• 대한영상의학회지
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• 제81권2호
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• pp.302-309
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• 2020

파브리병(Fabry disease)은 매우 드문 X-연관 유전 대사 질환으로 알파 갈락토시다아제(alpha galactosidase A)의 결핍으로 인하여 다양한 세포 및 기관에 글리코스핑고지질(glycosphingolipid)의 축적을 초래하는 질환이다. 심장 침범이 비교적 흔하며 비정상적인 지질침착으로 인한 심근 염증, 좌심실 비대 및 심근 섬유증을 일으킨다. 심장 침범은 환자 예후를 결정하는 중요한 요인이므로 이를 진단하는 것은 매우 중요하다. 심장 자기공명영상은 심실의 기능, 부피 측정을 위한 표준기법으로 알려져 있으며 심근의 조직 변화를 볼 수 있는 유용한 기법이다. 특히 최근 많이 쓰이는 T1 지도화 기법을 통한 심근 조영 전 T1 수치를 이용하여 파브리병의 심장 침범을 조기 진단할 수 있으며 자기공명영상을 이용한 심근 질량 측정으로 치료 모니터링을 할 수 있다. 심장 자기공명영상은 파브리병 환자에서 다양한 역할을 할 수 있을 것으로 생각되며 이에 대해 정리해보고자 한다.

• Journal of Chest Surgery
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• 제43권6호
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• pp.627-634
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• 2010

배경: 조직판막의 내구성은 심장 판막 수술에서 기계판막과 조직판막을 선택하는 중요한 기준이 된다. 본 연구는 조직판막이 사용된 대동맥판막치환술의 중기성적을 분석해 보았다. 대상 및 방법: 이 연구는 1990년 3월부터 2009년 3월까지 조직판막을 이용하여 대동맥판막치환술을 시행한 380명의 환자들을 대상으로 하였다. 술 후 평균 관찰 기간은 $46.7{\pm}40.8$월(0~196개월)이었으며 외래 경과 기록과 의무기록을 통하여 후향적으로 분석하였다. 결과: 총 380명의 환자에서 389예의 수술이 시행되었으며 환자들의 평균 연령은 $69{\pm}9$세, 남녀 비는 227 : 162였다. 조기 사망은 15명 (3.9%)이었다. 1년, 5년, 10년 생존율은 92.3%, 78.1%, 54.2%였다. 재수술의 l년, 5년, 10년 회피율(Freedom from reoperation)은 98.4%, 97.1%, 91.7%였으며 구조적 판막 손상 1년, 5년, 10년의 회피율(Freedom from structural valvular deterioration)은 96.1%, 92.3%, 88.0%였다. 수술 전 위험인자의 다변량 분석에서 젊은 나이 (p<0.001)가 재수술의 위험인자였으며 수술 후 판막 부하 최고속도(p=0.034)와 젊은 나이(p=0.029)가 구조적 판막 손상의 위험인자였다. 고령(p=0.001), 장시간의 심폐기사용(long bypass time) (p=0.035), 관상동맥 우회술을 동시에 실시한 경우(concomitant CABG) (p=0.003), 술 전 중등도 이상의 좌심실 기 능부전(Left ventricular ejection fraction, LVEF<40%) (p=0.003)이 조기 사망의 위험인자였으며 술 전 신기능저하(estimated glomerular filtration rate, eGFR<60 mL/min) (p=0.025)와 지속적인 좌심실비대(persistent left ventricular hypertrophy, LVH) (p=0.032)가 만기 사망의 위험인자였다. 결론: 이 연구를 통해 조직판막을 이용한 대동맥판막치환술은 재수술과 구조적 판막 손상 회피율 등의 측면에서 만족할 만하다고 할 수 있으며, 향후 더 많은 환자에서 보다 장기적인 연구가 필요할 것으로 생각된다.

• Investigative Magnetic Resonance Imaging
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• 제16권2호
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• pp.189-194
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• 2012

40세 남성에서 상대정맥의 폐쇄와 우폐동맥 협착을 동반한 종격동 종괴가 발견되어 병리학적 소견에 경화성 종격동염으로 진단되었고, 6개월 후 심장 자기공명영상(MRI)에서 비허혈성 심근병증으로 의심된 심장림프종의 예를 보고하고자 한다. 심장 초음파에서 제한적 양상의 심장 생리현상을 보였고 구혈율이 매우 감소되어 있었다. MRI에서 전반적인 심근비대와 조영제 주입 후 광범위한 지연조영증강이 있어 비허혈성 심근병증이 의심되었으며 심장근육 생검 후 림프종으로 확진되었다. 조영제 주입후 MRI에서 심근 기저부는 침범되지 않았고 전반적인 심내막 보존이 있었으므로 아밀로이드증과 구별되는 양상이고 종괴를 형성하지 않았기 때문에 심장종양 보다는 심근병증에 더 유사한 영상 소견을 보인 특이한 증례로서 심장림프종의 자기공명영상에서의 특징을 문헌고찰과 함께 보고하고자 한다.

• 한국임상수의학회지
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• 제33권4호
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• pp.221-224
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• 2016

An 8 month-old male Maltese (weighing 2.0 kg) was referred with loud heart murmur at routine physical exam in local animal clinic. Electrocardiogram found left ventricular hypertrophy pattern (4.5 mV R-wave). Diagnostic imaging studies revealed the elongation of left ventricle (LV) with classic triple bumps on the main pulmonary artery, aorta and left atrium on the ventrodorsal view of radiograph. Echocardiography revealed patent ductus arteriosus (PDA) duct and continuous turbulent shunt flow (maximal velocity 4.83 m/s) between the aorta and pulmonary artery with left to right direction. The PDA in this dog was successfully closed through femoral vein (transvenous approach) using a 5 mm Amplatz$^{(R)}$ Canine Duct Occluder. To the best of author's knowledge, this is the first case of PDA occlusion treated with Amplatz Canine Duct Occluder through femoral vein.

• 한국임상수의학회지
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• 제28권1호
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• pp.133-138
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• 2011

A 4-year-old female Fox Terrier dog (weighting 8 kg) with history of severe abdominal distention and anorexia was presented to Veterinary Teaching Hospital, Gyeongsang National University. In physical examination, grade 4/6 systolic murmur heard at the left heart base. Electrocardiography showed right ventricular enlargement (right axis deviation and deep S wave) and right atrial enlargement (P pulmonale). Diagnostic imaging studies revealed hypertrophy of right ventricle, enlargement of right atrium and tricuspid regurgitation and turbulence in the pulmonary artery in right parastenal short axis view. Based on the diagnostic findings, the dog was diagnosed as a case of valvular pulmonic stenosis.

• Journal of Chest Surgery
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• 제11권1호
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• pp.81-84
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• 1978

A 15 years Old girl was admitted with chief complaints of intermittent claudication of lower extremity, dizziness, and headache for 5 years. On admission, malignant hypertension was noted in the upper part of body [190-150/120-110] but femoral & dorsalis pedis pulse could not palpate. Once she had experienced C. V. A. due to hypertension of upper part, about years ago. On auscultation, systolic murmur was audible along the left sternal border. E.C.G. Showed left ventricular hypertrophy pattern, and others within normal limit. Retrograde aortography demonstrated diffuse narrowing of entire thoracic aorta with underdeveloped lower abdominal aorta [below the renal artery] & both common lilac artery, and rich collaterals, but normally visualized greater arteries in the aortic arch. On left posterolasteral thoracotomy, entire descending thoracic aorta revealed marked narrowing with mild perivascular adhesion, but no mediastinal pleura adhesion. These findings suggest as congenital type of atypical coarctation in the entire thoracic aorta with mild secondary change. But histopathology was showed the findings of chronic non-specific aortitis, later. Dacron by pass graft was performed with end to side anastomosis between graft and aortic wall. After operation, all her preoperative symptoms & signs were disappeared, and discharged with good general condition.

• Journal of Chest Surgery
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• 제20권2호
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• pp.404-410
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• 1987

An 18-day-old male neonate with hypoplastic left heart syndrome underwent surgical intervention by modification of the Norwood procedure on September 23, 1986. Hypoplastic left heart syndrome is a serious congenital cardiac anomaly that has a fatal outlook if left untreated. Included in this anomaly are [1] aortic valve atresia, and hypoplasia of the ascending aorta and aortic arch, [1] mitral valve atresia or hypoplasia, and [3] diminutive or absent left ventricle. Patent ductus arteriosus is essential for any survival, and there is usually a patent foramen ovale. Coarctation of the aorta is frequently associated with the lesion.z With a limited period of cardiopulmonary bypass, deep hypothermia, and circulatory arrest, the ductus arteriosus was excised. The main pulmonary artery was divided immediately below its branches, and the distal stump of the divided pulmonary artery was closed with a pericardial patch. The aortic arch was incised, and a 1 5mm tubular Dacron prosthesis was inserted between the main pulmonary artery and the aortic arch. A 4mm shunt of polytetrafluoroethylene graft was established between the new ascending aorta and the right pulmonary artery to provide controlled pulmonary blood flow. Following rewarming, the heart started to beat regularly, but the patient could not be weaned from cardiopulmonary bypass. At autopsy, the patient was found to have hypoplasia of the aortic tract complex with mitral atresia and aortic atresia. A secundum atrial septal defect was noted. Right atrial and ventricular hypertrophy was present, and the left ventricle was entirely absent. Although unsuccessful in this case report, continuing experience with hypoplastic left heart syndrome will lead to an improvement in result.

• Journal of Chest Surgery
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• 제28권6호
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• pp.601-605
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• 1995

Clinical evaluations were performed on 54 cases of the traumatic sternal fracture, those were admitted and treated at the department of Thoracic and Cardiovascular Surgery in Chosun University Hospital during the past 5 years period from January 1990 to December 1994. The frequency was about 7.8% of the nonpenetrating chest trauma. The ratio of male to female was 2 : 1 in male predominance. Average age was 45.7 years old and age distribution was from 18 to 80 years old. The most common cause in the sternal fracture were high decelerating injury[ 38 cases . The most common fracture site was sternal body. Associated intrathoracic organ injuries were cardiac contusion[11cases , hemopneumothorax and pulmonary contusion. Added, thoracic cage and extrathoracic organ injuries were rib fracture, head injuries, thoracic spinal fracture and long bone fracture. Abnormal ECG findings were sinus bradycardia[4 cases , sinus tachycardia[3 cases , bundle branch block, atrial fibrillaton, left ventricular hypertrophy and myocardial ischemia. The average days of admission was 19.1 days. All patients were treated with conservative treatment. The complications after treatment were atelectasis[2 cases ,empyema[1 cases , ARDS[1 cases .

• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.84-87
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• 2016

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.

• 한국임상수의학회지
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• 제26권2호
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• pp.155-159
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• 2009

암컷 잡종견(2년령, 체중 4.3 kg)이 운동불내성, 야행성 기침, 심잡음을 주증상으로 강원대학교 수의학부대학 부속동물병원에 내원하였다. 전수축기성 잡음이 양쪽에서 청진되었으며, 영상진단에서 대동맥 변위, 폐동맥협착, 심실중격결손, 심방중격결손, 양심실의 비대, 대동맥하협착이 확인되었다. 이러한 소견들을 바탕으로 대동맥하 협착증을 동반한 팔로오증으로 진단을 내렸다. 이는 국내에서 진단된 대동맥하 협착증과 복합된 팔로오증의 첫 번째 증례보고이다.