Elevated pulmonary pressure and right ventricular (RV) dysfunction are the hallmarks of pulmonary vascular disease in animal models and human patients with pulmonary arterial hypertension (PAH). Monocrotaline models of...
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Prognostic value of right pulmonary artery distensibility in dogs with pulmonary hypertension

Chan IP, Weng MC, Hsueh T, Lin YC, Lin SL

The right pulmonary artery distensibility (RPAD) index has been used in dogs with pulmonary hypertension (PH) caused by heartworm infection, myxomatous mitral valve disease, or patent ductus arteriosus. We hypothesized...
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Pulmonary Arterial Hypertension and Pregnancy: Single Center Experience in Current Era of Targeted Therapy

Lim K, Chang SA, Oh SY, Lee JH, Song J, Kang IS, Huh J, Park SJ, Park SW, Kim DK

OBJECTIVES: To report our experiences in pregnant patients with pulmonary arterial hypertension (PAH) who were treated with targeted therapy. METHODS: From 2011 to 2017, women who decided to maintain pregnancies in...
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Pulmonary Hypertension in Newborn Infants with Vein of Galen Malformation

Song WS, Oh MY, Cho JY, Kim CY, Suh DC, Jung E, Kim EA, Kim KS, Lee BS

OBJECTIVE: Vein of Galen malformation (VGM) is a rare cerebrovascular malformation. Children with VGM presenting in newborn period demonstrate various clinical courses from normal development to severe neurologic impairment or...
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Thyroid Hormone Resistance in a Preterm Infant with a Novel THRB Mutation

Bae JY, Kim DY, Kwon YD, Song YH, Lim HH, Park HD, Lim JW

Resistance to thyroid hormone (RTH) is a condition caused by a mutation in the thyroid hormone receptor gene. It is rarely reported in individuals with no family history of RTH...
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Central extracorporeal membrane oxygenation and early rehabilitation for persistent severe pulmonary hypertension following pulmonary endarterectomy

Seong GM, Hong SB, Huh JW, Lim CM, Koh Y, Lee JW, Jung SH, Park DW, Lee JS

Chronic thromboembolic pulmonary hypertension is potentially curable with a pulmonary endarterectomy. However, approximately 20% of patients have persistent pulmonary hypertension after pulmonary endarterectomy, which is a major risk factor for...
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Sudden Death due to Rupture of Pulmonary Trunk Aneurysm in a Patient with Eisenmenger Syndrome

Kim SZ, Lee S

Proximal pulmonary artery aneurysms and dissection are rare and life-threatening conditions, which are usually detected only during autopsy examination in cases of sudden death. These pathological entities often occur as...
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A Rare Case of Lethal Prenatal-Onset Infantile Cortical Hyperostosis

Kim ST, Kim H, Kim HH, Lee NH, Han Y, Sung SI, Chang YS, Park WS

Infantile cortical hyperostosis, or Caffey's disease, usually presents with typical radiological features of soft tissue swelling and cortical thickening of the underlying bone. The disease can be fatal when it...
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Acute Hemodynamic Changes after Single Administration of Udenafil in Pulmonary Arterial Hypertension: a Phase IIa Study

Chang SA, Kim HK, Chang HJ, Kim DK

BACKGROUND AND OBJECTIVES: Udenafil, a new phosphodiesterase-5 inhibitor (PDE5i), has been used to treat erectile dysfunction. Given the proven benefit of PDE5i in pulmonary arterial hypertension (PAH), we evaluated serial...
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Chronic Thromboembolic Pulmonary Hypertension: Endovascular Treatment

Ahn CM, Hiromi M

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare cause of pulmonary hypertension; less than 5% of pulmonary hypertension is caused by recurrent pulmonary thromboembolism (PTE). By definition, CTEPH happens within...
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The Role of Autophagy in Eosinophilic Airway Inflammation

Lee J, Kim HS

Autophagy is a homeostatic mechanism that discards not only invading pathogens but also damaged organelles and denatured proteins via lysosomal degradation. Increasing evidence suggests a role for autophagy in inflammatory...
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A Case of Unilateral Vocal Fold Paralysis Caused by Ortner's Syndrome

Park SH, Park HS, Bae WY, Lee DK

The causes of vocal cord paralysis include iatrogenic injury during thyroid or cervical surgery, heart and chest surgery, and tumorous lesion such as laryngeal cancer and lung cancer. In addition...
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