Surgical outcomes of interrupted aortic arch with ventricular septal defect in neonates and infants / 中华胸心血管外科杂志

Objective:To summarize the surgical outcomes of interrupted aortic arch with ventricular septal defect (IAA/VSD) in neonates and infants.Methods:This was a retrospective review of clinical data of 123 neonates and infants who received surgery for IAA/VSD from Jan 2009 to Jan 2019. Median age at repair was 48 days. Forty-four patients (36%) were neonates. One-hundred-and-twenty-two patients (99%) underwent standard aortic arch reconstruction with VSD closure, and one neonate (1%) underwent staged Yasui operation. Risk factors for early mortality was analyzed by decision tree model.Results:Early mortality after surgery was 13%. Duration of cardiopulmonary bypass longer than 135 min, surgery received during neonatal period and before 2016 was identified as higher risk group for mortality. Median follow-up time was 3.5 years (range, 1-10 years). Freedom from aortic arch obstruction at 6 months, 1 year, 5 years after surgery was 75%, 72% and 72% respectively. Freedom from left ventricular outflow tract (LVOT) obstruction at 6 months, 1 year, 5 years after surgery was 91%, 83% and 73% respectively. A total of 17 patients received 21 reoperations. The patient who received Yasui operation experienced no residual obstruction during the follow-up.Conclusion:Early outcomes after surgery for IAA/VSD in neonates and infants are satisfactory. However, patients with standard aortic arch reconstruction have a higher risk for aortic and LVOT obstruction, and require multiple reoperations.

Single-stage repair of interrupted aortic arches and associated cardiac anomalies in neonatal: the outcomes and follow-up / 中华胸心血管外科杂志

Objective:This study defined mid-term results of a policy of single-stage repair of interrupted aortic arch associated cardiac anomalies.Methods:Between October 2012 and June 2019, 38 patients with interrupted aortic arch were evaluated for short- and mid-term results after surgical treatment, the average age of patients was(12±7) days and the mean body weight was(3.3±0.5)kg.29 patients belonged to IAA type A, and 7 patients to type B, and 2 patients belonged to IAA type C, 30 patients were complicated with ventricular septal defect, atrial septal defect, and patent ductus arteriosus, two complicated with bicuspid aortic valve, four complicated with Taussig-Bing malformation and two with double outlet right ventricle, one with truncus arteriosus(A4), one with aortopulmonary window, one with left ventricle outflow tract obstruction. Single-stage repair was performed in all patients with cardiac anomalies.Results:Early mortality was 5.2%(2 cases), one was die for low cardiac output syndrome, the other die of septic shock. Median CPB time was 138 min(90-305 min), and median aortic cross clamping time was 68 min(47-163 min). Hospital day was 25d( 13-52 days).32 patients were followed-up, median time 34 months(9-85months). 2 patients(6.0%) were mild pulmonary stenosis pressure(20-25 mmHg), 3 patients(9.3%) suffer aortic anastomosis mild stnosis(21-44)mmHg.1 patient(3.1%) LVOTO was repaired ten months after the repair of interrupted aortic arch. The left broncus of of one patient was mild stenosis but without dyspnea.Conclusion:Single-stage end -to-side anastomosis repair of interrupted aortic arches in neonatal and repair of associated cardiac anomalies is safe and have low effective with low motality.

Chinese expert consensus on surgical treatment of congenital heart disease (11): Coarctation of the aorta and interrupted aortic arch / 中国胸心血管外科临床杂志

@#Coarctation of the aorta and interrupted aortic arch are congenital anomalies affecting the aortic arch. Because of the poor natural prognosis, many patients will need early surgical repair or even emergency surgery. With the improvement of the surgical techniques, cardiopulmonary bypass techniques and perioperative intensive care, surgical mortality has now dramatically decreased. However, aortic arch restenosis, left ventricular outflow tract obstruction and long-term hypertension are problems that may be still encountered during the follow-up period. By reviewing large amounts of literature and discussing among experts, we achieved a consensus on many aspects of the management strategy. We hope this consensus will help Chinese colleagues further improve the overall surgical outcomes of coarctation of aorta and interrupted aortic arch.

Staged Repair after Hybrid Palliation for Interrupted Aortic Arch with Systemic Outflow Tract Obstruction / 대한흉부외과학회지

Surgical management of interrupted aortic arch (IAA) with systemic outflow tract obstruction is clearly a challenge. If both ventricles are adequate, the Yasui operation is a useful option. Otherwise, a staged approach through initial hybrid palliation and delayed biventricular repair, tailored to the degree of obstructed outflow, serves to avoid a high-risk neonatal procedure. Herein, we present a patient with IAA and severe systemic outflow tract obstruction whose treatment involved hybrid palliation, followed by a Yasui operation.

Short and midterm results of surgical treatment of interrupted aortic arch / 中华外科杂志

Objective@#To analyze the early and midterm results of surgical treatment of interrupted aortic arch (IAA) with double-ventricular procedure.@*Methods@#The data of the 68 cases with the main diagnosis of IAA with biventricular structure from June 2009 to June 2017 at Department of Cardiac Surgery, Guangdong General Hospital was collected, including 46 cases of type A, 22 cases of type B. There was no type C case. Except for 5 cases without patent ductus arteriosus or other intracardiac malformations, the remaining 63 cases were combined with intracardiac deformity. The age of operation was 8 days to 18 years, including 28 cases(41.2%) of newborns, 32 cases(47.1%) within 1 year old, 6 cases(8.8%) from 1 to 15 years old, and 2 cases(2.9%) above 15 years old. Of the 5 patients with no intracardiac malformations, 4 patients were treated with a left-posterior thoracic lateral incision for primary surgical correction with end-to-side anastomosis, and 1 adult patient was treated with a median sternotomy incision to complete the anastomosis with the assist of extracorporeal circulation. Of the 63 patients with intracardiac malformation, 2 underwent reconstruction of the aortic arch via posterolateral left thoracic incision merely; 2 patients underwent staged surgery to correction the IAA and intracardiac malformation; the remaining 59 patients underwent the one-stage procedure of IAA and intracardiac malformation correction with deep hypothermic circulatory arrest and 43 cases of selective cerebral perfusion. A total of 55 cases of end-to-end or end-to-side anastomosis were performed in all aortic arch reconstruction. There were 6 cases of connection the arch to descending aorta with autogenous pulmonary artery conduit, and 1 case with homemade bovine pericardial conduit. And there were 5 cases of anastomosis enlargement with autogenous pulmonary artery patch, and 1 case with left subclavian artery flap patch. There were 1 case performed with D. K.S. operation for the severe hypogenetic aortic valve and ascending aorta, and 1 case with left ventricular double outlet channel operation for the severe subvalvular aortic stenosis.@*Results@#Follow-up to 9 years, 4 deaths (5.9%) related to circulatory system diseases: 3 cases died within 30 days after surgery (1 case was neonatal, 2 cases were 1 to 12 months old); one died 8 months after surgery for low cardiac output syndrome. In the early postoperative period, 11 cases (16.2%) of residual aortic arch obstruction (pressure gradient >30 mmHg) were observed, of which 3 cases were moderate (4.4%); follow-up to 5 years after operation was residual obstruction in 3 cases and moderate in 2 cases. There were 3 cases with aortic valve and subvalvular obstruction (differential pressure >30 mmHg), 3 cases were followed up to 5 years after aortic valve and subvalvular obstruction, and 2 cases were moderate or above. To date, 5 patients have undergone 6 reoperations of the cardiovascular system: 3 cases because of pulmonary artery stenosis, 2 cases because of severe aortic arch stenosis, and 1 case because of aortic subvalvular obstruction.@*Conclusions@#One-stage surgical repair of IAA is safe and clinically effective in the neonatal and infant. But some patients still requires reoperation for re-obstruction from subvalvular or aorta arch. For some patients, the choice of aortic arch reconstruction depends on the specific situation.

Surgery treatment of Berry syndrome / 中华胸心血管外科杂志

Objective The purpose of this manuscript was to compare the outcomes of different surgical methods of Berry syndrome and to figure out the occurrence of postoperative morbidity.Methods From January 2003 through December 2017,nineteen infants with Berry syndrome underwent one-stage repair at Shanghai Children's Medical Center.There were 12 male and 7 female.Among them,six patients were neonates.The IAA morphology was type A in 17 patients and type B in 2 patients.The APW morphology was type Ⅰ a in 6 patients,type Ⅱ b in 11 patients,and type Ⅲ in 2 patients.Preoperative mechanical ventilation was required in 4 patients.Three different surgical correction techniques were employed to repair the APW and AORPA,including intra-aortic baffle in 8 patients,right pulmonary artery (RPA) detachment in 6 patients,and RPA angioplasty with aortic cuff in 5 patients.The descending aorta was then anastomosed to the aortic arch by an end-to-side anastomosis with a patch augmentation in the anterior wall.Results The mean CPB and aortic cross-clamp time was (146.7 ± 63.5)minutes (range,79 to 260 minutes) and (74.3 ± 27.4)minutes(range,46 to 147 minutes),respectively.There were 3 deaths.One patient died of severe pneumonia and multiorgan dysfunction on postoperative day 26.One patient suffered severe low cardiac output syndrome after surgery and died on postoperative day 1.One patient died of congestive heart failure at 2 months after discharge.Reoperations were required in 3 patients during the follow-up period.RPA arterioplasty with bovine pericardial patch augmentation was performed in 2 patients for RPA restenosis.Lecompte maneuverer was performed in 1 patient to release the compression of the RPA from the ascending aorta.Conclusion The mortality of one-stage repair of Berry syndrome was high.Surgical correction should be performed as soon as diagnosed.An intra-aortic baffle patch is suitable for type Ⅱ a APW defect patients beyond the neonatal period.Pulmonary hypertension crisis is important after surgery.RPA restenosis is the main reason for reoperation.

One-stage surgical repair of interrupted aortic arch in neonates and young infants:surgical experiences, follow-up re-sults and subsequent treatments / 中华胸心血管外科杂志

Objective To discuss one-stage surgical repair of interrupted aortic arch ( IAA) in neonates and young in-fants, summarize follow-up results and subsequent treatments.Methods From September 2010 to December 2014, a series of 38 consecutive neonates and young infants ( M/F =26/12 ) with IAA were admitted in our surgical group to receive surgical treatment, with a median age of 11 days(1 day to 5 months) and a median body weight of 4.1 kg(1.8 to 5.8 kg).IAA was type A in 24 cases, type B in 13 cases and type C in 1 case.Associated cadiovascular anomalies were common except 2 cases, including atrial septal defect(n=23), ventricular septal defect(n=36), left ventricular outlet tract obstruction(LVOTO)(n=7), anomalous left pulmonary artery from aorta(n=1), truncus arteriosus(n=1).There were 5 cases of airway stenosis in 28 cases of CT angiography and 3D airway reconstruction.All the 38 cases were admitted with differential cyanosis.36 cases were recieved one-stage surgical repair except 2 cases died before operation.35 cases of IAA associated with intracardiac anom-alies were repaired through a median sernotomy.Cardiopulmonary bypass(CPB) was performed with two canulations in ascend-ing aorta and main pulmonary artery.Selective cerebral perfusion(n=21) or circulatory arrest(n =14) was carried out with body temperature of 18℃-23℃.After the patent ductus arteriosus tissue was completely resected , a continuous end-to-end or end-to-side suturing with 7/0(8/0) prolene was performed.In 1 type B cases with expected excessive anastomotic tension, the left subclavian artery was cut off to release the aortic arch .The associated anomalies were repaired during the cooling or re-warming period.Chest close was delayed to carry out no more than 24-72 h postoperatively in 16 cases.The other one type A IAA case without intracardiac anomaly was repaired through a standard left posterior lateral incision .Patients were strictly fol-lowed up with a standard protocol.Complications such as aortic arch restenosis, new LVOTO, new airway stenosis, and so on, were specialy focused on during the follow-up period.If needed, reoperation or transcatheter intervention was applied to treat the complications.Results Two cases died before operation and another 2 died in the early period postoperatively because of pulmonary hypertension crisis and severe tracheal stenosis, respectively.Sudden death was in 1 case during the follow-up peri-od.Median CPB time was 138 min(105-208 min) and median selective cerebral perfusion or circulatory arrest time was 24 min(16-35 min) .Mechanical ventilation time was 2-25 days.Blood pressure difference between the upper and lower limbs was less than 20 mmHg in all the patients before discharge.29 cases(85.29%) was followed up for 28 months(9 -60 months).3 cases were received reoperation in 5 cases of newly detected LVOTO.One case received reoperation in the 7 preop-erative LVOTO cases because of newly detected LVOTO(blood pressure gradient more than 40 mmHg).Two cases with aortic arch restenosis received percutaneous transcatheter ballon dilation .One case with new left main bronchus stenosis after opera-tion was strictly followed up.Conclusion IAA is a rare and severe congenital heart disease.Positive surgical repair should be performed after definite diagnosis being made .Although satisfactory results can be obtained with one-stage primary operation , IAA is a kind of progressive chronic disease.New postoperative LVOTO should be focused on becasue it will need reoperation or even the third operation .

Role of systolic blood pressure gradient of limb test in diagnosing neonatal aorta anomaly / 中华实用儿科临床杂志

Objective To determine the effectiveness of systolic blood pressure gradient between the right arm and the right leg(SBPG) tests in the diagnosis of neonatal aorta anomaly(AoA).Methods The SBPG of infants suspected of having critical congenital heart diseases were collected prospectively,who were admitted to Guangdong General Hospital from January 2013 to December 2015.The results of SBPG test were compared with those of echocardiography or cardiac computed tomography (golden standard).The rates of true positive,true negative,false positive,false negative were calculated under cutoff values of 5 mmHg(1 mmHg =0.133 kPa),10 mmHg,15 mmHg and 20 mmHg,respectively.Receiver operating characteristic (ROC) curves was used to compare tests of different cutoff and the areas under the ROC curve were also calculated.Results Among 664 enrolled infants,67 cases were confirmed by golden standard test.The systolic blood pressure in the right arm,the legs and SBPG in AoA group and non-AoA group were (88.0±20.4) mmHgvs.(73.4±9.3) mmHg (P＜0.01),(66.1 ±10.1) mmHg vs.(69.0 ±9.7) mmHg(P＞0.05) and (22.6±17.8) mmHgvs.(2.3 ±4.8) mmHg(P ＜0.01),respectively.In these patients,31 cases(46.3％),31 cases(46.3％),27 cases(40.3％) and 21 cases(31.3％) were diagnosed of AoA,and 36 cases(53.7％),36 cases (53.7％),40 cases(59.7％),and 46 cases (68.7％) were missed by SBPG tests of 5 mmHg,10 mmHg,15 mmHg and 20 mmHg,respectively (P ＜ 0.01).The rates of true negative among those groups were 94.1％,99.5％,99.7％ and 100.0％,and the areas under ROC curve were 0.656,0.722,0.695 and 0.657,respectively (P ＜ 0.01).Conclusions Almost half of AoA infants could be screened out by SBPG test.The cutoff of 10 mmHg could probably be used to screen potential AoA infants,with higher true positive rate and lower false positive rate.

One-Stage Repair of an Interrupted Aortic Arch with an Aortopulmonary Window in a Premature Neonate

Interrupted aortic arch with an aortopulmonary window is a rare congenital entity that is associated with high morbidity and mortality, especially in premature low-birth-weight infants, and the proper timing of surgical correction remains a matter of debate. We present the case of a premature infant weighing 1.6 kg who successfully underwent one stage surgical repair to treat interrupted aortic arch with an aortopulmonary window. The therapeutic management of this patient is described below, and a review of the literature is presented.

Complete Repair of Double Outlet Right Ventricle and Interrupted Aortic Arch (Arch Reconstruction+Arterial Switch Operation+Intra-ventricular Rerouting) after Bilateral Pulmonary Artery Banding / 日本心臓血管外科学会雑誌

We performed bilateral pulmonary artery banding (BPAB) through a median sternotomy on a four-day-old male infant with a double-outlet right ventricle (DORV) and interrupted aortic arch (IAA) who was delivered at 40 weeks of gestation. After urinary output improved, definitive repair was carried out 5 days later. Intra-ventricular rerouting was followed by arterial switch with the Lecompte maneuver. The aortic arch was reconstructed with direct anastomosis and the right ventricular outflow tract was augmented with a patch. The sternum was left open at the end of the procedure and the chest was closed on post-operative day (POD) 4. The patient was discharged from hospital on POD 78 after receiving treatment for pneumonia and chylothorax.

Prenatal ultrasonic diagnosis of fetal interrupted aortic arch / 中华超声影像学杂志

Objective To evaluate the value of prenatal ultrasound in the interrupted aortic arch (IAA), and analyze the reasons of misdiagnosis and improve diagnostic ratio of this kind of defects. Methods Ultrasonic characteristics were analyzed in 16 fetuses with aortic arch anomalies, which were compared with ultrasound image and autopsy results of the normal fetus. The relativity of fetal IAA and abnormal chromosome was also analyzed. Results Among the 16 cases, 15 cases were confirmed by anatomy and the accuracy of ultrasound diagnosis was 94% (15/16), of which one case was serious constriction of aortic arch, one case was error type, three cases were obtained explicit type due to unsatisfactory results of prenatal ultrasound. The coincidence rate of ultrasonic type was 73% (11/15). All of the corrected diagnosed cases were detected with ventricular septal, of which 5 cases were detected with complete endocardial cushion defect, 9 cases were found extra cardiac malformations. Among the 7 karyotype check cases, 3 cases were diagnosed with Trisomy 18, 1 case Trisomy 13, while the rest 3 cases normal. The incidence rate of Trisomy 18 was 43 % (3/7). Conclusions Prenatal ultrasound shows a high accuracy in diagnosing fetal interrupted aortic arch (IAA ), but there’s a need to be improved in explicit typing. It is difficult to identify fetal interrupted aortic arch (IAA) and serious constriction of aortic arch (COA). The incidence rate of Trisomy 18 in the abnormal chromosome of fetal IAA is high.

Arch Reconstruction with Autologous Pulmonary Artery Patch in Interrupted Aortic Arch

Various surgical techniques have been developed for the repair of an interrupted aortic arch. However, tension and Gothic arch formation at the anastomotic site have remained major problems for these techniques: Excessive tension causes arch stenosis and left main bronchus compression, and Gothic arch configuration is related to cardiovascular complications. To resolve these problems, we adopted a modified surgical technique of distal aortic arch augmentation using an autologous main pulmonary artery patch. The descending aorta was then anastomosed to the augmented aortic arch in an end-to-side manner. Here, we report two cases of interrupted aortic arch that were repaired using this technique.

Complete Repair of Truncus Arteriosus and Interrupted Aortic Arch (Arch Reconstruction + Rastelli Operation) after Bilateral Pulmonary Artery Banding / 日本心臓血管外科学会雑誌

A baby girl delivered at 41 weeks of gestation with persistent truncus arteriosus (PTA) and interrupted aortic arch (IAA) type A was referred to our institute for surgical intervention. Bilateral pulmonary artery banding (BPAB) proceeded through a median sternotomy at the age of 11 days to control excessive pulmonary blood flow. Thereafter, she gained weight under continuous prostaglandin E1 (PGE 1) infusion. Definitive repair proceeded at the age of 2 months. Cardiopulmonary bypass was established through a redo-median sternotomy, with two arterial cannulae (brachiocephalic artery and descending aorta). The aortic arch was reconstructed with direct anastomosis. The orifice of the pulmonary artery was removed from the arterial trunk and the defect in the aortic wall was directly closed. A ventricular septal defect was closed under cardioplegic arrest via a right ventriculotomy. The continuity from the right ventricle to the pulmonary artery was made using a hand-made, extended polytetrafluoroethylene (ePTFE) conduit with a bicusp. The sternum was left open at the end of the procedure and the chest was closed on post-operative day (POD) 3. She was weaned from mechanical ventilation on POD 4 and the postoperative course was uneventful. She was discharged on POD 49.

Interrupted Aortic Arch and Aortopulmonary Window Associated with Complete Atrioventricular Septal Defect.

We report a rare case of a neonate with interrupted aortic arch, aortopulmonary window and complete atrioventricular septal defect. To the best of our knowledge, this unusual triad has not been previously described. The main question of the surgical strategy for CAVSD, in setting of associated defects, is to classify the CAVSD as balanced or unbalanced.

One-stage surgical repairs for interrupted aortic arch associated with cardiac defects in infants / 中华胸心血管外科杂志

Objective To sum up the clinical information and outcomes of one-stage surgical repairs for interrupted aortic arch (IAA) associated with cardiac defects through median stemotomy in infant.Methods From August 2005 to January 2012,23 IAA patients,aged 18 days to 3 years [mean age (8.61 ± 11.81) months],body weight 3.3-13.0 kg [mean (6.61 ± 3.26) kg] were reviewed underwent one-stage repair.There were 12 male and 11 female.The anatomic subtype ineluded type A 20 (87.0％),type B 3 (13.0％),and no type C in the records.All cases included ventricular septal defect and patent ductus artefious,some also with artirical-pulmonary window,aterical septal defect,bicuspid or unicuspid aortic valve,and subvalvular aortic stenosis.Diagnosis was determined in of the patients and suspected in by echocardiography.Also,64 layers helico-CT was employed to make a definite diagnosis for 18 patients and cardiac catheterization was used for 4 patients.All patients with cardiac anomalies underwent one-stage repair through median sternotomy.The aortic continuity was reestablished by anastomosis between the descending aortic segment and aortic arch.Results In all 23 cases,21 were successful.There was 2 (8.69％) postoperative death:one was due to surgical hemorrhage and severe low cardiac output during perioperative stage and the other was 2-month old due to crisis of pulmonary hypertension.CPB time was ranged from 53-215 min [(129.76 ± 38.98) min],and aortic crossclamp time was 34-125 min[(74.47 ± 24.30) min].The length of stay in ICU postoperatively was 96h averagely.The postoperative complications included severe low cardiac output syndrome in 3 patients,hypoxemia in 13,pneumonia in 7,and supraventricular tachycardia in 12.21 patients were followed up from 2 months to 6 years and were in good condition without recoactation.Conclusion The outcomes of early and medium term for one-stage repair of IAA and associated cardiac anomalies through median stemotomy is excellent.Technique of extended anastomosis between the descending aortic segment and aortic arch may reduce the incidence of recoarctation.It is simplified the procedure and improved life quality of patients.

Sudden decrease in end-tidal carbon-dioxide in a neonate undergoing surgery for type B interrupted aortic arch.

Interruption of the aortic arch is a rare anomaly affecting 1% of children with congenital heart disease. The systemic circulation is ductal dependent and is determined principally by the ratio of the resistances in the systemic and the pulmonary vascular bed. Any increase in the pulmonary vascular resistance may increase the dead space ventilation due to acute pulmonary hypoperfusion. We report a case where sudden decreases in the end-tidal carbon-dioxide due to pulmonary hypoperfusion mimicked accidental endotracheal tube extubation in an infant undergoing repair of interrupted aortic arch.

Single-stage repair of coarctation of aorta or interrupted arch and associated intracardiac defects through median sternotomy / 中华胸心血管外科杂志

Objedtve To summarize the experience of single-stage repair of coarctation of aorta(CoA) or interrupted aortic arch (IAA)and associated intracardiac defects through median stemotomy.Methods From Jan 2007 to Jul 2008,a total of 24 pa-tients with CoA or IAA and associated intracardisc defects were surgically repaired in single-stage through median stermotomy,inchud-ing 9 coanctation of aorta,12 coarctation with aortic arch hypoplasia,and 3 interrupted aorlic arch,.The associated intracardiac de-fects were Taussing-Bing anomaly 4,non-restricted VSD 22,subaortic stenosis 1 and pulmonary vein stenosis 1.The age ranged form 1 to 99 months (average 16 months) and the body weight ranged from 4 to 19 kg(average 9.3 kg).Aortic arch reconstruction was performed by hypothermic continuous low flow bypass using regional perfusion for all patients.Three patients with LAA and 9 patients with CoA underwent end-to-end ansetomosis.Of the 12 patients with coarctation and aortic arch hyipoplasia,8 patiellts underwent ex-tended end-to-end anastomosis,2 patients underwent end-to-side anastomosis and 2 patients underwent aortoplasfy.Results 2 cases were dead. One infant with Taussig-Bing type heart was dead of severe infection after 47 days postoperative,the other one who associ-ated with LAA and VSD dead of pulmonary hypertension crisis due to pneumonia after 15 days postoperative.No patient presented neu-rdogieal complication and renal insufficiency during the perioperation.2 cases presented recurrent respiratory problem.During the 18months follow-up,no patient presented with recoarctation except one with pressure gradient more than 20 mm Hg.Conclusion Pa-tients with coarctation of aorta or interrupted aortic arch and associsted intracardisc defects should be surgically treated as early as pos-sible when diagnosis was mode.Single-stage sortic arch reconstruction through median stemotomy using continuous regional perfusion is an effective and safe procedurd.Sufficient resection of ductus,extensive dissection of thoracic vessels and optimal tissus-tissue anas-tomosis techmique are very important for successful repair and avoiding recoarctation.

Anesthesia for noncardiac surgery in a neonate with interrupted aortic arch: A case report / 대한마취과학회지

Interrupted aortic arch (IAA) is a rare congenital cardiovascular anomaly in which there is no direct continuity between the aortic arch and the descending aorta. A patent ductus arteriosus (PDA) usually feeds the descending aorta. But, as ductus constricts, IAA is lethal in nearly 100% of the cases, if untreated. The cause of death is a combination of greatly increased left to right shunt with increased pulmonary blood flow, which results in heart failure, pulmonary edema, and sequelae of the reduced perfusion of all structures distal to the interruption. During the noncardiac surgery in such patients, adequate managements with prostaglandin infusion, careful manipulation of the pulmonary-systemic blood flow ratio, inotropic support, and aggressive treatment of acidosis should be provided to ensure optimal outcomes of subsequent surgical reconstruction. We present the anesthetic experience of abdominal surgery in a neonate with a type A IAA combined with PDA and ventricular septal defect.

Evaluation of Diagnosis and Preoperative Management of 53 Cases with Interrupted Aortic Arch in Infancy / 实用儿科临床杂志

Objective To evaluate diagnosis and preoperative management of cases with interrupted aortic arch(IAA)in infancy.Methods Fifty-three infants who were admitted to our hospital from Jan.2001 to Nov.2007 were involved.Clinical data,findings of echocardiogram(Echo),spiral CT,MRI,angiocardiography,preoperative management,surgical repair and postoperative outcome were analyzed retrospectively.Results There were 38 boys and 15 girls,weighted 2.18-10.0(4.32?1.60)kg,aged 1 day to 12(3.05?3.53)months,of which 50.94% were neonates.Symptoms at presentation were 90.57% with tachypnea and 83.02% with difficulty in feeding.Eighty three point zero two percentage cases had different degree of congestive heart failure,37.74% of which were in grade Ⅲ heart function.All cases had weakened femoral pulse.All cases were performed Echo,38 cases of them diagnosed as IAA,6 cases as IAA or severe coarctation of aorta(CoA);they were diagnosed as IAA by CT,and 9 as severe CoA who were diagnosed as IAA via CT or operation.Thirty-three cases were performed CT,of which 15 underwent surgical repair,cardiovascular abnormalities revealed by CT were the same as those in surgical findings.Three cases were taken MRI,and 7 cases were performed angiocardiography.According to the results of Echo,CT,MRI,angiocardiography and surgical findings,35 cases were type A,15 cases were type B and 3 cases were type C.Preoperative treatment included maintaining patent ductus areriosis,management of heart failure and supportive treatment.After proper preoperative management of medication,most cases with congestive heart failure were improved.Twenty-six cases underwent surgical repair,16 survived,10 died du-ring perioperative stage.Main cause of death was severe low cardiac output.Conclusions Value of Echo in diagnosis of IAA is limi-ted.Combination of Echo with CT or MRI is a convenient and safe way to diagnose IAA,it can replace the traditional method of Echo combined with angiocardiography.Proper preoperative management is helpful to patients with IAA to pass to surgical repair,and makes for successful operation.

A Case of Interrupted Aortic Arch Diagnosed by Fetal Echocardiography / 대한산부인과학회잡지

Interrupted aortic arch is a extreme type of coarctation of aorta and mostly associated with other cardiac anomalies. Unless there were no proper management, about 90% of babies would be dead in their neonatal period. A 28-year-old nulliparous woman was refered to our hospital for fetal echocardiography at 23 weeks' gestation with an abnormal prenatal ultrasonographic findings. We found interrupted aortic arch(type A), which was located in distal portion of the left subclavian artery, and ventricular septal defect. After 5 months follow up, the baby was vaginally delivered at 40 weeks' gestation. This baby was delivered at 40 weeks' gestation of a male infant with APGAR scores of 8 and 9 at 1 and 5 minutes, respectively. Neonatal echocardiography and electron beam tomography revealed interrupted aortic arch(type A), ventricular septal defect(subaortic type), patent ductus arteriosus, and atrial septal defect(ostium secondum defect). At the 10th day of birth, the infant was performed the operation of end to end anastomosis of desending aorta, patch repair of atrial septal defect, ligation of patent ductus arteriosus, and direct closure of patent foramen ovale.