• The Korean Journal of Physiology and Pharmacology
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• 제20권6호
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• pp.641-647
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• 2016

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by vascular remodeling of pulmonary arteries (PAs) and increased vascular resistance in the lung. Monocrotaline (MCT), a toxic alkaloid, is widely used for developing rat models of PAH caused by injury to pulmonary endothelial cells; however, characteristics of vascular functions in MCT-induced PAH vary and are not fully understood. Here, we investigated hypoxic pulmonary vasoconstriction (HPV) responses and effects of various vasoconstrictors with isolated/perfused lungs of MCT-induced PAH (PAH-MCT) rats. Using hematoxylin and eosin staining, we confirmed vascular remodeling (i.e., medial thickening of PA) and right ventricle hypertrophy in PAH-MCT rats. The basal pulmonary arterial pressure (PAP) and PAP increase by a raised flow rate (40 mL/min) were higher in the PAH-MCT than in the control rats. In addition, both high $K^+$ (40 mM KCl)- and angiotensin II-induced PAP increases were higher in the PAH-MCT than in the control rats. Surprisingly, application of a nitric oxide synthase inhibitor, L-$N^G$-Nitroarginine methyl ester (L-NAME), induced a marked PAP increase in the PAH-MCT rats, suggesting that endothelial functions were recovered in the three-week PAH-MCT rats. In addition, the medial thickening of the PA was similar to that in chronic hypoxia-induced PAH (PAH-CH) rats. However, the HPV response (i.e., PAP increased by acute hypoxia) was not affected in the MCT rats, whereas HPV disappeared in the PAH-CH rats. These results showed that vascular contractility and HPV remain robust in the MCT-induced PAH rat model with vascular remodeling.

• Journal of Chest Surgery
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• 제22권5호
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• pp.775-780
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• 1989

This study concerns the late results observed at follow-up[average: 32.5 months] of 23 patients, in whom Patent Ductus Arteriosus with pulmonary hypertension among 55 patients. They were operated from May 1984 to July 1987 in Chonnam University Medical School. There was predominance of woman [2.5:1]. No operative death occurred. All of patients subside preoperative symptoms, but 1 patient diagnosed recannalization of duct at follow-up. Systolic murmur was found over the pulmonary area in 5 patients [22 %], but it may be not related to systolic pulmonary arterial pressure[SPAP]. Also, SPAP were normalized in 74 % of patients and had mild hypertension in 26 % of patients. Although the patients had Patent Ductus Arteriosus with pulmonary hypertension, successful surgical correction was carried out safely in all instance but one, by ligation and facilitated by hypotensive anesthesia.

• Journal of Chest Surgery
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• 제48권1호
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• pp.52-54
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• 2015

Idiopathic pulmonary arterial hypertension eventually leads to right-sided heart failure and sudden death. Its mortality rate in children is still high, despite improvements in pharmacological therapy, and therefore novel treatments are necessary. The Potts shunt, which creates an anastomosis between the left pulmonary artery and the descending aorta, has been proposed as a theoretically promising palliative surgical technique to decompress the right ventricle. We report the case of a 12-year-old girl with suprasystemic idiopathic pulmonary hypertension and right ventricular failure who underwent a Potts shunt for palliation with good short-term results.

• Tuberculosis and Respiratory Diseases
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• 제59권2호
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• pp.170-178
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• 2005

배 경 : 특발성 폐동맥고혈압(idiopathic pulmonary arterial hypertension, IPAH)과 만성혈전색전성 폐고혈압(chronic thromboembolic pulmonary hypertension, CTEPH)은 드문 질환이지만 만성적인 폐동맥 고혈압을 유발하는 중요한 질환이다. 두 질환은 임상소견 및 검사소견이 유사한 질환으로 감별이 어렵지만 치료의 차이 때문에 감별이 꼭 필요한 질환이다. 한 대학병원에서 경험한 두 질환의 임상상의 유사점과 차이점을 분석하고자 하였다. 대상 및 방법 : 서울아산병원에서 1995년부터 2002년까지 IPAH로 진단 받은 환자 33명과 CTEPH으로 진단 받은 환자 22명에 대해서 작성된 프로토콜과 의무기록을 검토하여 증상, 신체검사, 심전도, 흉부단순촬영, 폐기능검사, 심초음파, 핵의학검사, 심도자검사 등의 임상소견을 비교, 분석하였다. 결 과 : 나이의 중앙값은 IPAH군에서 33세(6~70세)로 CTEPH군의 52세(27~78세)보다 적었다. 성별은 IPAH군은 33명중 여자가 25명(76%)으로 남자보다 많았고 CTEPH군은 22명중 남자가 12명(55%)으로 성비에 차이가 없었다. 흉부단순촬영상 계측치, 심전도에서 계측치, 폐기능검사에서 폐활량과 폐확산능 그리고 심초음파에서 삼첨판최고역류속도는 두 군간에 차이가 없었다. 폐관류스캔에서는 IPAH군의 28.1%의 환자가 정상소견을 보였고 71.9%의 환자가 폐색전증의 낮은 가능성 소견을 보였다. 이에 반해 CTEPH군에서는 22명 모든 환자에서 폐색전의 높은 가능성소견이 관찰되었다. 결 론 : CTEPH과 IPAH는 임상증상이나 일반적인 검사소견이 유사한 질환이지만, 나이와 성별분포 및 폐관류 스캔소견에 차이를 보여 이러한 차이점들이 두 질환의 감별에 도움을 줄 것으로 사료된다.

• 대한임상검사과학회지
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• 제55권4호
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• pp.269-275
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• 2023

심장초음파검사는 폐동맥고혈압 진단에 유용한 비침습 방법으로 심장초음파 검사자의 교육상태, 지식수준, 경력에 따라 영향을 받는다. 이 연구의 목적은 폐동맥고혈압으로 진단받은 환자의 심장초음파 영상을 경력이 다른 검사자 간의 측정값 일치도를 비교하는 것이다. 2020년 1월 1일부터 2020년 12월 31일까지 S 병원에서 폐동맥고혈압으로 진단받고 심장초음파 검사를 받은 148명 환자의 심장초음파 영상을 심장초음파검사 경력이 다른 3명의 검사자가 재측정하였다. 각 검사자가 측정한 심장초음파 측정값을 분석한 결과, 이산변수 분석에서 검사자 간 일치도 평가는 검사자 3과 심장내과 전문의 그룹에서 모두 우수한 일치도를 보여 검사 경력이 많을수록 측정값 예측에 유리했다. 또한, 연속변수에서 모든 검사자는 구조적으로 복잡한 우심실의 측정값보다 내면이 단순하고 명확한 우심방의 측정값에서 좋은 일치도를 보였다. 본 연구는 국내 최초로 심장초음파를 시행하는 임상병리사 중 경력이 다른 임상병리사들이 심장초음파 영상을 재측정한 측정값의 일치도를 분석한 것이다.

• Korean Circulation Journal
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• 제48권12호
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• pp.1145-1147
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• 2018

• Archives of Plastic Surgery
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• 제49권3호
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• pp.369-372
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• 2022

Reduction mammaplasty is the gold standard treatment for gigantomastia. We report one female patient with juvenile gigantomastia associated with severe pulmonary hypertension where her pulmonary pressure decreased significantly after the surgery, improving her quality of life. A 22-year-old female patient with gigantomastia since 10 years old, tricuspid regurgitation, and pulmonary thromboembolism antecedent was admitted to the emergency department. Her oxygen saturation was 89%. Acute heart failure management was initiated. An echocardiogram reported left ventricle ejection fraction (LVEF) of 70% with severe right heart dilation, contractile dysfunction, and arterial pulmonary pressure (PASP) of 110 mm Hg. A multidisciplinary team considered gigantomastia could generate a restrictive pattern, so a Thorek reduction mammoplasty with Wise pattern was performed. Presurgical measurements were: sternal notch to nipple-areola complex, right 59 cm, left 56 cm. Three days after surgery, the patient could breathe without oxygen support. In the outpatient follow-up, patient referred reduction of her respiratory symptoms and marked improvement in her quality of life. Six months after surgery, a control echocardiogram showed a LVEF of 62% and PASP of 85 mm Hg. Pulmonary hypertension may be present in patients with gigantomastia. Reduction mammoplasty may be a feasible alternative to improve the cardiac signs and symptoms in patients with medical refractory management.

• Journal of Chest Surgery
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• 제23권1호
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• pp.32-40
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• 1990

Surgical correction of congenital cardiac defects in infants and children with an elevated pulmonary arterial pressure or pulmonary vascular resistance carries a significant early postoperative mortality. And accurate assessments of cardiac output is critically important in these patients. From April 1988 through September 1989, serial measurements of cardiac index, ratio of pulmonary-systemic systolic pressure, ratio of pulmonary-systemic resistance, central venous pressure, left atrial pressure, and urine output during the first 48 hours after the cardiac operation were made in 30 congenital cardiac defects associated with pulmonary hypertension. Cardiac index showed significant increase only after 24 hour postoperatively and this low cardiac performance in the early postoperative period should be considered when postoperative management is being planned in the risky patients. There were no variables which showed any significant correlation with cardiac index. In 12 cases[40%], pulmonary hypertensive crisis developed during the 48 hours postoperatively, and they were treated with full sedation, hyperventilation with 100 % 0y and pulmonary vasodilator infusion. In all patient with preoperative pulmonary hypertension, surgical placement of a pulmonary artery catheter is desirable to allow prompt diagnosis of pulmonary hypertensive crisis and to monitor subsequent therapy.

• 대한소아치과학회지
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• 제42권1호
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• pp.75-79
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• 2015

폐동맥 고혈압은 좌우단락을 가진 선천성 심장질환의 흔한 합병증으로 폐동맥 고혈압 환자에서 높아진 폐혈관 저항은 생명에 위협을 초래한다. 전신마취시의 양압환기는 폐동맥압을 높이고, 폐혈류량은 감소시키므로 저산소증을 유발할 수 있으며, 이는 폐동맥 고혈압 환자에게서 불리하게 작용할 수 있다. 따라서 폐동맥 고혈압 환자에서는 양압환기보다는 자발호흡이 보다 안전할 것으로 생각된다. 만 5세 남환이 심한 저체중으로 본원으로 의뢰되었으며, 내원 당시 환아의 몸무게는 11 kg이었고, 209년 팔로사징후로 완전 교정 수술을 받은 의과적 병력이 있었으며, 2007년부터 현재까지 고혈압 약을 복용하고 있다고 하였다. 환아는 다발성 우식을 가지고 있었으며, 치료는 경비캐눌라를 사용한 세보플루란 흡입 진정 하에 진행하엿다. 치료 내내 환아는 정상적인 생징후와 자발호흡을 유지하였으며, 이후에도 폐동맥 고혈압과 연관된 다른 합병증은 보이지 않았다. 폐동맥 고혈압 환자에서의 세보플루란 흡입 진정의 안전한 사용 증례를 통해서, 세보플루란 흡입 진정이 심혈관계 질환을 가진 환자들에서 전신마취의 대안으로 사용될 수 있는 가능성을 보여주었다.

• Tuberculosis and Respiratory Diseases
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• 제85권1호
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• pp.1-10
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• 2022

Pulmonary hypertension (PH) is a condition of increased blood pressure in the pulmonary arteries and is diagnosed with an increased a mean pulmonary artery pressure ≥25 mm Hg. This condition may be associated with multiple clinical situations. Based on pathophysiological mechanisms, clinical presentation, hemodynamic profiles, and treatment strategies, the patients were classified into five clinical groups. Although there have been major advances in the management of PH, it is still associated with significant morbidity and mortality. The diagnosis and treatment of PH have been performed mainly by following European guidelines, even in Korea because the country lacks localized PH guidelines. European treatment guidelines do not reflect the actual status of Korea. Therefore, the European diagnosis and treatment of PH have not been tailored well to suit the needs of Korean patients with PH. To address this issue, we developed this guideline to facilitate the diagnosis and treatment of PH appropriately in Korea, a country where the consensus for the diagnosis and treatment of PH remains insufficient. This is the first edition of the guidelines for the diagnosis and treatment of PH in Korea, and it is primarily based on the '2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.' with the acceptance and adaptation of recent publications of PH.