• BMB Reports
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• v.47 no.6
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• pp.311-317
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• 2014

microRNAs (miRNAs) are a class of small, non-coding RNAs that play critical posttranscriptional regulatory roles typically through targeting of the 3'-untranslated region of messenger RNA (mRNA). Mature miRNAs are known to be involved in global cellular processes, such as differentiation, proliferation, apoptosis, and organogenesis, due to their capacity to target multiple mRNAs. Thus, imbalances in the expression and/or activity of miRNAs are involved in the pathogenesis of numerous diseases, including pulmonary arterial hypertension (PAH). PAH is a progressive disease characterized by vascular remodeling due to excessive proliferation of pulmonary artery endothelial cells (PAECs) and pulmonary artery smooth muscle cells (PASMCs). Recently, studies have evaluated the roles of miRNAs involved in the pathogenesis of PAH in these pulmonary vascular cells. This review provides an overview of recent discoveries on the role of miRNAs in the pathogenesis of PAH and discusses the potential for miRNAs as therapeutic targets and biomarkers of PAH.

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.93-96
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• 2010

A 47-year-old male patient in whom atrial septal defect (ASD) had been diagnosed 15 years previously was admitted for cardiac catheterization. He had definite cyanotic lips and nail beds and severe pulmonary arterial hypertension (PAH). He had received medical treatment only for the last few years after being diagnosed with Eisenmenger syndrome. After cardiac catheterization, he received iloprost inhalation therapy pre and postoperation and was discharged after successful surgical closure of the ASD.

• YAKHAK HOEJI
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• v.54 no.2
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• pp.126-133
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• 2010

This study was conducted to analyze cost-utility of bosentan versus iloprost indicated for pulmonary arterial hypertension (PAH) in a Korean healthcare setting from a payer's perspective. We constructed a Markov model to estimate total costs and outcomes for 1-year time horizon in a hypothetical cohort of 50-year-old patients with PAH. Base analysis showed that bosentan resulted in KW 5.5 billions saving and 18 quality-adjusted life year (QALY) gains per 100 patients compared to iloprost. Bosentan as a dominant strategy was found to be robust through various sensitivity analyses.

• Tuberculosis and Respiratory Diseases
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• v.46 no.6
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• pp.846-855
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• 1999

Background : Secondary pulmonary hypertension is an important final endpoint in patients with chronic hypoxic lung disease, accompanied by deterioration of pulmonary hemodynamics. The clinical diagnosis of pulmonary hypertension and/or cor pulmonale could be difficult, and simple noninvasive evaluation of pulmonary artery pressures has been an relevant clinical challenge for many years. Doppler echocardiography might to be a more reliable method for evaluating pulmonary hemodynamics in such patients in terms of the accuracy, reproducibility and easiness for obtaining an appropriate echocardiographic window than M-mode echocardiography. The aim of this study was to assess echocardiographic parameters associated with pulmonary arterial hypertension, defined by increasing right ventricular systolic pressure(RVSP), calculated from trans-tricuspid gradient in patients with chronic hypoxic lungs. Method : We examined 19 patients with chronic hypoxic lung disease, suspected pulmonary hypertension under the clinical guidelines by two dimensional echocardiography via the left parasternal and subcostal approach in a supine position. Doppler echocardiography measured RVSP from tricuspid regurgitant velocity in continuous wave with 2.5MHz transducer and acceleration time(AT) on right ventricular outflow tract in pulsed wave for the estimation of pulmonary arterial pressure. Results : On echocardiography, moderate to severe degree of pulmonary arterial hypertension was defined as RVSP more than 40mmHg, presenting tricuspid regurgitation. Increased right ventricular endsystolic diameter and shortened AT were noted in the increased RVSP group. Increased RVSP was correlated negatively with the shortening of AT. Other clinical data, including pulmonary functional parameters, arterial blood gas analysis and M mode echocardiographic parameters were not changed significantly with the increased RVSP. Conclusion : These findings suggest that shortened AT on pulsed doppler can be useful when quantifying pulmonary arterial pressure with increased RVSP in patients with chronic lung disease with hypoxemia. Doppler echocardiography in pulmonary hypertension of chronic hypoxic lungs is an useful option, based on noninvasiveness under routine clinical practice.

• Korean Journal of Radiology
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• v.20 no.6
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• pp.976-984
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• 2019

Objective: To establish diagnostic criteria for pulmonary arterial hypertension (PAH) in children by using parameters obtained through noninvasive cardiac computed tomography (CCT). Materials and Methods: We retrospectively measured parameters from CCT images of children from a single institution in a multiple stepwise process. A total of 208 children with mean age of 10.5 years (range: 4 days-18.9 years) were assessed. The variables were classified into three groups: the great arteries; the ventricular walls; and the bilateral ventricular cavities. The relationship between the parameters obtained from the CCT images and mean pulmonary arterial pressure (mPAP) was tested and adjusted by the children's body size. Reference curves for the pulmonary trunk diameter (PTD) and ratio of diameter of pulmonary trunk to ascending aorta (rPTAo) of children with CCT images of normal hearts, adjusted for height, were plotted. Threshold lines were established on the reference curves. Results: PTD and rPTAo on the CCT images were significantly positively correlated with mPAP (r > 0.85, p < 0.01). Height was the body size parameter most correlated with PTD (r = 0.91, p < 0.01) and rPTAo (r = -0.69, p < 0.01). On the basis of the threshold lines on the reference curves, PTD and rPTAo both showed 88.9% sensitivity for PAH diagnosis, with negative predictive values of 93.3% and 92.9%, respectively. Conclusion: PTD and rPTAo measured from CCT images were significantly correlated with mPAP in children. Reference curves and the formula of PTD and rPTAo adjusted for height could be practical for diagnosing PAH in children.

• Journal of Chest Surgery
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• v.50 no.5
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• pp.378-381
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• 2017

Patients with an atrial septal defect (ASD) and severe pulmonary arterial hypertension (PAH) are considered ineligible for defect closure surgery because of the risk of right ventricular decompensation and death after the operation. We report the case of a patient with large ASD and severe PAH who was able to undergo defect closure surgery successfully following long-term use of combined oral sildenafil and beraprost.

• Journal of Chest Surgery
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• v.17 no.2
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• pp.250-256
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• 1984

Eisenmenger syndrome is a condition which systemic arterial blood oxygen unsaturation occurs if obstruction in the pulmonary capillaries raises the pulmonary vascular resistance and pulmonary arterial pressure to or beyond systemic levels and then a significant right to left shunt develops across a preexisting cardiac septal defect or an aortopulmonary communication-We have experienced 3 cases of similar condition. Case I is 24 year old man who has had cyanosis and dyspnea on exertion since childhood. His pulmonary arterial pressure was 110/80mmHg. He was operated under diagnosis of the mitral stenosis and tetralogy of Fallot, but it was finally discovered that he had patent ductus arteriosus and ventricular defect was closed with perforated prosthetic patch, but the patient expired due to right heart failure low cardiac output. Case II was 16 year old female who had pulmonary hypertension of 110/85mmHg. She was diagnosed as Eisenmenger syndrome combining with atrial septal defect and patent ductus arteriosus. Case III was 20 year old male. His pulmonary arterial pressure was 110/70mmHg and the underlying defect was patent ductus arteriosus.

• Tuberculosis and Respiratory Diseases
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• v.63 no.4
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• pp.346-352
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• 2007

Background: Pulmonary hypertension is one of the cardiovascular complications of in COPD. However, a diagnosis of pulmonary hypertension requires an invasive test, such as right heart catheterization. NT-proBNP is a cardiac hormone that is elevated when a cardiac volume or pressure overload is present. It was hypothesized that NT-proBNP might play a role in detecting of pulmonary hypertension in COPD patients. Method: The 31 COPD patients, who underwent all of NT-proBNP, echocardiography, and spirometry in Seoul National University Bundang Hospital during the period from November 2003 to July 2005, were retrospectively analyzed. Result: Of the 31 COPD patients, 9 patients had pulmonary hypertension. A significant positive correlation was observed between the NT-proBNP and pulmonary arterial pressure (r=0.589, p=0.002). However, there was no significant correlation observed between the $FEV_1$ and NT-proBNP and $FEV_1$ and pulmonary arterial pressure. Conclusion: NT-proBNP might indicate the presence of pulmonary hypertension in COPD patients.

• Neonatal Medicine
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• v.28 no.1
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• pp.1-6
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• 2021

Persistent pulmonary hypertension of the newborn (PPHN) is a consequence of the failure of a decrease in the elevated pulmonary vascular resistance after birth. Pulmonary vasodilators, including inhaled nitric oxide (iNO), have been the mainstream of targeted therapy for PPHN, but no drugs have been proven to be effective in preterm infants with PPHN. The fetus remains hemodynamically stable despite lower arterial oxygen tension and pulmonary blood flow as compared to full-term newborns. This adaptation is due to the lower oxygen requirement and high oxygen-carrying capacity of fetal circulation. The immature lungs of preterm infants are more vulnerable to reactive oxygen species, and the response of pulmonary vascular dilatation to blood oxygen tension is blunted in preterm infants. Recently, iNO has been reported to be effective in a selected group of preterm infants, such as those with prolonged preterm rupture of membrane-oligohydramnios-pulmonary hypoplasia sequence. PPHN in preterm infants, along with maximum supportive treatment based on fetal physiology and meticulous assessment of cardiovascular function, is in dire need of new treatment guidelines, including optimal dosing strategies for pulmonary vasodilators.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.238-242
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• 1986

The prognosis of patients with VSD and pulmonary hypertension is at least partially related to the reversibility of pulmonary hypertension after surgery. To predict postoperative pulmonary arterial pressure, immediate postbypass values were compared with preoperative hemodynamic data in 18 surgically closed VSD patients aged 6 to 80 months. The following results were obtained. 2] There was a good correlation between preoperative PP/PS and postoperative PP/PS in patients aged over 24 months [r=0.685, p<0.05], and in patients whose QP/QS were over 2.0 [r=0.686, p<0.01]. 3] There was also a good correlation between pump time and postoperative PP in total patients [r=0.697, p<0.005]. 4] Direct correlations were found between preoperative PP/PS and immediate postbypass PP/PS in patients aged over 24 months and in patients whose QP/QS>2.0, but the effect of CPB would participate in some degree. So it may be necessary to reevaluate their relationship after the effect of CPB have been disappeared.