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Objective: Shone's complex comprises of a combination of congenital cardiac anomalies causing obstructions in the left ventricle's inflow and outflow tracts. This systematic review aims to evaluate the clinical features and surgical outcomes of Shone's complex., Methods: An electronic literature search of PubMed and Scopus was performed to identify relevant studies related to the presentation, management, and outcomes of Shone's complex. Two reviewers independently performed selection. Data on study characteristics, participant demographics, interventions, outcomes, and follow-up durations were extracted and analyzed., Results: A total of 691 papers were identified, with 18 studies included in the final analysis. The majority of the studies (n = 12) focused on the pediatric age group. The most common clinical presentations were coarctation of the aorta (n = 17) and mitral stenosis (n = 12). Surgical interventions often involved staged approaches, prioritizing outflow before inflow obstructions. Mitral valve repair was preferred over replacement due to better long-term outcomes (n = 8). Biventricular repair was recommended due to improved postoperative outcomes, but often needed reoperations. Reoperations were common, primarily due to recurrent coarctation (n = 10), subaortic stenosis (n = 8), and mitral valve dysfunction (n = 7). Pulmonary hypertension (n = 10) and arrhythmias (n = 11) were significant complications. Most patients were in modified Ross/NYHA functional class 1 on follow-up. Mortality rates ranged from 4 to 28%, with better outcomes associated with early and strategic surgical interventions., Conclusion: Early diagnosis and biventricular repair were associated with better outcomes while transplantation was often an eventuality. Standardized diagnostic criteria, long-term follow-up, and consensus guidelines are needed to improve the management of this congenital heart disease., (© 2024. The Author(s), under exclusive licence to The Japanese Association for Thoracic Surgery.)

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.

Objectives: The outcomes of the single-stage surgical repair of aortic arch hypoplasia (AAH) and/or coarctation of the aorta (CoA) associated with ventricular septal defect (VSD) remain controversial, especially in a lower middle-income country. This study reports the results of a single-stage repair protocol at our institution for AAH/CoA with VSD using selective cerebral perfusion., Methods: This retrospective study included 100 consecutive patients who underwent single-stage repair via median sternotomy using selective cerebral perfusion for AAH/CoA with VSD from July 2010 to March 2017., Results: The patients consisted of 65 males and 35 females. The median age of the patients was 67 days (range 4-2266 days); the median weight was 3.8 kg (range 2.1-15 kg). The average cardiopulmonary bypass time was 132 ± 28 min, the aortic cross-clamp time was 92 ± 23 min and the selective cerebral perfusion time was 33 ± 10 min. The survival rate of all patients was 94.7 ± 2.3%, with an in-hospital mortality of 5% and no late mortality at a median follow-up of 37 months (range 4-96 months). Four patients required reoperation due to recoarctation. The overall event-free survival rate following surgery was 87.1%. The median pressure gradient across the anastomosis at the last follow-up was 8.3 ± 2.8 mmHg. Multivariate logistic regression analysis revealed proximal aortic arch obstruction as a predictor of mortality (odds ratio = 3.8). The aortic isthmus diameter was identified as a predictor for reintervention by Cox regression (hazard ratio = 6.7)., Conclusions: Single-stage repair for AAH/CoA with VSD is safe and feasible in a developing country., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Introduction: Neonatal management of aortic coarctation with ventricular septal defect is still under debate between the one-stage full repair by sternotomy versus the staged repair of the coarctation first by thoracotomy (with or without banding the pulmonary artery) followed later by subsequent closure of the ventricular septal defect., Evidence Acquisition: The aim of this review was to synthesize the evidence in literature since 1980 for the neonatal population. A meta-analysis compared mortality between the two strategies., Evidence Synthesis: The analysis did not find a superiority of a strategy over the other regardless of the surgical era studied. Recoarctation rates of both strategies are presented and a management algorithm is suggested., Conclusions: Instead of comparing between the two strategies, a case-adapted management considering the anatomy of the ventricular septal defect and of the aortic arch is discussed to address this association of lesions though presenting with a wide range of settings.

Background: Patients with coarctation of the aorta have a high prevalence of intracranial aneurysms (IA) and suffer subarachnoid hemorrhage (SAH) at younger ages than the general population. American Heart Association/American College of Cardiology guidelines recommend IA screening, but appropriate age and interval of screening and its effectiveness remain a critical knowledge gap., Methods and Results: To evaluate the benefits and cost-effectiveness of magnetic resonance angiography screening for IA in patients with coarctation of the aorta, we developed and calibrated a Markov model to match published IA prevalence estimates. The primary outcome was the incremental cost-effectiveness ratio. Secondary outcomes included lifetime cumulative incidence of prophylactic IA treatment and mortality and SAH deaths prevented. Using a payer perspective, a lifetime horizon, and a willingness-to-pay of $150 000 per quality-adjusted life-year gained, we applied a 3% annual discounting rate to costs and effects and performed 1-way, 2-way, and probabilistic sensitivity analyses. In a simulated cohort of 10 000 patients, no screening resulted in a 10.1% lifetime incidence of SAH and 183 SAH-related deaths. Screening at ages 10, 20, and 30 years led to 978 prophylactic treatments for unruptured aneurysms, 19 procedure-related deaths, and 65 SAH-related deaths. Screening at ages 10, 20, and 30 years was cost-effective compared with screening at ages 10 and 20 years (incremental cost-effectiveness ratio $106 841/quality-adjusted life-year). Uncertainty in the outcome after aneurysm treatment and quality of life after SAH influenced the preferred screening strategy. In probabilistic sensitivity analysis, screening at ages 10, 20, and 30 years was cost-effective in 41% of simulations and at ages 10 and 20 in 59% of simulations., Conclusions: Our model supports the American Heart Association/American College of Cardiology recommendation to screen patients with coarctation of the aorta for IA and suggests screening at ages 10 and 20 or at 10, 20, and 30 years would extend life and be cost-effective.

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., Competing Interests: The authors declare that they have no competing interests., (Copyright © 2020 Wangping Chen et al.)

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.

Patients who undergo coarctation repair during infancy have excellent early survival but long-term survival is unknown. We aimed to describe the long-term survival of patients with coarctation repaired during infancy and determine predictors of mortality. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium for patients with coarctation who underwent surgical repair before 12 months of age between 1982 and 2003. Long-term transplant-free survival was obtained by linkage with the National Death Index and the Organ Sharing Procurement Network. Kaplan Meier survival plots were constructed, and univariate and multivariable analyses were performed to determine predictors of mortality. We identified 2,424 coarctation patients who met inclusion criteria. At 20 years postoperatively, 94.5% of all patients and 95.8% of those discharged after initial operation remained alive, respectively. Significant multivariable predictors of mortality included surgical weight <2.5 kg (hazard ratio [HR] 3.70, 95% confidence interval [CI] 2.19 to 6.24), presence of a genetic syndrome (HR 2.40, 95% CI 1.13 to 5.10), and repair before 1990 (HR 1.91, 95% CI 1.09 to 3.34). None of the other factors examined including age at repair, gender, coarctation type, or surgical approach were found to be statistically significant. Over half of the deaths were due to the underlying congenital heart disease or other cardiovascular etiology. Overall long-term survival of patients who undergo coarctation repair during infancy is excellent. However, patients do experience small continued survival attrition throughout early adulthood. Ongoing monitoring of this cohort is necessary to assess late mortality risk., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives: The main challenge of aortic coarctation (CoA) repair in infants is to obtain durable results without morbidity. We aimed to describe predictors of aortic arch reintervention after aortic CoA repair., Methods: Between January 2000 and March 2014, we retrospectively included consecutive infants with isolated CoA or CoA with ventricular septal defect (CoA + VSD) who had surgical repair of the aortic arch before 3 months of age., Results: Five hundred and thirty patients were included: 308 (58%) patients had isolated CoA and 222 (42%) patients had CoA + VSD. Three hundred and eighty-five patients (72.6%) had CoA repair, 51 patients (9.6%) had CoA repair with closure of VSD and 94 patients (17.8%) had CoA repair with pulmonary artery banding. Mean age at operation was 13 ± 1.6 days, with 294 patients (55.5%) operated on before 2 weeks. Median follow-up was 7.57 years. Sixty-one patients (11.5%) needed reintervention on the aortic arch. Freedom from aortic arch reintervention was 90% at 1 year and 88.5% at 5 years. Proportions of aortic arch reintervention were similar in the different surgical strategy groups (P = 0.80). However, in patients receiving prostaglandin E1 (PGE1), the end-to-end repair was at higher risk of recoarctation compared to the extended end-to-side repair (P = 0.033). The risk factors of aortic arch reintervention were age at repair <15 days (P = 0.034) and the need for PGE1 infusion at surgery (P = 0.0043)., Conclusions: CoA repair in young infants has an overall good outcome. The use of PGE1 may modify the aortic arch anatomy and mask the boundaries of the resection to be performed. PGE1 treatment should be studied more specifically in another study to improve preoperative management., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: To examine the contemporary long-term outcome after coarctation repair., Methods: This is a retrospective cohort study of 834 patients aged ≥16 years who underwent coarctation repair under single-centre follow-up. Repair was performed at a median age of 3 years (lower-upper quartile: 1 month to 15 years) by surgery in 83% (690/834) and angioplasty/stenting in 17% (144/834). Survival was compared with an age- and gender matched normal population. Other outcomes included arch reintervention, aortic valve intervention, ascending aortic intervention, and residual/re-coarctation and resting hypertension at latest follow-up., Results: After a median follow-up of 27 years (lower-upper quartile: 18-36), there were 38 late deaths (5%, 38/834). Overall survival was 99%, 88% and 65% at 30, 50 and 70 years of age, respectively, significantly reduced compared with a matched normal population (standardised mortality ratio: 3.20, log-rank: p<0.001). Thirty per cent (246/834) required ≥1 arch reintervention, 13% (111/834) an aortic valve intervention and 5% (43/834) an ascending aortic intervention. Freedom from aortic valve and ascending aortic intervention was 83% and 92% at 50 years and 53% and 81% at 70 years of age, respectively. Residual/re-coarctation (gradient ≥25 mm Hg or repair site/diaphragm ratio ≤70%) at latest follow-up was present in 60% (282/474) and resting hypertension in 57% (379/661)., Conclusions: Long-term survival in contemporary adult survivors of coarctation repair is significantly lower than a matched normal population with accelerated decline after the third decade. Nearly 60% of patients eventually develop hypertension, whereas approximately 50% require further invasive cardiovascular treatment by 50 years of age. Our risk-stratifying data may enable personalised follow-up strategies for this common congenital heart condition., Competing Interests: Competing interests: Yd’Ud is a consultant for Actelion and MSD., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

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., (© 2019 Wiley Periodicals, Inc.)

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., (Copyright © 2018 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: Surgical repair of coarctation of the aorta (CoA) is often possible through left thoracotomy and without the use of cardiopulmonary bypass. Recent studies reporting the outcome after CoA repair through left thoracotomy are limited. Therefore, the aim of this study is to evaluate the results of CoA repair through left thoracotomy in children who were operated on in our centre over the past 21 years., Methods: From January 1995 to December 2016, 292 patients younger than 18 years underwent primary CoA repair through left thoracotomy at our 2 institutions. Peri- and postoperative data and follow-up data collected from our hospital and the referring hospitals were retrospectively reviewed., Results: Median age at operation was 64 days (range 2 days-17 years). Most patients underwent the resection of the CoA followed by an (extended) end-to-end anastomosis (93%). Six patients died perioperatively and 2 more patients died during the follow-up, of which 7 patients had other major comorbidities. Actuarial survival was 97% at 5 years, 96% at 10 years and 96% at 15 years. Second arch interventions due to recoarctation were performed in 9.9% (n = 29) of patients, consisting of balloon dilatation in all but 2 patients. Recoarctation occurred significantly more often after initial repair in the neonatal period (21%) and could occur as late as 14 years after initial surgery. There were 7 re-recoarctations, and 14% of patients were on hypertensive medication during the follow-up., Conclusions: Repair of CoA through left thoracotomy is a safe procedure with low rates of mortality. The long-term follow-up is necessary due to the significant risk of recoarctation requiring reintervention.

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., (Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.)

Background: Coarctation of the aorta can be associated with hypoplasia of the proximal transverse aortic arch. One approach to manage this condition is via left thoracotomy and extended end-to-end anastomosis with the expectation that the proximal arch will grow over time. Our preferred approach is to augment the aorta via midline sternotomy. We hypothesized that this approach is safe, durable, and allows reliable growth of the aorta., Methods: We identified the records of patients with biventricular anatomy who had coarctation of the aorta, hypoplasia of the proximal transverse arch, and no other cardiac lesion that would mandate cardiopulmonary bypass use and midline sternotomy. The records of 62 such patients operated on between 2005 and 2016 were retrospectively reviewed. Patient demographics, clinical variables and outcome data were collected and analyzed using SAS 9.4. Data are presented as median (interquartile range [IQR])., Results: Sixty-two patients (23 girls [37%]) underwent repair at 10 (IQR, 5 to 21) days of life. Forty-nine (79%) patients were on prostaglandin infusion to maintain ductal patency. Fifteen (24%) patients presented in shock with end organ dysfunction, 17 (27%) were on inotropes, and 26 (42%) were mechanically ventilated. The proximal transverse arch was 41% (IQR, 34% to 47%) of the size of ascending aorta as measured by echocardiography (z-score, -5 [IQR, -5.8 to -4.3]). Following median sternotomy, repair was carried out on cardiopulmonary bypass (41 [IQR, 37 to 47] minutes). The arch was reconstructed with (n = 26 [42%]) or without (n = 36 [58%]) coarctectomy usually using homograft patch aortoplasty (n = 58 [94%]). In all but 2 patients, repair was undertaken with circulatory arrest (27 [IQR, 22 to 31] minutes). Patients were extubated 4 (IQR, 3 to 5) days later and discharged home in 12 (IQR, 8 to 18) days. There was no mortality, and 8 morbidity events (3 recurrent nerve injury, 2 chylothorax, 1 phrenic nerve injury, 1 seizure, and 1 superficial wound infection) in 7 (11%) patients. All patients are alive at 41 (IQR, 11 to 64) months of follow-up. Reintervention was required in 6 (10%) patients (5 catheter based and 3 surgical) for recurrent distal coarctation. Reintervention-free survival at 1, 3, and 5 years was 87%. Only 1 child was currently on antihypertensive therapy, and all were in New York Heart Association functional class I symptoms. At last echocardiogram, the proximal transverse arch was 97% (IQR, 84% to 103%) of the diameter of the ascending aorta (z-score, 0.8 (IQR, 0.3 to 1.3]), ejection fraction was 70% (IQR, 60% to 76%), and only 2 patients had significant left ventricular hypertrophy., Conclusions: Arch augmentation via median sternotomy is a safe and effective procedure that can be accomplished with low morbidity and mortality. The reconstructed arch retains excellent growth potential resulting in a very favorable physiologic outcome., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Several techniques have been described to correct coarctation associated with distal arch hypoplasia. However, in neonates, residual gradients are frequently encountered and influence long-term outcome. We reviewed our experience with an alternative technique of repair combining carotid-subclavian angioplasty and extended end-to-end anastomosis., Methods: From 1998 through 2014, 109 neonates (median age, 9 days) with coarctation and distal arch hypoplasia (n = 106) or type A interrupted aortic arch (n = 3) underwent repair using this technique. Thirty patients had isolated lesions (group 1), 44 associated ventricular septal defect (group 2), and 35 associated complex cardiac lesions (group 3). Median follow-up was 98 months., Results: Repair was performed via left thoracotomy in 97%. There was one procedural-related death (0.9%) and overall five patients died during index admission (4.6%). Ten deaths were recorded at follow-up. Actuarial five-year survival was 86% (100% in group 1, 91% group 2, and 66% in group 3). Recurrent coarctation (clinical or invasive gradient >20 mm Hg) developed in 15 patients, all but 2 successfully treated by balloon dilatation. Freedom from any reintervention (dilatation or surgery) at five years was 86%. Only two patients were on antihypertensive drugs at last follow-up., Conclusions: This combined technique to correct distal arch hypoplasia and isthmic coarctation results in low mortality and acceptable recurrence rate. It preserves the left subclavian artery and allows enlargement of the distal arch diameter. Late outcome is excellent with very low prevalence of late arterial hypertension.

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., (Copyright © 2017 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Today, surgical repair has almost doubled the 30-year survival rate in patients with coarctation of the aorta (CoA), and 72% to 98% of patients now reach adulthood. Possible late complications include malignant hypertension, left ventricular dysfunction, aortic valve dysfunction, recurrent CoA, and aneurysm formation with risk of rupture. Treating postoperative CoA-related aneurysms with observation alone is associated with a mortality rate of 36%, compared with 9% for surgical repair. Even in the best surgeons' hands, aortic surgery has associated complications, and the complexity of reoperative surgery makes the risks substantially greater. For patients with CoA-related aneurysm, endovascular treatment constitutes a good alternative to reoperative surgery because it poses a lower risk of morbidity and mortality. Implanting an endograft has been shown to be successful in treating CoA and related aneurysms, producing excellent intermediate outcomes and minimal morbidity and mortality. Despite evidence that using covered stents improves outcomes, the superiority of any particular stent type has yet to be established. With a variety of endografts available, the decision of which stent to use depends on anatomy, availability, and operator preference.

Objectives: The aim of our prospective randomized study was to compare modified reverse aortoplasty (MRA) and extended end-to-end anastomosis (EEA)., Methods: We have assessed the operative correction results in 54 infants with coarctation of the aorta and distal aortic arch hypoplasia who underwent primary repair in our institute between July 2013 and February 2014. All of the patients were <12 months old when they had the operation. Patients were randomly assigned to 2 arms: modified reverse subclavian flap angioplasty (MRA group; n = 27) or extended end-to-end anastomosis (EEA group; n = 27)., Results: We found a significant difference in residual arterial hypertension between the groups: 2 (7.7%) patients in the MRA group and 8 (30.8%) patients in the EEA group, respectively (P = 0.03). Risk factors for arterial hypertension were precoarctation area rigidity and endocardial fibroelastosis. During the last follow-up visit, recoarctation was observed in 1 (3.8%) patient in the MRA group and in 2 (7.7%) patients in the EEA group, respectively (P = 0.50). Cox regression analysis showed that the only risk factor for recoarctation was the patient's low weight before surgery [odds ratio (95% confidence interval) 0.016 (0.001-0.51), P = 0.047]. An aortic aneurysm developed in 2 (7.7%) patients in the MRA group; however, no significant difference was found between the groups (P = 0.15)., Conclusions: The results of surgical repair of coarctation of the aorta using modified reverse subclavian flap angioplasty and extended anastomosis were comparable in mortality rates and early postoperative complications. Nevertheless, MRA may be beneficial in reducing residual arterial hypertension at the mid-term follow-up visit., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: To investigate survival and freedom from reintervention after aortic coarctation repair in Maltese patients and to compare cardiovascular mortality in coarctation repair survivors with that in the general population., Design: All 72 aortic coarctation patients with any type of repair, born by end-1997 and logged in the local database were included. Trends in timing and type of repair were determined by comparing patients born before and after 1985. Kaplan-Meier analyses of survival and reintervention-free survival were performed on the 59 repair survivors with complete follow-up data (mean follow-up 26.13 ± 9.62 (range 1.05-44.55 years). Cardiovascular mortality in repair survivors was compared with that in 438 age- and sex-matched general Maltese controls., Results: Patients born after 1985 underwent repair at a younger age (median age 0.18 vs 13.96 years; P < .001), with less patch aortoplasties in favor of end-to-end anastomosis or transcatheter stenting. Among the 59 long-term follow-up patients, there were 7 cardiovascular deaths and 10 patients needed reintervention. Estimated mean survival was 40.33 years (95% CI 37.71, 42.95) with a survival rate of 67.5% at 40 years from repair. Estimated mean reintervention-free survival was 38.13 years (95% CI 34.52, 41.75) with freedom from reintervention rate of 77% at 30 years. Patients repaired aged <10 years required earlier reintervention (estimated mean reintervention-free survival 35.12 years (95% CI 29.54, 40.71) vs 40.80 years (95% CI 37.16, 44.37); P = .04). There was an excess of cardiovascular deaths among repaired coarctation subjects compared to the general population (11.9% vs 1.4%; P < .001) and survival in coarctation patients was significantly lower (67.90 years (95% CI 60.28, 75.52) vs 85.78 years (95% CI 83.12, 88.44); P < .001)., Conclusions: Despite earlier diagnosis and repair, contemporary coarctation repair survivors remain at increased risk of cardiovascular death. An important proportion require repair site reintervention. Specialist follow-up and aggressive cardiovascular risk factor management are mandatory to improve long-term outcomes., (© 2017 Wiley Periodicals, Inc.)

Objectives: Coarctation of the aorta (CoA) in adolescents and adults is often complicated by other cardiac or aortic problems and may carry additional surgical difficulties. Limited studies have reported the surgical outcomes of CoA repair in this particular patient population. We reviewed our contemporary experience of open surgical management of CoA in adolescents and adults., Methods: From 2008 to 2016, a total of 60 adolescents and adults (mean age, 32 years) underwent CoA repair at our centre. Of this cohort, 43 patients only underwent CoA repair (isolated group), while the remaining 17 underwent other concomitant cardiac procedures (concomitant group). Ascending-to-descending aortic bypass was the preferred repair technique in the concomitant group. Aortic valve replacement and a Bentall/David procedure were the two most frequently combined procedures., Results: There were no in-hospital deaths in the isolated group compared with three in the concomitant group (0% vs 17.6%, P  = 0.029). The median intensive care unit stay (1 vs 2 days, P <0.01) and postoperative hospital stay (6 vs 9 days, P <0.01) were significantly longer in the concomitant group. Postoperative respiratory failure occurred more frequently in the concomitant group (0% vs 17.6%, P  = 0.029). The isolated group had better overall survival during follow-up ( P  = 0.037). The 5-year overall survival rate was 98% in the isolated group and 82% in the concomitant group., Conclusions: Surgical management of coarctation in adolescents and adults can achieve similar satisfactory outcomes as their neonatal counterparts, but the complexity of the concomitant procedures is a risk factor for this particular patient population., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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., (© 2016 Wiley Periodicals, Inc.)

Objective: Long-term sequelae and events after coarctation repair are well described. However, the predictive value of variables from clinical follow-up investigation for late events and survival has rarely been investigated., Methods: All patients who participated in the prospective cross-sectional COALA Study in 2000 with a structural clinical investigation including blood pressure measurement and symptom-limited exercise test were contacted for reevaluation of survival, current clinical status and major cardiovascular events., Results: Of 273 eligible patients, 209 were available for follow-up. Nine patients had died at a median age of 46years (range 30-64years), five of them due to cardiovascular complications. Late mortality after surgical intervention was 5.7% with a median age of 41years (range 16-64years). Twenty-five patients had a major cardiovascular event: 12 had procedures at the aortic valve or aortic arch, 8 had procedures for restenosis, 2 had endocarditis, 2 had a cerebrovascular insult and 1 an aortic dissection. The presence of bicuspid aortic valve (p=0.009), brachial-ankle blood pressure gradient >20mmHg (p<0.001) and reduced left ventricular function (p=0.002) correlated with major cardiovascular events., Conclusion: Surgical correction of coarctation of the aorta shows fairly low mortality in the long-term follow-up. Late morbidities include recoarctation, but also the consequences of the hemodynamics produced by a congenital bicuspid aortic valve, presence of which is predictive for aortic valve procedures: however the predictive value of clinical variables is limited., (Copyright © 2016. Published by Elsevier Ireland Ltd.)

Background: Left-sided cardiac obstructions represent 15% of congenital heart disease (CHD). The treatment in adults is surgical; however, balloon dilation by interventional catheterization can alleviate the symptoms in pediatric patients to allow them to reach the target height. The aim was to determine the survival and the factors associated with reintervention in patients with CHD with left-sided obstruction treated with balloon angioplasty., Methods: A cohort study was conducted in patients aged 4 to 17 years with left-sided heart obstruction (valvular stenosis [VS], supravalvular aortic stenosis [SAS], coarctation of the aorta [CA]) successfully treated with balloon angioplasty. The follow-up was of 10 years and the outcome variable was the restenosis with reintervention criteria. Pediatric stage at the time of the procedure, nutritional status, residual gradient, and presence of genetic syndromes were considered prognostic variables. For statistical analysis, measures of central tendency and dispersion were used. Chi squared was employed in qualitative variables and Kruskal-Wallis in quantitative variables., Results: We had a total of 110 patients: 40% had CA, 35% VS, and 25% SAS. 39% required reintervention: 80% in SAS, 35% in CA, and 14% in VS., Conclusion: The intervention balloon is a stopgap measure that allows patients with left-sided obstructions to reach the target height.

Objectives: Coarctation with hypoplastic aortic arch can be treated with resection and extended end-to-end anastomosis (REEEA) as well as end-to-side anastomosis (ESA). The aim of the study was to review our experience with these techniques in newborns and infants and to assess mid-term outcome with regards to morbidity, mortality, and reintervention rate in relation to operative access and technique., Patients and Methods: Retrospective review of hospital charts and surgical reports from 183 consecutive newborns and infants with coarctation and hypoplastic aortic arch with or without ventricular septal defect between 1996 and 2013. Median age at surgery was 15 days (0-345). Lateral thoracotomy was used as operative access in 111 patients; 72 patients had a median sternotomy, 71 of them with cardiopulmonary bypass (ESA n = 30, REEEA n = 41). Fifty-two patients (28.4%) had an additional ventricular septal defect closure. Follow-up data were available for 75.96% with a median follow-up of 6.3 years (0.2-18.16 years)., Results: Thirty-day mortality was 0.54% with no late mortality occurring during follow-up. There was 1 severe complication: paraplegia and cerebral hypoxemia after REEEA. Freedom from mortality and reintervention at 10 years was 99.27% and 90.12%, respectively. Lateral thoracotomy as operative access was a risk factor for recurrent obstruction (P = .03)., Conclusions: REEEA and ESA were safe and effective treatments in newborns and infants. In borderline cases, aortic arch reconstruction should be performed through a median sternotomy on bypass., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: Aortic coarctation presenting during adult life most frequently represents cases of re-coarctation, following previous transcatheter or surgical therapy, or missed cases of native coarctation. In the area of evolving endovascular therapy, we believe that there is still a place for durable open repair by means of resection and interposition grafting. We, therefore, evaluated our results in adult patients with primary aortic coarctation or complications of a previous coarctation repair., Methods: A total of 38 patients were operated between 1989 and 2014. Median age was 43 years (range 18-69 years), and 20 were male (52.6%). Seventeen patients (44.7%) had recurrent coarctation or dilatation after previous repair during childhood; the remaining 21 (55.3%) had primary coarctation diagnosed at adult age. Data were retrospectively reviewed and analysed for indications, type of repair, operative details and outcomes., Results: Resection and interposition grafting was performed primarily with the use of left-left bypass (mean cross-clamping time 41 ± 13 min). There were no in-hospital deaths, stroke, spinal cord ischaemia, renal or respiratory failure. No patient had evidence of symptomatic aortic re-coarctation or pseudoaneurysm formation on follow-up. Long-term survival after 20 years was 94.7%., Conclusions: Open surgical repair of primary, recurrent or complicated adult aortic coarctation by interposition grafting is a safe and feasible therapeutic option, providing durable long-term results and excellent long-term survival., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Coarctation of the aorta (CoA), a juxtaductal obstructive lesion in the descending aorta and commonly associated with hypoplasia of the aortic arch occurs in 5-8% of patients with congenital heart disease. Since the initial surgical corrections in the 1950, surgical and transcatheter options have constantly evolved. Nowadays, transcatheter options are widely accepted as the initial treatment of choice in adults presenting with native or recurrent CoA. Surgical techniques are mainly reserved for patients with complex aortic arch anatomy such as extended arch hypoplasia or stenosis or para-CoA aneurysm formation. Extended aneurysms can be covered by conformable stents but stent implantation may require preparative vascular surgery. Complex re-CoA my best be treated by an ascending to descending bypass conduit. The following review aims to describe current endovascular and surgical practice pointing out modern developments and their limitations.

Background: Coarctation of the aorta is a congenital narrowing of the upper descending aorta. The approximate incidence is 4% in live-born children with congenital heart disease. This study aimed to describe the surgical outcome and survival of patients undergoing congenital aortic coarctation repair via subclavian flap aortoplasty (group 1) or resection with end-to-end anastomosis (group 2)., Methods: We retrospectively reviewed the clinical outcomes of 105 infants who underwent repair of aortic coarctation between 2000 and December 2012. Fifty patients (group 1) underwent subclavian flap aortoplasty and 55 (group 2) underwent resection with end-to-end anastomosis. Procedure details and early results were collected by retrospective review of hospital and clinic data., Results: The mean age of patients in group 1 was 6.73 ± 1.1 vs. 6.76 ± 1.2 months in group 2, and the mean weight was 6.01 ± 1.3 vs. 5.9 ± 1.0 kg, respectively. There were no significant differences among the intra- and postoperative variables in the 2 groups. Six patients in group 1 had a peak systolic gradient >20 mm Hg. The recurrence rate in group 1 was 12% vs. 1.8% in group 2 (p < 0.05). Overall mortality was 2.8%. Survival in group 1 was 96% vs. 98.2% in group 2., Conclusion: Repair of aortic coarctation in infancy by resection with end-to-end anastomosis can be performed with a low mortality rate and a low incidence of recoarctation, and it provides the optimal prognosis for coarctation in infancy., (© The Author(s) 2016.)

Background: There is no systematic assessment of available evidence on effectiveness and comparative effectiveness of balloon dilatation and stenting for aortic coarctation., Methods and Results: We systematically searched 4 online databases to identify and select relevant studies of balloon dilatation and stenting for aortic coarctation based on a priori criteria (PROSPERO 2014:CRD42014014418). We quantitatively synthesized results for each intervention from single-arm studies and obtained pooled estimates for relative effectiveness from pairwise and network meta-analysis of comparative studies. Our primary analysis included 15 stenting (423 participants) and 12 balloon dilatation studies (361 participants), including patients ≥10 years of age. Post-treatment blood pressure gradient reduction to ≤20 and ≤10 mm Hg was achieved in 89.5% (95% confidence interval, 83.7-95.3) and 66.5% (44.1-88.9%) of patients undergoing balloon dilatation, and in 99.5% (97.5-100.0%) and 93.8% (88.5-99.1%) of patients undergoing stenting, respectively. Odds of achieving ≤20 mm Hg were lower with balloon dilatation as compared with stenting (odds ratio, 0.105 [0.010-0.886]). Thirty-day survival rates were comparable. Numerically more patients undergoing balloon dilatation experienced severe complications during admission (6.4% [2.6-10.2%]) compared with stenting (2.6% [0.5-4.7%]). This was supported by meta-analysis of head-to-head studies (odds ratio, 9.617 [2.654-34.845]) and network meta-analysis (odds ratio, 16.23, 95% credible interval: 4.27-62.77) in a secondary analysis in patients ≥1 month of age, including 57 stenting (3397 participants) and 62 balloon dilatation studies (4331 participants)., Conclusions: Despite the limitations of the evidence base consisting predominantly of single-arm studies, our review indicates that stenting achieves superior immediate relief of a relevant pressure gradient compared with balloon dilatation., (© 2016 American Heart Association, Inc.)

Congenital heart disease (CHD) is the most common birth defect, affecting about 0.8% of live births. Advances in recent decades have allowed >85% of children with CHD to survive to adulthood, creating a growing population of adults with CHD. Little information exists regarding survival, demographics, late outcomes, and comorbidities in this emerging group, and multiple barriers impede research in adult CHD. The National Heart, Lung, and Blood Institute and the Adult Congenital Heart Association convened a multidisciplinary working group to identify high-impact research questions in adult CHD. This report summarizes the meeting discussions in the broad areas of CHD-related heart failure, vascular disease, and multisystem complications. High-priority subtopics identified included heart failure in tetralogy of Fallot, mechanical circulatory support/transplantation, sudden cardiac death, vascular outcomes in coarctation of the aorta, late outcomes in single-ventricle disease, cognitive and psychiatric issues, and pregnancy., (Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Aims: Stent placement as treatment for coarctation of the aorta (CoA) has become a more common choice in the last 20 years. Clinical results of CoA stenting are usually reported in small retrospective case series. This systematic review provides an overview of clinical experience with stenting for CoA., Methods and Results: A systematic review of the reports published between January 1990 and December 2014 after stenting a CoA was performed with a focus on relief of obstruction and lowering of blood pressure. Study and patient characteristics were extracted, as well as pre- and post-stenting aortic diameter in mm, systolic pressure gradient (SPG) and pre- and post-stenting systolic blood pressure (mmHg), periprocedural and follow-up complications. Forty-five articles met the inclusion criteria. Three outcomes were extracted from the articles - aortic diameter, systolic pressure gradient and blood pressure. Diameter increased from 6.4 mm (5.6, 7.3) to 15.1 mm (14.5, 15.7), pressure gradients decreased from 40 mmHg (35, 42) to 4 mmHg (3, 5) and systolic blood pressure decreased from 153 mmHg (148, 158) to 132 mmHg (127, 136). Stent migration was the most common periprocedural complication (2.4%), and mortality was low (0.4%)., Conclusions: Stenting is an effective treatment with regard to immediate relief of obstruction and direct lowering effect on blood pressure. However, there is a lack of evidence regarding late effectiveness concerning durable blood pressure lowering, and limited information on periprocedural and late complications. This observation calls for a systematic and longer prospective follow-up of patients after CoA stenting.

Objectives: This study sought to determine whether several anatomic or evolving characteristics of the coarctation may create challenging conditions for treatment., Background: Stent repair of coarctation of aorta is an alternative to surgical correction., Methods: We analyzed our 21-year experience in the percutaneous treatment of complex coarctation of aorta. Adverse conditions for treatment were as follow: 1) complete interruption of the aortic arch (n = 11); 2) associated aneurysm (n = 18); 3) complex stenosis (n = 30); and 4) the need for re-expansion and/or restenting (n = 21). Twenty patients (33%) belonged to more than 1 group. Ten interruptions were type A and 1 was type B. The mean length of the interrupted aorta was 9 ± 11 mm. The associated aneurysms were native in 8 patients and after previous intervention in 10 patients. Aneurysm shapes were fusiform in 8 patients and saccular in 10. The following characteristics defined complex stenosis as long diffuse stenosis, very tortuous coarctation, or stenosis involving a main branch or an unusual location. Patients previously stented at an early age, required re-expansion and/or restenting after reaching 16 ± 5 years of age., Results: Two patients had died by 1-month follow-up. The remaining 58 patients did well and were followed-up for a mean period of 10 ± 6 years. Late adverse events occurred in 3 patients (5%). All remaining patients are symptom-free, with normal baseline blood pressure. Imaging techniques revealed good patency at follow-up without associated aneurysm or restenosis. The actuarial survival free probability of all complex patients at 15 years was 92%., Conclusions: Stent repair of complex coarctation of aorta is feasible and safe. Initial results are maintained at later follow-up., (Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Aims: Previous cohort studies of patients with coarctation of the aorta (CoA) demonstrate reduced long-term survival. Improved surgical outcomes in children and evolution of adult congenital heart disease (ACHD) services have resulted in improved survival in patients with other CHDs. We hypothesise that for young adult patients with CoA long-term outcomes have improved in the contemporary era., Methods: 151 patients (58% men) with simple CoA followed up at a tertiary ACHD service in Sydney, Australia, from 1993 to 2013 were included. We documented mortality and major morbidity such as the need for re-intervention for re-coarctation or aneurysms., Results: 140 patients (mean age 35±15 years) underwent CoA repair at median age of 5 (IQR 0-10) years. Initial surgical strategy included end-to-end repair in 43, subclavian flap aortoplasty in 28 and patch aortoplasty in 31 patients (and was not documented in 28 cases). 6 patients had endovascular repair, 4 had interposition tube grafts and 11 were unrepaired. There were a total of seven deaths at a median age of 60 years. Actuarial survival was 98% at 40, 98% at 50 and 89% at 60 years of age. Re-coarctation occurred in 34% and descending aortic aneurysms were noted in 18%. Patients with end-to-end repair had lower rates of significant re-coarctation or descending aortic aneurysms (p=0.026 and <0.001, respectively). 66% had bicuspid aortic valve and 44% were hypertensive., Conclusions: Patients with CoA who reach adolescence demonstrate very good long-term survival up to age 60 years. Long-term morbidity is common, however, related largely to aortic complications and late hypertension., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

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., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: The surgical mortality associated with repair of coarctation of the aorta (CoA) over a 25-year period was examined. Risk factors for discharge mortality were evaluated as well as the surgical techniques and its evolution over the period studied., Methods: Utilizing the pediatric cardiac care consortium, we conducted a retrospective review of patients less than 18 years of age submitted between 1982 and 2007. Variables reviewed included weight at birth, age and weight at the operation, type of coarctation repair, associated cardiac anomalies, year of repair, center-specific volume, postoperative length of stay, and in-hospital mortality., Results: There were 7,860 patients submitted with the procedural code for repair of CoA. Forty-five percent underwent repair within 30 days of life (n = 3,549), including 1,444 patients who were operated upon within the first 7 days of life (18% of all patients). Seventy percent (n = 5,528) of patients had an isolated CoA (iCoA). The overall mortality for the entire group was 4.2% (n = 331), decreasing to 2.0% (n = 114) for iCoA (P < .0001). A hypoplastic aortic arch occurred in 4.6%, with a mortality of 10.6%. Coarctectomy with an end-to-end reconstruction was the most common procedure performed. Multivariable modeling for discharge mortality was significant for diagnosis of ventricular septal defect, operative weight, operative year, and diagnosis of aortic arch hypoplasia., Conclusions: Operative repair of CoA is accomplished with a low mortality, although certain subgroups have persistently inferior outcomes. The techniques utilized for aortic reconstruction have evolved, with coarctectomy and an end-to-end anastomosis becoming the dominant surgical procedures., (© The Author(s) 2015.)

Background: To present perioperative and long-term results of percutaneous treatment of adult isthmic coarctation of the aorta by means of a self-expandable closed-web uncovered nitinol stent (Sinus-XL, Optimed, Esslingen, Germany)., Methods and Results: Preoperative, perioperative, and long-term clinical and computed tomographic angiography data were collected and analyzed prospectively. A total of 52 consecutive patients were treated with the Sinus-XL stent. Mean age was 36.6 (21-67) years, peak invasive trans-coarctation of the aorta gradient was 54.7 ± 9.9 mm Hg, and upper body hypertension unresponsive to medical treatment was present in all patients. Mean stent diameter and length were 24.2 mm (22-28 mm) and 70.4 mm (40-80 mm), respectively. Eight patients (15.4%) required coarctation of the aorta predilatation. All patients underwent poststent dilatation with a noncompliant balloon. Postoperative peak gradient (3.3 ± 2.5 mm Hg) was reduced significantly (P < 0.001) and minimal aortic diameter was increased significantly (4.6 ± 1.9 versus 18.6 ± 2.5 mm; P < 0.001). All patients were discharged home (mean hospitalization, 3.5 days). At follow-up (47.6 months; 12-84), 1 (1.9%) noncardiovascular mortality was reported. Aortic computed tomography confirmed the absence of stent collapse and secondary migration and documented stability in aortic diameter (18.3 ± 2.7 mm). Thirty patients (57.7%) were completely weaned-off antihypertensive medications and their use dropped from 2.6 to 0.9 drugs/patient (P < 0.001). Ankle-brachial pressure index increased from 0.75 to 0.98 (P < 0.001)., Conclusions: Adult coarctation of the aorta treatment by means of a self-expandable uncovered stent is safe and durable. The peculiar stent design maintains adequate localized radial strength over time with minimal trauma on the adjacent aortic wall and negligible device-related complications. Blood pressure control optimization is immediate and persistent even at long-term follow-up., (© 2015 American Heart Association, Inc.)

Background: Adult post-coarctectomy patients (CoA) demonstrate increased cardiovascular morbidity and mortality. The carotid intima-media thickness (CIMT), a marker for atherosclerosis, is increased in CoA. The aim was to evaluate the predictive value of CIMT for cardiovascular events., Methods and Results: Consecutive CoA patients were prospectively studied during 10.1±0.7 years follow-up. At baseline and follow-up echocardiography, MRI imaging and CIMT imaging were performed, while cardiovascular events were registered. CIMT data were compared with controls. The composite endpoint included: myocardial infarction, cerebrovascular events (CVAs), and (sudden) cardiac death. 160 CoA patients were studied (median age 31.7 (18-74 years), 64% male). Events occurred in 11 patients (7%), five (3%) with myocardial infarction, four (2.5%) with an ischemic CVA and two (1%) died suddenly. An increased CIMT (≥0.8 mm) (HR=15.44, P=<0.001) was predictive for the occurrence of cardiovascular events. Baseline CIMT was increased in CoA compared to controls (0.64±0.12 mm vs 0.57±0.07 mm, P=0.005). CIMT progression rates were similar (0.0091±0.016 mm/year vs 0.0097±0.018 mm/year, P=0.84). Signs of atherosclerosis occurred significantly earlier in CoA patients., Conclusion: The contemporary cardiovascular event rate in CoA is 11% in 10 years. Atherosclerosis seems to appear earlier in CoA patients as compared to controls. CoA patients with a CIMT exceeding 0.8mm have a fifteen fold higher cardiovascular risk. CIMT seems to be a useful tool for cardiovascular risk assessment in CoA., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Background: The purpose of this study was to determine the rate of reinterventions from birth to adulthood after end-to-side anastomosis technique for interrupted and hypoplastic aortic arches. Multicenter data have shown that more than half of patients require arch reintervention in the 2 decades after repair., Methods: The follow-up of 170 consecutive patients undergoing end-to-side repair through sternotomy for interrupted aortic arch (95) or coarctation (75) in one institution between 1985 and 2012 was reviewed. Associated lesions included ventricular septal defect (143) and bicuspid aortic valve (50). One-stage repair was performed in 158 patients (93%)., Results: There were 12 hospital deaths (7%), and 4 early arch reinterventions (2 for bronchial compression). Eleven hospital survivors (7%) were lost to follow-up. After a mean of 10 ± 6 years, there were 9 late deaths. Eighteen-year survival was 93% (95% confidence interval: 87 to 96). Eight patients had bronchial compression, 5 during initial stay and 3 after hospital discharge; 2 of them required surgery. Eighteen-year freedom from arch reoperation was 87% (95% confidence interval: 76 to 93). An additional 10 patients underwent balloon dilation, for an 18-year freedom from reintervention (balloon dilation or surgery) of 77% (95% confidence interval: 65 to 85). At last follow-up, 24 patients (16%) had an echocardiographic gradient greater than 25 mm Hg. Blood pressure was recorded in 105 patients, and only 11 (10%) were hypertensive., Conclusions: The end-to-side anastomosis technique for repair of aortic arch lesions results in a low rate of mortality, arch reoperation, and late hypertension. The development of arch obstruction requiring balloon dilation warrants continuous follow-up of these patients., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Management of a ventricular septal defect (VSD) at time of coarctation of the aorta (CoA) repair remains controversial, with recent studies advocating concomitant repair of both defects. We evaluated the surgical management and mortality for patients undergoing CoA repair associated with a VSD., Methods: We retrospectively reviewed data submitted to the Pediatric Cardiac Care Consortium of patients undergoing repair of CoA from 1982 to 2007. The cohort was divided into three groups: CoA repair plus VSD closure (group 1); CoA repair plus pulmonary artery band (group 2); and CoA repair without repair of VSD (group 3). Variables reviewed included era, age, and weight at repair, and in-hospital mortality., Results: There were 7,860 patients who underwent repair of CoA, of whom 2,022 had an associated VSD (25.7%). Mortality after CoA repair with and without an associated diagnosis of VSD was 8.3% versus 2.1% (p < 0.001). Mean age at repair for group 1 (n = 286) and group 2 (n = 472) was 87.4 days and 21.6 days, respectively (p = 0.004), and median weight was 3.31 kg and 3.30 kg, respectively (p = 0.130). Discharge mortality for group 1 and group 2 was similar, at 8.7% and 9.1%, respectively (p = 0.852). Patients with CoA/VSD who had neither VSD closure nor pulmonary artery banding (group 3) had a hospital mortality of 7.9%., Conclusions: The association of CoA and VSD is common. A strategy of concomitant VSD closure at CoA repair does not result in worse discharge mortality when compared with pulmonary banding with anticipated staged repair of the VSD. These outcomes support continued evaluation of a one-stage approach., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Hybrid operations combining open and endovascular techniques have evolved for patients with complex aortic and coexisting cardiovascular disease. Our objectives were to describe the repair techniques and assess the outcomes in patients undergoing hybrid repair for aneurysm associated with previous aortic coarctation., Methods: From 2004 to 2012, 14 patients underwent hybrid repair by elephant trunk with endovascular completion (n = 5), frozen elephant trunk (n = 8), or antegrade stent grafting (n = 1). The mean age at surgery was 45 ± 13.5 years. Of the 14 patients, 8 underwent supra-aortic arterial revascularization (ascending to subclavian bypass in 5, carotid-subclavian bypass in 2, or ascending to carotid and subclavian bypass in 1). Ten patients had a bicuspid aortic valve, 5 underwent concomitant aortic valve replacement, and 1 underwent valve repair. Six had a hypoplastic arch. Other procedures included ascending aortic repair (n = 4), coronary artery bypass grafting (n = 1), ascending to descending bypass (n = 1), and subclavian aneurysm repair (n = 1). One operation was an emergency, the others were elective. The mean maximum aneurysm diameter was 5.9 ± 1.5 cm. Data were obtained from a prospective database and chart review., Results: No perioperative mortality, stroke, renal failure, or paraplegia occurred. One patient required prolonged intubation, another required reoperation for postoperative bleeding. Two endoleaks required repeat intervention. The mean length of stay was 9 ± 5.5 days. One late death occurred from hypertensive crisis and associated disseminated intravascular coagulation. At a mean follow-up of 26 months, no aortic growth was found., Conclusions: Hybrid repair of postcoarctation repair aneurysm is a safe and effective, less-invasive treatment option for patients with complex anatomy and/or concomitant cardiac disease., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Cardiovascular magnetic resonance (CMR) is ideal for assessing patients with repaired aortic coarctation (CoA). Little is known on the relation between long-term complications of CoA repair as assessed by CMR and clinical outcome. We examined the prevalence of restenosis and dilatation at the repair site and the long-term outcome in patients with repaired CoA., Methods and Results: CMR imaging and clinical data for adult CoA patients (247 patients aged 33.0 ± 12.8 years, 60% male), were analyzed. The diameter of the aorta at the repair site was measured on CMR and its ratio to the aortic diameter at the diaphragm (repair site-diaphragm ratio, RDR) was calculated. Restenosis (RDR≤70%) was present in 31% of patients (and significant in 9% [RDR<50%]), and dilatation (RDR>150%) in 13.0%. A discrete aneurysm at the repair site was observed in 9%. Restenosis was more likely after resection and end-end anastomosis, whereas dilatation after patch repair. Systemic hypertension was present in 69% of patients. Of the hypertensive patients, blood pressure (133 ± 20/73 ± 10 mm Hg) was well controlled in 93% with antihypertensive therapy. Mortality rate over a median length of 5.9 years was low (0.69% per year, 95% CI: 0.33-1.26), but significantly higher than age-matched healthy controls (standardised mortality ratio 2.86, CI 1.43-5.72, p<0.001)., Conclusion: Restenosis or dilatation at the CoA repair site as assessed by CMR is not uncommon. Medium term survival remains good, however, albeit lower than in the general population. Life-long follow-up and optimal blood pressure control are likely to secure a good longer term outlook in these patients., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Objective: To review the surgical outcome of the Yasui operation in patients with adequate-sized ventricles and ventricular septal defect (VSD) associated with obstructions of the aortic arch and left ventricular outflow tract (LVOT)., Methods: Since 1985, 17 patients have undergone the Yasui operation at our institution. Interrupted aortic arch was present in 11 patients and coarctation of the aorta/hypoplastic arch was present in 6. Twelve patients had aortic stenosis, and 5 patients had aortic atresia. The minimum diameter of the LVOT and the z-score in patients with aortic stenosis were 3.7 ± 0.4 mm and -9.2 ± 1.2, respectively. Primary repair was performed in 6 patients, and 11 patients were staged, with bilateral pulmonary artery banding (PAB) in 8, arch repair with PAB in 2 and Norwood operation in 1. The mean age and body weight at the time of the Yasui operation was 4.7 ± 5.3 months and 4.5 ± 1.8 kg, respectively. The ascending aorta and aortic arch were reconstructed by Damus-Kaye-Stansel (DKS) anastomosis with graft interposition in 2, DKS with direct anastomosis in 6 and Norwood-type reconstruction in 9. VSD was enlarged in 6 patients. Right ventricle to pulmonary artery continuity was established with a valved conduit in 14 patients, the Lecompte manoeuvre in 2 patients and another method in 1 patient. The mean duration of the follow-up was 7.6 ± 9.2 years., Results: There was 1 early death due to myocardial infarction and 1 late death due to non-cardiac cause. The actuarial survival at 10 years was 87.8%. Six patients underwent reoperation, including 5 conduit exchanges, 2 LVOT repairs and 2 aortic arch repairs. The freedom from reoperation for all causes at 5 and 10 years were 71.3 and 28.5%, respectively. In the last echo study, LVOT flow velocity was 1.2 ± 0.8 m/s, and neoaortic valve regurgitation was mild in 1 patient and trivial or absent in the remaining patients., Conclusions: The results of the Yasui operation were excellent, showing low mortality and good mid-term left ventricular function without outflow tract stenosis or neoaortic valve insufficiency. Bilateral PAB as initial palliation is a useful option in symptomatic neonates.

Objectives: The objective of our study was to review the long-term outcomes of patients undergoing surgical repair of aortic coarctation., Background: Surgical repair of aortic coarctation has been performed at the Mayo Clinic, Rochester, Minnesota, for over 60 years., Methods: Between 1946 and 2005, 819 patients with isolated coarctation of the aorta underwent primary operative repair. Medical records were reviewed and questionnaires mailed to the patients., Results: Mean age at repair was 17.2 ± 13.6 years. The majority (83%) had pre-operative hypertension. Operations included simple and extended end-to-end anastomosis (n = 632), patch angioplasty (n = 72), interposition grafting (n = 49), bypass grafting (n = 30), and subclavian flap or "other" (n = 35). Overall early mortality (<30 days) was 2.4%. In the previous 30 years (n = 225), there were no operative deaths. Mean follow-up was 17.4 ± 13.9 years, with a maximum of 59.3 years. Actuarial survival rates were 93.3%, 86.4%, and 73.5% at 10, 20, and 30 years, respectively. When compared to an age- and sex-matched population, long-term survival was decreased (p < 0.001). Older age at repair (>20 yrs) and pre-operative hypertension were associated with decreased survival (p < 0.001). Patients age <9 years age at repair had significantly less hypertension at 5 to 15 years of follow-up (p < 0.001). Rates of freedom from re-intervention on the descending aorta were 96.7%, 92.2%, and 89.4% at 10, 20, and 30 years, respectively. Younger age at time of repair (p < 0.001) and an end-to-end anastomosis technique (p < 0.001) were independently associated with lower rates of re-intervention on the descending aorta., Conclusions: Primary repair of isolated coarctation of the aorta was performed with a low rate of mortality. However, long-term survival was reduced compared with that in an age- and sex-matched population, and many patients required further reoperation. These findings emphasize that patients with aortic coarctation need early recognition and intervention, as well as lifelong informed follow-up., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: Coarctation accompanied by cardiac lesions is a complex clinical situation due to the presence of two different pathologies that necessitate surgical treatment. An individual strategy, according to the severity of the disease, is important to reduce perioperative mortality and morbidity., Methods: We report here on 25 patients with coarctation accompanied by cardiac lesions who were treated by various surgical approaches. Coarctation and associated disease were treated in 14 patients in a single stage by an ascending-to-descending bypass (n = 11) or by a hybrid procedure (n = 3). The remaining 11 patients underwent a two-stage operation for their treatment. Six of these 11 patients who had coronary artery disease or signs of congestive heart failure were first operated for their cardiac disease, whereas in the remaining five patients, who did not have any congestive signs, coarctation repair was performed first., Results: All the patients were male, between the ages of 20 and 24 years, except for one 45-year-old woman. The mean cross-clamp times, cardiopulmonary bypass times and operation times were 52 ± 14.5, 102.3 ± 28.5 and 174 ± 24.8 min in the extra-anatomical bypass group; 29.8 ± 11.7, 55.5 ± 17.6 and 116 ± 22 min in the two-stage groups and 49 ± 19.8, 63 ± 18.7 and 159 ± 21.3 min in the hybrid patients, respectively. One patient who underwent extra-anatomical bypass died on the 14th postoperative day. There were no events during the follow-up period for the other patients. Also, there were no gradients between the extremities and no graft-related complications., Conclusions: As a consequence of the progress in the development of endovascular techniques, hybrid treatment is becoming a more popular option for the treatment of coarctation accompanied by cardiac diseases. Two-stage procedures and extra-anatomical bypass might be alternative techniques if endovascular procedures are contraindicated or failing.

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.

Aortic coarctation represents a distinct anatomic obstruction as blood moves from the ascending to the descending aorta and can present in a range of ages from infancy to adulthood. While it is often an isolated and discrete narrowing, it can also be seen in the more extreme scenario of severe arch hypoplasia as seen in the hypoplastic left heart syndrome or in conjunction with numerous other congenital heart defects. Since the first description of an anatomic surgical repair over sixty years ago, an evolution of both surgical and transcatheter therapies has occurred allowing clinicians to manage and treat this disease with excellent results and low morbidity and mortality. This review focuses on the current state of both transcatheter and surgical therapies, paying special attention to recent data on long-term follow-up of both approaches. Further, current thoughts will be explored about future therapeutic options that attempt to improve upon historical long-term outcomes.

Background: For neonates with atrioventricular septal defect and aortic arch obstruction including coarctation of the aorta, we sought to determine whether a difference in outcomes exists after a primary neonatal versus staged surgical repair (neonatal arch repair with delayed intracardiac repair)., Methods: This retrospective cohort study included consecutive neonates with atrioventricular septal defect and aortic arch obstruction who underwent cardiac surgery before 28 days of age at six centers from 1990 to 2009. Characteristics and outcomes between patients undergoing neonatal versus staged repair were compared., Results: Of 66 study patients, 31 (47%) underwent primary neonatal repair and 35 (53%) underwent staged repair. At baseline echocardiogram, a greater percentage of neonatal repair patients had relative unbalanced ventricular size (56% versus 35%, p = 0.02). There were no other differences in demographic characteristics, cardiac anatomical or functional details, or surgical technique. Those undergoing neonatal repair tended to be more likely to have at least moderate left atrioventricular valve regurgitation early after repair (42% versus 19%, p = 0.05) and to have at least one major in-hospital complication (42% versus 20%, p = 0.06). After the initial cardiac operation, compared with the neonatal repair group, patients undergoing staged repair had greater survival (87% versus 57% at 6 years, log-rank p = 0.02) and freedom from the first unplanned cardiac reoperation (69% versus 45% at 6 years, log-rank p = 0.005)., Conclusions: For neonates with atrioventricular septal defect and aortic arch obstruction, when compared with neonatal repair, a staged approach was associated with improved survival and lower morbidity., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Neonatal coarctation of the aorta (CoA) is often associated with hypoplastic left-sided cardiac structures. Limited data are available regarding the long-term clinical outcomes. Our purposes were to assess the following in neonates with CoA and left-sided cardiac hypoplasia: (1) left-sided cardiac structure growth over time, (2) echocardiographic parameters predicting reintervention, and (3) long-term outcomes and reintervention rates. Data were collected for all patients born with isolated CoA, along with a mitral or aortic Z-score of <-2, who underwent repair before 2 months of age from January 1993 to April 1997. Recent follow-up data were available for 51 of 63 patients (81%) aged 15.4 ± 1.5 years (range 11 to 18). Of the 51 patients, 46 (90%) had a recent echocardiogram, all with normal left ventricular systolic function. The mitral and aortic valve annulus Z-scores increased significantly from the initial measurements: -3.29 ± 1.54 to -0.94 ± 0.58 (p <0.0001) and -3.25 ± 1.98 to 0.08 ± 1.81 (p <0.0001). No significant change was seen from intermediate (6.1 ± 1.6 years) to the latest follow-up examination. Of the 51 patients, 12 (24%) required reintervention at 3 ± 4.5 years; 9 for repeat CoA, 4 for subaortic stenosis, and 2 for aortic stenosis (some in combination). The initial echocardiographic parameters were not associated with reintervention. The overall freedom from reintervention was 76% at 15 years of follow-up. In conclusion, the long-term outcomes after isolated CoA repair with associated left-sided cardiac hypoplasia were excellent. Both aortic and mitral valve sizes had increased substantially by intermediate follow-up but tended to normalize afterward. Although 24% of the patients required reintervention, significant left ventricular inflow or outflow tract obstruction was uncommon., (Copyright © 2013 Elsevier Inc. All rights reserved.)