• Tuberculosis and Respiratory Diseases
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• 제85권1호
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• pp.1-10
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• 2022

Pulmonary hypertension (PH) is a condition of increased blood pressure in the pulmonary arteries and is diagnosed with an increased a mean pulmonary artery pressure ≥25 mm Hg. This condition may be associated with multiple clinical situations. Based on pathophysiological mechanisms, clinical presentation, hemodynamic profiles, and treatment strategies, the patients were classified into five clinical groups. Although there have been major advances in the management of PH, it is still associated with significant morbidity and mortality. The diagnosis and treatment of PH have been performed mainly by following European guidelines, even in Korea because the country lacks localized PH guidelines. European treatment guidelines do not reflect the actual status of Korea. Therefore, the European diagnosis and treatment of PH have not been tailored well to suit the needs of Korean patients with PH. To address this issue, we developed this guideline to facilitate the diagnosis and treatment of PH appropriately in Korea, a country where the consensus for the diagnosis and treatment of PH remains insufficient. This is the first edition of the guidelines for the diagnosis and treatment of PH in Korea, and it is primarily based on the '2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.' with the acceptance and adaptation of recent publications of PH.

• Clinical and Experimental Pediatrics
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• 제53권6호
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• pp.688-693
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• 2010

An increase in the number of preterm infants and a decrease in the gestational age at birth have resulted in an increase in the number of patients with significant bronchopulmonary dysplasia (BPD) and secondary pulmonary hypertension (PH). PH contributes significantly to the high morbidity and mortality in the BPD patients. Therefore, regular monitoring for PH by using echocardiography and B-type natriuretic peptide (BNP) or N-terminal-proBNP must be conducted in the BPD patients with greater than moderate degree to prevent PH and to ensure early treatment if PH is present. In the BPD patients with significant PH, multi-modality treatment, including treatment for correcting an underlying disease, oxygen supply, use of diverse selective pulmonary vasodilators (inhaled nitric oxide, inhaled prostacyclins, sildenafil, and endothelin-receptor antagonist) and other methods, is mandatory.

• Neonatal Medicine
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• 제25권3호
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• pp.91-95
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• 2018

Bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) is increasingly being recognized as a cause of morbidity and mortality in preterm infants. Recently, BPD-PH has also been shown to have additional long-term negative effects on neurodevelopmental outcomes and right ventricular function. Several significant risk factors associated with the development of BPD-PH have been identified. A screening strategy for BPD-PH is needed for infants presenting more than one risk factor. In addition, an early echocardiogram within 14 days of age may be a useful tool to identify infants at high-risk for BPD-PH. We have reviewed recent progress in research concerning clinical characteristics, presentation, and outcomes of BPD-PH and have suggested direction for future studies.

• Journal of Cardiovascular Imaging
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• 제26권4호
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• pp.189-200
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• 2018

Pulmonary hypertension (PH) is defined as resting mean pulmonary artery pressure ${\geq}25mmHg$ and is caused by multiple etiologies including heart, lung or other systemic diseases. Evaluation of right ventricular (RV) function in PH is very important to plan treatment and determine prognosis. However, quantification of volume and function of the RV remains difficult due to complicated RV geometry. A number of imaging tools has been utilized to diagnose PH and assess RV function. Each imaging technique including conventional echocardiography, three-dimensional echocardiography, strain echocardiography, computed tomography and cardiac magnetic resonance imaging has-advantages and limitations and can provide unique information. In this article, we provide a comprehensive review of the utility, advantages and shortcomings of the multimodality imaging used to evaluate patients with PH.

• The Korean Journal of Physiology and Pharmacology
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• 제17권1호
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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.

• Archives of Pharmacal Research
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• 제26권8호
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• pp.612-619
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• 2003

In this study, we evaluated the effects of oral administration of DA-8159, a selective phosphodiesterase-5 inhibitor, on the development of pulmonary hypertension (PH) induced by monocrotaline (MCT). Rats were administered either MCT (60 mg/kg) or saline. MCT-treated rats were divided into three groups and received orally administered vehicle, or 1 mg/kg or 5 mg/kg of DA-8159, twice a day for twenty-one days. The MCT group demonstrated increased right ventricular weights, medial wall thickening in the pulmonary arteries, myocardial fibrosis and the level of plasma cyclic guanosine monophosphate (cGMP), along with decreased body weight gains. However, DA-8159 markedly and dose-dependently reduced the development of right ventricular hypertrophy and medial wall thickening. DA-8159 also amplified the increase in plasma cGMP level and significantly increased the level of lung cGMP, compared with the MCT group. Although the body weight gain was still lower from the saline-treated control group, DA-8159 demonstrated a significant increase in body weight gains, in both 1 mg/kg and 5 mg/kg groups, when compared with the MCT group. In myocardial morphology, MCT-induced myocardial fibrosis was markedly prevented by DA-8159. These results suggest that DA-8159 may be a useful oral treatment option for PH.

• Tuberculosis and Respiratory Diseases
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• 제47권2호
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• pp.150-160
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• 1999

연구배경: CREST 증후군은 전신성 경화증의 이형으로 전신성 경화증에 비교하여 병의 진행이 늦은 것으로 알려져 있다. 하지만 CREST 증후군 환자의 약 50%에서 폐나 심장질환의 증거 없이 폐고혈압이 발생하며, 이러한 환자들의 이년 생존률은 40%로 폐고혈압이 없는 환자에 비해 예후가 매우 나쁘다. 그러나 CREST 증후군에 동반되는 폐고혈압의 원인과 병리기전에 대해서는 전혀 알려진 바 없으며 막연히 원발성 폐고혈압의 이형(variant form) 또는 이차성 폐고혈압으로 분류되고 있다. 총상병변(plexiform lesion) 내 증식된 내피세포의 클론성 검증은 폐고혈압을 원발성과 이차성으로 나누는 기준이 되고 있다. 이에 저자들은 CREST 증후군이 동반된 폐고혈압 환자의 총상병변 내피세포의 클론성을 분석함으로써 CREST 동반된 폐고혈압이 원발성인지 이차성인지를 알아보고자 하였다. 방법: 폐고혈압을 동반한 CREST 증후군 여자환자에서 얻은 폐조직의 파라핀 block을 사용하여 microdissection 방법으로 총상병변내에 증식된 내피세포를 분리한후 X-염색체의 methylation pattern을 이용하여 클론성 분석을 시행하였다. 같은 방법으로 중막 비후를 보이는 혈관의 혈관 평활근과 폐실질의 클론성도 분석하였다. 결과: 14개 총상병변의 '증식된 내피세포'의 클론성 모두 다클론성 이었다. 또한 중막비후가 현저한 5개 폐동맥의 평활근세포 클론성도 모두 다클론성 이었다. 결론: 이상의 결과는 CREST 증후군에 동반되는 폐고혈압이 원발성 폐고혈압과는 다른 병리 기전을 갖는 이차성 폐고혈압임을 시사한다.

• Neonatal Medicine
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• 제18권1호
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• pp.96-103
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• 2011

목적: 극소 저체중 출생아에서 폐고혈압증은 기관지 폐 이형성증의 대표적인 심폐관련 합병증으로 높은 사망률과 합병증과 연관되어 있으나, 이에 대한 연구는 드문 실정이다. 이에 본 저자들은 기관지 폐 이형성증을 가진 극소 저체중 출생아에서 폐고혈압증의 유병률, 경과 및 치료뿐 아니라 예후 및 이와 관련된 인자들에 대해 확인해 보고자 하였다. 방법: 2000년 1월부터 2007년 7월까지 성균관대학교 의과대학 삼성서울병원 신생아 집중치료실에 입원한 출생 체중 1,500g 미만 환아 865명 중 28일 이상 산소 치료를 필요로 하였던220명을 대상으로 하였다. 심장 초음파 검사상 삼첨판 역류 속도 3 m/s 이상이 확인되면서, 우심실 비대 혹은 심실 중격이 평형함이 관찰되는 경우를 폐고혈압으로 진단하였으며, 이들을 대조군과 비교하였다. 환아들의 의무기록을 후향적으로 분석하여, 인구학적, 주산기 인자, 합병증, 사망률을 비교하였으며, 폐고혈압증을 가진 환아 중 사망군과 생존군으로 나누어 이들의 특성 및 관련인자를 분석하였다. 결과: 기관지 폐 이형성증을 가진 VLBWI 218명 중 14명(6%)에서 폐고혈압증이 발생하였으며, 모두 중등도 이상의 기관지 폐 이형성증에서 발생하였다. 폐고혈압증은 평균 생후 133일(40-224일)에 진단 받았으며, 중증 기관지 폐 이형성증, 부당 경량아, 외부 환아는 폐고혈압증 발생과 관련된 인자이다. 기관지 폐 이형성증에서 발생한 폐고혈압증의 사망률은 57%이며, 중증 기관지 폐 이형성증은 사망의 중요한 원인이다. 생존한 환아에서 회복되는데 까지 걸리는 시간은 평균 3개월이었다 결론: 극소 저체중 출생아에서 기관지 폐 이형성증과 관련된 폐고혈압증은 높은 사망률 및 합병증을 보이며, 이들의 발생 및 예후는 기관지 폐 이형성증 정도와 연관성이 있다. 이들의 조기진단을 위해 중등도 이상의 기관지 폐 이형성증 환아에서 정기적인 선별검사 및 퇴원 이후에도 장기간 지속적인 추적 관찰이 필요하다.