• 제목/요약/키워드: Congenital sinus

검색결과 126건 처리시간 0.026초

발살바동 동맥류 파열의 임상적 고찰 (Clinical features and surgical results of ruptured sinus of valsalva aneurysm)

  • 이태호;이동원;조준용;현명철;이상범
    • Clinical and Experimental Pediatrics
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    • 제49권3호
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    • pp.287-291
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    • 2006
  • 목 적 : 발살바동 동맥류는 선천성으로 발생하며 비교적 드문 질환이나 파열시 급성 심부전으로 진행되어 사망할 수 있으므로 수술만이 유일한 치료방법이다. 이에 저자는 지난 14년간 발살 바동 동맥류 파열로 수술을 받은 환자들을 대상으로 임상적 특징 및 수술 결과에 대하여 알아보고자 하였다. 방 법 : 이번 연구는 1990년 1월부터 2004년 2월까지 경북대학교병원에서 발살바동 동맥류 파열로 진단 받은 17명의 환자를 대상으로 임상증상, 이학적 소견, 과거병력, 동반 심질환 유무, 수술소견 및 장기적인 예후에 대하여 조사하였다. 결 과 : 남자가 13례, 여자가 4례 였으며, 평균연령은 $30{\pm}12.5$세(10-59세)였다. 술전 시행한 초음파 검사상 동반된 심질환으로는 심실 중격 결손이 8례(모두 이중연관 동맥하 결손), 대동맥 폐쇄부전이 11례에서 관찰되었다. 수술 소견상 누공의 위치는 우관상 동맥동-우심실 13례, 우관상 동맥동-우심방 1례, 비관상동맥동-우심실 2례 그리고 비관상 동맥동-우심방이 1례였으며, 심실 중격 결손이 14례에서 관찰되었으며, 모두 이중연관 동맥하결손이었다. 수술방법은 13례에서 첩포봉합, 4례에서 단순봉합을 실시하였으며, 대동맥판막 치환술은 4례, 대동맥판막 성형술은 2례에서 시행되었다. 수술로 인한 사망은 한 례도 없었으며, 추적 관찰기간($40{\pm}49$개월) 중 심질환으로 사망한 례도 없었다. 결 론 : 발살바동 동맥류는 많은 환자에서 심실 중격 결손, 특히 이중연관 동맥하 결손을 동반하므로, 심초음파 검사를 시행할 때 이 결손을 찾기 위해 세밀한 주의가 필요하다. 나아가서 크기가 작은 이중연관 동맥하 심실 중격 결손의 치료 방침도 재조명할 필요가 있을 것이다.

선천성 횡격막 무발육증 수술치험 1례 (Congenital Agenesis of Left Diaphragm: Surgical Repair - Report of a Case -)

  • 이종락;이신영
    • Journal of Chest Surgery
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    • 제24권12호
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    • pp.1238-1241
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    • 1991
  • Agenesis of the hemidiaphragm is unusual congenital anomaly associated with a high mortality. A case of congenital agenesis of left diaphragm was experienced in 22-day old male patient who was dyspneic and cyanotic on admission. Emergency exploration through the left eight interspace thoracotomy showed complete agenesis of the left diaphragm. The stomach and transverse colon covered with peritoneal sac was partially herniated into left hemithorax. The left lung was slightly hypoplastic. This neonate had no intestinal malrotation. The defect was reconstructed using Dacron graft. Dacron patch was sutured with interrupted Ethibond to chest wall anteriorly, esophagus aorta and costomediastinal sinus medially, and the tenth rib posterolaterally. Postoperatively, Extubation was performed at 1st day, but some respiratory difficulty was noted. Severe dyspnea was occurred at postoperative 11th day and so reintubation was done. Intermittently ventilatory support and intravenous alimentation were continued for 9 days after that. Thereafter he had no respiratory problems at discharge.

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Congenital midline cervical cleft: An easily misdiagnosed disease

  • Kang, Byungkwon;Kim, Byungjun
    • 대한두개안면성형외과학회지
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    • 제21권6호
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    • pp.372-375
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    • 2020
  • Congenital midline cervical cleft is a rare congenital disease. The disease is often misdiagnosed as a branchial cleft deformity, thyroglossal duct cyst, or other skin diseases. It has the following characteristics: skin defect at the midline of the anterior neck, a skin tag at the upper end of the lesion, and a blind sinus tract at the caudal aspect with or without mucoid discharge. Treatment is usually for aesthetic purposes; therefore, early surgical en bloc resection with Z-plasty or W-plasty is recommended to reduce recurrence and scar formation.

승모판막대치술후 합병한 좌심실 파열 보고 (Lt. Ventricular Rupture Complicated with Mitral Valve Replacement -One case report-)

  • 김병열
    • Journal of Chest Surgery
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    • 제15권2호
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    • pp.250-253
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    • 1982
  • A persistent left superior vena cava draining into the left atrium associated with atresia of the coronary sinus-ostium, ASD, and PDA is a rare congenital anomaly. The patient was a 4 year-old female whose complaints were frequent URI and exertional dyspnea. The congenital heart anomaly was suspected at 2 months of her age. Chest films showed cardiomegaly [C-T ratio, 75%]. EKG, Echocardiography, cardiac catheterization and angiocardiography were performed. Open heart surgery was done under impression of LV-RA shunt, bilateral superior vena cavae, and ASD. At the time of operation, huge LA and RA, inferior vena caval defect of a secundum type ASD [1.5 x 3cm in diameter], absence of innominate vein, atresia of the coronary sinus-ostium, and persistent LSVC draining into LA were noted. Direct suture closure of ASD and ligation of LSVC were done. The patient`s postoperative course was somewhat eventful: systolic murmur at apex remained. Four months after the operation, congestive heart failure attacked a few times. PDA that was overlooked at the time of open heart surgery was detected through postoperative cardiac catheterization in.4 months later. Emergent operation for closure of PDA was performed on the day of recatheterization. After that, patient`s heart failure was easily controlled without any notable problem.

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대동맥판막 폐쇄부전증을 동반한 대동맥판막 상부 협착증 (Supravalvular Aortic Stenosis with Aortic Regurgitation)

  • 김정태;이철주;소동문;한정선
    • Journal of Chest Surgery
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    • 제32권6호
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    • pp.591-594
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    • 1999
  • 대동맥판상부 협착증은 발살바동 상부에서부터 협착이 존재하는 비교적 흔치않은 선천성 질환이다. William 증후군없이 선천적으로 대동맥판 상부 협착증과 대동맥 좌관상판엽의 형성부전으로 인한 대동맥판 폐쇄부전증으로 진단받은 39세 여자환 悶“\ulcorner대동맥판막 치환술 및 Vascutek graft를 이용한 판탈롱 대동맥성형술을 시행 하였다. 환자의 술후경과는 좋았고 술후 9일째 퇴원하였다.

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고리췌장을 동반한 초극소저체중출생아에서 나타난 굴기능부전증후군 1례 (A Case of Sick Sinus Syndrome in Extremely Low Birth Weight Infant with Annular Pancreas)

  • 김지은;바우어 지그프리드;부윤정;이장훈;장기영;최병민;박문성
    • Neonatal Medicine
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    • 제18권2호
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    • pp.395-398
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    • 2011
  • 굴기능부전증후군은 매우 드물지만 신생아에서도 발생할 수 있으며 소아 환자의 경우 대부분 선천심장병이 있거나 심장수술 후에 발생한다. 그러나 이와 같은 심질환의 선행 요인이 없는 신생아에서도 발생할 수 있기 때문에 이에 대한 인지가 필요하다. 굴기능부전증후군은 대부분 특발성으로 발생하며 증상도 다양하고 24시간 심전도 검사상에서도 다양한 형태로 관찰된다. 본 증례에서는 초극소저체중출생아에서 선천심장병 없이 발생한 굴기능부전증후군에 대해 보고하였다. 환아는 심전도상 굴느린맥과 함께 심방세동이 동반된 느린맥과 빠른맥이 반복되어 관찰되었으며 무호흡, 산소포화도의 저하, 청색증의 징후를 나타냈다. 증상이 있는 굴기능부전증후군의 경우 대부분 심장박동조율기의 삽입이 필요하나 본 환아는 당시 체중 750 g으로 심장박동조율기의 삽입에 실패하여 약물 치료를 시작하였다. Isoproterenol과 atropine으로 치료하면서 환아의 체중이 1,500 g을 넘어서자 증상과 심전도상의 호전을 보였다. 향후 본 환아에서처럼 심장박동조율기의 삽입이 어려운 소아 환자의 경우에 대한 치료 체계에 대해 많은 연구가 필요하다.

Technique of Coronary Transfer for TGA with Single Coronary Artery

  • Kim, Tae Ho;Jung, Jae Jun;Kim, Yong Han;Yang, Ji-Hyuk;Jun, Tae-Gook
    • Journal of Chest Surgery
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    • 제47권6호
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    • pp.529-532
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    • 2014
  • An eight-day-old neonate was diagnosed with dextro-transposition of the great arteries, atrial septal defect, patent ductus arteriosus, and a single sinus origin of the coronary arteries. The single coronary artery originated from the left sinus (sinus 2), had a proximal left circumflex arterial branch, and passed anteriorly to the right side of the aorta, further branching into the right coronary and left anterior descending arteries. We successfully performed an arterial switch operation and coronary transfer by tube graft reconstruction with autologous aortic tissue to treat the dextro-transposition of the great arteries and atrial septal defect with a single-sinus origin of the coronary arteries.

Embolization of the Device to the Left Pulmonary Artery after the Interventional Closure of Ruptured Sinus of Valsalva Aneurysm

  • Choudhry, Lalit Kumar;Rao, Vinay M;Gnanamuthu, Birla Roy;Agrawal, Vishal;Shankar, Ravi;Prasath, Ram
    • Journal of Chest Surgery
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    • 제48권3호
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    • pp.202-205
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    • 2015
  • Formation of an aneurysm in the sinus of Valsalva of the aortic root is usually due to an area of congenital weakness in its wall. This aneurysm may progressively dilate and rupture into any of the cardiac chambers or into the pericardial cavity. Though this is conventionally treated by surgery, interventional therapy using various closure devices is becoming more common. Embolization of these closure devices may occur. We report a case of embolization of such a device into the left pulmonary artery which during surgical retrieval, unmasked the hidden ventricular septal defect (VSD). Therefore one has to be cautious while making a diagnosis of rupture of the sinus of Valsalva of right coronary sinus without VSD.

심외성 발살바동 동맥류-수술치험 1례- (Extracardiac Aneurysm of the Sinus of Valsalva - A case report -)

  • 신성현;장원채;나국주;안병희;김상형
    • Journal of Chest Surgery
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    • 제31권3호
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    • pp.304-307
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    • 1998
  • Valsalva동 동맥류는 발생빈도가 낮은 질환으로 대부분이 선천성이며 동양인에서 보다 빈번하게 발생하는 것으로 보고되고 있다. Valsalva동 동맥류는 심장내로 진행되어 우심실이나 심방으로 파열되는 경우가 대부분이고 심외성 Valsalva동 동맥류는 발생빈도가 매우 낮다. 심외성 Valsalva동 동맥류는 대부분 대동맥판폐쇄부전을 야기하고 동맥류에 의한 압박으로 우심실 유출로 협착, 심근 허혈 및 심근 경색 등을 일으킬 수 있으며 심낭내로 파열된 경우 심인성 쇼크나 돌연사를 일으키므로 매우 주의를 요하며 확진되면 외과적으로 교정하는 것이 바람직하다. 저자들은 좌관상동맥동 및 무관상동맥동에 발생한 동맥류에 의해 심근허혈 및 대동맥판폐쇄부전이 발생하였던 예를 외과적으로 치료하여 양호한 성적을 얻었기에 문헌고찰과 더불어 보고하고저 한다.

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선천성 및 후천성 심질환의 개심술 (A Clinical Evaluatuin on Open Heart Surgery of Congenital and Acquired Heart Disease)

  • 김근호
    • Journal of Chest Surgery
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    • 제12권1호
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    • pp.33-42
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    • 1979
  • The present study reports 41 cases of congenital and acquired heart diseases, who received open heart surgery under extracorporeal circulation [ECC] by Sarns Heart-Lung-Machine [HLM] at the Department of Thoracic and Cardiovascular Surgery, Hanyang University Hospital during the` period between July 1975 and February 1979. The priming of pump oxygenator was carried out by the hemodilution method using Hartman`s solution, whole blood, and fresh human plasma. The rate of hemodilution was in the average of 50.8 ml/kg. ECC was performed at the average perfusion flow rate of 85.0 ml/kg/min [2.43 L./ kg/2] and at moderate hypothermia. In the total cardiopulmonary bypass, arterial pressure ranged between 55 mmHg and 90 mmHg, but generally maintaining over 70 mmHg. Patient age ranged between 2 and 54 year old, in congenital heart diseases, between 2 and 28, in acquired heart diseases, between 17 and 54 Sex ratio of male to female was 20:21. The cases include a case of pulmonary valvular stenosis, 4 cases of atrial septal defect, 9 cases of ventricular septal defect, 9 cases of tetralogy of Fallot, 5 cases of pentalogy of Fallot, 3 cases of atypical multiple anomalies 7 cases of mitral stenosis or insufficiency, a case of myxoma in left atrium, and a case of ruptured aneurysm of Valsalva`s sinus. The surgical managements were 16 valvulotomy for pulmonary valvular stenosis, 2 Teflon patch graft closure and 5 simple suture closure of atrial septal defect, 16 Teflon patch graft closure and 5 simple suture closure of ventricular septal defect, 12 pericardial patch graft for infundibular stenosis of right ventricle, one anastomosis between left superior vena cava and right atrium, 2 open mitral commissurotomy, 5 mitral valve replacement using Starr-Edward`s ball valve, porcine xenograft by Hancock, by Carpentier-Edward, or Angell-Shiley, one removal of left atrial myxoma, and a repair of ruptured aneurysm of Valsalva`s sinus. Four [9.7%] out 41 cases expired postoperatively and the rest of 37 cases survived with satisfactory results. The causes of death were one coronary embolism in tetralogy of Fallot, 2 postoperative lower cardiac output in atypical multiple anomalies, and one right heart failure in large: ventricular septal defect with pulmonary hypertension.

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