• 제목/요약/키워드: Tricuspid valve stenosis

검색결과 49건 처리시간 0.024초

선천성 복잡 심기형 환자의 외과적 교정술시 동종이식편의 적용에 관한 연구 (Application of Homograft in the Surgical Correction of Complex Congenital Cardiac Malformations)

  • 지현근
    • Journal of Chest Surgery
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    • 제28권11호
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    • pp.1038-1044
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    • 1995
  • We have been used cryopreserved homograft valves for right ventricular outflow tract[RVOT reconstruction since November 1993. The homograft valves were harvested from the hearts of brain dead patients or hearts of transplant recipients. There were 12 male and 10 female patients. Their ages ranged from 5 months to 13 years[mean age,39.2 $\pm$ 37.4 months and the weight ranged from 5 to 48kg [mean weight, 13.7$\pm$ 9. l kg . The diagnoses included pulmonary atresia with ventricular septal defect [n=14 , tetralogy of Fallot[n=4 , truncus arteriosus[n=3 , and double outlet right ventricle with pulmonic stenosis[n=l .Monocuspid homograft patches were used for RVOT widening or REV[reparation l`etage ventriculaire operations in 4 patients. We also used homograft as valved conduits for RVOT reconstruction in 17 patients and left ventricular outflow tract reconstruction in anatomically corrected transposition in 1 patient. Among them size-reducing technique [converting a tricuspid valved conduit into a bicuspid valved conduit were applied to six patients for the correction of size mismatching. The mean follow-up period was 10.6 $\pm$ 5.4 months. There was one operative death[4.5% due to bleeding and one reoperation for removal of vegetation on the homograft leaflet. Postoperative echocardiography documented no significant homograft insufficiency and RVOT obstructions.In short-term, the homograft valves provide excellent hemodynamic characteristics, even though further studies are necessary to evaluate the long-term results.

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2세 이하의 대동맥교약증 (Surgical Treatment of Coarctation of Aorta Less Than 2 Years Old)

  • 홍은표
    • Journal of Chest Surgery
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    • 제26권8호
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    • pp.604-608
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    • 1993
  • Coarctation of aorta is rather common congenital cardiovascular disease in the western contries, but it is known to be less than 2 % in Korea. From June 1986 to December 1992, seven patients of surgically treated coarctation of aorta who were less than 2 years old, were experienced at Department of Thoracic and Cardiovascular Surgery, Yeungnam University Hospital. The patients included six male and one female, with ages in the range of one month and 24 months. Four patients were preductal type and three juxtaductal. Associated cardiac anomalies were present in all patients and they were PDA[6 cases], ASD[3], VSD[2], bicuspid aortic valve[2], aortic stenosis[1], mitral regurgitation[1], and tricuspid regurgitation[1]. The operative procedures were four end to end anastomosis and three subclavian flap aortoplasty. Mean aortic cross clamping times were 37.3 minutes in patients with end to end anastomosis and 30.3 minutes in patients with subclavian flap aortoplasty. There were two operative deaths in patients who were treated with subclavian flap aortoplasty and pulmonary artery banding. One patient who had been treated with subclavian flap aortoplasty was complicated with postoperative mild paraplegia in lower limb. Pulmonary artery banding has been disappointing in our patients, and the data was suggestive that earlier total repair of complicated coarctation might improve survival.

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Handheld Echocardiography in a Clinical Practice Scenario: Concordances Compared to Standard Echocardiographic Reports

  • Gustavo Gavazzoni Blume;Luka David Lechinewski;Isabela Pedroza Vieira;Nadine Clausell;Giovana Paludo Bertinato;Paulo Andre Bispo Machado-Junior;Pedro Goulart Berro;Lidia Ana Zytynski Moura;Teresa Tsang
    • Journal of Cardiovascular Imaging
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    • 제30권1호
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    • pp.25-34
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    • 2022
  • BACKGROUND: The purpose of this study was to assess the utility of a handheld device (HH) used during common daily practice and its agreement with the results of a standard echocardiography study (STD) performed by experienced sonographers and echocardiographer. METHODS: A prospective follow-up was conducted in an adult outpatient echocardiography clinic. Experienced sonographers performed the STD and an experienced echocardiographer performed the HH. STD included 2-dimensional images, Doppler and hemodynamics analysis. Hemodynamic assessment was not performed with the HH device because the HH does not include such technology. The images were interpreted by blinded echocardiographers, and the agreement between the reports was analyzed. RESULTS: A total of 108 patients were included; and the concordance for left ventricle (LV) ejection fraction (EF), wall motion score index, LV and right ventricle (RV) function, RV size, and mitral and aortic stenosis was excellent with κ values greater than 0.80. Wall motion abnormalities had good concordance (κ value 0.78). The agreement for LV hypertrophy, mitral and aortic regurgitation was moderate, and tricuspid and pulmonary regurgitation agreements were low (κ values of 0.26 and 0.25, respectively). CONCLUSIONS: In a daily practice scenario with experienced hands, HH demonstrated good correlation for most echocardiography indications, such as ventricular size and function assessment and stenosis valve lesion analyses.

대동맥폐동맥창 (Aortopulmonary Window)

  • 김동진;민선경;김웅한;이정상;김용진;이정렬
    • Journal of Chest Surgery
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    • 제39권4호
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    • pp.275-280
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    • 2006
  • 배경: 대동맥폐동맥창은 매우 드문 질환이며 동반 심기형을 보이는 경우가 흔하다. 좌우 단락에 의한 과다한 폐 혈류량으로 조기 교정이 필수이나, 정확한 진단과 수술적 교정으로 좋은 결과를 얻을 수 있다. 본 연구에서는 과거 20년간의 경험한 대동맥폐동맥창을 정리하였다. 대상 및 방법: 1985년 3월부터 2005년 1월까지 16명(남 10, 여 6)의 환자가 대동맥폐동맥창으로 진단되어 수술적 교정을 시행하였고,수술당시 평균연령은 $157.8{\pm}245.3$ ($15.0{\sim}994.0$)일이었고, 평균 체중은 $4.8{\pm}±2.5$ ($1.7{\sim}10.7$) kg이었다. 동반 심기형은 동맥관개존증(8예), 심방중격결손증(7예), 대동맥궁단절증(5예), 심실중격결손증(4예), 난원공(3예), 삼첨판막역류증(3예), 승모판역류증(2예), 대동맥판역류증(1예), 대동맥축착증(1예), 좌측상대정맥(1예), 우심증(1예)이었다. 수술 방법은 대동맥폐동맥창의 분리 절단 후 대동맥 부위를 일차 봉합 또는 첩포 봉합하고 폐동맥 부위를 일차 봉합 또는 첩포 봉합하거나(11예), 대동맥폐동맥창의 분리 절단 없이 동맥 내에서 첩포 봉합술을 하거나(3예), 대동맥궁단절 및 대동맥축착을 동반한 환자에서 하대동맥을 직접 대동맥 창에 봉합하였다. (2예). 결과: 사망한 경우가 1예 있었다. 환자는 기관지 분지부에서 상방 2.5 cm정도 심한 협착과 기관유래기관지가 우상엽과 연결되어 있었던 경우로, 기관성형술(sliding tracheoplasty)시행 후 과다 출혈로 술 후 5일째 사망하였다. 복잡 대동맥폐동맥창 환자의 입원 기간 및 중환자실 체류 기간이 더 길었으며, 재수술(5예)과 합병증의 빈도도 더 높았다. 재수술은 좌폐동맥 협착(4예), 우폐동맥 협착(2예), 주폐동맥 협착(1예) 등이 원인이었다. 평균 추적 기간은 $6.8{\pm}5.6$ (57.0일$\sim$16.7년)년이었고, 생존 환자의 추적 기간 동안 NYHA 기능분류는 모두 I이었다. 결론: 연구자 등은 대동맥폐동맥창으로 진단된 16명의 환자에 대한 분석을 통해 조기 수술로 술 후 양호한 결과를 확보할 수 있음을 확인하였으며, 동반 심기형의 적절한 수술적 교정이 만기 예후를 좌우하는 것을 알 수 있었다. 수술 후 재협착의 빈도를 줄이기 위한 수술 전략의 재고가 요구된다.

단심실 -III C Solitus 형의 수술치험- (Surgical Repair of Single Ventricle (Type III C solitus))

  • naf
    • Journal of Chest Surgery
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    • 제12권3호
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    • pp.281-288
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    • 1979
  • For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

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Ebstein 기형의 수술 -2례 보고- (Surgical Repair for Ebstein's Anomaly)

  • naf
    • Journal of Chest Surgery
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    • 제12권3호
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    • pp.289-296
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    • 1979
  • For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

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교정형 대혈관 전위증의 수술적 치료 (Operative Treatment of Congenitally Corrected Transposition of the Great Arteries(CCTGA))

  • 이정렬;조광리;김용진;노준량;서결필
    • Journal of Chest Surgery
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    • 제32권7호
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    • pp.621-627
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    • 1999
  • 배경: 본 연구에서는 교정형 대혈관전위증으로 양심실교정이 가능하여 개심술을 시행받은 65례의 환아를 대 상으로 질환의 해부학적 특성, 수술방법 및 결과를 분석하였다. 대상 및 방법: 1984년부터 1998년 9월까지의 교정형 대혈관전위증으로 양심실교정을 받은 65례의 환자들을 대상으로 하였으며 주로 환자의 의무기록을 참조하여 후향적으로 동반기형에 따른 수술법의 차이 및 각각의 수술법에 따른 합병증등 단기 및 중장기 성 적을 분석하였다. 결과 평균 연령은 5.4$\pm$4.8세(2개월~18세)이었으며 남:녀비는 39:26이었다. 형태로는 {S,L,L}이 53례{I,D,D}가 12례였다. 폐동맥 협착이나 폐쇄가 동반되지 않았던 경우가 13례(20%)였으며 심실 혹은 심방중격결손증과 폐동맥 협착증이 동반된 경우가 26례(40%)였고 심실중격결손증과 폐동맥 폐쇄증이 동반된 례가 26례(40%)였다. 술전 경도이상의 삼첨판막 폐쇄부전을 보인 환자는 38.5%(26/65)였다. 완전교정 술전 단락술은 22명의 환자에서 24차례 시행하였다. 교정술의 방법으로 폐동맥심실유출로 협착이 없던 13례 에서는 7례에서 심실 혹은 심방 중격결손증만을 폐쇄하였고, 3례에서 삼첨판막치환술을, 3례에서 좌심실을 체순환으로 사용하는 중복치환술(Senning+Rastelli, Senning+REV, Senning+ASO)을 시행하였다. 심실,혹은 심방 중격결손증과 폐동맥 협착이 있던 26명의 환자에서는 24례에서 우심실을 체순환으로 사용하는 기존의 방법 (Rastelli술식 9례, 심실중격결손증 폐쇄후 폐동맥판막절개술 7례, REV형 술식 4례, 중격결손 폐쇄후 폐동맥 판막하 근육절제가 3례, 삼첨판막치환술이 1례)으로 수술하였으며 2례에서 중복치환술(Senning+Rastelli, Mustard+REV)을 시행하였다. 심실중격결손증과 폐동맥폐쇄증이 있었던 26명의 환자에서는 18명에서 심실중 격결손증을 폐쇄하고 Rastelli술식을 시행하였고, 7례에서 중복치환술(Senning+Rastelli)을 시행하였으며, 1례는 심실중격결손증을 폐쇄하고 REV형 술식을 시행하였다. 술후 추적 기간동안 우심실을 체순환으로 사용한 환 자들에서의 삼첨판막폐쇄부전은 술전 평균 1.3$\pm$1.4도에서 2.2$\pm$1.0도로 통계적으로 의미있게 증가하였다 (p<0.05). 그러나 중복치환술을 시행받은 환자들은 술후 삼첨판막의 폐쇄부전의 증가가 없었다. 술전 완전 방 실차단을 보인 환자는 2례(3.1%)있었으며 술후 새롭게 발생한 완전방실차단은 7례(10.8%)있었다. 술후 기타 장,단기 합병증으로는 폐동맥심실유출로도관(conduit) 재협착이 10례, 혈전증(판막: 2례, 인조혈관: 1례, 폐동 맥: 1례)이 4례, 2주 이상의 지속적 흉관배액이 4례, 유미흉이 3례, 출혈에 의한 재수술이 3례, 기타 급성 신 부전, 종격동염, 횡경막신경 마비가 각각 2례씩 있었으며, 중복치환술을 받은 환자들과 전통적 술식으로 수 술받은 환자에서 술후 합병증의 차이는 없었다. 65명의 환자를 평균 54$\pm$49개월(0~177개월)간 추적관찰하였 으며, 수술 초기에 사망한 환자는 13명으로 20.0%(13/65)의 수술사망율을 보였으며 3명의 환자가 추적기간중 사망하여 24.6%(16/65)의 전체사망율을 보였다. 중복치환술을 받은 환자의 수술사망율은 33.3%(4/12)였다. 술 후 1년, 5년, 10년 누적생존율은 각각 75.0$\pm$5.6%, 75.0$\pm$5.6%, 69.2$\pm$7.6%였다. 가장 흔한 사망원인으로는 술 후 저심박출증후군으로 8례였으며 삼첨판막 폐쇄부전이 심해져 심부전으로 사망한 경우도 5례로 사망의 중 요 원인이었다. 결론 저자들은 본 연구를 통하여 기존의 형태학적 우심실을 체순환으로 사용하는 방법과 형태학적 좌심실을 체순환으로 사용하는 해부학적 교정법인 중복치환술중 어느 것이 우월하다고 결론지을 수는 없었으나 해부학적 교정술이 삼첨판막의 폐쇄부전의 진행을 막을수 있다는 면에서 이점이 있으리라 사 료되며 보다 세분화된 적응증의 결정과 아울러 장기적인 추적관찰 및 비교가 필요하리라 생각된다

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개심술(開心術) 2,000례의 임상적 고찰 (Clinical Experiences of Open Heart Surgery)

  • 김하늘루;박경택;곽기오;한일용;소영환;최강주;이양행;조광현
    • Journal of Chest Surgery
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    • 제31권12호
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    • pp.1183-1194
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    • 1998
  • 배경: 인제 대학교 의과대학 부산 백병원 흉부외과학 교실에서는 1985년 9월부터 1997년 9월까지 총 2,000례의 개심술을 시행하였다. 대상 및 방법: 전체 2,000례의 개심술 중 선천성 심질환이 1532례, 후천성 심질환이 468례였다. 연령별 분포는 선천성 심질환에서는 생후 9일에서 68세 까지였고, 후천성 심질환에서는 11세부터 66세 까지였다. 결과: 선천성 심질환군은 심실중격 결손증(VSD)이 940례, 심방중격 결손증(ASD)이 324례, 팔로 4징증(TOF)이 112례, 폐동맥 협착(PS)이 46례, 심내막상 결손(ECD)이 38례, 발살바동 파열(Valsalva sinus rupture)이 15례, 완전 대혈관 전위증(TGA) 과 양대혈관 우심실 기시증(DORV) 각각 4례 등으로 구성되었다. 선천성 심질환군에서는 근치적 수술후 3.1%의 사망률을 보였다. 후천성 심질환군 468례 중 심장 판막질환이 381례, 허혈성 심질환이 48례, 심장종양이 12례, Annuloaortic ectasia가 8례, 박리성 대동맥류가 16례 등이었다. 381례의 판막질환 중 단일 판막 치환술이 226례(대동맥 판막 치환술 36례, 승모판막 치환술 188례, 삼첨판막 치환술 2례), 이중 판막 치환술이 71례(대동맥 판막 치환술 과 승모판막 치환술), 승모판막 치환술과 삼첨판막 성형술을 동시에 시행한 경우가 54례, 이중 판막치환술과 삼첨판막 성형술을 동시에 시행한 경우가 18례 등이었다. 사용된 인공판막은 총 466개 였다. 승모판막 치환술에 사용된 인공판막은 St. Jude Medical 판막이 123개, Carpentier-Edwrads 판막이 90개, CarboMedics 판막이 65개, Sorin 판막이 42개, 기타 판막이 16개였다. 대동맥 판막 치환술에 사용된 인공 판막은 St. Jude Medical 판막이 68개, CarboMedics 판막이 36개, Carpentier-Edwards 판막이 14개, 기타 판막이 9개 였다. 관상동맥 우회술(CABG)은 48례에서 시행되었다. 혈관 이식편의 수는 단일 혈관 이식이 14례, 이중 혈관 이식이 21례, 삼중 혈관 이식이 10례, 사중 혈관 이식이 3례였다. 결론: 술후 재원 기간내 사망률은 비청색증 선천성 심질환에서 2.0%, 청색증 선천성 심질환에서 15.5%, 후천성 심질환에서 5.1%였다. 전체 사망률은 2,000례 중 72명이 사망하여 3.6%였다

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총폐정맥 환류 이상증에 대한 술후 장기성적 검토 (Long-term Results Following Surgical Repair of Total Anomalous Pulmonary Venous Return)

  • 원태희
    • Journal of Chest Surgery
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    • 제28권6호
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    • pp.565-570
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    • 1995
  • Seventy-three patients with isolated total anomalous pulmonary venous connection the patients associated with other major cardiac anomalies such as single ventricle, DORV[Double Outlet Right Ventricle and large VSD[Ventricular Septal Defect were excluded were underwent surgical repair from January 1980 through October 1993. There were 45 boys and 28 girls. The mean age at operation was 19.9 months[range 6 days to 24 years and mean body weight was 7.1kg[range 2.6kg to 45kg . The anomalous locations of connection were supracardiac in 38, cardiac in 21, infracardiac in 5, and mixed in 9. In 38 patients[52% , the venous drainage was obstructed. The obstruction ratios according to the connection type were as follows: 53%[28/38 in supracardiac, 52%[11/21 in cardiac, 100%[5/5 in infracardiac, 22%[2/9 in mixed type. The associated cardiac anomalies were persistent left SVC[2 , tricuspid valve regurgitation[3 , cor triatriatum[1 , and mitral cleft[1 . And associated noncardiac anomalies were imperforate anus[1 and Neil Weightman syndrome[1 . The operative mortality was 23%. The causes of death were pulmonary hypertensive crisis, perioperative myocardial failure, pneumonia with sepsis, arrhythmia and etc. The statistically significant factors in postoperative mortality were the pulmonary venous obstruction and age [p<0.01 . The operative mortality was high in groups of age under 1 month and pulmonary venous obstruction. The mean follow-up was 27.1 months. There were two late deaths. The first patient was three months old boy with supracardiac type and severe obstructive symptoms. The postoperative echocardiography was showed anastomotic stenosis and reoperations were performed twice but the patients expired due to pneumonia and sepsis. The second patient was three month old boy with supracardiac type and total correction was done and was doing well postoperatively. Eight years later, he expired suddenly due to arrhythmia. But all the other patients were in NYHA Fc I and received no medications. The 5-year survival rate excluding early expired patients is 97.1 $\pm$ 0.03 %. In conclusion, although the operative mortality of total anomalous pulmonary venous connection was relatively high compared to other major cardiac anomalies, we could expect excellent long-term results by early surgical correction.

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수정 대혈관 전위 환자에서 예후에 영향을 주는 인자들에 대한 중기적 고찰 (Evaluation of Prognostic Factors in Corrected Transposition of the Great Arteries at Mid-term Follow-up)

  • 송영환;권혁주;김기범;강수정;배은정;노정일;윤용수;이정렬;김용진;노준량
    • Clinical and Experimental Pediatrics
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    • 제46권2호
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    • pp.154-161
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    • 2003
  • 목 적 : 수정 대혈관 전위 환자에서는 동반 심장 기형, 우심실 기능, 삼첨판 판막 기능, 부정맥 등이 예후에 영향을 주는 것으로 알려져 있다. 이 연구에서는 중기간의 관찰을 통해 여러 예후 인자들을 비교 분석하였다. 방 법 : 서울대학교병원에서 C-TGA로 진단 받은 94명을 대상으로 후향적 임상 기록 고찰을 시행하였다. 대상 환자들은 남아가 58명, 여아가 36명이었고, 추적 관찰 시 최종 평균 연령은 $12{\pm}9$세였으며, 평균 추적 관찰 기간은 $9{\pm}6.4$년이었다. 결 과 : 대상 환자 94명 중에 심장 수술은 72명이 시행 받았고, 고전적 교정술이 55례, 해부학적 교정술이 17례였다. 동반된 심장 기형(삼첨판 역류, 폐동맥 협착이나 폐쇄, 심실 중격 결손, 임상군), 심장 수술 여부와 종류, 완전 방실 전도 차단을 예후인자로 설정하였고, 예후 인자들 중에서 삼첨판 역류, 폐동맥 협착, 심실 중격 결손은 중등도 이상인 경우만 양성으로 채택하였다. 본 연구에서는 삼첨판 역류만이 생존율에 영향을 주는 예후인자였으며, Ebstein 기형과 고도 방실 전도 차단이 삼첨판 역류와 연관성이 있었다. 삼첨판 부전이 있는 환자의 20년 생존율은 35%로, 없는 환자 77%에 비교해서 유의한 차이를 보였고, 수술 직후 사망을 제외한 소그룹 분석에서도 삼첨판 부전이 있는 경우와 없는 경우의 20년 생존율이 각각 48%와 87%로 유의한 차이를 보였다. 20년 생존율 분석과 삼첨판 역류와의 연관성 분석에서 고전적 교정술과 해부학적 교정술간에 유의한 차이는 없었다. 결 론 : 삼첨판 부전은 수정 대혈관 전위 환자의 중요한 예후인자이며, Ebstein 기형과 방실 전도 장애가 삼첨판 부전에 영향을 줄 수 있다. 이 밖에 심장 수술, 동반 심기형 등 다른 예후인자에 대한 정확한 평가를 위해서는 장기적인 추적 관찰이 필요하다.