• Title/Summary/Keyword: pulmonary arterial hypertension

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Association of PPARGC1A Gene Variants with Hypertension in Korean Population

  • Jin, Hyun-Seok;Park, Sangwook
    • Biomedical Science Letters
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    • v.27 no.1
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    • pp.12-18
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    • 2021
  • Hypertension (HTN) is one of the cardiovascular disease risk factors. Peroxisome proliferator-activated receptor γ coactivator 1 alpha (PPARGC1A) is involved in a master modulator of mitochondrial biogenesis, and pulmonary arterial hypertension. In this study, we report results of PPARGC1A were associated with hypertension and its intermediate phenotype of systolic (SBP) and diastolic blood pressure (DBP) in the Korean population. In detail, identifying a susceptibility locus, 3 SNPs for HTN, 2 SNPs for SBP, 3 SNPs for DBP at P<0.05. Among them, rs1472095 in PPARGC1A gene statistically demonstrated one of the significant correlations with Hypertension (P-value=0.00359, OR=0.8, 95% CI=0.68~0.93). The minor allele (T) of PPARGC1A was statistically associated with the increased value of DBP, SBP, and the increase risk of hypertension. We aim to manifest a significant association between genetic variant in PPARGC1A and hypertension. This finding suggested that association of PPARGC1A genetic polymorphism and HTN accelerates our understanding of blood pressure control and underlines potential drug targets for treatment of hypertension.

Pulmonary Thromboendarterectomy Under Total Circulatory Arrest (완전순환정지를 이용한 폐동맥색전증의 수술 치험 1례)

  • Kim, Chang-Young;Kang, Chang-Hyeun;Ahn, Hyuk
    • Journal of Chest Surgery
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    • v.35 no.9
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    • pp.684-687
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    • 2002
  • Besides lung transplantation, pulmonary thromboembolectomy is the only effective therapeutic option for chronic thromboembolic pulmonary hypertension. It is however associated with a considerably high hospital mortality between 6.6 to 23%. Proper patient selection is critical when considering a patient for pulmonary thromboembolectomy. And It cannot be overemphasised that the key to the success of the operation is complete endarterectomy of the entire pulmonary arterial tree. We report that pulmonary thromboendarterectomy under total circulatory arrest was an effective and safe method in the surgical correction of the chronic thrornboernbolic pulmonary hypertension and enabled complete removal of superimposed peripheral organized thrombi in a good operative field.

Clinical and Hemodynamic Investigation of Atrial Septal Defect (심방중격결손증의 혈류역학적 상관관계 및 임상적 고찰)

  • 정상조
    • Journal of Chest Surgery
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    • v.24 no.5
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    • pp.445-450
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    • 1991
  • Forty eight patients diagnosed as atrial septal defect, had been operated from April 1986 to December 1990 at the Department of Thoracic and Cardiovascular Surgery, Inha University Hospital, were analysed retrospectively. Of the 48 patients, 12 patients, were male and 36 patients were female, Their ranged from 8 months to 51 years old, and the mean was 18.0 years old. The proceeding two symptoms were exertional dyspnea and frequent upper respiratory infection. But 10 patients [20.8%] were asymptomatic. Electrocardiographic findings were regular sinus rhythm in 100.0%, RVH in 29.2%, incomplete RBBB in 27.1%, and first degree AV block in 2.1%. Hemodynamic studies were performed in 38 of 48 patients. Significant pulmonary hypertension[over 50mmHg in systolic pulmonary arterial pressure], which is the most serious risk factor, was developed in 4 patients[8.3%]. There was statistical significance between size of defect[cm2/BSA-M2] and systolic pulmonary arterial pressure[sPAP] retrospectively[p=0.036]. We could not find the correlationship between age and Qs % size. Anatomically, secundum type constituted 97.9%, in which 43 patients were PFO type[91.6%] and 3 patients were IVC type[6.3%]. 38 of 48 ASD patients were repaired with patch closure[72.9%] and remains were repaired with direct closure[27.1%]. The operative result was excellent except two cases of transient postoperative arrhythmia. There was no operative mortality.

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The Senning Operation for Transition of the Great Arteries -a 7-year prospective study - (대혈관전위증의 Senning 수술요법)

  • Kim, Gi-Bong;No, Jun-Ryang
    • Journal of Chest Surgery
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    • v.22 no.5
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    • pp.753-759
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    • 1989
  • Recently, the arterial switch operation has received more interest and increased application for many patients with TGA [Transposition of the Great Arteries]. Not all patients, however, are suitable candidates for this approach, and its success will be measured against the Senning procedure. From June 1982 through June 1989, 48 consecutive patients underwent correction of TGA by a modification of the Senning procedure in our hospital. 34 were males and 14 females, with ages ranging from 3 months to 99 months [mean age 14 months]. The patients were divided into two groups, group I [TGA with intact ventricular septum or without significant pulmonary hypertension] and group II [TGA with significant pulmonary hypertension]. The hospital mortality was 9.5 % in group I and 55.6 % in group lI. 31 patients were discharged from the hospital after a Senning operation and have been followed a mean of 31.2 months. There were 5 late deaths, 3 in group I and 2 in group II. All patients were followed up with EKG, echocardiogram, and in several cases, Holter monitor and cardiac catheterization and angiography were also performed. There have been arrhythmias in 7, superior vena caval obstruction in 5, pulmonary venous obstruction in 2 and tricuspid insufficiency and/or right ventricular dysfunction in 16 patients. The Senning operation for Simple TGA can be performed with a low operative mortality and morbidity, but tricuspid insufficiency and/or depressed right ventricular function can be a problem with prolonged follow up.

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Pulmonary Thromboendarterectomy for Chronic Thromboembolic Pulmonary Hypertension in a Patient with Antiphospholipid Syndrome and Systemic Lupus Erythematosus (항인지질 증후군과 전신성 홍반성 루푸스 환자에게 발생된 만성 폐혈전색전성 폐동맥 고혈압에 대해 시행한 혈전내막제거술)

  • Kang, Pil-Je;Kim, Jeong-Won;Lee, Jae-Won
    • Journal of Chest Surgery
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    • v.40 no.12
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    • pp.867-870
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    • 2007
  • Antiphospholipid syndrome (APS) is defined as venous and/or arterial thromboses, recurrent fetal losses, thrombocytopenia in combination with repeatedly positive tests for the lupus anticoagulant (LAC), and anticardiolipin antibodies (aCL). The pulmonary manifestation is APS are relatively rare. We report a rare case of antiphopholipid syndrome with systemic lupus erythematosus in a patient who presented with pulmonary hypertension secondary to a chronic pulmonary thromboembolism. A bilateral thromboendarterectomy was performed satisfactorily and the incision was extended to the left intrapleural pulmonary artery.

Endovascular Treatment of a Systemic-to-Pulmonary Artery Fistula: A Case Report (체-폐동맥루의 혈관 내 치료: 증례 보고)

  • Eunbyul Lee;Dong Jae Shim;Doyoung Kim;Jung Whee Lee
    • Journal of the Korean Society of Radiology
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    • v.82 no.3
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    • pp.682-687
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    • 2021
  • Systemic-to-pulmonary artery fistulas are rare. This condition may be congenital, post-traumatic, or post-inflammatory and can cause infection, hemorrhage, or pulmonary hypertension. Here, we report a case of an intercostal-to-pulmonary artery fistula, incidentally detected during the evaluation of dyspnea in a 67-year-old female. Retrograde transcatheter coil embolization in a dilated draining pulmonary artery was initially attempted. However, another draining pulmonary artery developed after 5 months. The intercostal arteries or systemic feeders were successfully embolized through a transarterial access. At the 10-month follow-up, the abnormally dilated vessels had regressed, and dyspnea had improved. Sequential or simultaneous retro- and antegrade transcatheter embolization may successfully treat pleural arterio-arterial fistulas.

Changes in the Lung after Pulmonary Hypertension Induced by Obstruction of the Pulmonary Vein in Rats (흰쥐에서 폐정맥 폐쇄에 의해 유도된 폐동맥고혈압 발생 후의 폐장의 변화)

  • Jang Won-Chae;Jeong In-Suk;Cho Kyu-Sung;Oh Bong-Suk
    • Journal of Chest Surgery
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    • v.39 no.9 s.266
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    • pp.659-667
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    • 2006
  • Background: Experimental studies of vascular remodeling in the pulmonary arteries have been performed actively. These models required a persistent vascular insult for intimal injury induced by chronic hypoxia, monocrotaline intoxication or chronic air embolism and characterized medial hypertrophy and neointimal formation by active synthesis of the extracellular matrix protein. The purpose of this study was to determine the pattern of pulmonary vascular remodeling after obstruction of the pulmonary vein. Material and Method: Obstruction of the right pulmonary vein with a metal clip was performed in Sprague-Dawley rats $(352{\pm}18g,\;n=10)$ to cause pulmonary vascular disease. Fifteen days later, experimental studies were done and finally the both lungs and hearts were extirpated for experimental measurement. Pulmonary arterial pressure, weight ratio of right ventricle (RV) to left ventricle (LV) and ventricular septum (S) (RV/LV +S weight ratio), and pulmonary artery morphology (percent wall thickness, %WT) were evaluated and compared with normal control groups. Result: Pulmonary hypertension $(38{\pm}12mmHg\;vs\;13{\pm}4mmHg;\;p<0.05)$ and right ventricular hypertrophy (right ventricular/left ventricular and septal weight ratio, $0.52{\pm}0.07\;vs\;0.35{\pm}0.04;\;p<0.05$) with hypertrophy of the muscular layer of the pulmonary arterial wall (percent wall thickness, $22.4{\pm}6.7%\;vs\;6.7{\pm}3.4%;\;p<0.05$) were developed by 15 days after obstruction of the pulmonary vein. Conclusion: Obstruction of the pulmonary vein developed elevation of pulmonary blood pressure and medial hypertrophy of the pulmonary artery. These results are a part of the characteristic vascular remodeling. Theses results demonstrate that obstruction of the pulmonary vein can develope not only high pulmoanry blood flow of contralateral lung but also intima injury inducing vascular remodeling.

Epidemiology of PAH in Korea: An Analysis of the National Health Insurance Data, 2002-2018

  • Albert Youngwoo Jang;Hyeok-Hee Lee;Hokyou Lee;Hyeon Chang Kim;Wook-Jin Chung
    • Korean Circulation Journal
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    • v.53 no.5
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    • pp.313-327
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    • 2023
  • Background and Objectives: Pulmonary arterial hypertension (PAH) is a rare but fatal disease. Recent advances in PAH-specific drugs have improved its outcomes, although the healthcare burden of novel therapeutics may lead to a discrepancy in outcomes between developing and developed countries. We analyzed how the epidemiology and clinical features of PAH has changed through the rapidly advancing healthcare infrastructure in South Korea. Methods: PAH was defined according to a newly devised 3-component algorithm. Using a nationwide health insurance claims database, we delineated annual trends in the prevalence, incidence, medication prescription pattern, and 5-year survival of PAH in Korea. Cumulative survival and potential predictors of mortality were also assessed among 2,151 incident PAH cases. Results: Between 2002 or 2004 and 2018, the prevalence and incidence of PAH increased 75-fold (0.4 to 29.9 per million people) and 12-fold (0.5 to 6.3 per million person-years), respectively. The proportion of patients on combination PAH-specific drug therapy has also steadily increased up to 29.0% in 2018. Among 2,151 incident PAH cases (median [interquartile range] age, 50 [37-62] years; 67.2% female), the 5-year survival rate and median survival duration were 71.8% and 13.1 years, respectively. Independent predictors of mortality were age, sex, etiology of PAH, diabetes, dyslipidemia, and chronic kidney disease. Conclusions: This nationwide study delineated that the prevalence and incidence of PAH have grown rapidly in Korea since the early 2000s. The use of combination therapy has also increased, and the 5-year survival rate of PAH in Korea was similar to those in western countries.

A retrospective clinical study of isolated patent ductus arteriosus (동맥관 개존증의 임상적 고찰)

  • 곽영태
    • Journal of Chest Surgery
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    • v.17 no.4
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    • pp.593-606
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    • 1984
  • With the ligation of patent ductus arteriosus by Gross in 1938, surgeons first entered the field of congenital heart disease. Interruption of a ductus is one of the most satisfactory and curative operations in the field of surgery for congenital heart disease. 27 cases of isolated patent ductus arteriosus were operated from Jan. 1978 to July 1984 at the Department of Thoracic & Cardiovascular Surgery in Kyung-Hee University Hospital. Retrospective clinical analysis of these patients were: 1. Sex ratio, female: male, was 2:1. 2. Mean age at operation was 9.85\ulcorner.58 years. The youngest patient was a 23 month-old girl and the oldest one was a 24 year-old male. 3. More than half of the patients had less than 50 percentile of growth retardation. 4. Chief complaints of the patients were frequent URI [52%], dyspnea on exertion [33%], generalized weakness [22%], palpitation [7%], but 7 patients [26%] had no subjective symptoms. 5. Continuous machinery murmur could be heard at the 2nd or 3rd intercostal space on the left sternal border in 22 patients [81%]. The other S patients made systolic murmur with accentuation of the second heart sound and those were associated with pulmonary hypertension. 6. Radiologic findings of Chest P-A were cardiac enlargement in 15 patients [55%], enlargement of pulmonary conus and/or increasing density of pulmonary vascularity in 20 patients [74%]. 7. Electrocardiographic findings of the patients were within normal limit in 13 patients [48%], LVH in 4 patients [15%], biventricular hypertrophy in 3 patients [11%]. 8; echocardiogram was obtained from 11 patients. Ductus was directly visualized in 7 patients. Left atrial enlargement is the secondary change of left to right shunt, 10 patients had LA/Ao ratio more than 1.2. 9. Cardiac catheterization performed in 25 patients. The mean value of the results were:SO2[PA-RV]= 14.72\ulcorner6.01%, Qp/Qs=2.22\ulcorner.80, peak systolic pulmonary arterial pressure=48.28\ulcorner1.60 mmHg. 10. 26 patients were operated through the left posterolateral thoracotomy: closure of ductus by double ligation in 14 cases, triple ligation in 5 cases, and division with suture in 8 cases. One patient suffer from aneurysmal rupture of main pulmonary artery, endocarditis, hemopericardium was treated with cardiopulmonary bypass via median sternotomy and closure of ductus through the ruptured main pulmonary artery. 11.There was no death associated with the operation, but 3 cases were experienced with intraoperative rupture around the ductus resulting in massive bleeding. The other complications were transient hoarseness in one patient, atelectasis in left lower lobe in 3 patients, and postoperative systemic hypertension in 4 patients with unknown etiology. 12. Pulse pressure was reduced, 11.47+5.92 mmHg, postoperatively, as compare to preoperative status. 13. Intraoperative wedge lung biopsy from lingular segment for the evaluation of the pulmonary vascular disease was taken in S patients with severe pulmonary hypertension. The result was Heath-Edward grade I in one case, grade II in two cases, and grade III in two cases.

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A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis (원발성 담관성 간경변과 동반된 문맥폐고혈압 1예)

  • Kim, Se Joong;Lee, Eun Ju;Jung, Ki Hwan;Kang, Eun Hae;Lee, Sung Yong;Lim, Hong Euy;Yim, Hyung Joon;Lee, Sang Yeub;Kim, Je Hyeong;Shin, Chol;Shim, Jae Jeong;In, Kwang Ho;Kang, Kyung Ho;Yoo, Se Hwa
    • Tuberculosis and Respiratory Diseases
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    • v.62 no.5
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    • pp.421-426
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    • 2007
  • Portopulmonary hypertension (PPHTN) is a clinically and pathophysiologically distinct complication of advanced liver disease. PPHTN is characterized by the development of pulmonary arterial hypertension in association with advanced hepatic disease-related portal hypertension. A characteristic feature of PPHTN is an obstruction to the pulmonary artery flow caused by vasoconstriction, the proliferation of the endothelium and smooth muscle components of the vascular wall, as well as in situ thrombosis. This disorder is commonly underdiagnosed but the clinical implications are significant because it has substantial effects on survival and requires special treatment. We report a case of portopulmonary hypertension in a 53-year-old woman with primary biliary cirrhosis who presented with exertional dyspnea.