• Tuberculosis and Respiratory Diseases
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• 제60권2호
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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.

• BMB Reports
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• 제47권6호
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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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• 제53권1호
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• pp.93-96
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• 2010

15년전 심방중격 결손증 진단을 받은 47세 남자 환자가 심도자 검사를 위해 입원하였다. 환자는 입술과 손톱에서 명확한 청색증을 보이고 있었으며 심한 폐동맥 고혈압을 나타내고 있었다. 본 환자는 지난 수년간 아이젠멩거 증후군으로 진단되어 대증적 치료만을 받아오고 있었다. 심도자 검사 후 환자는 흡입형 Iloprost 치료를 시작 하였으며 성공적으로 심방중격결손증 수술을 받을 수 있었다. 환자는 수술 후에도 치료를 지속하였다.

• 약학회지
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• 제54권2호
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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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• 제46권6호
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• pp.846-855
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• 1999

연구배경 : 만성 저산소성 폐질환 환자의 폐동맥고혈압은 폐혈류 장애에 따른 악화에 이르는 주요 합병증이다. 폐동맥 고혈압과 이에 수반되는 폐성심의 임상적 진단은 때로 모호하여 폐동맥압의 비관혈적인 심초음파 검사의 중요성이 대두되고 있다. 최근 도플러 심초음파는 기존의 M모드 검사법에 비해 측정치의 정확도, 재생산성과 좋은 창을 얻을 수 있는 상대적인 장점이 있어 폐기종성 환자에서 보다 적용하기 용이한 검사법이다. 본 연구는 만성 저산소성 폐질환의 폐동맥 고혈압 예상 환자에서 도플러 검사법이 갖는 유의한 지표를 확인함에 목적이 있다. 방 법 : 대상환자는 만성 저산소성 폐질환을 보이며 임상소견상 폐동맥 고혈압이 예상되어 심초음파 검사를 시행받은 19명의 환자들을 대상으로 의의있는 폐동맥 고혈압 지표에 대해 조사하였다. 대상 환자들은 와위에서 좌흉골연이나 늑골하에 2.5MHz 진동자를 두고 도플러 및 M모드 심초음파를 시행하였고 특히, 삼첨판 역류시는 도플러 모드로 예측되는 우심실 수축기압(RVSP)이 40mmHg 이상인 군을 그 미만이거나 삼첨판 역류가 없었던 경우와 구분하여 다른 임상지표들에 대하여 비교, 분석하였다. 결 과 : 1) M모드 심초음파의 늑골하 사방관찰에서 RVSP 상승군이 대조군에 비해 수축기말 우심실 직경은 증가하였지만 그외 이완기말 우심실 직경, 수축기 및 이완기말 면적, 우심실 지유벽의 두께, 우심실 출구의 직경과 심실 중벽 두께 등은 두군간 차이가 없었다. 2) 도플러 모드에서 RVSP 상승군의 우심실 출구 수축기 가속기간(AT)은 $82{\pm}9msec$로 대조군 $96{\pm}16msec$에 비해 유의하게 단축되어 있었다. 3) 동맥혈 검사상 RVSP 상승군은 대조군에 비해 산소분압이 감소되어 있었으나 유의한 차이는 없었고 폐기능검사의 노력성 폐활량 및 1초 노력성호기량도 상승군에서 저하되었으나 두군간 유의한 차이는 없었다. 4) AT는 RVSP와 통계학적으로 유의한 역상관관계를 보여주었다. 결 론 : 만성 저산소성 폐질환에서 임상소견상 폐동맥 고혈압이 예상되는 경우 이를 규명하기 위해 심초음파 검사를 하여 컬러도플러로 삼첨판 역류가 확인되면 연속파 도플러로 RVSP를 예측할 수 있으며 이는 간헐파 도플러의 AT의 단축과 관련되어 있음을 확인하였다. 도플러 모드하의 RVSP 및 AT의 측정은 폐동맥 고혈압의 좋은 지표가 될 수 있을 것으로 예상된다.

• Korean Journal of Radiology
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• 제20권6호
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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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• 제50권5호
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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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• 제17권2호
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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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• 제63권4호
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• pp.346-352
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• 2007

연구 배경: 폐동맥 고혈압은 만성 폐쇄성 폐질환 환자에서 중요한 심혈관계 합병증이다. 뇌나트륨이뇨펩티드는 허혈성 심질환과 심부전의 경우에서 진단적 효율성과 예후 예측인자로서의 효율성이 잘 알려져 있고 우심에 압력 과부하가 있는 상황에서도 증가된다. 뇌나트륨이뇨펩티드의 측정이 만성 폐쇄성 폐질환 환자에서 폐동맥 고혈압의 진단에 유용성이 있는지 알아보고자 폐동맥압과 뇌나트륨이뇨펩티드와의 상관관계를 분석하였다. 연구 방법: 의무 기록 분석을 통하여 뇌나트륨이뇨펩티드, 심초음파, 폐기능 검사를 모두 실시한 만성 폐쇄성 폐질환 환자들을 대상으로 연구를 수행하였다. 뇌나트륨이뇨펩티드(NT-proBNP), 폐동맥압(PAP), 1초간 노력성 폐활량($FEV_1$)과의 상관관계를 분석하였다. 결과: 총 31명의 환자가 분석에 포함되었으며 폐동맥 고혈압이 동반된 환자는 9명(26.7%)이었다. 폐동맥압과 뇌나트륨이뇨펩티드 사이에 유의한 상관관계가 관찰되었다(r=0.589, p=0.002). 결론: 뇌나트륨이뇨펩티드는 만성 폐쇄성 폐질환 환자들에서 동반된 폐동맥 고혈압의 진단에 사용될 수 있을 것으로 사료된다.

• Neonatal Medicine
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• 제28권1호
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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.