• Tuberculosis and Respiratory Diseases
• /
• v.69 no.4
• /
• pp.237-242
• /
• 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
• /
• v.40 no.2
• /
• pp.147-152
• /
• 1993

Background: There is evidence that platelet is activated in chronic obstructive pulmonary disease and activated platelet with injured endothelium contribute to the pathogenesis of pulmonary hypertension, prognostic factor of chronic obstructive pulmonary disease. So, we have investigated platelet function further in chronic obstructive pulmonary disease and effect of platelet activation on pulmonary hypertension. Method: We studied platelet aggregation ratio and alpha-granule products such as platelet factor 4(PF4) and beta-thromboglobulin (${\beta}$-TG) in control subjects and COPD without and with pulmonary hypertension subjects. Result: 1) The platelet aggregation ratio (PAR) was $0.99{\pm}0.04$ in control subjects, $0.98{\pm}0.05$ in COPD without pulmonary hypertension subjects and $0.89{\pm}0.08$ in COPD with pulmonary hypertension subjects. The platelet aggregation ratio of COPD subjects was tend to decrease than that of control subjects and the ratio of COPD with pulmonary hypertension subjects was significantly lower than that of control subjects. 2) The platelet factor 4 (PF4, IU/ml) was $4.7{\pm}1.2$ in control subjects, $18.6{\pm}4.9$ in COPD without pulmonary hypertension subjects and $57.2{\pm}12.7$ in COPD with pulmonary hypertension subjects. The level of COPD subjects was significantly higher than that of control subjects and the level of COPD with pulmonary hypertension subjects was significantly higher than that of COPD without pulmonary hypertension subjects. 3) The beta-thromboglobulin (${\beta}$-TG, IU/ml) was $34.4{\pm}5.8$ in control subjects, $80.4{\pm}18.1$ in COPD without pulmonary hypertension subjects and $93.0{\pm}14.0$ in COPD with pulmonary hypertension subjects. The level of COPD subjects was significantly higher than that of conrtrol subjects and the level of COPD with pulmonary hypertension subjects was tend to increase than that of COPD without pulmonary hypertension subjects. 4) There was no correlation between the clinical parameters and PAR, PF4 and ${\beta}$-TG but there was significant correlation among PAR, PF4 and ${\beta}$-TG. Conclusion: The platelet is activated in chronic obstructive pulmonary disease and the platelet of COPD with pulmonary hypertension is tend to be activated more than that of COPD without pulmonary hypertension. So, activated platelet may involve in the pathogenesis and maintenance of pulmonary hypertension in COPD subjects and modulation of platelet activity that might reduce pulmonary hypertension needs to be determined.

• Tuberculosis and Respiratory Diseases
• /
• v.48 no.1
• /
• pp.67-71
• /
• 2000

Since its first description in 1951 by Mantz and Craig, pulmonary hypertension associated with portal hypertension has been observed more frequently. In a recent prospective study Hadengue et al. reported 2 % incidence of pulmonary hypertension in patients with portal hypertension. Thus this simultaneous occurrence can no longer be considered to be coincidental. The etiology remains unclear. It is most likely that vasoactive substances, normally metabolized by the liver, may have gained access to pulmonary circulation through portosystemic collaterals in portal hypertension. In genetically susceptible individuals, these substances could lead to pulmonary hypertension by inducing vasoconstriction or direct toxic damage to the wall of the small pulmonary arteries. A recent case of pulmonary hypertension in a 49-year-old woman with portal hypertension due to liver cirrhosis is reported as well as a review of the literature.

• Journal of Yeungnam Medical Science
• /
• v.25 no.1
• /
• pp.50-57
• /
• 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.

• The Journal of the Korean life insurance medical association
• /
• v.30 no.1
• /
• pp.21-23
• /
• 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.

• Sleep Medicine and Psychophysiology
• /
• v.17 no.2
• /
• pp.69-74
• /
• 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
• /
• v.67 no.3
• /
• pp.177-182
• /
• 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
• /
• v.26 no.2
• /
• pp.122-128
• /
• 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
• /
• v.52 no.2
• /
• pp.110-122
• /
• 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
• /
• v.55 no.6
• /
• pp.212-214
• /
• 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.