• Journal of Yeungnam Medical Science
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• v.34 no.2
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• pp.279-284
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• 2017

Pulmonary artery sarcoma (PAS) is a rare and fatal disease that often mimics chronic thromboembolic pulmonary hypertension (CTEPH); therefore, diagnosis of PAS is often delayed. Herein, a healthy 74-year-old man was presented with a 4-month history of dyspnea. Chest computed tomography showed wall thickening and stenosis in the main pulmonary artery as well as in both proximal pulmonary arteries. In order to differentiate between unusual CTEPH, vasculitis, and PAS, we performed right heart catheterization and pulmonary angiography. The mean pulmonary arterial pressure was 21 mmHg, and there was severe pulmonary artery stenosis. Thrombi on the pulmonary arterial wall lesions were observed in intravascular ultrasound and optical coherence tomography. Furthermore, the patient had a history of deep vein thrombosis. Therefore, we diagnosed unusual CTEPH. After 6 months of rivaroxaban anticoagulation therapy, a chest X-ray revealed a left lower lobe lung mass, and a positron emission tomography later showed hypermetabolic lesions in the main pulmonary artery wall, in both pulmonary arteries walls, in the lung parenchyma, and in the bones. A biopsy of the right proximal humerus lesion revealed undifferentiated intimal sarcoma. Pulmonary sarcoma is rare, but should be considered when differentially diagnosing main pulmonary artery wall thickening and stenosis. A positron emission tomography may aid in this diagnosis.

• 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.41 no.5
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• pp.591-597
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• 2008

Background: Pulmonary endarterectomy is widely accepted as a treatment for chronic thromboembolic pulmonary hypertension. Based on our experiences, we sought to find ways to reduce perioperative complications and to improve surgical outcomes in patients undergoing pulmonary endarterectomy. Material and Method: This study was designed as a retrospective analysis of 20 patients with pulmonary hypertension who underwent pulmonary endarterectomy between January 1998 and March 2008. All patients presented with chronic dyspnea. Deep vein thrombosis (DVT) was the major cause of chronic pulmonary thromboembolism (55%). Seventeen patients (85%) underwent inferior vena cava (IVC) filter placement. Thirteen patients underwent surgery under total circulatory arrest, while the others underwent surgery while on low flow cardiopulmonary bypass. Concomitant tricuspid annuloplasty was done in 6 patients (66%) whose tricuspid regurgitation was as severe as grade IV/IV. The mean follow-up duration was $45{\pm}32$ months. Result: Using of University of California, San Diego (UCSD), thromboembolism classification, 4 patients (20%) were type 1, 8 patients (40%) were type II, and 8 patients (40%) were type III. Right ventricular systolic pressure was reduced significantly from $77{\pm}29$ mmHg to $37{\pm}19$ mmHg after pulmonary endarterectomy (p<0.001). The degree of tricuspid regurgitation and the NYHA functional class were all improved postoperatively. Reperfusion edema occurred in 7 cases (35%). The incidence of reperfusion edema was higher in the UCSD type III group than in the other group (25% vs 50%, p=0.25) and the length of postoperative intensive care unit stay was longer in type III group ($5{\pm}2$ days vs $9{\pm}7$ days, p=0.07). The early mortality rate was 10%, and the late mortality rate was 15% (n=3); one death was due to progression of underlying non-Hodgkin's lymphoma, and the other deaths were related to recurrent thromboembolism and persistent pulmonary hypertension, respectively. Conclusion: Pulmonary endarterectomy, as a curative surgical method for treating chronic thromboernbolic pulmonary hypertension, should be performed aggressively in patients diagnosed with chronic thromboembolic pulmonary hypertension, and an effort should be made to reduce the frequency of perioperative complications and to improve surgical outcomes.

• Journal of Chest Surgery
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• v.13 no.3
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• pp.237-242
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• 1980

This is a case report of spontaneous regression of elevated pulmonary vascular resistance after the age of 6 years in a patient with partial endocardial cushion defect. The patient was first evaluated and considered to be highly risky for surgical correction because of obstructive type of pulmonary hypertension and presisting congestive heart failure at the age of 6 years, during which time medical treatment for congestive heart failure and sufferring from respiratory infection were only provided. Finally the patient was reevaluated at the age of 16 years, in January of 1980 when cardiac catheterization was revealed markedly reduced pulmonary vascular resistance and pulmonary hypertension as well. The patient was operated upon with uneventful postoperative recovery. So we report this case with review of the literatures regarding natural history regarding natural history of partial endocardial cushion defect.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.238-242
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• 1986

The prognosis of patients with VSD and pulmonary hypertension is at least partially related to the reversibility of pulmonary hypertension after surgery. To predict postoperative pulmonary arterial pressure, immediate postbypass values were compared with preoperative hemodynamic data in 18 surgically closed VSD patients aged 6 to 80 months. The following results were obtained. 2] There was a good correlation between preoperative PP/PS and postoperative PP/PS in patients aged over 24 months [r=0.685, p<0.05], and in patients whose QP/QS were over 2.0 [r=0.686, p<0.01]. 3] There was also a good correlation between pump time and postoperative PP in total patients [r=0.697, p<0.005]. 4] Direct correlations were found between preoperative PP/PS and immediate postbypass PP/PS in patients aged over 24 months and in patients whose QP/QS>2.0, but the effect of CPB would participate in some degree. So it may be necessary to reevaluate their relationship after the effect of CPB have been disappeared.

• Journal of Chest Surgery
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• v.12 no.2
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• pp.105-109
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• 1979

Since 1973, 40 patients with Patent Ductus Arteriosus were operated in The Department of Thoracic and Cardiovascular Surgery, Korea University Medical College. Clinical analysis of these cases: 1. Age ranged from 8 months old to 28 years old Sex ratio was 22 females to 18 males. 2. Pulse pressure widening above 50 mmHg was appeared in 27 patients. In 31 patients, continuous machinery murmur was heard on left second and third intercostal space, but 9 patients has holosystolic murmur on left sternal border. 3. Retrograde Aortography was performed in 11 patients and right heart catheterization, 17 patients, In 8 patients, pulmonary hypertension [above 30 mmHg in` systole] was noted. 3 out of 8 patients was combined with Ventricular Septal Defect. Severe pulmonary hypertension [above 80mmHg in systole] was presented in 3 patients. 4. In 38 patients, operative method was performed with multiple suture ligation of PDA, and in 2 patients, suture closure through pulmonary arteriotomy under cardiopulmonary bypass. 5. One operative death occurred in a patient in this group. Cause of death was right heart failure after multiple suture ligation of PDA and pulmonary artery bandings.

• Journal of Chest Surgery
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• v.15 no.3
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• pp.311-315
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• 1982

Between 1973 and 1981 a total of 38 patients with a adult Patent Ductus Arteriosus, resulting from a congenital heart disease. There were 10 males and 28 females and most were in their twenties. In 21 cases, typical Patent Ductus Arteriosus without pulmonary hypertension and in 17, atypical Patent Ductus Arteriosus with pulmonary hypertension were noted. Methods of surgical treatment were division, double ligation, triple ligation, or wrapping with Teflon felt and ligation. The number of patients for each surgical treatment were 23 cases with double ligation, 4 cases with triple ligation, 3 cases of wrapping with Teflon felt and ligation and 8 cases with division. Surgical mortality occurred in one case[2.6%].

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.1-6
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• 2010

Transient tachypnea of the newborn (TTN), respiratory distress syndrome (RDS), and persistent pulmonary hypertension of the newborn (PPHN) are the three most common disorders that cause respiratory distress after birth. An understanding of the pathophysiology of these disorders and the development of effective therapeutic strategies is required to control these conditions. Here, we review recent papers on the pathogenesis and treatment of neonatal respiratory disease.

• Proceedings of the Korean Society of Veterinary Clinics Conference
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• 2009.04a
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• pp.216-216
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• 2009

• Tuberculosis and Respiratory Diseases
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• v.68 no.5
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• pp.273-279
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• 2010

Background: Pulmonary hypertension is considered as a poor prognosis factor in patients with chronic obstructive pulmonary disease (COPD). There has been reported brain natriuretic peptide (pro-BNP) is related with increased right ventricular (RV) workloads. However, there are few studies that evaluate the relationship between BNP and pulmonary arterial pressure (PAP), RV function and St. George Respiratory Questionnaire (SGRQ) score in patients with COPD, and the effects of angiotensin converting enzyme inhibitor (ACEI) on these parameters. Methods: Pulmonary function test, echocardiography, blood BNP, and SGRQ score were evaluated in stabilized moderate degree COPD patients ($FEV_1$/FVC< 70%, $50%{\leq}FEV_1$ < 80%) aged 45 years and over, without worsening of symptoms within recent 3 months. After treating with ramipril 10 mg for 3 months, the same evaluation was repeated. Results: Twenty-two patients were included in this study. BNP was significantly correlated with PAP (Pearson coefficient ${\rho}=0.51$, p=0.02), but not with RV ejection fraction (EF) and predicted $FEV_1%$. The values for predicted $FEV_1%$ showed significant correlation with SGRQ total score and activity score, but not with BNP or PAP. After ramipril treatment, PAP showed significant decrease ($42.8{\pm}8.1$ vs. $34.5{\pm}4.5mm$ Hg p=0.0003), tricuspid annular plane systolic excursion significant increase ($21.5{\pm}3.3$ vs. $22.7{\pm}3.1mm$ p=0.009). BNP showed a tendency to decrease without statistical significance ($40.8{\pm}59.6$ vs. $18.0{\pm}9.1pg/mL$ p=0.55). SGRQ scores showed no significant change. Conclusion: BNP showed significant correlation with resting PAP, which means BNP could be used as markers for pulmonary hypertension. Treatment with ACEI didn't show significant change in the level of BNP, while pulmonary hypertension and RV function were improved.