• 제목/요약/키워드: Double Outlet Right Ventricle

검색결과 63건 처리시간 0.018초

복잡심기형의 외과적 치료 (Surgical treatement of complex cardiac malformations)

  • 조형곤
    • Journal of Chest Surgery
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    • 제19권1호
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    • pp.122-133
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    • 1986
  • Thirteen patients with cyanotic cardiac malformations having more complex intracardiac defects, hemodynamics and operative procedures than ones in Tetralogy of Fallot undertaken total surgical corrections from July 1981 to August 1985. The cases of corrective surgery for complex cardiac malformations were 3.9% of all congenital cardiac malformations and 12.6% of cyanotic cardiac malformations. Six patients died within 30 days after surgery. So operative mortality was 46%; Transposition of the great arteries, two of 4 patients, due to low cardiac output syndrome and tracheal bleeding ; Univentricular heart, one of 3 patients, due to bleeding; Corrected transposition of the great arteries, one of 2 patients, due to acute heart failure; Tricuspid atresia, one of 2 patients, due to low cardiac output syndrome; Double outlet right ventricle, one of single patient, due to respiratory failure. The cases of surgical correction for complex cardiac malformations are progressively increasing in numbers. The more accurate evaluation of anatomical condition and hemodynamics in preoperative diagnosis, studies on applicable surgical procedure and perioperative care of patients are necessary in the improvement of clinical and surgical results.

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폐동맥하 심실증격결손을 동반한 양대혈관 우심실기시중에서 동맥전환술의 중단기 결과 (Early and Midterm Results of Arterial Switch Operation for Double-Outlet Right Ventricle with Subpulmonary VSD)

  • 양승인;이형두;김시호;조광조;우종수;이영석;성시찬
    • Journal of Chest Surgery
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    • 제37권4호
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    • pp.313-321
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    • 2004
  • 폐동맥하 심실중격결손을 동반한 양대혈관 우심실기시증에서 동맥전환술은 심실내 교정의 제한된 적응증과 동맥전환술의 우수한 임상성적으로 인해 최근 이 기형의 선택적 치료법으로 자리잡고 있다. 이 기형에 대한 동맥전환술의 단기 및 중기 성적을 검토하였다. 대상 및 방법 1994년 8월부터 2002년 7월까지 8년 동안 동아대학교 병원에서 심실중격결손증 교정과 동맥전환술을 시행한 폐동맥하 심실중격결손을 동반한 양대혈관 우심실기시증 환자 13명을 대상으로 후향적 조사를 하였다. 양대혈관 우심실기시의 진단은 50% rule을 적용하였다. 중심나이와 평균체증은 각각 27일(범위, 3-120일)과 3.8$\pm$0.7kg (범위, 2.92-5.3kg)이었다. 대동맥궁기형은 6명(46.2%)에서 동반되었고 모두 일차 완전교정(one-stage repair)으로 교정되었다. 양대혈관의 위치관계는 좌우로 위치한 것이 8예(61.5%), 전후로 위치한 것이 5예(38.5%)였다. 관상동맥의 형태는 1LCx-2R과 좌관상동맥이 폐동맥의 뒤로 돌아가는 형태가 각각 6예씩(46.2%)이었으며 1명(7.7%)에서 벽속 좌관상동맥기형이 관찰되었다. 심실중격결손의 확장 및 패치를 이용한 우심실유출로 성형술이 각각 1명(7.7%)에서 시행되었다 좌우 대혈관 위치관계를 갖고 있는 3명(23.1%)을 제외한 모든 환자에서 Lecompte 술식을 시행하였다. 결과: 3예(23.1%)의 수술사망이 발생하였다. 3예 모두 대동맥궁기형을 갖고 있었던 경우였다. 수술 생존자의 평균추적기간 41.3$\pm$30.7개월(범위 1.7-79.1개월) 동안 만기사망은 1예(10%)로 술 후 5개월 뒤 중추신경계 합병증으로 사망하였다. 1예(10.0%)에서 압력차 30mmHg 이상의 폐동맥판 협착이 발견되었고 2예에서 좌폐동맥 협착으로 풍선확장술이 필요하였으며 이 중 1예(10%)에서 술 후 52개월만에 재수술이 필요하였다. 무증상의 중등도 대동맥판 폐쇄부전증이 1예(10%)에서 발견되었다. 수술사망을 포함한 5년 생존율은 68.3%였다. 결론: 대동맥궁기형을 동반한 폐동맥하 심실중격결손의 양대혈관 우심실기시증 환자에서는 높은 수술사망률을 보였으나 동반하지 않은 경우는 낮은 수술사망률과 재수술률을 보여 동맥전환술이 이 심기형에서 유용한 수술방법으로 고려될 수 있을 것이라고 생각한다.

고식적 목적 혹은 Fontan 수술 전단계로서 시행한 양방향성 상공정맥 폐동맥 단락술의 조기 임상 결과 (Bidirectional Cavopulmonary Shunt Operations as Palliation or Pre-Fontan Stage Operation - Early Results -)

  • 한재진
    • Journal of Chest Surgery
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    • 제25권4호
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    • pp.406-411
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    • 1992
  • Thirty-four patients were received bidirectional cavopulmonary shunt[BCPS] from Aug. 1989 to Apr. 1991 at Sejong General Hospital, Puchon, Korea. Their ages were from 43 days to 21 years old with 19 cases of infant, 10 from 1 to 5 years old and 5 cases above 6 years old. Their diagnoses were as follows: 13 cases with uni-ventricular heart, 9 tricuspid atresia, 6 double outlet of right ventricle, 4 pulmonary atresia with intact ventricular septum, and 2 transposition of great arteries with pulmonary stenosis. Among them, 10 patients had received other palliative operations before. The BCPS operations were performed under the cardiopulmonary bypass and 10 patients who had bilateral superior vena cava received bilateral BCPS. Other associated procedures were 9 cases of takedown of Blalock-Taussig shunt, 3 pulmonary artery angioplasty, 1 unifocalization, 1 repair of total anomalous pulmonary venous return, 1 Damus procedure, 1 relief of sub-aortic stenosis, 1 right ventricular outflow tract reconstruction and one case of tricuspid valve obliteration. There were 3 operative deaths[8.8%] and two late deaths. The remainders show good postoperative state and their oxygen saturation was increased significantly. Conclusively, the bidirectional cavopulmonary shunt is very effective and safe palliative or pre-Fontan stage operation for the many complex congenital anomalies with low pulmonary blood flow especially for the patients who have the risk of Fontan repair.

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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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Fontan 술식의 치험 23례에 관한 고찰 (Twenty three experiences of Fontan operation)

  • 안재호
    • Journal of Chest Surgery
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    • 제16권3호
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    • pp.342-348
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    • 1983
  • The Fontan principle of redirecting systemic venous blood into the pulmonary arteries via a conduit was initially proposed for Tricuspid Atresia, but it and its modifications have now gained a much wider application than initially intended. From September 1978 to July 1983, Atriopulmonary anastomosis has been performed in 23 patients, 2 months to 19 years of age, Seoul National University Hospital. The diagnoses were Tricuspid Atresia [TA] in 13, Univentricular Heart [UVH] in 9 and one case of Double Outlet of Right Ventricle [DORV]. Previous procedures included two Glenn shunts and one Blalock-Taussl8 shunt. Among these 23 patients, 10 patients had Right Atrial to Pulmonary Artery conduit, with a valve in 7 and without in 3. The remaining 13 patients had direct anastomosis between RA to PA. There were 14 early deaths, 7 of 13 TA patients, 6 of 9 UVH patients and one DORV, and the total hospital mortality was 60%. But there were no later deaths, 9 of these 23 patients survived operation and are presently alive. The Fontan procedure can be done with an acceptable low mortality with good functional results for Tricuspid Atresia and other complex lesion in foreign hospital, but till now our results revealed much higher risks. For our good operative results and effective patients selection, we must clarify the exact condition of pulmonary arterial system and accumulate much more experiences and technique.

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변형 Fontan수술의 수술결과 및 장기결과에 미치는 영향에 관한 연구 (Factors influences on early and late results of modified fontan operation)

  • 장병철;박영환;조범구
    • Journal of Chest Surgery
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    • 제19권4호
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    • pp.569-577
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    • 1986
  • Between October 1979 and June 1986, modified Fontan procedures have been performed on 22 patients by the Department of Thoracic and Cardiovascular Surgery, Yonsei University College of Medicine. Twelve patients had tricuspid atresia; one, congenital tricuspid stenosis; five, single ventricle; two, I-transposition of the great vessels; one, double outlet right ventricle, and one, pulmonary atresia with an intact ventricular septum. There were 9 operative deaths [mortality; 40.9%]. The causes of death were right heart failure in six patients and pulmonary venous hypertension in one who misdiagnosed preoperatively. Another two were deceased due to sepsis and cerebrovascular accident at postoperative 35 and 34 days in each. There were 7 patients below 4 years of age at the time of operation and among them 4 patients were deceased. The operative death was not related with patients` age above and below 4[p=0.211]. The relation between operative death of tricuspid anomaly and another cardiac malformations was statistically significant [p=0.048]. The operative procedures with or without valved conduit [woven dacron] was related significantly [p=0.043] in the case of the 21 of the patients, but the modified Fontan operation with a valved conduit was performed early stage in this series. Since 1982, we operated on 4 patients, doing a right atrium-right ventricle anastomosis without a conduit. All survived and remained in functional class I [NYHA]. The right atrial pressure [RAP] was elevated significantly after operation [mean 9.9$\pm$4.8 ~16.9$\pm$3.6 mmHg, p<0.001]. The relation between the postoperative RAP of the survival group [16.5$\pm$4.3 mmHg] and the group who died [17.4$\pm$2.2 mmHg] was statistically significant [p=0.047]. There was no relation between any operative death and any previous palliation. All patients were followed for 4 months to 80 months, except one who was lost to follow up at 2 months following surgery [mean 11.4 months, 238 patient. months]. All were in functional class I with 5 on medications and 7 not. One was reoperated at 70 months following the first operation, due to conduit stenosis. She was moderately impaired in activity, with hepatomegaly after the second operation.

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Polytetrafluoroethylene 인조혈관을 이용한 체-폐동맥 단락술의 조기성적 및 원격 성적 (Systemic-Pulmonary Shunts Using Microporous Polytetrafluoroethylene Prosthesis [Early and Late Results])

  • 장병철
    • Journal of Chest Surgery
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    • 제19권1호
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    • pp.50-57
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    • 1986
  • Sixty-nine patients with various types of cyanotic congenital heart disease underwent systemic-pulmonary artery shunts with a microporous polytetrafluoroethylene [PTFE] prosthesis between 1979 and 1985. Their ages ranged from 2 months to 39 years [mean$\pm$SD: 5.2$\pm$7.4, median: 3.3 years]. Diagnosis included the following: Tetralogy of Fallot, 45: Double outlet right or left ventricle, 11: Single ventricle, .5: Transposition of great vessels, 4: Tricuspid atresia, 3 and Pulmonary atresia with intact ventricular septum, 1. Forty-eight patients had subclavian-pulmonary artery anastomosis, 12 patients aorta-right pulmonary artery anastomosis, 6 patients aorta-main pulmonary artery anastomosis, and 3 patients descending aorta-pulmonary artery anastomosis. The PTEE graft of 3 mm in diameter was used in 1, 4 mm in 29, 5 mm in 35 and 6 mm in 4 patients. Ten patients were died within 30 days after operation [mortality rate: 14.5%]. Among them, 6 patients were operated in urgency due to cardiac arrest or severe anoxic spell after cardiac catheterization, and so surgical mortality of elective operation is 9.5%. The 59 survivors showed improvement of the arterial oxygen saturation [65.4% - 9.8%] and hemoglobin [18.8 gm/dl - 16.0 gm/dl] values [V<0.01]. The follow up period ranged from 1 month to 67 months, [752 patient-months] and during this periods there were 4 late shunt failures after 3 months postoperatively with 4 mm graft, and 2 with 5 mm graft. The over-all patency rate of 4 mm PTFE was 85.9$\pm$9.2% [SEM] in 12 months and 40.9$\pm$22.5% in 24 months. The over-all patency rate of 5 mm PTFE was 87.5$\pm$9.6% in 12 months and 58.3$\pm$24.6% in 36 months. The lowest systolic pressure in death group was 64.9$\pm$15.0 mmHg and in survival group, 86.4$\pm$12.1 mmHg [P<0.001]. We think that the PTFE graft is useful in palliative shunt operation, but the effectiveness of the 4 mm PTFE graft may be limited. The blood pressure also may play an important role in patency of Prosthesis.

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대동맥교약증의 임상연구 (Surgical Management of Coarctation of Aorta)

  • 김훈;강면식;홍유선;조범구;홍승록
    • Journal of Chest Surgery
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    • 제22권1호
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    • pp.59-66
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    • 1989
  • Coarctation of aorta, a well known congenital cardiovascular defect, can be recognized in the most instances by simple finding of physical examination. This condition shortens life if untreated, but it can be corrected surgically to render the patient functionally normal. It seems relatively rare in Asian. During Six-Year period from January, 1982 through June, 1988, twenty four consecutive operations for the coarctation of the aorta were performed at Yonsei Medical Center. The patients included 14 males and 10 females in the range, 2 months and 34 years old. Associated Cardiac anomalies were patient ductus arteriosus, ventricular septal defect, mitral valve regurgitation, aortic stenosis, double outlet right ventricle, corrected transposition of great vessel, etc. The preoperative main symptoms were frequent URI and dyspnea. Congestive heart failure was the most common symptom at the group younger than 2 year olds. Operative techniques for the coarctation of the aorta were prosthetic patch aortoplasty in 18 patients, resection & vascular graft interposition in 4, resection and end to-end anastomosis in 2. There was no operative death. Four patients had persistent or paradoxical hypertension, and one had postoperative paraplegia.

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Heart Transplantation in a Patient with Left Isomerism

  • Bang, Ji Hyun;Oh, You Na;Yoo, Jae Suk;Kim, Jae-Joong;Park, Chun Soo;Park, Jeong-Jun
    • Journal of Chest Surgery
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    • 제48권4호
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    • pp.277-280
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    • 2015
  • We report the case of a 37-year-old man who suffered from biventricular failure due to left isomerism, inferior vena cava interruption with azygos vein continuation, bilateral superior vena cava, double outlet of right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and isolated dextrocardia. Heart transplantation in patients with systemic venous anomalies often requires the correction and reconstruction of the upper & lower venous drainage. We present a case of heart transplantation in a patient with left isomerism, highlighting technical modifications to the procedure, including the unifocalization of the caval veins and reconstruction with patch augmentation.

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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