Hospital Survival After Surgical Repair of Truncus Arteriosus with Interrupted Aortic Arch: Results from a Multi-institutional Database.

Truncus arteriosus (TA) is a major congenital cardiac malformation that requires surgical repair in the first few weeks of life. Interrupted aortic arch (IAA) is an associated malformation that significantly impacts the complexity of the TA operation. The aim of this study was to (1) define the comorbid conditions associated with TA and (2) determine the hospital survival and morbidity of patients with TA with and without an IAA. Data was collected from the Vizient Clinical Database/Resource Manager, formerly University HealthSystem Consortium, which encompasses more than 160 academic medical centers in the United States. The database was queried for patients admitted from 2002 to 2016 who were ≤ 4 months of age at initial admission, diagnosed with TA, and underwent complete surgical repair during that hospitalization. Of the 645 patients with TA who underwent surgery, 98 (15%) had TA with an interrupted aortic arch (TA-IAA). Both TA and TA-IAA were associated with a high prevalence of comorbidities, including DiGeorge syndrome, prematurity, and other congenital malformations. There was no difference in mortality between TA and TA-IAA (13.7-18.4%, p value = 0.227). No comorbid conditions were associated with an increased mortality in either group. However, patients with TA-IAA had a longer post-operative length of stay (LOS) compared to those without IAA (30 versus 40.3 days, p value = 0.001) and this effect was additive with each additional comorbid condition. In conclusion, the addition of IAA to TA is associated with an increased post-operative LOS, but does not increase in-hospital mortality.

Multicenter experience with endovascular treatment of aortic coarctation in adults.

OBJECTIVE: The objective of this study was to evaluate outcomes of endovascular treatment of aortic coarctation in adults. METHODS: Clinical data and imaging studies of 93 consecutive patients treated at nine institutions from 1999 to 2015 were reviewed. We included newly diagnosed aortic coarctation (NCO), recurrent coarctation, and aneurysmal/pseudoaneurysmal degeneration (ANE) after prior open surgical repair (OSR) of coarctation. Primary end points were morbidity and mortality. Secondary end points were stent patency and freedom from reintervention. RESULTS: There were 54 (58%) male and 39 (42%) female patients with a mean age of 44 ± 17 years. Thirty-two patients had NCO (mean age, 48 ± 16 years) and 61 had endovascular reinterventions after prior OSR during childhood (mean, 30 ± 17 years after initial repair), including 50 patients (54%) with recurrent coarctation and 11 (12%) with ANE. Clinical presentation included asymptomatic in 31 patients (33%), difficult to control hypertension in 42 (45%), and lower extremity claudication in 20 (22%). Endovascular treatment was performed using balloon-expandable covered stents in 47 (51%) patients, stent grafts in 36 (39%) patients, balloon-expandable uncovered stents in 9 (10%) patients, and primary angioplasty in 1 (1%) patient. Mean lesion length and diameter were 64.5 ± 50.6 mm and 19.5 ± 6.7 mm, respectively. Mean systolic pressure gradient decreased from 24.0 ± 17.5 mm Hg to 4.4 ± 7.4 mm Hg after treatment (P < .001). Complications occurred in nine (10%) patients, including aortic dissections in three (3%) patients and intraoperative ruptures in two patients; type IA endoleak, renal embolus, spinal headache, and access site hemorrhage occurred in one patient each. The aortic dissections and ruptures were treated successfully by deploying an additional covered stent proximal to the site of dissection or rupture. Two patients died within 30 days of the index procedure. After a mean follow-up of 3.2 ± 3.1 years, nearly all patients (98%) were clinically improved and all stents were patent. Reintervention was needed in 10 (11%) patients. Freedom from reintervention at 5 years was 85%. Two additional patients died during follow-up of coarctation-related causes, including rupture of an infected graft and visceral ischemia. Patient survival at 5 years was 89%. CONCLUSIONS: Endovascular repair is effective with an acceptable safety profile in the treatment of NCO and postsurgical complications of coarctation after initial OSR. Aortic rupture is an infrequent (2%) but devastating complication with high mortality. Balloon-expandable covered stents are preferred for NCO, whereas stent grafts are used for ANE. The rate of reinterventions is acceptable, with high procedural and long-term clinical success.

Surgical repair of aortic coarctation in pediatric age: A single center two decades experience.

BACKGROUND: To evaluate early and long-term results of surgical treatment of aortic coarctation (CoAo) in infants and children. METHODS: A retrospective clinical review of patients less than 18 years with CoAo, undergoing surgery between 1995 and 2015. Data were retrieved from our institutional database, to identify preoperative and postoperative characteristics. Statistical analysis was performed by bivariate, Cox's, and logistic regression analysis. RESULTS: Three hundred forty-one consecutive patients (male/female: 192/149; the median age at surgery of 25 days; interquartile range [IQR], 10-143 days) were included; 187 patients were less than 1 month (54.9%); 276 underwent extended end-to-end anastomosis (EEEA) (80.9%). Hypoplastic aortic arch (HAA) occurred in 34.6% and bicuspid aortic valve in 21.1%. The isolated type was present in 249 (73.0%). Significant postoperative complications occurred in 5.6%. Thirty-day mortality was 1.4%. At a median follow-up of 10.2 years (IQR, 6-15 years; FU completeness, 91.2%), there were eight late deaths (2.6%), most in the complex type. Among 298 survivors, 284 (95.3%) were in NYHA class I; 10 (3.0%) were on antihypertensive treatment. Reinterventions on aortic arch occurred only in 4.5%, being HAA a significant risk factor for reoperation ( P = 0.00173). Freedom from mortality and reintervention on aorta at 21 years were 93.5% and 93.6%, respectively. CONCLUSIONS: Surgical repair of CoAo by EEEA without CPBP is a safe and low-risk procedure, concerning either early or late outcomes, despite the presence of HAA and neonatal age can influence recoarctation. Most patients are clinically well in the long-term, and only a few require antihypertensive therapy.

A single centre experience with an evolving approach for the repair of coarctation of the aorta.

BACKGROUND: Isolated coarctation of the aorta can be repaired by either lateral thoracotomy or sternotomy approach with end-to-end anastomosis. Most commonly, neonates with coarctation of the aorta also have hypoplasia of the arch, requiring median sternotomy and extended end-to-side anastomosis with arch augmentation. The aim of this study was to describe our experience as the institution adopted the median sternotomy approach for repair, by reviewing complications, mortality, and reintervention. METHODS: Retrospective chart review of 66 patients aged 0-1 year who had arch repair performed by a single surgeon over an 8-year period was performed. Median age at surgery was 22 days (4-232) and median weight was 3.08 kg (1.25-8.0). Forty-one (62%) patients underwent median sternotomy. RESULTS: There was 1 death from a noncardiac cause. Eighteen per cent of our patients were ≤2.5 kg. Vocal cord paresis occurred in 16% of patients under 2.5 kg and 9.5% of patients 2.5 kg or above at the time of surgery. Hypertension at 6-month follow-up was greater in patients under 2.5 kg (44%) than patients 2.5 kg or above (15%). Total surgical reintervention rate was 6%. For patients above 2.5 kg, the surgical reintervention rate was 5.4% and for patients below 2.5 kg, the surgical reintervention rate was 8.3%. CONCLUSION: We concluded that for neonates with coarctation of the aorta and hypoplastic arch, median sternotomy is a safe surgical approach with low morbidity and mortality with the possible advantage of reduced surgical re-intervention and mortality in the population below 2.5 kg.

Outcomes of thoracic endovascular aortic repair in adult coarctation patients.

BACKGROUND: Aortic coarctation (AC) is most commonly identified in pediatric patients; however, adults can present with late sequelae of untreated coarctation or complications of prior open repair. To date, there are limited data about the role of thoracic endovascular aortic repair (TEVAR) in this group of patients. The purpose of this analysis was to describe our experience with management of adult coarctation patients using TEVAR. METHODS: All TEVAR patients treated for primary coarctation or late sequelae of previous open repair (eg, pseudoaneurysm, recurrent coarctation or anastomotic stenosis related to index open coarctation repair) were reviewed. Demographics, comorbidities, procedure-related variables, postoperative outcomes, and reintervention were recorded. Computed tomography centerline assessments of endograft morphology were completed to delineate stent anatomy at the coarctation site. Survival and reintervention were estimated using life-table analysis. RESULTS: A total of 21 patients were identified (median age, 46 years [range, 33-71 years]; 67% male [n = 14]). Nine patients (43%) were treated for symptomatic primary (n = 6) or recurrent (n = 3) coarctation. Other indications included degenerative thoracic aneurysm (n = 6), pseudoaneurysm (n = 4), and dissection (n = 2). Technical success was 100% (95% confidence interval [CI], 84%-100%). No 30-day mortality or paraplegia events occurred; however, two patients (10%) experienced postoperative nondisabling stroke. In primary or recurrent coarctation patients with available computed tomography imaging (n = 8 of 9), nominal stent graft diameters were achieved proximal and distal to the coarctation (range, -0.4 to -1.2 mm of desired final stent diameter). Specific to the coarctation site, there was a significant increase in aortic diameter after TEVAR (before stenting, 11.5 [95% CI, 6.8-12.3] mm; after stenting, 15 [95% CI, 13.7-15.7] mm; P = .004). Concurrently, systolic arterial blood pressure at time of discharge was significantly lower (before stenting: 147 mm Hg; 95% CI, 137-157 mm Hg; after stenting: 124 mm Hg; 95% CI, 118-134 mm Hg; P = .02). For all patients, median clinical follow-up time was 8 months (interquartile range, 3-13 months; range, 1-106 months). Three endoleaks were detected, all of which were type II related to left (n = 2) or aberrant (n = 1) subclavian arteries. Four patients (19%) underwent reintervention (median time, 7 months; range, 2-12 months), with three of four being subclavian artery embolization; one was an aortic root replacement for ascending aneurysm with bicuspid aortic valve. One-year freedom from reintervention was 78% ± 9% (95% CI, 42%-92%). The 1- and 3-year survival was 95% ± 5% (95% CI, 71%-99%). One late death was related to complications from pre-existing congenital heart disease. CONCLUSIONS: Adult AC patients can be treated safely with TEVAR, and the annular constriction of an AC can be successfully dilated by the stent graft. Given these findings, a greater number of patients with longer term follow-up is warranted to further define the role of TEVAR in the management of adult AC patients.

Postnatal outcome of fetal isolated ventricular size disproportion in the absence of aortic coarctation.

OBJECTIVE: Cardiac ventricular size disproportion is a marker for aortic coarctation (CoA) in fetal life, but approximately 50% of fetuses do not have CoA after birth. The aim of this study was to evaluate the postnatal outcome of cases with fetal ventricular size disproportion in the absence of CoA after birth. METHODS: All cases with fetal isolated ventricular size disproportion diagnosed between 2002 and 2015 were extracted from a prenatal congenital heart defects regional registry. Cases were stratified according to presence or absence (non-CoA) of aortic arch anomalies after birth. Postnatal outcome of non-CoA cases was evaluated by assessing the presence of cardiac and other congenital malformations, genetic syndromes and other morbidity after birth. Non-CoA cases were further classified according to whether they had cardiovascular pathology requiring medication or intervention. RESULTS: Seventy-seven cases with fetal ventricular size disproportion were identified, of which 46 (60%) did not have CoA after birth. Of these, 35 did not require cardiovascular intervention or medication, whereas 11 did. Of the 46 non-CoA cases, six presented with clinical pulmonary hypertension requiring treatment after birth, cardiac defects were present in 24 cases and syndromic features were seen in four. Overall, 43% of all non-CoA children were still under surveillance at the end of the study period. CONCLUSIONS: The postnatal course of cases with fetal ventricular size disproportion is complicated by prenatally undetected congenital defects (46%) and pulmonary or transition problems (35%) in a significant number of cases that do not develop CoA. Proper monitoring of these cases is therefore warranted and it is advisable to incorporate the risks for additional morbidity and neonatal complications in prenatal counseling. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Adverse outcome of coarctation stenting in patients with Turner syndrome.

OBJECTIVES: This study examines the outcome and procedural outcomes of percutaneous stent angioplasty for aortic coarctation in patients with Turner syndrome (TS). BACKGROUND: TS occurs in 1 in 2,500 live-born females and is associated with aortic coarctation. METHODS: In this multicenter, retrospective cohort study, all patients with TS and a coarctation of the aorta, treated with percutaneous stent implantation were included. The procedural strategies were dictated by local protocols. Adverse events at short- and long-term follow-up and qualitative parameters concerning the stent implantation were assessed. RESULTS: In the largest study to date of TS patients receiving aortic stents, a total of 19 patients from 10 centers were included. Twelve patients were treated for native and 7 for recurrent coarctation. Age at intervention was 16.9 (7-60) years (median; min-max). The coarctation diameter increased significantly from 8.0 mm (2-12) pre-intervention to 15.0 mm (10-19) post-intervention (P < 0.001). Three (15.8%) adverse events occurred within 30 days of the procedure, including two dissections despite the use of covered stents, one resulting in death. At long-term follow-up (6.5 years, min-max: 1-16), two additional deaths occurred not known to be stent-related. CONCLUSIONS: Though percutaneous treatment of aortic coarctation in TS patients is effective, it is associated with serious morbidity and mortality. These risks suggest that alternative treatment options should be carefully weighed against percutaneous stenting strategies. © 2016 Wiley Periodicals, Inc.

Aortic Arch Reconstruction in Neonates with Biventricular Morphology: Increased Risk for Development of Recoarctation by Use of Autologous Pericardium.

BACKGROUND: The aim of this study was to analyze risk factors promoting development of recoarctation (Re-CoA) in neonates who survived aortic arch repair from an anterior approach. METHODS: Fifty consecutive neonates with biventricular morphology and ductal-dependent lower body perfusion who were discharged home following aortic arch repair with cardiopulmonary bypass between 2000 and 2012 were retrospectively reviewed. Arch anatomy was either interruption (n = 10) or hypoplasia with coarctation (n = 40). Aortic arch reconstruction was performed by using patch material (bovine pericardium, n = 30, homograft, n = 10, or glutaraldehyde-treated autologous pericardium, n = 7), and three patients underwent direct end-to-side anastomosis. Antegrade cerebral and continuous myocardial perfusion was performed in 39 and 21 patients, respectively. Kaplan-Meier freedom from Re-CoA was calculated. Morphologic and perioperative data indicating increased risk of Re-CoA by univariate analysis were included in multivariate Cox regression analysis. RESULTS: Mean follow-up was 5.3 ± 4.1 years. Re-CoA occurred in 13 patients and was treated successfully by balloon dilatation (n = 6) or surgery (n = 7). Freedom from Re-CoA after 1 and 5 years was 83 ± 5 and 79 ± 6%, respectively. Two patients died early after surgical repair of Re-CoA. The use of autologous pericardium for aortic arch augmentation was the only independent risk factor for development of Re-CoA (hazard ratio: 4.3 [95% confidence interval: 1.2-16.1]; p = 0.028). CONCLUSION: Re-CoA following neonatal aortic arch surgery can be treated by balloon dilatation or surgery, if adequate. In this study, the risk for development of Re-CoA was independently increased by the use of autologous pericardium during initial arch repair.

Intervention for recoarctation in the single ventricle reconstruction trial: incidence, risk, and outcomes.

BACKGROUND: Recoarctation after the Norwood procedure increases risk for mortality. The Single Ventricle Reconstruction (SVR) trial randomized subjects with a single right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt or a right ventricle-pulmonary artery shunt. We sought to determine the incidence of recoarctation, risk factors, and outcomes in the SVR trial. METHODS AND RESULTS: Recoarctation was defined by intervention, either catheter based or surgical. Univariate analysis and multivariable Cox proportional hazard models were performed with adjustment for center. Of the 549 SVR subjects, 97 (18%) underwent 131 interventions (92 balloon aortoplasty, 39 surgical) for recoarctation at a median age of 4.9 months (range, 1.1-10.5 months). Intervention typically occurred at pre-stage II catheterization (n=71, 54%) or at stage II surgery (n=38, 29%). In multivariable analysis, recoarctation was associated with the shunt type in place at the end of the Norwood procedure (hazard ratio, 2.0 for right ventricle-pulmonary artery shunt versus modified Blalock-Taussig shunt; P=0.02), and Norwood discharge peak echo-Doppler arch gradient (hazard ratio, 1.07 per 1 mm Hg; P<0.01). Subjects with recoarctation demonstrated comorbidities at pre-stage II evaluation, including higher pulmonary arterial pressures (15.4±3.0 versus 14.5±3.5 mm Hg; P=0.05), higher pulmonary vascular resistance (2.6±1.6 versus 2.0±1.0 Wood units·m(2); P=0.04), and increased echocardiographic volumes (end-diastolic volume, 126±39 versus 112±33 mL/BSA(1.3), where BSA is body surface area; P=0.02). There was no difference in 12-month postrandomization transplantation-free survival between those with and without recoarctation (P=0.14). CONCLUSIONS: Recoarctation is common after Norwood and contributes to pre-stage II comorbidities. Although with intervention there is no associated increase in 1-year transplantation/mortality, further evaluation is warranted to evaluate the effects of associated morbidities.

Balloon angioplasty of recoarctation of the neoaortic arch after the Norwood operation: factors affecting outcome and recurrence.

BACKGROUND: Balloon angioplasty (BA) is effective in relieving neoaorta recoarctation (reCoa) after the Norwood procedure. However, recurrence is not uncommon and risk factors for success and recurrence require further elucidation. We report the results of BA for reCoa following the Norwood procedure. We examine acute results and risk factors associated with success and recurrence after BA. METHODS: Patients who underwent BA between November 2000 and June 2010 were studied. Factors for immediate success and recurrence after BA were determined using logistic regression. Recurrence-free survival was evaluated using the Kaplan-Meier curve. RESULTS: Forty-seven angioplasties were performed in 39 patients. Mean age at catheterization was 5.0 ± 4.1 months. BA was successful in 40 angioplasties (85.1%). A higher preprocedure gradient across the coarctation (P = 0.04) and a higher ratio of balloon to descending aorta (P = 0.01) were associated with success. Six patients required redilation. Risk factors for recurrence included older age at Norwood (P = 0.02), younger age (P = 0.03), lower weight (P = 0.04) and smaller body surface area at balloon angioplasty (BA) (P = 0.04), and shorter duration between surgery and angioplasty (P = 0.03). Freedom from recurrence from the first catheterization was 82% after 6 months and 78% after 1 year. There were no neurologic sequelae or deaths. CONCLUSIONS: BA is effective acutely and long term with limited morbidity and mortality. Recurrence occurs usually within the first year. Delay in performing the initial angioplasty may be beneficial in reducing the risk of recurrence, but further study is needed.

The neonatal hypoplastic aortic arch: decisions and more decisions.

Neonatal patients with hypoplasia of the aortic arch constitute a heterogeneous group with a wide spectrum of severity. The milder end of the spectrum comprises patients with aortic coarctation and isthmus hypoplasia. At the other end of the spectrum are patients with severe transverse arch hypoplasia or hypoplastic left heart syndrome. The aim of this paper is to discuss the various strategies and surgical approaches available for this group of patients, focusing on the surgical decisions that influence individual patient management. Many of the things discussed are applicable to any neonatal arch problem. We also describe and discuss in detail our surgical technique for patients who undergo neonatal repair of a hypoplastic aortic arch via median sternotomy.

Ascending-to-descending bypass for simultaneous surgery of aortic coarctation with other cardiac pathologies.

BACKGROUND: The appropriate approach for aortic coarctation associated with other cardiac diseases necessitating surgery is still controversial. The aim of this study was to evaluate the results after simultaneous surgery performed via median sternotomy and consisting of extra-anatomical ascending-to-descending aortic bypass and various other cardiac procedures. METHODS: Between January 1999 and February 2009, 13 consecutive patients with aortic coarctation coexistent with other cardiac diseases necessitating surgery underwent simultaneous surgery via median sternotomy. An extra-anatomical ascending-to-descending aortic bypass for coarctation repair was performed in all patients accompanied by various cardiac procedures (5 aortic root and valve replacement; 2 aortic valve replacement; 2 coronary artery bypass grafting; 2 mitral valve repair; 1 aortic valve replacement and coronary artery bypass grafting; 1 mitral and tricuspid valve repair). There were 3 women and 10 men with a mean age of 52 years (range 25-69). Two patients had recurrent or residual coarctation 37 and 46 years after previous surgical repair, respectively. RESULTS: Early mortality was 0 and there was only 1 late death during the follow-up of up to 11 years. New York Heart Association (NYHA) functional class improved on average from 2.4 to 1.2. At the last follow-up, blood pressure measured at the upper and lower extremities showed no gradient in any patient, indicating a durable function of the extra-anatomical bypass. Only 3 patients were on reduced antihypertensive therapy; 8 patients were on the same medication and 1 patient required increased medication therapy compared with the medication prior to surgery. CONCLUSIONS: Ascending-to-descending bypass can be performed via median sternotomy simultaneously with various cardiac procedures without considerable extension of the procedure. The operative and long-term results are excellent, and this approach can be recommended as the procedure of choice in patients with aortic coarctation and additional cardiac diseases necessitating surgery.

Subclavian flap aortoplasty and preservation of left upper extremity circulation using an interposition graft.

OBJECTIVES: To introduce a surgical technique to maintain left upper limb blood flow after subclavian flap aortoplasty (SFA). METHODS: Five patients (9 to 23 months of age) with a diagnosis of long-segment aortic coarctation underwent conventional SFA. A Gore-tex graft was interposed between the stump and the proximal descending aorta to maintain perfusion of subclavian artery. RESULTS: All patients had a patent Gore-tex graft and normal blood flow of the subclavian artery and left upper limb. One patient expired and four others were discharged with a mean follow-up of 48 months. On follow-up all patients had normal development of the left upper limb and no signs of limb ischemia. Echo findings revealed normal arch flow with normal flow in the Gore-tex graft and left upper extremity. CONCLUSIONS: Interposing a Gore-tex graft between the subclavian artery stump and proximal descending aorta concomitant with SFA can be safely performed in infants with long-segment aortic coarctation, with preservation of left upper extremity circulation.

Late outcomes of reintervention on the descending aorta after repair of aortic coarctation.

BACKGROUND: After repair of aortic coarctation, patients may develop restenosis, aneurysms, and pseudoaneurysms at the site of prior repair. We assessed the outcomes of late reintervention on the descending aorta after aortic coarctation repair. METHODS AND RESULTS: From March 1954 to July 2008, 130 patients had operations or endovascular procedures on the descending aorta after previous coarctation repair. We excluded patients who had complex left-sided cardiac lesions or interrupted aortic arch. Mean age at reintervention was 32±24 years and 28% were female. The interval between coarctation repair and reintervention was 17±13 years. Seventy-four percent of patients had hypertension. Reasons for reintervention were restenosis (n=122 [94%]), aneurysm (n=4 [3%]), and pseudoaneurysm (n=4 [3%]). Ninety-five patients (73%) underwent operative procedures including an extra-anatomic conduit (n=41), patch repair (n=32), interposition graft (n=14), end-end anastomosis (n=6), and subclavian flap (n=2). Thirty-five patients underwent endovascular treatment (balloon dilatation, n=22 or stenting, n=13). There was no early mortality. In the surgical group, 5 patients required early reoperation for bleeding and 5 patients had early vocal cord paralysis. One patient in the endovascular group had aortic rupture at the time of intervention requiring urgent operation. Survival was 97% at 10 years. At 5 years, freedom from a second repeat procedure on the descending aorta was 96% in the surgical group and 72% in the endovascular group (P<0.001). Five years after reintervention, fewer patients required treatment for hypertension (57% versus 74%, P<0.001) and a median of 1 antihypertensive medication was prescribed compared with a median of 2 medications preintervention. CONCLUSIONS: Operative and endovascular management of recoarctation can be performed safely with good late outcomes.

Pathology of the aortic arch in hypoplastic left heart syndrome: surgical implications.

Aortic arch reconstruction plays an important role in the success of the Norwood procedure (NP) for hypoplastic left heart syndrome (HLHS). This study investigated the cardiac specimens to determine the etiology of distal aortic arch obstruction after the NP for HLHS and to locate coarctation of the aorta in HLHS untreated by surgery. This study examined 17 cardiac specimens: 9 that had NP and 8 not treated by surgery. The findings after NP showed frequent failure to resect the coarctation segment completely and failure to extend the augmentation patch into the descending aorta. Five (62.5%) of the eight hearts not treated by surgery had significant periductal coarctation of the aorta. After NP for nine patients, three (33%) had residual coarctation of the aorta. To minimize the risk of recurrent or persistent aortic arch obstruction after NP and to improve the long- and short-term outcome, the ductal tissue and the coarctation segment encircling the aortic lumen should be resected. The distal wall incision should be extended at least 5 mm beyond the distal aspect of the ductal tissue. These steps could avoid major aortic arch obstruction, promote growth of the native aortic tissue, and avoid ventricular dysfunction.

Treatment of complex coarctation and coarctation with cardiac lesions using extra-anatomic aortic bypass.

BACKGROUND: Coarctation of the aorta with cardiac lesions or complex coarctation is a formidable challenge for cardiac surgeons. Extra-anatomic bypass allows simultaneous intracardiac repair or an alternative approach for patients with complex coarctation. METHODS: Between July 1997 and March 2008, 43 patients with coarctation of the aorta underwent extra-anatomic bypass grafting, including 10 ascending-to-descending aorta bypasses and 33 ascending aorta-to-infrarenal abdominal aorta bypasses. Forty patients had additional cardiovascular disorders and concomitant procedures performed including aortic valve replacement, mitral valve replacement, coronary artery bypass grafting, closure of ventricular septal defect and patent ductus arteriosus, ascending aorta repair, and the Bentall procedure. The other three patients had complex coarctation of the aorta, including a long-segment coarctation in two cases, and descending aortic aneurysm in one. RESULTS: Two patients died perioperatively: one due to air embolism during the cardiopulmonary bypass; one due to septic shock. There were no late deaths. Complications included laparotomy for mechanical ileus in one and re-exploration for bleeding in one case. There were no strokes or paraplegia and no grafted-related complication during follow-up period. Systolic blood pressure dropped from 160 +/- 27 mm Hg before surgery to 114 +/- 16 mm Hg postoperatively. Only two patients with mild hypertension postoperatively needed oral medicine. CONCLUSIONS: Extra-anatomic aortic bypass via median sternotomy or median sternotomy-laparotomy can be performed with low morbidity and mortality. It is a preferable single-stage approach for patients with concomitant complex coarctation and cardiovascular disorders.

Endovascular stent placement is an acceptable alternative to reoperation in selected infants with residual or recurrent aortic arch obstruction.

OBJECTIVE: To describe endovascular stent placement in infants as a technically feasible option in circumstances where surgery is considered less favorable. BACKGROUND: Endovascular stent placement has become established as a first line therapy for native coarctation of the aorta or recoarctation in older children where stents capable of expansion to adult size can be placed safely. Surgery remains the therapy of choice in infants and young children. The management of aortic arch obstruction in infants is, however, frequently complicated by complex anatomy or clinical condition that may make surgery or further surgery an unattractive option. There is little reported data and the implications thereof of transcatheter stent placement in aortic arch obstruction in infants. METHODS: Between August 2004 and November 2009, 11 patients had aortic arch obstruction treated with endovascular stent placement. The median age and weight at first stent placement was 46 days (range 3-399 days) and 4 kg (range 1.4-8 kg), respectively. In 10 patients, surgical intervention preceded transcatheter stent placement. Four had complex aortic arch obstruction and seven had recoarctation. RESULTS: Reduction in peak systolic gradient to <10 mm Hg was achieved in seven of 10 patients with an improvement in aortic artery diameter to >90% of adjacent aorta in all. The diameter of the arch obstruction increased from a median of 1.60 to 4.90 mm (P = 0.001) and the peak systolic gradient from 45 mm Hg to 8 mm Hg (P < 0.0001). Adverse events occurred in two patients one who required further surgical revision and a second who required placement of a second stent. The median follow up was 3.60 years (range 0.4-5.5 years) with two patients having died at 1.34 and 1.42 years poststent placement. Of the nine patients alive, six have since undergone further angioplasty at a median time interval of 0.77 years (range 0.17-2.76 years). Long-term complications occurred in none. CONCLUSIONS: Endovascular stent placement in infants is technically feasible with good results achievable even in small babies. It should be considered as a therapeutic option in complex cases when surgical alternatives are less favorable.

Bailout stenting for critical coarctation in premature/critical/complex/early recoarcted neonates.

BACKGROUND: Surgical repair of critical coarctation can be problematic in premature, critical, complex, or early postoperative neonates. OBJECTIVES: We aimed to review our experience with stent implantation to defer urgent surgery to an elective time. METHODS: Fifteen neonates with severe aortic coarctation: five premature-hypotrophic (1,400-2,000 g), six critical and complex cardiac malformation, four early (1 day [0-2 days]; median [range]) after surgical coarctectomy or complex arch reconstruction. Bare coronary stents (diameter 4.0 [3.5-5.0] mm; length 10 [8-16] mm) were used. Stents were removed surgically depending on clinical needs. RESULTS: Adequate aortic flow was obtained in 15 patients. The femoral artery was preserved in 13/15 patients. Two deaths occurred before stent removal and were nonprocedure related. In patients with simple stented coarctation, the stent was removed after 2.8 [0.2-5.0] months. In complex cardiac malformation, stents were finally removed 3.0 [0.2-78] months after implantation. SURGICAL TECHNIQUE: simple coarctectomy end-to-end in eight, extensive arch patch reconstruction in four. One patient is awaiting stent removal. The final maximum systolic velocity (cw-Doppler) across the aortic arch was 1.7 [1.2-2.5] m/sec. CONCLUSIONS: In premature/critical/complex neonates with severe coarctation, bailout stenting followed by early or late surgical coarctectomy appears a promising concept.

Retrospective cohort study of prenatally and postnatally diagnosed coarctation of the aorta (CoA): prenatal diagnosis improve neonatal outcome in severe CoA.

Introduction: Prenatal diagnosis of congenital heart disease (CHD) leads to improved outcome but not mortality rate. This may not be the case for coarctation of the aorta (CoA). The objective of this study is to estimate the effect of a prenatal diagnosis of CoA by comparing neonates with CoA by the time of diagnosis.Materials and methods: The study included 38 neonates with CoA diagnosed prenatally and 102 neonates diagnosed postnatally. The postnatal group was divided into two subgroups: (1) severe CoA: symptoms of CoA within the first 7 days (n = 43) and (2) mild CoA: symptoms within the 8-28th day (n = 34). The neonates diagnosed more than 28 days after delivery were excluded from the study (n = 25). Severe CoA was defined as CHD diagnosed postnatally with clinical symptoms that presented in the first week after birth. Mild CoA was defined as CHD that presented clinical symptoms later than 7 days of life.Results: Prostaglandins were initiated at lower doses (p < .001) in the prenatal group. Severe postnatal CoA was associated with more frequent Neonatal Intensive Care Unit (NICU) visits than mild postnatal CoA (p = .005). The length of hospitalization of neonates with severe postnatal CoA was 10 days longer than compared to the prenatal group, but the difference was not statistically significant. The highest mortality rate was in the severe postnatal CoA group (18.6%) which was significantly higher than the mortality rate in the prenatal group (p = .005).Conclusion: 1. Prenatal identification of fetuses at increased risk of developing CoA may reduce mortality and improve outcome only in neonates with severe CoA (symptoms of CoA within the first 7 days after birth); 2. Prenatal diagnosis of severe CoA was associated with lower prostaglandin doses and lower mortality rate.

Application of Modified Sliding Anastomosis in the Repair of Aortic Coarctation.

OBJECTIVES: To evaluate the early and midterm results of a modified sliding anastomosis technique in patients with aortic coarctation. MATERIALS AND METHODS: In this study, we reported a new repair method and compared the early and midterm outcome(s) with a conventional surgical approach for the management of patients with aortic coarctation. Forty-eight aortic coarctation patients with a narrowed segment length longer than 2 cm were operated at our department's pediatric surgical division. Excision of the coarctation and end-to-end anastomosis was carried out in twenty-five patients (control group). In contrast, a modified sliding technique was used for twenty-three cases in the observation group. Other accompanying cardiac anomalies simultaneously repaired included ventricular septal defect and patent ductus arteriosus. All patients received 1.5-10 years of postoperative echocardiographic follow-up. RESULTS: This is a retrospective study carried out between January 2005 and June 2018. The study population consisted of forty-eight patients, which included twenty-six male and twenty-two female patients, with an average age of 5.2 ± 1.9 months (range, 28 days to 1 year). There was no mortality. The operative time, the number of intercostal artery disconnection, the drainage volume, and arm-leg systolic pressure gradient postoperation were less in the observation group as compared to the control group (p < 0.05). Also, cases with an anastomotic pressure gradient exceeding 10 mmHg during follow-up were less in the observation group as compared to the control group (p < 0.05). The postoperative complications encountered were chylothorax (control group 2 cases vs. observation group 0) and pulmonary atelectasis (control group 4 cases vs. observation group 1). They all, however, recovered after conservative treatment. Three patients in the control group underwent balloon angioplasty (reintervention) postoperative 2-4 years due to an increase in the anastomotic pressure gradient (>20 mmHg). After reintervention, the anastomotic pressure gradient reduced to 14 mmHg, 15 mmHg, and 17 mmHg, respectively. CONCLUSIONS: For long segment aortic coarctation patients (longer than 2 cm), the use of the modified sliding anastomotic technique effectively helps to retain more autologous tissues, enlarge the diameter of the anastomosis, and decrease anastomotic tension and vascular injury. Therefore, this technique provides a new idea for the surgical treatment of aortic coarctations.