One-stage repair of cardiac and arch anomalies without circulatory arrest

Between 1999 and 2002, 23 patients underwent single-stage complete repair of cardiac anomalies and aortic arch obstruction, without circulatory arrest. Median age was 1.2 years. Intracardiac defects included ventricular septal defect in 9, double-outlet right ventricle in 6, d-transposition of the great arteries and ventricular septal defect in 2, subaortic obstruction in 3, and atrial septal defect in 3. Fourteen patients had coarctation of the aorta, 6 had coarctation with hypoplastic aortic arch, and 3 had interrupted aortic arch. Simple techniques were employed such as cannulation of the ascending aorta near the innominate artery and maintaining cerebral and myocardial perfusion. After correction of arch obstruction, intracardiac repair was undertaken. The mean cardiopulmonary bypass time was 169 min, aortic crossclamp time was 51 min, and arch repair took 16 min. There was no operative mortality or neurological deficit. In follow-up of 1-43 months, no patient had residual coarctation. This simplified technique avoids additional procedures, reduces ischemic time, and prevents problems related to circulatory arrest.     

复杂性动脉导管未闭的外科治疗

目的 :介绍复杂性动脉导管未闭 (PDA)诊断和手术治疗。方法 :采用胸骨正中切口。一期治疗PDA和心内伴发畸形。依据导管粗细 ,肺动脉扩张程度 ,采用结扎法和深低温低流量法经肺动脉缝闭PDA。要确认PDA周围的解剖 ,包括主动脉、左、右肺动脉 ,以免误扎。结果 :本组 72例 ,女性占6 7% ;PDA直径 0 3～ 1 8cm。伴发心内畸形中以室间隔缺损为主占 6 0 % ,法洛四联症 18%。死亡 1例 ,系合并室间隔缺损伴主动脉弓离断 ,误扎生命依赖导管。结论 :一期治疗PDA及伴发的心内畸形 ,避免二次手术的损伤 ,为安全、有效的治疗方法。及时发现恰当处理PDA ,决定治疗的结果 ,需特别引起重视。     

Enlarging angioplasty of the aortic arch with an pulmonary artery autograft patch. Interest in a single-stage repair of the syndrome of coarctation and interruption of the aortic arch]

Single stage repair of syndromes of coarctation and interruption of the aortic arch is a routine procedure in many surgical centres with good immediate results. The classical technique of aortic repair is based on the principles of Crafoord's extended resection anastomosis. Recoarctation is not an unusual long-term complication. A technique of enlarging angioplasty of the aorta using a patch of pulmonary artery has been developed and used in 22 neonates with obstruction of the aortic arch associated with ventricular septal defect with an average age and body weight of 15 days and 2.9 Kg respectively. The ventricular septal defect was closed surgically during the same procedure. Total circulatory arrest was not used in these children and all had aortic repairs with selective cerebral perfusion with moderate hypothermia (28-30 degrees C). This technique was used without any procedure-related early morbidity. No early or late deaths were observed in this series. Two patients were reoperated during the first year after the initial procedure: one for residual ventricular septal defect and the other for supraventricular pulmonary stenosis. Two patients, one of whom was reoperated, developed supraventricular pulmonary stenosis with a gradient of over 60 mmHg. These stenoses were observed in the first cases operated and were essentially due to the technique of pulmonary artery reconstruction. Over a median follow-up period of 10 months, no recoarctations were observed: the Doppler ultrasound study showed an isolated mean systolic gradient of 6 +/- 12 mmHg. The authors conclude that angioplasty of the aortic arch with an enlarging patch of pulmonary artery autograft during single stage surgery of syndromes of coarctation and interruption of the aortic arch provides a harmonious and durable repair of the aortic arch.     

Results of surgical correction of ventricular septal defects in infants]

Of a consecutive series of 91 babies aged less than 2 years, operated on for closure of a ventricular septal defect during the last four years, the 61 cases with a minimum post operative follow up period of 18 months were retained for review. Closure of the ventricular septal defect was carried out directly 41 times, and after pulmonary artery banding in the other 20 cases, using deep hypothermia with a short period of circulatory arrest and cardiopulmonary bypass.     

Repair of Coarctation-Related Aortic Arch Aneurysm and Ventricular Septal Defect in an Adolescent

A saccular aortic arch aneurysm that is secondary to aortic arch coarctation and that is accompanied by a ventricular septal defect is a rare combination in the adolescent patient. Total simultaneous repair of all of these conditions is desirable, because of the higher morbidity and mortality rates of staged procedures—particularly when resection of the saccular aneurysm is delayed.Herein, we discuss the case of a 16-year-old boy who underwent simultaneous surgical correction of these malformations. With the aid of cardiopulmonary bypass on the beating heart, the coarctation and the aneurysmal segment were resected, and a tubular Dacron graft was interposed. The ascending aorta and femoral artery were both then cannulated to ensure whole-body perfusion during cardiopulmonary bypass. The ventricular septal defect was closed with the patient under cardioplegic arrest. After 10 days, he was discharged from the hospital without sequelae. We conclude that single-staged repair of cardiac abnormalities and of an aortic arch aneurysm that is secondary to coarctation of the aortic arch can be performed safely and effectively in adolescent and adult patients by use of our technique.Key words: Aorta/surgery, aortic aneurysm/physiopathology/surgery/ultrasonography, aortic coarctation/complications/physiopathology/surgery/ultrasonography, coronary disease/surgery, magnetic resonance angiography, methods, time factorsUnrepaired coarctation of the aorta can lead to hypertensive cardiovascular sequelae, such as coronary disease, heart failure, stroke, aortic dissection or rupture, and premature death.¹ Although narrowing of the aortic arch is sometimes seen when surgery is performed on congenital cardiac abnormalities, aortic coarctation that persists into adulthood is unusual. Coarctation that originates between the arch vessels is rare, constituting approximately 1% of all coarctation cases.² Aortic coarctation is sometimes accompanied by a fusiform or saccular aneurysm, which is either congenital or secondary to the hemodynamic disturbance caused by the coarctation. Native aortic coarctation alone is usually repaired surgically through a left thoracotomy, but aortic arch coarctation with saccular aneurysms and concomitant cardiac disorders in adolescent patients still present dilemmas for the surgeons.³ To decrease potential complications of anatomic repair, various techniques have been proposed,^3,4 such as extra-anatomic ascending aorta-to-descending aorta bypass, or the use of deep hypothermic circulatory arrest during a simultaneous or staged surgical approach. In our patient, the single-staged repair of a ventricular septal defect (VSD) and a large saccular aortic arch aneurysm that was secondary to an unusually located coarctation of the aortic arch was carried out via median sternotomy.     

Risk factors associated with arterial switch operation for transposition of the great arteries

INTRODUCTION AND OBJECTIVES: The present study was undertaken to determine the risk factors for early mortality following an arterial switch operation. PATIENTS AND METHOD: From January 1994 through October 2003, 78 pediatric patients underwent surgical repair. Simple transposition was present in 48 patients (61.5%), 29 (37.2%) had an associated ventricular septal defect, and one had a Taussig-Bing anomaly. The risk factors analyzed were: the patient's age and weight at the time of the intervention, repair of a coexisting ventricular septal defect, coronary artery anatomical pattern, duration of cardiopulmonary bypass, duration of aortic cross-clamping, and duration of circulatory arrest. All factors were evaluated for strength of association with the duration of mechanical ventilation, the length of intensive care unit stay, and mortality. RESULTS: Overall, the early mortality rate was 9% (7/78). Some 14 patients (17.9%) underwent simultaneous repair of a ventricular septal defect. Patients with an intramural coronary artery (n=3, 3.8%) or a single coronary ostium (n=5, 6.4%) were the only ones who had a significant (P<.05) mortality risk, at 50% (4/8). Circulatory arrest was implemented in 53 (68%) patients. There were significant correlations between the duration of circulatory arrest and the ventilator support time (r=0.3, P<.05) and the duration of stay in the intensive care unit (r=0.3, P<.05). CONCLUSIONS. The risk of early death was increased when more complex coronary artery anatomical variants were present. As the period of circulatory arrest lengthened, the mechanical ventilation time and duration of intensive care unit stay increased.     

Interruption of the aortic arch in adults.

The interruption of the aortic arch is a rare cardiac congenital disease; such patients may occasionally survive to adulthood without surgery. The associated intracardiac malformations may modify survival. Our aim is to report three young adults (18-19 years of age) with interruption of the aortic arch. Two had type C, and the other had type B with subpulmonic ventricular septal defect and pulmonary valve insufficiency. We review 106 cases collected from the medical literature into 3 groups: 1) the whole group of patients; 2) patients with isolated interruption of the aortic arch, and 3) patients with interruption of the aortic arch associated with ventricular septal defect. In the whole group we found 18 cases of interruption of the aortic arch type A, and 25 cases of interruption of the aortic arch type B; 37 cases of isolated interruption of the aortic arch and 43 cases associated with ventricular septal defect. Fifty percent of the patients died before 15 days of life (0.042 years). According to the cumulative frequency graphic, only 5% of the patients survived beyond the age of 5 years. We found no information to relate patient's survival rate and anatomic type of the interruption of the aortic arch. From adolescence, the survival of the patients with interruption of the aortic arch associated to septal ventricular defect was 7%, and a 14% survival was found in patients with isolated interruption of the aortic arch. No statistical difference was found between the means of the ages of these two groups (P > 0.25).     

Complete repair of the interrupted aortic arch by an anterior approach. Apropos of 9 cases

Interrupted aortic arch is a duct-dependent heart disease, and this is why the use of prostaglandins E, which keep the ductus arteriosus open to feed the descending aorta, has transformed its formerly sombre prognosis. The authors report the cases of 9 neonates and infants presenting with interrupted aortic arch associated with ventricular septal defect who underwent total repair of the malformations under extracorporeal circulation. Five children were less than 10 years' old. The aortic lesions were of type A in 2 cases (interruption distal to the left subclavian artery) and of type B in 7 cases (interruption between the left carotid and subclavian arteries). In the second group the lesions were of type B1 in 5 cases (without retro-oesophageal right subclavian artery) and of type B2 in 2 cases (right retro-oesophageal artery distal to the interruption). There was one death (11 p. 100). So far, no child has been reoperated upon for residual aortic obstruction or reopened ventricular septal defect. This technique of total repair in one stage, with closure of the ventricular septal defect, seems to be preferable to the two-stage technique in which stage one includes, in addition to aortic arch repair, cerclage of the pulmonary artery, a procedure with numerous disadvantages and a higher overall mortality rate.     

Interrupted aortic arch: an eight-year experience

INTRODUCTION: Interrupted aortic arch (IAA) is a rare congenital heart defect with a high mortality rate in the neonatal period. Surgical correction of associated intracardiac anomalies can be performed in a one-stage (primary) or two-stage approach. OBJECTIVES: Case review of children with IAA operated in our center and to evaluate the surgical outcomes and the occurrence of complications. METHODS: A retrospective chart review of children operated from June 1998 to October 2006. RESULTS: Twelve children (nine girls and three boys) were operated. Nine patients had ventricular septal defect with septal malalignment, two had univentricular hearts and two had transposition of the great arteries. Primary correction was performed in eleven patients (aged between two and 38 days), including two Norwood procedures. There was no early mortality. The follow-up time ranged from 30 days to six years (median 2.6 years), with late mortality of 33%. Two children required reintervention for aortic arch restenosis, which was successfully treated by catheterization. DISCUSSION: Our experience is that early primary repair of IAA has low early and late mortality and is the method of choice most situations. Late mortality depends the severity of associated anomalies.     

Emergency surgical repair of acute aortic arch dissection. Apropos of 14 cases

Among 70 patients operated upon for acute dissection of the ascending aorta between 1977 and 1984, 14 (age range 40-72 years) benefited from emergency aortic arch repair motivated by the presence in, or extension to, this segment of the portal of entry, or by lesions of the supra-aortic main vessels. The operation was performed under cardiopulmonary bypass in all cases. Cerebral protection was ensured in 9 patients by deep hypothermia alone or with circulatory arrest for a mean period of 5 minutes (7 cases), and in 5 patients by continuous brain perfusion (carotid blood flow 4 ml/kg/min; perfusion pressure 70 mmHg). Repair consisted of ascending aorta replacement combined with gluing of the arch whenever possible, or bevelled resection in the concavity of the arch, or complete aortic arch replacement with reimplantation of the cervico-cerebral vessels when necessary. In every case the gelatin-resorcin-formalin glue was used to reinforce the aortic wall or the areas with sutures. Operative mortality was nil. Hospital mortality was 28.5%. In patients operated upon under deep hypothermia alone or associated with circulatory arrest the main complications were neurological (4 cases) and respiratory (3 cases) disorders; these were responsible for the death of 3 patients. A fourth patient died of mediastinitis. No neurological or respiratory complication occurred in patients who had brain perfusion. The mean follow-up period was 37 months (range 7-84 months). Late mortality was nil. All but one patient (sequelae of paraplegia) are in excellent clinical condition. Post-operative angiography alone or combined with computerized tomography showed satisfactory blood distribution in all cases and no aneurysm formation on the dissected aorta despite a persistent false lumen.     

Echocardiographic assessment of interrupted aortic arch

BACKGROUND: In patients with interrupted aortic arch echocardiography provides detailed information about the anatomy of the aortic arch and the associated cardiac anomalies. Only a few reports have evaluated the reliability of this non-invasive diagnostic procedure by correlation with angiographic and surgical findings. METHODS: From 1988 through 1993, 45 infants with interrupted arch underwent surgical repair (mean age 13.02 days). Of the patients, 33 had interruption of the arch between the left common carotid and subclavian arteries; 25 patients had a ventricular septal defect, and the remaining 20 had coexisting complex congenital heart defects. Preoperative diagnosis was made exclusively by echocardiography in 25 of the patients. Accuracy of echocardiographic diagnosis was evaluated retrospectively by comparing preoperative studies with angiography and surgical reports. We then investigated whether the morphologic features of the interrupted arch might influence surgical procedure or outcome. RESULTS: Intracardiac anatomy was accurately diagnosed by echocardiography in all cases; in 2 patients angiography provided additional information concerning the morphology of the aortic arch. Operative notes described differences in morphology of the arch in 7 patients, but these did not influence the surgical procedure. Direct anastomosis of the interrupted segments was possible in 38 patients, and 36 patients underwent primary intracardiac repair. Echocardiographic measurements revealed that the diameter of the ascending aorta was related to the number of vessels originating from the proximal aortic arch. The distance between the interrupted segments was significantly different according to the site of interruption, but not between cases with an isolated ventricular septal defect versus those with complex heart disease. It did not influence the method of arch repair, nor was it related to recurrent or residual obstruction. CONCLUSION: Preoperative echocardiography offers accurate and complete diagnosis in the critically ill neonate with interrupted aortic arch and associated intracardiac abnormalities.     

主肺动脉窗及合并心脏畸形的外科手术治疗经验总结

目的:回顾分析主肺动脉窗及合并心脏畸形的临床特征,总结外科手术治疗的经验和效果.方法:自1997-01至2011-05,共有46例主肺动脉窗患者接受了外科手术治疗,平均年龄(3.2±2.5)岁(0.1～16)岁,平均体重(11.4±6.2)kg(4～47)kg.合并的心脏畸形包括右肺动脉起自升主动脉7例、二尖瓣关闭不全7例、主动脉瓣下隔膜5例、动脉导管未闭6例、主动脉弓中断5例、室间隔缺损4例、房间隔缺损4例、右室双出口3例、三尖瓣关闭不全2例、右冠状动脉起自肺动脉2例、法乐四联症1例.43例患者在体外循环下手术,其中23例经主动脉切口补片修补,12例经肺动脉切口修补,4例经主肺动脉窗修补,2例行切断缝合.其余3例患者在非体外循环下行主肺动脉窗缝扎(1例)或切断缝合(2例).合并心脏畸形同时矫治.结果:早期死亡3例,平均随访5年,1例患者术后一年死于肺动脉高压,其余患者无再次手术及残余分流,主动脉和肺动脉发育正常.结论:主肺动脉窗一经诊断,应尽早外科手术治疗,体外循环下经主动脉切口补片修补是首选的方法.     

主动脉手术体外循环的初步体会

10例主动脉手术在体外循环下进行。降主动脉手术4例,升主动脉手术5例,主动脉弓离断(B型)手术1例。根据不同手术分别采用低温体外循环,深低温停循环或左心转流。本组死亡3例。本文讨论了左心转流的方法,深低温停循环的安全时限,B型主动脉弓离断手术的体外循环管理,及防止人工血管渗血的问题。     

大血管手术中的体外循环管理

目的:总结各种大血管手术中的体外循环(extracorporeal circulation,ECC)方法及管理经验.方法:体外循环下完成各种大血管手术21例,升主动脉人造血管置换、Bentall手术、全主动脉弓置换(象鼻术)+带膜支架植入手术,21例患者采用改良心肌保护液灌注,均用深低温停循环+顺行性脑灌注方法或逆行灌...     

Lung function after deep hypothermic cardiopulmonary bypass in infants

There is increasing concern about neurologic injury due to deep hypothermia with low flow during repair of complex congenital heart defects in neonates and infants. Twenty infants with ventricular septal defect and pulmonary hypertension were randomly assigned to cardiac repair under deep hypothermia with circulatory arrest or deep hypothermia with low flow. Measurements of static pulmonary compliance, airway resistance, and respiratory index were performed before institution of cardiopulmonary bypass and at 5 minutes and 2 hours after cessation of cardiopulmonary bypass. Both groups had significant pulmonary dysfunction in terms of static pulmonary compliance, airway resistance, and respiratory index. There was greater impairment of pulmonary compliance and respiratory index after deep hypothermia with low flow, and this group required longer intensive care unit stay.     

Isolated cerebral and myocardial perfusion during aortic arch repair in neonates

OBJECTIVES: To prevent possible neurologic injury after hypothermic circulatory arrest, aortic arch obstruction with cardiac defects is repaired in one stage using isolated cerebral and myocardial perfusion (ICMP). This study investigated serum S-100 protein(S-100) levels in neonates undergoing ICMP. METHODS: Between February 2000 and January 2001, 19 neonate patients underwent repair of critical congenital heart defects. Seven of these patients with aortic coarctation(n = 3) or interrupted aortic arch (n = 4) with ventricular septal defect(ICMP group) underwent primary total repair. An arterial cannula was inserted either into the ascending aorta or into a polytetrafluoroethylene graft which was anastomosed to the innominate artery. During arch repair, a cross-clamp was placed between the innominate and left carotid arteries, and an end-to-end arch anastomosis was performed with cerebral perfusion and heart beating. During ICMP the flow was reduced to maintain a radial artery pressure of 30-45 mmHg. The remaining 12 patients underwent complete transposition of great arteries(n = 9) or total anomalous pulmonary venous connection(n = 3) using a cardiopulmonary bypass(CPB) with flow of 150-180 ml/kg/min(control group). Sequential blood samples for S-100 determinations were taken after induction of anesthesia, 30 min after aortic declamping(post-ACC), 30 min after CPB, and 24 hr after CPB. RESULTS: There were no early and late deaths. Neurologic symptoms were not observed in any patients. Mean ICMP time in ICMP group was 17 +/- 4 min. In all patients, S-100 showed the highest value post-ACC and then declined with time. There were no differences in S-100 between the groups at any other time point. CONCLUSIONS: Selective cerebral perfusion through the innominate artery may be able to maintain brain circulation.     

Management of traumatic sinus of valsalva-right ventricular fistulae

Residual intracardiac defects following penetrating injuries to the heart require thorough invasive evaluation and strong consideration for operative repair based upon the degree of hemodynamic compromise. Traumatic aortico-right ventricular fistulae result in high flow left-to-right shunts, and are associated with early onset of congestive failure. Operative repair requires utilization of total cardiopulmonary bypass, ventriculotomy and aortotomy to allow for thorough inspection of the ventricular septum, aortic valve cusps, and coronary ostia. Obliteration of both ends of the fistula (septal and sinus of Valsalva repair) minimizes the risk of recurrence.     

Intrapulmonary channel for one-stage correction of aortic arch obstruction

There are several methods of surgical repair of aortic coarctation or interruption; the optimal technique is still controversial. The purpose of this study was to assess a new surgical method: intrapulmonary channel for one-stage repair of aortic coarctation or interruption associated with intracardiac anomalies. Between 1993 and 1995, 4 patients with aortic coarctation or interruption and intracardiac anomalies received one-stage surgical correction. Their ages ranged from 5 to 26 years (mean, 16 years). The aortic arch lesions were preductal coarctation in 2, and type B interruption in 2. Coexisting anomalies consisted of patent ductus arteriosus in 4, ventricular septal defect in 3, and aortopulmonary window in 1. An intrapulmonary channel was constructed in all patients, and co-existing anomalies were corrected simultaneously. There was no hospital death or late mortality. A cerebral complication occurred in one patient because of air embolism. Mean follow-up was 9.5 years (range, 8.5-11.5 years). There was no evidence of recoarctation or late aneurysm formation. For selected patients with aortic coarctation or interruption and intracardiac anomalies, an intrapulmonary channel might be an option for one-stage correction.     

Interrupted aortic arch. A series of 15 patients

Between 1983 and 1989, 15 children underwent surgical repair of interrupted aortic arch at 1 to 20 days of age. The anatomical form was a Celoria and Patton type B in all patients with an associated perimembranous ventricular septal defect in all but one who had multiple ventricular septal defects, and patent ductus arteriosus. Six children had a retro-esophageal right subclavian artery, two had subaortic stenosis and two had a right-sided descending thoracic aorta. In two children with severe hypoplasia of the ascending aorta the repair was performed in one stage with two deaths due to left ventricular failure. In the other B cases, a two-stage repair was carried out. The reconstruction of the aortic arch varied according to the individual case. All children had pulmonary artery banding. Seven children survived longer than 30 days. Six of them later underwent a complete repair. The only survivors were those patients in whom the neo-aortic arch grew harmoniously. The authors conclude that: a two-stage repair gave disappointing results in this series of consecutive patients, mainly because of the poor quality of the reconstruction of the aortic arch by thoracotomy.     

Congenital heart disease: diagnostic approaches, surgical indications and operative results

Our management policies of the main congenital cardiac defects which require emergency surgical intervention in neonate and infancy were reported. Total anomalous pulmonary venous drainage (TAPVD): Recent refinements in two-dimensional (2-D) echocardiography have made it possible to operate for this anomaly using only this modality. When a combination of coarctation is suspected and/or the location of the drainage of the pulmonary vein is obscure, catheterization and angiocardiography are added for detailed diagnosis. It is, however, our policy not to perform right-sided angiocardiography for patients younger than three months of age. Symptomatic cases are operated urgently, although not always on an emergency basis. In our experience, a 12 approximately 24 hour delay with intensive cardiorespiratory and metabolic care may improve the preoperative conditions considerably and increase the chances of a successful surgical repair. Coarctation of the aorta (CoA) and interruption of the aortic arch (IAA): Diagnosis of CoA by echocardiography and aortography by radial artery injection is well established. In neonates and infants with CoA or CoA + patent ductus arteriosus (PDA) and/or ventricular septal defect (VSD), emergency repair of coarctation (usually with subclavian flap aortoplasty) without pulmonary artery banding (PAB) is undertaken on the day of established diagnosis. If a combination of complex cardiac anomalies such as transposition of the great arteries (TGA) and Taussig-Bing anomaly is suspected, catheterization and angiocardiography are added for the detailed diagnosis. Repair of coarctation combined with PAB has been our choice of procedure in these infants. If IAA is suspected, catheterizaton, angiographic and detailed echocardigraphic studies are performed to define the precise anatomy of the aortic arch and associated intracardiac lesions, paying particular attention to the left ventricular outflow tract, as soon as such patients become clinically stable by intensive medical treatment using prostaglandin E1 (PGE1), digitalis and diuretics. Once the diagnosis is established surgical treatment should be carried out without delay. Initial palliation by aortic arch reconstruction with PAB followed by two-stage definitive intracardiac repair have been our choice of procedure in neonates and infants with IAA without severe left ventricular outflow tract obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)