• Tuberculosis and Respiratory Diseases
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• v.48 no.6
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• pp.973-979
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• 2000

A glycogen storage disease(GSD) type I is a metabolic disease caused by a deficiency in one of the components of the glucose-6-phosphatase(G-6-Pase) system. This disorder results in hypoglycemia, hepatomegaly, lactic acidemia, hyperlipidemia, and hyperuricemia. Comon long(-)term complications include growth retaradation, gout, hepatic adenomas, osteoporosis and renal disease. However the cardiovascular system is rarely involved, and only six cases of pulmonary hypertension associated with GSD I have been reported in the literature. We experienced a case of pulmonary hypertension with type I GSD. A 31-year-old rnan, who had discovered type I GSD and received portocaval shunt operation 22 years ago, was admitted to the hospital with the chief complaint of dyspnea. Echocardiographic examination and cardiac catheterization revealed severe pulmonary hypertension. Nitric oxide and oral prostacycline derivative(beraprost) were tried without acute favorable response. After one year with beraprost, dyspnea, exercise capacity and hemodynamic parameters were improved. We report this case with a review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.43 no.2
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• pp.274-279
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• 1996

Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension in which the primary abnormality is obliterative obstruction of pulmonary veins, especially venules. Clinicaly, we should suspect this disease in the case of congestive cardiac failure with pulmonary hypertension, chronic interstitial pulmonary edema, and normal or elevated wedge pressure on cardiac catheterization. We experience a case of pulmonary hypertension due to pulmonary veno-occlusive disease. A 55-years -old woman developed progressive dry cough and dyspnea for 3 months. Physical examination showed normal heart sounds, diffuse crackles in the whole lung fields. The liver was not palpable and pitting edema was absent. The diagnosis was made by chest HRCT, 2-D echocardiography, normal pulmonary capillary wedge pressure on cardiac catheterization, and confirmed by thoracoscopic lung biopsy. This patient was treated with vasodilator(calcium antagonist) and with mild symptomatic improvement. We reported a case of pulmonary veno-occlusive disease with review of literatures.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.610-614
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• 2003

Pulmonary hypertension may be associated with variable conditions such as the hyperkinetic state or pulmonary vascular obstruction. In these, stenosis of the individual pulmonary veins without any cardiac or vascular malformation is very rare. We experienced stenosis of individual pulmonary veins in a 10 months old boy who was admitted with recurrent dyspnea and cyanosis and then underwent angiogram and a lung perfusion scan.

• Journal of Chest Surgery
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• v.23 no.4
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• pp.659-666
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• 1990

We evaluated 68 patients with mitral valve disease who underwent mitral valve replacement from April, 1986, to December, 1988. Among them 20 patients showed increased systolic pulmonary arterial pressure greater than 60 mmHg and postoperative hemodynamic data were obtained in 19 patients average 13 months after mitral valve replacement. The results were as followings. 1. Average pulmonary systolic pressure decreased from 84.8$\pm$4.5 preoperatively to 33. 0$\pm$1.9mmHg postoperatively[P<0.001]. 2. Average pulmonary vascular resistance index decreased from 1425$\pm$148 preoperatively to 287+35.8 dyne * sec * cm2 * m postoperatively[P<0.001]. 3. Average cardiac index rose from 1.927$\pm$0.169 preoperatively to 2.625$\pm$0.159 L/min/m2 postoperatively [P <0.005]. This study shows that pulmonary hypertension and the increased pulmonary vascular resistance index due to mitral valve disease can regress significantly after mitral valve replacement.

• The Journal of the Korean life insurance medical association
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• v.30 no.1
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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.

• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.689-695
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• 2009

Purpose : To examine the effect of bosentan, a dual endothelin receptor (ER) antagonist, on the development of monocrotaline (MCT)-induced pulmonary hypertension in rats by especially focusing on the pulmonary vascular morphology changes. Methods : Sprague-Dawley rats were treated as follows: controls received a subcutaneous saline injection, MCT-treated rats received a subcutaneous MCT injection, and bosentan-treated rats received a MCT injection followed by treatment with bosentan (20 mg/kg/day). To assess the effects of ER blockade on the time course, the animals were exsanguinated, and their hearts and lungs were dissected after 7, 14, or 28 days. Results : The mean body weights of the MCT- and bosentan-treated rats were significantly lower than that of the control rats on days 7, 14, and 28. Bosentan administration significantly inhibited the progression of right ventricular hypertrophy on day 28 (right ventricle/[left ventricle＋septum]: $0.71{\pm}0.10$ in MCT-treated rats vs. $0.49{\pm}0.09$ in bosentan-treated rats; P<0.05). Quantitative analysis of peripheral pulmonary arteries revealed that the increase in medial wall thickness after MCT injection was significantly attenuated in the bosentan-treated rats on day 28 ($49.96{\pm}10.06%$ in MCT-treated rats vs. $47.09{\pm}10.48%$ in bosentan-treated rats; P<0.05). In addition, the increase in the number of intra-acinar muscular arteries after MCT injection was reduced by bosentan on days 14 and 28. Conclusion : Bosentan administration in intermediate doses exerts inhibitory effects on lung vascular hypertrophy and right ventricular hypertrophy during the development of MCT-induced pulmonary hypertension in rats.

• Tuberculosis and Respiratory Diseases
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• v.59 no.2
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• pp.170-178
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• 2005

Background : Idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare but significantly imperative in inducing chronic pulmonary hypertension. Clinically, it is difficult to distinguish between IPAH and CTEPH. However, the treatment of pulmonary hypertension is different depending on the disease. The present study was performed to analyze the similarities and differences in clinical features between IPAH and CTEPH. Methods : During a nine-year period, thirty-three patients with IPAH and twenty-two patients with CTEPH were enrolled. Symptoms, physical findings, chest radiograph, electrocardiograph, pulmonary function test, echocardiograph, perfusion lung scan, right heart catheterization results were analyzed between both the groups. Results : The median age of IPAH group was 33 (6~70) years that was lower than that (52(27~80) years) of CTEPH group. Amongst the IPAH patients, there was female predominance (76 %) and there was no sex difference between the patients with CTEPH. Both the groups exhibited similarity in the results of chest radiograph, electrocardiograph, pulmonary function test, and echocardiograph. In the perfusion lung scan, all IPAH patients exhibited findings with normal (28%) or low probability (72%) of pulmonary embolism and all CTEPH patients exhibited findings with high probability of pulmonary embolism. Conclusion : Although IPAH and CTEPH bear similarities in terms of symptoms, physical signs and general investigation results, there were differences in age distribution, sex predominance and results of perfusion lung scan.

• Journal of Chest Surgery
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• v.20 no.4
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• pp.688-694
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• 1987

At the Dept. of Thoracic and Cardiovascular Surgery of Pusan National University Hospital, postoperative cardiac catheterizations were performed in 12 patients of ventricular septal defect with severe pulmonary hypertension [Pp/Ps>0.75], who were operated during the period from July 1981 to Dec. 1986. The mean age of the patients preoperatively was 12.4 [range: 4-18] year-old and the mean follow-up duration was 25.8 [range: 8-53] month per patient. In comparison with the preoperative data, the systolic pulmonary artery pressure [SPAP] was decreased from 103.6*18.4 to 70.4*35.9 mmHg [p<0.01] and the Pp/Ps was decreased from 0.89*0.10 to 0.58*0.27 [p<0.01]. But the Rp/Rs and Rp were not meaningfully changed, from 0.31*0.16 and 7.6*0.4 unit to 0.41*0.32 and 8.0*6.6 unit, respectively. The preoperative Qp/Qs was bellow 2.0[mean: 1.6] in 3 out of 4 cases whose postoperative Rp/Rs and Rp were above 0.75 and 15 unit, respectively. On the contrary, the preoperative Qp/Qs was above 2.0 [mean: 3.5] in all of the 8 cases, whose postoperative Rp/Rs and Rp were below 0.50 and 10 unit, respectively.

• Tuberculosis and Respiratory Diseases
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• v.59 no.1
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• pp.5-13
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• 2005

Chronic obstructive pulmonary disease (COPD) is a chronic progressive disease, characterized by irreversible airflow limitation, with a partially reversible component. The pathological abnormalities of COPD are associated with lung inflammation, imbalances of proteinase and antiproteinase, and oxidative stress, which are induced by noxious particles and gases in susceptible individuals. The physiological changes of COPD are mucus hypersecretion, ciliary dysfunction, airflow limitation, pulmonary hyperinflation, gas exchange abnormalities, pulmonary hypertension, cor pulmonale and systemic effects. The airflow limitation principally results from an increase in the resistance of the small conducting airways and a decrease in pulmonary elastic recoil due to emphysematous lung destruction. This article provides a general overview of the pathophysiology of COPD.

• Journal of Chest Surgery
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• v.26 no.2
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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.