• Tuberculosis and Respiratory Diseases
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• 제69권4호
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• pp.237-242
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• 2010

Pulmonary vascular disease is a category of disorders, including pulmonary hypertension, pulmonary embolism or chronic thromboembolic pulmonary hypertension, pulmonary vasculitis, pulmonary vascular disease secondary to chronic respiratory disease, and pulmonary vascular tumor and malformations. This article reviews the recent advances in this wide spectrum of pulmonary vascular diseases.

• Tuberculosis and Respiratory Diseases
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• 제40권2호
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• pp.147-152
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• 1993

연구배경 : 만성폐쇄성폐질환에서 혈소판이 활성화되어 있는 근거가 있으며 이 질환의 예후 인자로 잘 알려진 폐동맥 고혈압이 활성화된 혈소판 및 손상된 내피세포가 관여하리라는 보고가 있다. 이에 만성폐쇄성폐질환에서 혈소판 활성화를 알아보고 혈소판 활성화가 폐동맥 고혈압에 미치는 영향을 알아보고자 연구를 시행하였다. 방법 : 대조군 및 만성폐쇄성폐질환의 폐동맥 정상혈압군과 고혈압군을 대상으로 혈소판 응집비 및 혈소판의 alpha-granule에 함유되어 있는 Platelet factor 4와 $\beta$-thromboglobulin을 측정하여 다음과 같은 결과를 얻었다. 결과: 1) Platelet aggregation ratio (PAR)는 대조군 $0.99{\pm}0.04$, 폐동맥 정상혈압군 $0.98{\pm}0.05$, 폐동맥 고혈압군 $0.89{\pm}0.08$으로 감소하는 추세를 보였으며, 폐동액 고혈압군에서 대조군보다 통계학적으로 의미 있게 감소 하였다(p<0.05) (Table 2, Fig. 1). 2) Platelet factor 4 (PF4, IU/ml)는 대조군 $4.7{\pm}1.2$, 폐동맥 정상혈압군 $18.6{\pm}4.9$, 폐동맥 고혈압군 $57.2{\pm}12.7$으로 만성폐쇄성폐질환에서 대조군보다 통계학적으로 의미 있게 증가 하였으며(p<0.01), 폐동맥 고혈압군에서 폐동맥 정상혈압군보다 통계학적으로 의미 있게 증가 하였다(p<0.01) (Table 2, Fig. 2). 3) Beta-thromboglobulin (${\beta}$-TG, IU/ml)은 대조군 $34.4{\pm}5.8$, 폐동맥 정상혈압군 $80.4{\pm}18.1$, 폐동맥 고혈압군 $93.0{\pm}14.0$으로 만성폐쇄성폐질환에서 대조군보다 통계학적으로 의미 있게 증가 하였으며(p<0.01) 폐동맥 고혈압군에서 폐동맥 정상혈압군보다 통계학적인 의미는 없지만 증가하는 경향을 보였다(Table 2, Fig.3). 4) 임상 소견과 PAR, PF4 및 ${\beta}$-TG 사이에는 상관 관계가 없었으나 PAR, PF4및 ${\beta}$-TG 사이에는 통계학적으로 의미 있는 상관관계를 보였다 (Table 3). 결론 : 만성폐쇄성폐질환에서 혈소판이 뚜렷이 활성화되어 있었으며 폐동맥 고혈압군에서 좀더 활성화되는 경향을 보였다. 따라서 만성폐쇄성폐질환에서 혈소판 활성화가 폐동맥 고혈압의 유발 및 유지에 관여할 것으로 사료되며 치료에 대한 연구가 필요할 것으로 사료된다.

• Tuberculosis and Respiratory Diseases
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• 제48권1호
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• pp.67-71
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• 2000

문맥고혈압에 동반된 폐고혈압은 비교적 드문 질환으로 1951년 Mantz와 Craig가 처음 보고한 후 꾸준히 보고되고 있으나 국내엔 아직 보고된 바가 없다. 최근 간경변증으로 인한 복수와 호흡곤란을 주소로 내원한 49세 여자 환자에서 폐고혈압이 동반되어 있었던 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Yeungnam Medical Science
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• 제25권1호
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• pp.50-57
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• 2008

Pulmonary hypertension is an increase in blood pressure in the pulmonary artery, pulmonary vein or pulmonary capillaries. Depending on the cause, pulmonary hypertension can be a severe disease with markedly decreased exercise tolerance and right-sided heart failure. Pulmonary hypertension can present as one of five different types: arterial, venous, hypoxic, thromboembolic, or miscellaneous. Chronic obstructive pulmonary disease with severe pulmonary hypertension is a rare disease. A 52-year-old man presented with a complaint of aggravating dyspnea. The mean pulmonary arterial pressure was 61.5 mmHg by Doppler echocardiogram. The patient was prescribed diuretics, digoxin, bronchodilator, sildenafil, bosentan and an oxygen supply. However, he ultimately died of cor pulmonale. Thus, diagnosis and early combination therapy are important.

• 보험의학회지
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• 제30권1호
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• pp.21-23
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• 2011

Chronic thromboembolic pulmonary hypertension(CTEPH) is known as class IV pulmonary hypertension. Unlike other subtype of pulmonary hypertension, surgical treatment such as pulmonary endarterectomy is well known therapeutic strategy. Also there's oral disease-modifying drug which is developed lately. According to recent article, the prognosis of CTEPH is markedly improved. If prognosis of certain disease is improved, insurance rating should be altered. Whether rating change is necessary or not, mortality analysis of CTEPH was performed from recently published source article, Estimated extra-risks are MR of 525% and EDR of 37‰. In conclusion, the extra-risks of CTEPH are still very high.

• 수면정신생리
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• 제17권2호
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• pp.69-74
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• 2010

Obstructive sleep apnea syndrome is associated with significant cardiovascular morbidity and increased mortality. However, it was controversial whether obstructive sleep apnea syndrome could cause pulmonary hypertension. The controversy was resolved by several studies that have shown pulmonary hypertension in 20% to 40% of patients with obstructive sleep apnea syndrome without underlying other cardiopulmonary diseases and reductions in pulmonary arterial pressure in patients with obstructive sleep apnea syndrome after treatment with nocturnal continuous positive airway pressure. Recent studies provide strong evidence for endothelial dysfunction in obstructive sleep apnea syndrome and pulmonary hypertension. Endothelin-1 is a 21 amino acid peptide with diverse biologic activity such as highly potent vasoconstrictor and mitogen regulator that may play a key role in obstructive sleep ap-nea syndrome and pulmonary hypertension. Continuous positive airway pressure therapy is moderately effective in reducing pulmonary arterial pressure. Further researches are needed to assess the therapeutic efficacy of pharmacologic therapy with agents that inhibit the action of endothelin-1 in obstructive sleep apnea syndrome patients with pulmonary hypertension.

• Tuberculosis and Respiratory Diseases
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• 제67권3호
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• pp.177-182
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• 2009

Pulmonary arterial hypertension is a progressive, symptomatic, and ultimately fatal disorder for which substantial advances in treatment have been made during the past decade. This article reviews the recent advances in the field of pulmonary arterial hypertension (PAH). Epidemiology, genetics, treatment and prognosis will be the main focus of this update.

• Journal of Chest Surgery
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• 제26권2호
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• pp.122-128
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• 1993

This series compromised 31 patients with pulmonary hypertension of 282 patients of ventricular septal defect(VSD) who underwent operation at the department of Thoracic and Cardiovascular Surgery in Chonnam University Hospital, from January, 1986 to December, 1991. Pulmonary hypertension was noted in 59 of 280 cases of VSD. Of them, 31 cases underwent cardiac catheterization on postoperative 8th to 77th month. Age at operation was ranged from 10 months to 29 years (mean 9.13 years). 17 patients were male and 14 patients were female. Results of follow-up studies were as follows: Cardiothoracic ratio was decreased from 0.59${\pm}$0.04 to 0.54${\pm}$0.03 (p=NS). Postoperative systolic pulmonary arterial pressure (PAPs), mean pulmonary arterial pressure (PAPm), and systolic right ventricular pressure (RVPs) were decreased significantly (p<0.001). And also Rp/Rs was decreased from 0.37${\pm}$0.21 to 0.14${\pm}$0.06 (p<0.02). However, systemic arterial pressure (SAP), right atrial pressure (RAP), and pulmonary capillary wedge pressure (PCWP) were changed insignificantly. There were significant relations of follow-up period with the decrement of PAP(p<0.005). In contrary, ther were no relations between the decrement of PAP and the age at operation. These data suggested that the long-term hemodynamic changes remained to be determined in some of the patients, even though they Were asymptomatic, with pulmonary hypertension.

• Korean Circulation Journal
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• 제52권2호
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• pp.110-122
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• 2022

Pulmonary hypertension is a rare and progressive illness with a devastating prognosis. Promising research efforts have advanced the understanding and recognition of the pathobiology of pulmonary hypertension. Despite remarkable achievements in terms of improving the survival rate, reducing disease progression, and enhancing quality of life, pulmonary arterial hypertension (PAH) is not completely curable. Therefore, an effective treatment strategy is still needed. Recently, many studies of the underlying molecular mechanisms and technological developments have led to new approaches and paradigms for PAH treatment. Management based on stem cells and related paracrine effects, epigenetic drugs and gene therapies has yielded prospective results for PAH treatment in preclinical research. Further trials are ongoing to optimize these important insights into clinical circumstances.

• Clinical and Experimental Pediatrics
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• 제55권6호
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• pp.212-214
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• 2012

Rubinstein-Taybi syndrome (RTS) is characterized by peculiar facies, mental retardation, broad thumbs, and great toes. Approximately one-third of the affected individuals have a variety of congenital heart diseases. They can also have upper airway obstruction during sleep, due to hypotonia and the anatomy of the oropharynx and airway, which make these patients susceptible to obstructive sleep apnea (OSA). In our case, pulmonary hypertension was caused, successively, by congenital heart defects (a large patent ductus arteriosus and arch hypoplasia) and obstructive sleep apnea during early infancy. The congenital heart defects were surgically corrected, but persistent pulmonary hypertension was identified 2 months after the operation. This pulmonary hypertension was due to OSA, and it was relieved by nasal continuous positive airway pressure. This case is the first report of pulmonary hypertension from OSA in a young infant with RTS.