Fallot 四徵症의 根治手術

曺洸鉉 인제대학교 1985 仁濟醫學 Vol.6 No.1

One of the most common of the more serious congenital malformations of the heart is the tetralogy of Fallet, which is said as the watershed between the simple and complex cardiac anomalies. And, therefore, all cardiac surgeons have been much interested in surgical approach of this anomaly. Although Stensen deserves credit for the first description in 1972 of what is new termed the tetralogy of Fallot, nevertheless it is Etienne-Louis Arthur Fallot (1888) of Marseille, France, whose name is characteristically attached to this congenital cardiac disorder. In the original description of this, Fallot stated, "This malformation consists of a true anatomopathological type represented by the following tetralogy: (1) stenosis of the pulmonary artery; (2) interventricular communication; (3) deviation of the origin of the aorta to the right; (4) hypertrophy, almost always concentric, of the right ventricle. Failure of obliteration of the foramen ovale mar ocassionally be added in a wholly accessory manner." Despite the fact that accurate clinical diagnosis could often be established after these contributions by fallot, nevertheless, many years passed before definitive treatment of this was initiated by Blalock and Taussig (1945) with the establishment of the subclavian artery to pulmonary artery anastomosis. Thereafter, several types of this kind of palliative operation were established by Potts (1946), Waterston (1962), Sellors (1948), Brock (1948) and Glenn (1954). In an imaginative and daring effort, in 1954, Lillehei using controlled crass-circulation, carried out the first intracardiac repair of this anomaly by closing the VSD and relieving the pulmonary stenosis under direct vision. The results of surgery for this have steadily improved over the years, thanks to important contributions of many surgeons. Nevertheless because of its protean physiologic and anatomic presentations, tetralogy of Fallot continues to offer challenges to cardiologists and cardiac surgeons. Auther wanted to study and summarize about the radical operation of this anomaly with literature review.

TOF 완전교정술후 발생한 심실빈맥의 외과적 절제술 -치험1례보고-

장병철,김정택 대한흉부심장혈관외과학회 1996 Journal of Chest Surgery (J Chest Surg) Vol.29 No.6

14세 남자환자로 최근 3년간 반복된 심계항진을 주소로 내원하였다. 환자는 8년전에 TOF로 진단받고 완전교정수술을 받았다. 수술 전 심도자 검사에서 우폐동맥폐쇄와 그에 따른 우심실압력상승을 보였고 전기 생 리학검사에서는 우심실유출로의 중격부위 에서 기 원하는 회귀성 심실빈맥으로 진단되 었다 한 개의 양극성 전극을 이용한 심실외막전기도검사에서 심실빈맥의 회귀성회로는 첩포 원위부 좌측 의 심실중격쪽 심근에서 가장 빨리 나타나 시계방향으로 청포주위를 돌아 우심실유출로 우측 심근을 활 성화시키는 것으로 나타났다. 또한 64채널 심장전기도시스템을 이용한 심실심외막전기도 검사에서는 우심실유출로의 첩 포주위를 )20 msec 주기로 시계반대방향으로 회귀하는 심실빈맥을 관찰할 수도 있었 다. 수술은 우심실유출로의 첩포와 섬유화된 조직을 제거하고 우심실유출로 상연에서 폐동맥 판막륜까지 절개를 하여 회귀성 회로가 차단되도록 하였다. 폐동맥 판막륜과 심실중격사이 에는 회귀성 회로가 완전히 차단되도록 냉동병소를 만들었다. 우폐동맥이 발달되지 않아 우폐동맥재건술은 하지 못하였다. 수술후 전기생리학 검사에서는 더 이상심 퓟窄팀\ulcorner유발되지 않았다. 본 증례에서는 TOF완전교정수술후 우심실유출로 첩포와 일부 우심실 심근주위에 slow conduction지 대가 형성되고 이것에 의한 macro-reentry심실빈맥이 발생한 것으로 생각된다. 약물로서 치료되지 않을 때는 catheter ablation이나 외과적 절제를 고려해야 한다. 외과적 절제를 할 때에는 우심실유출로와 폐동 맥 판막륜사이의 심근조직손상을 최소화하면서 우심실유출로재건을 하는것이 중요하다.성 거부반응 1례, 크기불일치 에 의한 만성 이식부전증이 1례, 천식 발작에 의한 호흡부전이 1례, 지주막하출혈 1례, 체액 성 거부반응이 의심되는 경우가 1례 였다. 이상의 단기간성적을 통해 심장이식분야에서 더욱 발전을 요하며, 특히 이식공여자 및 수혜자의 선택 이나 술후 감염관리 및 체 액성거부반응치료 등에서 더욱 진전이 있어야 하겠다.07m', 동맥 혈 산소포화도는 83.0$\pm$3.8 % 이었다. 우폐동맥의 지름은 9.0$\pm$ 1.5 mm, 좌폐동맥의 지름은 7.7 $\pm$2.Omm, 폐동맥 지수는 197.3$\pm$57.1 mm2/m2, McGoon 비는 1.76$\pm$0.32 였다 수술후 전 환자에서 폐동맥 압력을 측정하였으며 평균 폐동맥 압은 12.8 $\pm$3.6mmHg이 었다. 환자의 성장에 따라 폐동맥의 지름도 증가하였으나 폐동맥 지수나 McGoon비는 증가하지 않았다. 또한 환자의 나이, 술후 동맥혈내 산소포화도의 증가 정도, 혈관 성형술 여부, 수술후 심도자 및 혈큰조 영술까지의 기간 등은 수술후의 폐동맥 지수의 증가에 영향을미치지 못하였으며 단지 수술전의 폐동맥 의 크기가 매우 작을 경우(McGoon 비 1.2 미만)에는 폐동맥 지수가 유의한 증가를 보였는데 이는 수술 \ulcorner 동반된 폐동맥 성형술등의 영향이 있었음을 감안하여야 할 것으로 생각되었다. 한편 수술전 폐동맥 크기에 대한 지표로서 폐동맥 지수(PAI)와 McGoon 비(MGR)와는 다음과 같은 유의한 상관관계가 있 음을 알 수 있었다 A 14-year-old male patient with previous surgical repair of tetralogy of Fallot was admitted with hemodynamically significant ventricular tachycardia (VT). On preoperative electrophysiologic study (EPS), the morphology of documented VT was RBBB of vertical axis with 320 msec cycle length. The endocardial mapping during VT delineated the origin of VT at right ventricular outflow tract (RVOT), where the patch was attached. The clinical VT had a clockwise reentry circuit around the patch with the earliest activation at the same site seen during the preoperative EPS. The previously placed right ventricular outflow patch and fibrous tissue were removed. During a postoperative EPS, it was no longer possible to induce the VT. Ventricular tachycardia following repair of tetralogy of Fallot seen in this patient was caused by a macro-reentry around the right ventricular outflow patch. We were able to ablate the VT with the aid of a detailed mapping of its epicardial activation sequence.

유아기에 시행한 팔로씨 사증의 심실절개를 통한 완전교정술

이정렬,김준성,김용진,노준량,배은정,노정일,윤용수,안규리 대한흉부외과학회 2004 Journal of Chest Surgery (J Chest Surg) Vol.37 No.2

배경: 유아기적 팔로씨 사증(Tetralogy of Fallot)의 완전교정술은 아직도 논란의 여지가 있으며 우심실절개를 통한 완전교정술 또한 많은 이견이 있다. 이 연구는 팔로씨 사증의 유아기 우심실절개를 통한 완전 교정술의 성적을 보여주는 연구이다. 대상 및 방법: 1990년 1월부터 2002년 4월까지 840명의 환아가 서울대학교병원에서 팔로씨 사증에 대한 수술을 시행받았으며 이 중 1세 미만에서 일차 완전교정술을 시행한 유아들 160명을 대상으로 조사하였다. 환아들의 평균연령은 8.1 2.6개월(3∼12)이었다. 완전 교정술은 모든 환자군에서 전체 우심실 길이의 30%를 넘지 않는 길이의 작은 우심실절개를 통해 시행하였으며 78명의 환자(49%)에서 경판막륜 첩포술(transannular patch)을 시행하였다. 결과: 4명의 환아(사망률: 2.5%)에서 조기 사망을 하였으며 만기 사망은 없었다. 모든 생존 환아에 대해 추적 관찰이 가능하였으며 모든 환아에서 현재 NYHA class I이나 II의 상태였다. 20명의 환아에서 재수술이 필요하였으며 1년과 10년 actuarial freedom from reoperation rate는 각각 94%와 87%였다. 2차원적 도플러 초음파검사를 시행하였을 때 거의 모든 환아들에게서 좋은 우심실 기능을 보여주었다. 결론: 이러한 결과는 팔로씨 사증의 완전 교정술을 유아기에도 적은 사망률과 합병증으로 만족할 만한 결과를 얻을 수 있다는 것을 제시하였다. 또한 이러한 환자군에서 우심실절개를 통한 심장 내의 병변을 안전하게 교정할 수 있으며 이로 인한 우심실 기능의 부전도 적다는 것을 보여주었다. Background: This study describes our surgical results of transventricular complete repair of tetralogy of Fallot in infants. Material and Method: Eight hundred and forty children underwent complete repair of TOF between January 1990 and April 2002 in our institute. One hundred sixty infants of them were included to this survey. Mean age at repair was 8.1±2.6 months (3~12). Correction was accomplished through a short right ventriculotomy less than 30% of ventricular height in all patients. A transannular patch was necessary in 78 patients (49%). Result: There were four early deaths. There were no late deaths. Follow-up with mean duration of 66 months was completed in all survivors. All patients are currently in New York Heart Association functional class I or II. Twenty patients required late reoperations. Actuarial freedom from reoperation at 1 and 10 years were 94% and 87% respectively. Two-dimensional and Doppler echocardiographic follow-up studies showed good right ventricular function in all patients except three. Conclusion: Our results suggested that early complete repair of TOF yield the acceptable results with low mortality and morbidity. Transventricular repair of intracardiac pathology can be safely applied to these patient population, yielding good postoperative right ventricular function.

Type 4 Tetralogy of Fallot with Pulmonary Hypertension in an American Shorthair Cat

김현진,Jihyun Kim,김태중,김하정 한국임상수의학회 2022 한국임상수의학회지 Vol.39 No.6

A 2-year-old, spayed female, American shorthair cat presented with acute weight loss, tachypnea, and dyspnea. The cat had grade V holosystolic murmur and systemic hypotension. Echocardiography showed a 9 mm defect in the ventricular septum, left-to-right dominant bi-directional shunt, right ventric- ular hypertrophy, pulmonary stenosis, pulmonary hypertension, and overriding aorta. The cat was diagnosed with a Tetralogy of Fallot. The cat was treated with furosemide, pimobendan, ramipril, and sildenafil. Treatment reduced pulmonary infiltration, pulmonary vessel enlargement, and main pulmonary artery bulging. However, right-to-left flow increased over time and right ventricular outflow tract velocity was elevated. Currently, the patient has maintained an improved state for 1 year. This case report described a severe inherited feline Tetralogy of Fallot case that was successfully managed for a long time.

우측상대정맥 없이 좌측 상대정맥이 좌심방으로 유입되는 활로씨 사징증의 수술치험 1례

김혁,김병일,김남수,김영학,정원상,강정호,지행옥,이철범,전석철,Kim, Hyuk,Kim, Byoung-Il,Kim, Nam-Su,Kim, Young-Hak,Chung, Won-Sang,Kang, Jung-Ho,Jee, Heng-Ok,Lee, Chul-Bum,Jeon, Seok-Chol 대한흉부심장혈관외과학회 1999 Journal of Chest Surgery (J Chest Surg) Vol.32 No.12

A persistent left superior vena cava draining into the coronary sinus is the most benign and widely encountered abnormality and can easily be explained embryologically as the persistence of the usual pattern of vnous circulation in the embryo,. However a persistent left superior vena cava draining into the left atrium with absent right superior vena cava is an extremely rare anomaly. We report this situation in an infant with tetralogy of Fallot. The most common approach has traditionally been intraatrial baffle repair to create a tunnel to the right atrium or rerouting of the left superior vena cava flow by directly anastomosing the left superior vena cava to the right atrium In the present study the left superior vena cava was transposed to the left pulmonary artery after the correction of tetralogy of Fallot. The most common approach has traditionally been intraatrial baffle repair to create a tunnel to the right atrium or rerouting of the left superior vena cava flow by directly anastomosing the left superior vena cava to the right atrium. In the present study the left superior vena cava was transposed to the left pulmonary artery after the correction of tetralogy of Fallot.

팔로4증 환자의 운동부하 검사시 심폐기능과 혈역학 요인의 비교

조수영,김영일 한국스포츠학회 2009 한국스포츠학회지 Vol.7 No.1

성인 20 세 이상 활로 4징증의 수술성적

정윤섭 대한흉부심장혈관외과학회 1990 Journal of Chest Surgery (J Chest Surg) Vol.23 No.2

Between January, 1970 and August, 1989, a total of 81 patients whose age were more than 20 years of life, received total correction for tetralogy of Fallot. This report analyzed 70 patients among them and excluded the remaining 11 patients whose clinical data could not be found. Their mean age was 25.750.39 years[range 20 \ulcorner50]. The clinical manifestations were cyanosis and clubbing [64 pts], frequent URI[40 pts], anoxic spell [19 pts], infective endo-carditis[4 pts], brain abscess[3 pts], pulmonary tuberculosis[3 pts] and CHF, chest tightness, nephrotic syndrome, left hemiplegia, and tamponade. The types of right ventricular outflow tract obstruction were combined[46 pts], pure infundibular [21 pts] and pure valvular[3 pts]. Associated cardiovascular anomalies were PFO [27 pts], ASDi8 pts], LSVC[8 pts], aortic regurgitation [5 pts], right aortic arch, coronary artery anomalies, PDA and dextrocardia. Hospital mortality was 5.7%. The causes of death ware low cardiac output [2 pts], aggravation of CRF[1 pts] and brain damage[1 pts]. There was one late death because of residual intracardiac shunt and congestive heart failure. During the follow-up period, 16 patients were lost and the remaining 49 patients were asymptomatic and leading normal lives. Residual intracardiac shunt was detected in 5 patients with radionuclide single pass study but all of them had Qp / Qs ratio less than 1.5.

Tetralogy of Fallot: A Surgical Perspective

Tom R. Karl 대한흉부외과학회 2012 Journal of Chest Surgery (J Chest Surg) Vol.45 No.4

Tetralogy of Fallot (TOF) is an index lesion for all paediatric and congenital heart surgeons. In designing an appropriate operation for children with TOF, the predicted postoperative physiology must be taken into account, both for the short and long term. A favourable balance between pulmonary stenosis (PS) and pulmonary insufficiency (PI) may be critical for preservation of biventricular function. A unified repair strategy to limit both residual PS and PI is presented, along with supportive experimental evidence. A strategy for dealing with coronary anomalies and some comments regarding best timing of operation are also included.

Severe Form of Tetralogy of Fallot: Late Presentation

Sherif Moustafa,Nanette Alvarez,Michael S. Connelly,Timothy Prieur,David J. Patton,Farouk Mookadam 한국심초음파학회 2014 Journal of Cardiovascular Imaging (J Cardiovasc Im Vol.22 No.4

Outcome of Staged Repair of Tetralogy of Fallot with Pulmonary Atresia and a Ductus-dependent Pulmonary Circulation: Should Primary Repair Be Considered?

Hyungtae Kim,성시찬,장윤희,Wonkil Jung,이형두,Ji Ae Park,허업 대한흉부외과학회 2011 Journal of Chest Surgery (J Chest Surg) Vol.44 No.6

The tetralogy of Fallot (TOF) with pulmonary atresia (PA) and a ductus-dependent pulmonary circulation (no major aorto-pulmonary collateral arteries (MAPCAs)) has been treated with staged repair or primary repair depending on the preference of surgeons or institutions. We evaluated the 19-year outcome of staged repair for this anomaly to find out whether our surgical strategy should be changed. Materials and Methods: Forty-four patients with TOF/PA with patent ductus arteriosus (PDA) who underwent staged repair from June 1991 to October 2010 were included in this retrospective study. The patients with MAPCAs were excluded. The average age at the first palliative shunt surgery was 40.8±67.5 days (range: 0∼332 days). Thirty-one patients (31/44, 70%) were neonates. The average weight was 3.5±1.6 kg (range: 1.6∼8.7 kg). A modified Blalock-Taussig (BT) shunt was performed in 38 patients, classic BT shunt in 4 patients, and central shunt in 2 patients. Six patients required concomitant procedures: pulmonary artery angioplasty was performed in 4 patients, pulmonary artery reconstruction in one patient, and re-implantation of the left pulmonary artery to the main pulmonary artery in one patient. Four patients required a second shunt operation before the definitive repair was performed. Thirty-three patients underwent definitive repair at 24.2±13.3 months (range: 7.3∼68 months) after the first palliative operation. The average age at the time of definitive repair was 25.4±13.5 months (range: 7.6∼68.6 months) and their average weight was 11.0±2.1 kg. For definitive repair, 3 types of right ventricular outflow procedures were used: extra-cardiac conduit was performed in 30 patients, trans-annular patch in 2 patients, and REV operation in 1 patient. One patient was lost to follow-up after hospital discharge. The mean follow-up duration for the rest of the patients was 72±37 months (range: 4∼160 months). Results: Ten patients (10/44, 22.7%) died before the definitive repair was performed. Four of them died during hospitalization after the shunt operation. Six deaths were thought to be shunt-related. The average time of shunt-related deaths after shunt procedures was 8.7 months (range: 2 days∼25.3 months). There was no operative mortality after the definitive repair, but one patient died from dilated cardiomyopathy caused by myocarditis 8 years and 3 months after the definitive repair. Five-year and 10-year survival rates after the first palliative operation were 76.8% and 69.1%, respectively. Conclusion: There was a high overall mortality rate in staged repair for the patients with TOF/PA with PDA. Majority of deaths occurred before the definitive repair was performed. Therefore, primary repair or early second stage definitive repair should be considered to enhance the survival rate for patients with TOF/PA with PDA.