Truncus arteriosus is a rare and highly lethal cardiac anomaly characterized by a single arterial trunk emerging from the heart and supplying the coronary, systemic, and pulmonary circulations, The first successful correction of truncus arteriosus was reported by McGoon et al. in 1968 and was based on experimental work reported by Rastelli et al. in 1967 in which a conduit consisting of a homograft of the ascending aorta and aortic valve was used to establish continuity between the right ventricle and the pulmonary arteries, Modification of this procedure using a Dacron tube valved with porcine xenograft instead of a homograft have resulted in the current definite treatment for truncus arteriosus. This report describes an 3 years and 4 months old boy with heart failure from type I truncus arteriosus who was diagnosed as the V. S. D. with pulmonary hypertension preoperatively and underwent corrective surgery employing the Rastelli procedure using a Dacron conduit valved with canine xenograft, but died due to massive bleeding from the anastomosis sites in operating room.
Lee, Jong Uk;Jang, Woo Sung;Lee, Young Ok;Cho, Joon Yong
Journal of Chest Surgery
/
v.49
no.2
/
pp.112-114
/
2016
The half-turned truncal switch (HTTS) operation has been reported as an alternative to the Rastelli or $r{\acute{e}}paration$$\grave{a}$$l^{\prime}{\acute{e}}tage$ ventriculaire procedures. HTTS prevents left ventricular outflow tract (LVOT) obstruction in patients with complete transposition of the great arteries (TGA) with a ventricular septal defect (VSD) and pulmonary stenosis (PS), or in those with a Taussig-Bing anomaly with PS. The advantages of the HTTS procedure are avoidance of late LVOT or right ventricular outflow tract (RVOT) obstruction, and of overstretching of the pulmonary artery. We report the case of a patient who underwent HTTS for TGA with VSD and PS, in whom there was no LVOT obstruction and only mild aortic regurgitation and mild RVOT obstruction, including observations at 12-year follow-up. Our experience with long-term follow-up of HTTS supports a solution for late complications after the Rastelli procedure.
One hundred and one patients with tetralogy of Fallot who were older than 16 years of age underwent a total correction of the anomaly between May, 1964 and July, 1987. This group comprised 14.9% of the 679 consecutive patients who had repair of the tetralogy at our institution during the same period. Of the 101 patients, 8 had a previous shunt procedure for palliation. The preoperative mean hemoglobin value was 16.9*1.0% and the mean systemic oxygen saturation, 84.4*0.9%. In 76 patients[75.2%], a type II ventricular septal defect was seen whereas in 14 patients[13.9%], the defect was type I. In 72 patients[71.3%], other cardiac anomalies were present which included patent foramen ovale in 37.6%, atrial septal defect in 8.99b, vegetations in 6.9%, right sided aortic arch in 5.9% and coronary artery anomaly in 5.0%. The right ventricular outflow obstruction was caused most commonly by combination of infundibular and valvular stenosis[74.3%], followed by isolated infundibular stenosis[19.8%] and valvular stenosis [5.9%] alone in order. The preoperative mean diameter of the pulmonary valve ring size was 10.2*0.5 mm in diameter. A transannular patch enlargement of the right ventricular outflow tract was performed in 28 patients and, in 12 a pericardial monocusp was utilized. Major anomalous aorto-pulmonary vessels were encountered in 5 patients which were detected before or during the operation. In 3 patients, they were ligated beforehand to control the flooding of the operative field. Postoperatively, the mean systolic pressure gradient between the right ventricle and the main pulmonary artery was 16.2*2.3 mmHg and the mean systolic pressure- ratio between the right and the left ventricle was 45.3*2.0%. Perioperative complications including bleeding in 8.9%, pleural effusion in 7.9%, dysrrhythmia in 4.9%, and residual VSD in 4.0%. Operative mortality was 8.9%. There has been no operative death in the recent 65 cases since 1981. There were 2 late deaths, 68 and 113 months after surgery. There were 2 late detachment of the VSD patch during the follow-up period. Of the 6 patients with patch detachment found during the postoperative period, 3 had subacute bacterial endocarditis before or after the operation indicating The serious nature of this complication. Two of these patients subsequently underwent a successful reoperation.
Since we first performed open heart surgery on December 30, 1986, 126 cases were operated on up to August 31, 1989. Among the 126 cases, 65 cases were congenital heart disease of which 63 were acyanotic disease, and 61 cases were acquired heart disease, most of which were valvular heart disease. The age distribution of congenital heart disease was from 1 years 2 months to 48 years, and males had a slightly higher incidence. The age of acquired heart disease was from a minimum of 15 years to a maximum of 68 years, and the male to female ratio was 1;1.5. Midsternotomy was performed in all cases, and the aortic cannula was inserted through ascending aorta and the venous cannula inserted into the SVC and IVC through the right atrium. Vent was inserted through the right superior pulmonary vein. Cardioplegia solution was used in all cases; it was composed of sodium bicarbonate 3.5 ampule, KCL 14 mEq, 2% lidocaine 2.5 ml, 20 % albumin 50 ml and heparin 1000 units mixed to 950 ml with Hartman solution, and was made to 4oC and infused 10 ml per Kg every 20 minutes. The congenital heart disease had a variety of VSD in 32 cases, ASD 23 cases, PS 6 cases, PDA 2 cases, and one case each of Ebsteins anomaly and tricuspid atresia. The operations performed for acquired heart disease were 4 cases of OMC, 33 cases of MVR, and 5 cases of AVR, and 1 case of AVR with CABG. DVR was perfomed in 13 cases, and triple valve replacement was done in 1 case. Other than these, excision of LA myxoma was 2 cases, and repair of traumatic VSD and removal of a pulmonary embolism were one case each. The surgical mortality was 5 cases[4%], all of which occurred in valve replacement cases. Follow-up study revealed 2 late deaths. One died after a traffic accident and one died due to sepsis after he had received a gastrectomy for ulcer bleeding. The remaining patients were in good condition.
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.
Sinus of Valsalva aneurysm is a rare cardiac anomaly and a long-term survival after surgical treatment has not been well established. This study was designed to evaluate the long-term surgical results after the repair of sinus Valsalva aneurysm. Material and Method: From April 1991 to November 2003, 35 patients (23 male, 12 female, mean age 35.2 years, range 11∼64) underwent operation for sinus of Valsalva aneurysm. Twenty six patients (74.3%) were in the New York Heart Association (NYHA) class III∼IV before surgery. In preoperative echocardiogram, mean EF was 63.32 $\pm$ 11.43% and nine patients (25.7%) were in AR grade III∼IV. Direct closure, patch closure of ruptured sinus Valsalva were performed in fourteen patients (46.7%), sixteen patients (53.3%) respectively. Aortic valve replacement, valvuloplasty were performed in five patients (14.3%), three patients (8.6%) respectively. Three patients (8.6%) underwent the Bentall procedure. Concomitant procedures were performed in 15 patients (42.9%), which were closure of VSD and ASD. Mean CPB time and ACC time were 116.79 $\pm$ 38.79 and 81.2 $\pm$ 28.97 minutes. Result: There was no operative mortality. One patient (2.9%) developed complete heart block that required a permanent pacemaker implantation. Three patients (8.6%) required reoperation due to a recurred rupture of the sinus Valsalva aneurysm and developed aortic insufficiency. Mean follow-up time was 58.55 $\pm$ 38.38 months. There was one late death. Actuarial 5 year freedom rate from reoperation was 87.1 $\pm$ 7%. Conclusion: Surgical treatment for sinus of Valsalva aneurysm is safe and has satisfactory long-term results.
Background: Anatomic correction of transposition of the great arteries by means of the arterial switch operation is now accepted as the therapeutic method of choice. This retrospective study attempts to assess the results of the neonatal arterial switch operation for transposition of the great arteries performed by our newly established institution. Materials and methods: 33 consecutive neonates underwent the arterial switch operation between October 1991 to November 1997. There were 27 neonates with transposition and intact ventricular septum, 3 with ventricular septal defect, and 3 with Taussig-Bing anomaly. The mean age was 10.9$\pm$7.9 days and mean body weight was 3.29$\pm$0.44kg. Results: Overall postoperative hospital mortality was 30.3% (10 patients). The mortality has improved with time; 75% (6 patients) among first 8 consecutive patients before 1994, 20% (2 patients) among 10 patients in 1994 and 1995, and 13.3% (2 patients) among 15 patients since 1996. Univariated analysis of risk factors revealed that earlier date of the operations and one of preoperative events were determinants for operative death. There were two late deaths. A mean follow-up of 17.4$\pm$16.5 months was achieved in all 21 survivors. All were in New York Heart Association functional class I. One patient had mild pulmonary stenosis and two had mild aortic valve regurgitation on their echocardiography. Conclusions: We concluded that we should continue to perform arterial switch operation for neonates with transposition of the great arteries because the mortality of the operation has been improved and the operative survivors have good functional results with low incidence of late complications.
Background: Interrupted aortic arch is a rare congenital heart anomaly which still shows high surgical mortality. In this study, we investigated the causes of and the risk factors for mortality to improve the surgical outcomes for this difficult disease entity. Material and Method: From 1984 to 2004, 42 patients diagnosed as IAA were reviewed retrospectively. Age, body weight at operation, preoperative diagnosis, preoperative PGE1 requirement, type of interrupted aortic arch, degree of left ventricular outflow stenosis, CPB time, and ACC time were the possible risk factors for mortality. Result: There were .14 hospital deaths. Preoperative use of PGE1, need for circulartory assist and aortic cross clamp time proved to be positive risk factors for mortality on univariate analysis. Preoperative left ventricular outflow stenosis was considered a risk factor for mortality but it did not show statistical significance (p-value=0.61). Causes of death included hypoxia due to pulmonary banding, left ventricular outtract stenosis, infection, mitral valve regurgitation, long cardiopulmonary bypass time and failure of coronary transfer failure in TGA patients. Conclusion: In this study, we demonstrated that surgical mortality is still high due to the risk factors including preoperative status and long operative time. However preoperative subaortic dimension was not related statistically to operative death statistically. Adequate preoperative management and short operation time are mandatory for better survival outcome.
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.
Kim Dong-Jin;Min Sun-Kyung;Kim Woong-Han;Lee Jeong-Sang;Kim Yong-Jin;Lee Jeong-Ryul
Journal of Chest Surgery
/
v.39
no.4
s.261
/
pp.275-280
/
2006
Background: Aortopulmonary window (APW) is a very rare congenital heart anomaly, often associated with other cardiac anomalies. It causes a significant systemic to pulmonary artery shunt, which requires early surgical correction. Accurate diagnosis and surgical correction will bring good outcomes. The purpose of this study was to describe our 20-year experience of aortopulmonary window. Material and Method: Between March 1985 and January 2005, 16 patients with APW underwent surgical repair. Mean age at operation was $157.8{\pm}245.3$ ($15.0{\sim}994.0$) days and mean weight was $4.8{\pm}2.5$ ($1.7{\sim}10.7$) kg. Patent ductus arteriosus (8), atrial septal defect (7), interruptedaortic arch (5), ventricular septal defect (4), patent foramen ovate (3), tricuspid valve regurgitation (3), mitral valve regurgitation (2), aortic valve regurgitation (1), coarctation of aorta (1), left superior vena cavae (1), and dextrocardia (1) were associated. Repair methods included 1) division of the APW with primary closure or patch closure of aorta and pulmonary artery primary closure or patch closure (11) and 2) intra-arterial patch closure (3). 3) Division of the window and descending aorta to APW anastomosis (2) in the patients with interrupted aortic arch or coarctation. Result: There was one death. The patient had 2.5 cm long severe tracheal stenosis from carina with tracheal bronchus supplying right upper lobe. The patient died at 5th post operative day due to massive tracheal bleeding. Patients with complex aortopulmonary window had longer intensive care unit and hospital stay and showed more morbidities and higher reoperation rates. 5 patients had reoperations due to left pulmonary artery stenosis (4), right pulmonary artery stenosis (2), and main pulmonary artery stenosis (1). The mean follow-up period was $6.8{\pm}5.6$ (57.0 days$\sim$16.7 years)years and all patients belonged to NYHA class 1. Conclusion: With early and prompt correction of APW, excellent surgical outcome can be expected. However, optimal surgical method needs to be established to decrease the rate of stenosis of pulmonary arteries.
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