Coarctation Of The Aorta Repair Research Articles

Outcome and Impact of Associated Left-Sided Cardiac Lesions in Coarctation of the Aorta Diagnosed During Fetal Life

Significant effort has been put into the optimization of the antenatal diagnosis of coarctation of the aorta (CoA). However, although left-sided cardiac lesions are known to cluster, the necessity to intervene postnatally for other left-sided cardiac lesions has not been reported in a cohort of fetuses with suspected CoA. We report a study of all 89 fetuses with antenatally suspected and postnatally confirmed diagnosis of CoA who underwent CoA repair as the primary procedure at a single tertiary congenital heart disease center over 10 years (January 1, 2010, to December 31, 2019). Almost 1 in 5 patients (18%) had to undergo surgery and/or transcatheter intervention on additional left-sided cardiac lesions (14%) and/or reintervention on the aortic arch (12%) during follow-up to median age of 2.85 years. Freedom from intervention at 5 years was 78% (95% confidence interval [CI] 67 to 88%) if reintervention on CoA was excluded, and 72% (95% CI 60 to 82%) if this was included. Five-year survival was 95% (95% CI 90 to 100%). Furthermore, 20% of affected infants had genetic (10%) and/or extracardiac (16%) abnormalities. Our study highlights the need for comprehensive antenatal counseling, including the prognosis of primary repair of CoA and the potential development of additional left-sided cardiac lesions, which may be difficult to diagnose prenatally even in expert hands or impossible to diagnose because of the physiology of the fetal circulation.

Abstract 9310: Aortic Arch Shape After Coarctation Repair Predicts Exercise Capacity: A Multicenter Analysis

Introduction: Patients with coarctation of the aorta (CoA) have decreased exercise capacity despite successful repair with no anatomic obstruction. Altered aortic arch shape after surgical repair may increase resistance to flow and affect flow distribution. This study correlates parameters of aorta size and flow with exercise capacity after successful CoA repair. Methods: We retrospectively reviewed patients with repaired CoA at 4 centers who had cardiac magnetic resonance imaging (CMR) and an exercise stress test with respiratory exchange ratio ≥ 1.09 within 1 year. Patients with significant valve disease, outflow tract obstruction, or need for cardiac bypass outside of their arch repair were excluded. Aorta size mismatch (D AAo /D DAo ), defined by the ratio of ascending (AAo) to proximal descending aorta (DAo) diameter, and DAo flow as a percentage of AAo flow, were measured from CMR. Each parameter was correlated with percent predicted peak oxygen consumption (VO2 max). Results: Forty-six patients (age 26 ± 8.4 years) with left ventricular ejection fraction (LVEF) > 52%, met inclusion criteria. Larger D AAo /D DAo and lower %DAo flow correlated significantly with lower VO2 max (Fig). Compared to those with normal VO2 max (n=25), patients with VO2 max <90% predicted (n=21) had a primary repair at an older age (p=0.03) and smaller DAo diameter/m 2 (p=0.04). There were no significant differences in prevalence of arch obstruction by echocardiogram, LVEF, hypertension, blood pressure gradient at rest or exercise, or AAo diameter/m 2 between groups. Conclusions: Aorta size mismatch, due to AAo dilation or small DAo, and associated decreased %DAo flow, correlate significantly with decreased exercise capacity after CoA repair in patients with normal left ventricular systolic function. CMR measurements of aorta geometry and flow can inform the definition of a successful repair and suggest that indications for re-intervention may not be limited to discrete re-coarctation.

Cardiovascular Morbidity and Mortality in Adult Patients With Repaired Aortic Coarctation.

BackgroundThe long‐term burden of cardiovascular disease after repair of coarctation of the aorta (CoA) has not been elucidated. We aimed to determine the incidence of and risk factors for cardiovascular events in adult patients with repaired CoA. Additionally, mortality rates were compared between adults with repaired CoA and the general population.Methods and ResultsUsing the Dutch Congenital Corvitia (CONCOR) registry, patients aged ≥16 years with previous surgical or transcatheter CoA repair from 5 tertiary referral centers were included. Cardiovascular events were recorded, comprising coronary artery disease, stroke/transient ischemic attack, aortic complications, arrhythmias, heart failure hospitalizations, endocarditis, and cardiovascular death. In total, 920 patients (median age, 24 years [range 16–74 years]) were included. After a mean follow‐up of 9.3±5.1 years, 191 patients (21%) experienced at least 1 cardiovascular event. A total of 270 cardiovascular events occurred, of which aortic complications and arrhythmias were most frequent. Older age at initial CoA repair (hazard ratio [HR], 1.017; 95% CI, 1.000–1.033 [P=0.048]) and elevated left ventricular mass index (HR, 1.009; 95% CI, 1.005–1.013 [P<0.001]) were independently associated with an increased risk of cardiovascular events. The mortality rate was 3.3 times higher than expected based on an age‐ and sex‐matched cohort from the Dutch general population (standardized mortality ratio, 3.3; 95% CI, 2.3–4.4 [P<0.001]).ConclusionsThis large, prospective cohort of adults with repaired CoA showed a high burden of cardiovascular events, particularly aortic complications and arrhythmias, during long‐term follow‐up. Older age at initial CoA repair and elevated left ventricular mass index were independent risk factors for the occurrence of cardiovascular events. Mortality was 3.3‐fold higher compared with the general population. These results advocate stringent follow‐up after CoA repair and emphasize the need for improved preventive strategies.

Ascending Aortoiliac Bypass for One-Stage Repair of Adult Aortic Coarctation With Concomitant Cardiac Lesions.

To evaluate the efficacy and safety of ascending aortoiliac bypass surgery for one-stage repair of adult coarctation of the aorta (CoA) and concomitant cardiac disease. From March 2012 to October 2018, 51 consecutive CoA patients were treated with ascending aorta to bilateral iliac artery bypass concomitant with and cardiac surgerical procedures performed for a variety of reasons. A Y-shaped graft was used for the bypass procedure. We evaluated early outcomes, including postoperative death, systolic blood pressure and differences between upper and lower limb blood pressure. The average age was 41years and 64.7% of patients were men. Simultaneous cardiac procedures included aortic valve replacement, ventricular septal defect repair, Bentall procedures and Wheat procedures. No deaths occurred in the early postoperative period. Three patients had delayed healing at the site of the abdominal lower quadrant incisions. The average systolic pressure in the upper limb and the average difference between the upper- and lower-limb blood pressure decreased significantly after surgery (162.7 ± 13.4mmHg vs 128.4 ± 6.7mmHg, P = .000; 69.6 ± 15.6mmHg vs 8.7 ± 7.6mmHg, P = .000, respectively); The systolic blood pressure in the lower limb increased after bypass surgery (93.1 ± 6.2mmHg vs 119.6 ± 7.7mmHg, P = .000). The follow-up rate was 100%, with an average follow-up time of 61months. Six patients (11.8%) had graft stenosis or occlusion. Three patients (5.9%) underwent endovascular embolectomy. In our small series, ascending aortoiliac bypass for one-stage repair of CoA with concomitant cardiac lesions appears safe and efficacious in effectively reduceing differences between upper and lower limb systolic blood pressure. Further study with larger sample size and longer follow-up is needed.

Open anatomical repair for primary coarctation of the aorta in adults.

Although endovascular repair has become an alternative treatment for coarctation of the aorta (CoA) in adults, open repair provides concomitant repair of other cardiac complications, including post-stenotic aneurysm, ascending aortic aneurysm, and intracardiac diseases. We evaluated open anatomical repair for CoA repair in adults. Eleven patients (6 men, age range 21-63years) underwent primary CoA repair. Complicating conditions included post-stenotic aortic aneurysm in the descending aorta in 5 patients (45.5%) and ascending aortic aneurysm in 3 (27.3%). Two patients (18.2%) had a bicuspid aortic valve, and one (9.1%) had a quadricuspid aortic valve. Ventricular septal defect was detected in 1 patient (9.1%). Eight patients (72.7%) underwent descending aorta replacement through a left thoracotomy, comprising partial cardiopulmonary bypass in 4 and deep hypothermic circulatory arrest in 4. Of those, the left subclavian artery was reconstructed in 4 patients. The remaining 3 patients (27.3%) underwent total arch replacement, through a median sternotomy in 1 and using a combination of median sternotomy and thoracotomy in 2. No in-hospital mortality was observed. No spinal cord ischemia or neurological events were encountered, but 1 patient (9.1%) who underwent CoA repair via median sternotomy and thoracotomy required prolonged ventilatory support for more than 48h. During the follow-up of 90months (interquartile range 65-124months), no patient died or required reintervention for the repaired segment. CoA in adults could be anatomically repaired with graft replacement both through the median sternotomy, the left thoracotomy, and the combination of both approaches, according to the complicated aortic or intracardiac lesions.

Abstract 15688: Computational Fluid Dynamics Assessment of Exercise in Patients With Successfully Repaired Coarctation of the Aorta

Introduction: Patients with coarctation of the aorta (CoA) have decreased exercise capacity even after successful repair with no anatomic obstruction. Our previous work demonstrated a robust negative correlation of aorta size mismatch, defined by ascending (AAo) to descending aorta (DAo) diameter ratio (D AAo /D DAo ), and exercise capacity (VO2 max), suggesting geometry alters arch resistance. This study aims to identify the hemodynamic impact of aorta size mismatch with simulations of rest and exercise using computational fluid dynamics (CFD). Methods: An IRB-approved retrospective chart review identified patients with CoA repair who had a cardiac MRI and exercise stress test within 6 months. Each aorta was segmented and scaled to a body surface area of 1 m 2 . CFD was used to simulate rest (3 L/min/m 2 ) and exercise (9 L/min/m 2 ). Outlet resistance was tuned to match patient-specific DAo flow split derived from in vivo MRI and assumed unchanged for exercise. Power loss was calculated between AAo and DAo and correlated with D AAo /D DAo . Results: The patients (ages 12-41 years, mean 26.8±8.6), had a VO2 max ranging from 47 to 126% predicted (mean 92±20%). Power loss had a significant negative correlation with D AAo /D DAo at rest (p=0.001) and exercise (p&lt;0.01). The ratio of power loss in exercise to rest positively correlated with D AAo /D DAo (p&lt;0.05) (Figure). Conclusions: The relationship of power loss with aorta size mismatch is largely driven by altered flow distribution dependent on AAo and DAo size. There is a disproportionate increase in power loss with exercise compared to rest with a larger aorta size mismatch. This suggests arch geometry independently impacts power loss in exercise which is captured by the measurement of aorta size mismatch and can be a target for optimizing aortic arch repair. Figure: Ratio of power loss in exercise to rest correlates with D AAo /D DAo . Example aortic arches are shown with low power loss (green) and high power loss (red) with corresponding VO2.

Neurological complications in aortic coarctation: Results of a Nationwide analysis based on 11,907 patients

BackgroundPatients with coarctation of the aorta (CoA) are at increased risk of periprocedural and late neurological complications. Based on a nationwide inpatient dataset, we assessed the prevalence and risk factors for neurological complications in this cohort. Method and ResultsWe included all CoA patients hospitalized in England between 1997 and 2015. The risk for procedural complications, subarachnoid bleeding and ischemic stroke were quantified, and risk factors investigated. Overall, 11,907 patients (60% male) with CoA were identified. The median age at first presentation was 0.6 years with a median follow-up period of 14.4 years. Throughout the study period, 8456 surgical or interventional procedures were performed in 6796 patients. The neurological complication rate for primary surgical or interventional repair in patients born after 1997 was 0.05% and 0.2%, respectively. During a total follow-up period of 146,295 patient-years, late neurological complications such as subarachnoid bleeding and cerebral infarction occurred in 225 patients (0.15%/patient-year). The median age for subarachnoid bleeding was 29 years and 57 years for ischemic stroke. While, arterial hypertension (p = .0014), current smoking (p = .02), dyslipidaemia (p = .007) and diabetes (p = .037) were significantly related to the risk of ischemic stroke, only arterial hypertension emerged as significant risk factor for subarachnoid bleeding. ConclusionsNeurological complication during primary CoA repair are extremely rare in the current era. In contrast, many CoA patients are afflicted by late complications such as subarachnoid bleeding and ischemic stroke at a relatively young age, emphasizing the need for meticulous and pro-active blood pressure control and reduction of cardiovascular risk factors.

Aortic Arch Enlargement and Coarctation Repair of Preserving the Lesser Curvature of the Aortic Arch Through a Left Thoracotomy in Neonates.

The aim of this study was to analyze the aortic arch repair technique for preserving the lesser curvature without cardiopulmonary bypass through a left thoracotomy in neonates with coarctation of the aorta (CoA) and a hypoplastic distal aortic arch (HDAA). HDAA was defined as z-score of the aortic arch < - 2.5. Twenty-four neonates with CoA and HDAA were retrospectively studied. Patients underwent enlargement of the HDAA associated with CoA repair when their z-scores were < - 4. They were divided into 2 groups on the basis of the preoperative z-score of the distal aortic arch: group 1 (n = 14), z-score ≥ - 4; group 2 (n = 10), z-score < - 4. Twenty-two patients had intracardiac defects. Follow-up ranged from 0.9 to 20.1years (median 11.6years). The z-scores of the distal aortic arch were significantly smaller in group 2 than group 1 (- 5.09 ± 1.05 vs - 3.19 ± 0.36, p < 0.001). There were no hospital deaths and no hypertension. All dimensions of the aortic arch in both groups revealed significant catch-up growth (p < 0.02). All patients showed a pressure gradient ≤ 5mmHg across the aortic arch and between the arms and legs at the latest follow-up. Two patients showed an angulated arch deformity over 10years later. This technique provided good catch-up growth and a low incidence of reobstruction and deformity of reconstructed aortic arch in both groups. These results suggest that this modification might be considered as one choice of technique for CoA and HDAA.

Neonatal management of prenatally suspected coarctation of the aorta.

(a) To determine the false-positive rate among newborns with prenatally suspected coarctation of the aorta (CoA) within the UCLA Health system, (b) to compare patient and maternal interventions and outcomes between false-positive cases and normal controls, and (c) to determine the timing of clinical presentation of CoA. We performed a single-center, retrospective case control study of all fetuses with suspected isolated CoA who underwent both fetal echocardiographic evaluation and subsequent delivery at UCLA between January 1, 2011, and December 31, 2018. Maternal and neonatal medical records were reviewed for demographic and clinical data, for cases of suspected CoA and for controls. A separate review of our institution's surgical database was performed to identify characteristics of all patients (neonatal and pediatric) with isolated CoA who underwent surgical repair during the same time period. Among the 50 fetal cases of isolated suspected CoA who delivered at our institution, 47 patients (94%) were found to be normal (false positives). Compared with normal controls, patients with suspected CoA were more likely to have delayed maternal bonding, delayed feeding, admission to the intensive care unit, performance of neonatal echocardiograms, initiation of intravenous fluids and initiation of prostaglandin E1, and a longer length of hospital stay. Among the 38 patients undergoing CoA repair at our institution during the study period, four patients were prenatally diagnosed and no patient presented clinically with symptoms before 48 hours after delivery. Compared with normal controls, patients with prenatally suspected coarctation are more likely to have delayed maternal bonding, delayed feeding, more frequent neonatal echocardiograms, and longer length of hospital stay. Further refinement of neonatal management may improve postnatal care.

Diagnosis and Surgical Repair for Coarctation of the Aorta With Intracardiac Defects: A Single Center Experience Based on 93 Infants.

Introduction: This study aims to summarize the clinical characteristics of coarctation of the aorta (CoA) associated with intracardiac anomalies in infants.Methods: Medical records of 93 infants who were diagnosed with CoA and intracardiac anomalies from August 2009 to August 2018 were retrospectively reviewed.Results: All of the 93 infants underwent single-stage repair for CoA associated with intracardiac anomalies. The mean operative time was 264.6 ± 57.1 min, and the time of ICU stay was 7.0 ± 4.1 days. The residual transcoarctation pressure gradient before discharge was lower than the pressure gradient prior to surgery (48.3 ± 17.5 vs. 22.4 ± 9.6 mmHg, P < 0.01). Early death before discharge was found in five infants. The mean follow-up time of 88 hospital survivors was 40.0 ± 26.4 months, and no subsequent death occurred in the follow-up. Transcoarctation pressure gradient of the 88 survivors in their last follow-up was 19.6 ± 10.5 mmHg. The pressure gradient of 27 cases was higher than 20 mmHg. Significant lower limb retardation was observed in four cases; therefore, balloon angioplasty consult was recommended. The cumulative recoarctation-free survival rate in 3-year follow-up was 73.5%.Conclusions: To avoid secondary operations in early period, single-stage repair of CoA associated with intracardiac anomalies was effective and safe, and the outcomes in early to mid-term follow-up were satisfactory.

Early Postnatal Echocardiography in Neonates with a Prenatal Suspicion of Coarctation of the Aorta

Coarctation of the aorta (COA) is suspected prenatally when there is ventricular asymmetry, arterial disproportion, and hypoplasia of the aortic arch/isthmus. The presence of fetal shunts creates difficulty in prenatal confirmation of the diagnosis so serial echocardiography after birth is necessary to confirm or refute the diagnosis. The first neonatal echocardiogram in prenatally suspected cases of COA was assessed for prediction of neonatal COA repair (NCOAR). This included morphological assessment, measurement of the aortic arch and calculation of the distal arch index (DAI = distance between left common carotid and left subclavian artery/diameter of thedistal arch). NCOAR was undertaken in 23/60 (38%) cases. Transverse arch, aortic isthmus z-score, and DAI had an area under the receiver operator curve of 0.88 (95% CI 0.77-0.98), 0.86 (95% CI 0.75-0.96), and 0.84 (95% CI 0.74-0.95), respectively for the prediction of NCOAR. Using transverse arch z-score threshold < - 3 gave sensitivity 100%, NPV: 100%, specificity 76%; aortic isthmus z-score < - 3: NPV 92%, specificity 62% and DAI > 1.4: NPV 88%, specificity 78%. The size of the distal aortic arch in infants with a common origin of the innominate artery and left common carotid artery who did not require COA repair was similar to the NCOAR cases (p = 0.22). The early postnatal assessment of the size and morphology of the aortic arch can assist in risk stratification for development of neonatal COA. The branching pattern of the head/neck vessels impacts on the size of the distal aortic arch adding to the complexity of predicting COA based on vessel size.

P1380 tissue Doppler and speckeled tracking of left ventricle post coarctation repair

Abstract Introduction Left ventricular dysfunction was observed after repair of coarctation of the aorta (COA) before development of left ventricular concentric hypertrophy. Aim The aim of this study was to assess the the LV functions after COA repair. Methods Thirty pediatric patients with COA repair underwent echocardiographic examinations using Conventional echocardiography, tissue doppler image (TDI) and 2D-STE. Results The LV systolic functions assessed by TDI and 2-D-STE showed a significant reduction of (S׳׳LW LV, IVA at LW LV, IVA at the septum, longtudinal strain (LS) basal and global radial (GRS) in cases compared to control group . There is significant increase in GCS in cases compared to control group, the conventional Doppler derived MPI of LV in post coarctation repair cases was significantly prolonged compared to control. Analysis of mitral annulus velocities including E", A", E"/A" ratio and E/E′ ratio revealed worsening of the left ventricular diastolic mechanics in the post COA repair cases compared to healthy controls. The E" wave velocity (at the left ventricular lateral wall) was significantly lower in post COA repair in cases compared with controls , E"/A" ratio was significantly lower in post COA repair cases compared with controls . The E/E′ ratio was significantly higher in the post COA repair cases compared to the control group . Conclusions LV systolic and diastolic dysfunction was fount in patient after COA repair. LV systolic function by 2-D-STE Cases Control P value Mean SD mean SD GLS -21.51 2.79 -22.63 2.99 .094 LS basal -19.94 2.83 -22.63 2.99 &amp;lt;0.01 (S) LS Mid -21.08 2.75 -21.87 2.78 .273 LS apical -23.52 4.22 -21.74 4.23 .109 GRS 22.58 5.24 49.01 10.08 &amp;lt;0.01 (S) RS basal 20.92 8.29 43.56 18.95 &amp;lt;0.01 (S) RS mid 25.03 7.59 56.80 14.27 .223 RS apical 21.78 8.34 46.66 15.11 &amp;lt;0.01 (S) GCS -20.09 2.66 -16.73 2.78 &amp;lt;0.01 (S) CS basal -21.49 2.70 -16.98 4.69 &amp;lt;0.01 (S) CS mid -21.27 4.09 -15.83 3.67 &amp;lt;0.01 (S) CS apical -17.51 4.63 -17.39 4.57 .920

P2741Predictors of subtle left ventricular systolic dysfunction after treatment of coarctation of the aorta

Abstract Background With the advent of the term “Heart Failure with Preserved Ejection Fraction - HFpEF”, more and more evidence has emerged supporting the importance of Speckle Tracking Echocardiography in measurement of Left ventricular (LV) Strain to assess subtle myocardial systolic dysfunction in such patients. Aim: To assess Global Longitudinal stain (GLS) of the LV in patients with Coarctation of the Aorta (CoA) after surgical or percutaneous treatment and predict the variables affecting the occurrence of subtle myocardial dysfunction in these patients. Patients and methods This was a cross sectional observational study that included 77 patients who presented to Ain Shams University hospital for follow up post treatment of Coarctation of the Aorta. All of these patients underwent intervention (surgical or catheter based) in the period between January 2005 and December 2017 and aged between 15 and 40 years at the time of the study. The patients underwent a detailed transthoracic echocardiogram, including Speckle Tracking using Phillips Q Lab version 7.1 software. All patients had normal systolic functions as measured by LV ejection fraction using Modified Simpsons' method. Correlations between variables were studied using “Independent paired T test and Chi square test”. Results The mean follow up duration was 13.19±3 years. The least accepted GLS value using the Phillips Q Lab version 7.1 software was −16.4%. Accordingly, the study group was divided into two groups (Normal GLS and Abnormal GLS). Nineteen patients had a low GLS, representing 24% of the study population. There was no age nor sex predilection between the two groups. Age at first intervention correlated positively with GLS (p=0.01), meaning that earlier intervention lead to better LV strain as the GLS is a negative value. Patients with Bicuspid Aortic Valve and those having Left ventricular Hypertrophy had significantly lower GLS (p=0.001). Patients with continuous abdominal Aortic flow had significantly lower GLS as compared to patients with pulsatile flow (p=0.005) (see figure). The occurrence of complications, e.g. stent fracture or recoarctation caused a significant reduction in GLS (p=0.012). Type of intervention, Order of interventions and age at second and third interventions did not significantly affect the GLS. CoA dimensions by MSCT and presence of Hypertension both before and after treatment did not affect the GLS. Effect of Aortic flow on GLS Conclusions Although most patients post CoA repair have normal LV ejection fraction, a good proportion of them have impaired LV global longitudinal strain and are thus prone to HFpEF. Earlier age at intervention, absence of complications and LV hypertrophy, as well as presence of pulsatile Abdominal Aortic flow lead to better LV performance as measured by GLS. LV strain derived from Speckle Tracking Echocardiography should be an integral part of follow up of CoA patients after repair. Acknowledgement/Funding None

Exercise Capacity in Asymptomatic Adult Patients Treated for Coarctation of the Aorta

A reduced exercise capacity is a common finding in adult congenital heart disease and is associated with cardiovascular morbidity and mortality. However, data on exercise capacity in patients after repair of coarctation of the aorta (CoA) are scarce. Furthermore, a high rate of exercise-induced hypertension has been described in CoA patients. This study sought to assess exercise capacity and blood pressure response in asymptomatic patients long-term after CoA repair in relation to left ventricular and vascular function. Twenty-two CoA patients (age 30 ± 10.6 years) with successful surgical repair (n = 12) or balloon angioplasty (n = 10) between 3 months and 16 years of age with a follow-up of > 10 years underwent cardiopulmonary exercise testing at a mean follow-up of 23.9 years. Exercise capacity (peak oxygen uptake; VO2peak) and blood pressure response were compared to age- and gender-matched reference values. Left ventricular function and volumetric analysis was performed using cardiovascular magnetic resonance imaging. CoA patients showed preserved exercise capacity compared to the healthy reference group, with a VO2peak of 41.7 ± 12.0 ml/kg/min versus 44.9 ± 6.7 ml/kg/min. VO2peak/kg showed a significant association with age (p < 0.001) and male gender (p ≤ 0.001). Exercise-induced hypertension occurred in 82% of CoA patients, and was strongly related to left ventricular mass (p = 0.04). Of the 41% of patients who were normotensive at rest, 78% showed exercise-induced hypertension. No significant correlation was found between peak exercise blood pressure and age, BMI, age at time of repair, LVEF, or LV dimensions. Exercise capacity is well preserved in patients long-term after successful repair of coarctation of the aorta. Nevertheless, a high number of patients develop exercise hypertension, which is strongly related to systemic hypertension. Regular follow-up, including cardiopulmonary exercise testing, and aggressive treatment of hypertension after CoA repair is strongly advised.

Increased blood pressure is associated with increased carotid artery intima-media thickness in children with repaired coarctation of the aorta.

The intima-media thickness of the common carotid artery (cIMT) is a good noninvasive surrogate marker for cardiovascular disease. Regular cIMT monitoring in children with congenital heart disease has great potential. We sought to determine which anthropomorphic and haemodynamic variables were significantly associated with the cIMT in paediatric patients with obesity and children with repaired coarctation of the aorta (CoA). We measured the cIMT in 143 children aged 5 to less than 18 years including normal weight controls (n = 44), children with overweight/obesity (n = 73) and children with repaired CoA (n = 26). cIMT was compared and the association between the cIMT and patient characteristics, including obesity and blood pressure (BP), was investigated. BMI z score, sex and the presence of CoA were significant independent predictors of cIMT. The cIMT was significantly greater in children with overweight/obesity (0.53 ± 0.06 mm) relative to normal weight controls (0.51 ± 0.04 mm), as well as in CoA patients with abnormally high BP (0.57 ± 0.08 mm) versus CoA patients with normal BP (0.52 ± 0.05 mm) and controls (0.51 ± 0.04 mm). CoA patients with normal BP did not have significantly increased cIMT. cIMT was positively associated with BMI z score, male sex and CoA repair in children. The increased cIMT in children with repaired CoA was because of those with abnormally high BP, which was masked in clinic for most of these patients. These findings warrant further investigations into the cIMT and other atherosclerotic risk factors to determine their potential clinical impact in these highly susceptible patients.

Increased aortic wall stiffness is predictive of aortic dilation in adult patients following coarctation of the aorta repair

Patients after repaired coarctation of the aorta are at risk for dilation and dissection of both the ascending and descending aorta. Histologic abnormalities seen are indistinguishable from patterns seen with Marfan syndrome, and lead to a stiff aortic wall, characterized by decreased distensibility, strain, and increased B-stiffness. We sought to analyze the relationship of aortic wall stiffness measured by cardiac MRI to aortic dilation following coarctation of the aorta repair. Fifty-two adult patients (26 females) with history of previous coarctation of the aorta repair who had undergone a recent cardiac MRI were identified. Studies and charts were reviewed retrospectively. Aortic strain, distensibility, and B-stiffness values were measured at level of ascending aorta and descending aorta. Relationships between measurements of aortic stiffness were compared to aortic dimensions. Median age at coarctation repair was 0.2 years (range 0.1–36 years). Types of repair included subclavian artery flap (44%), end-to-end anastomosis (33%), and patch angioplasty (15%). Time from repair to cardiac MRI was 25 years (range 1–42). By cardiac MRI, 38% had a dilated ascending aorta defined as a Z-score of >2.5. On average the ascending aorta was stiffer than the descending aorta for all three parameters. For both the ascending and descending aorta the 3 parameters of aortic stiffness were predictive of increasing aortic dimensions (P value < 0.01). By univariate analysis, risk factors for a dilated ascending aorta defined as a Z-score of >2.5 included older age at the time of initial repair, presence of a bicuspid aortic valve, and worse parameters of ascending aorta stiffness: % strain, distensibility, and B-stiffness, and a lower descending aorta % strain. By multivariate analysis, older age at initial repair and both a lower % strain at both the ascending and descending aorta remained statistically significant.

Hospital Costs Related to Early Extubation After Infant Cardiac Surgery

The Pediatric Heart Network Collaborative Learning Study (PHN CLS) increased early extubation rates after infant tetralogy of Fallot (TOF) and coarctation of the aorta (CoA) repair across participating sites by implementing a clinical practice guideline (CPG). The impact of the CPG on hospital costs has not been studied. PHN CLS clinical data were linked to cost data from Children's Hospital Association by matching on indirect identifiers. Hospital costs were evaluated across active and control sites in the pre- and post-CPG periods using generalized linear mixed-effects models. A difference-in-difference approach was used to assess whether changes in cost observed in active sites were beyond secular trends in control sites. Data were successfully linked on 410 of 428 eligible patients (96%) from four active and four control sites. Mean adjusted cost per case for TOF repair was significantly reduced in the post-CPG period at active sites ($42,833 vs $56,304, p < 0.01) and unchanged at control sites ($47,007 vs $46,476, p= 0.91), with an overall cost reduction of 27% in active versus control sites (p= 0.03). Specific categories of cost reduced in the TOF cohort included clinical (-66%, p < 0.01), pharmacy (-46%, p= 0.04), lab (-44%, p < 0.01), and imaging (-32%, p < 0.01). There was no change in costs for CoA repair at active or control sites. The early extubation CPG was associated with a reduction in hospital costs for infants undergoing repair of TOF but not CoA. This CPG represents an opportunity to both optimize clinical outcome and reduce costs for certain infant cardiac surgeries.

Long-Term Outcomes of Coarctation Repair Through Left Thoracotomy

Optimal surgical approach for repair of coarctation of the aorta (CoA) remains controversial. This study aimed to evaluate reintervention rates and its predictors by using a strategy of resection with extended end-to-end anastomosis (REEEA) through left thoracotomy. A retrospective analysis was performed for all patients who underwent isolated CoA repair or simultaneous repair of CoA and ventricular septal defect repair by REEEA between January 2000 and December 2015 at Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois. Patients with complex congenital heart disease were excluded. Transverse arch hypoplasia was defined as echocardiographic z-score lower than-2 or by documentation in medical or operative reports. Reintervention was defined as the need for balloon angioplasty or reoperation. Hypertension was defined as antihypertensive medication use or blood pressure greater than or equal to the 95th percentile. A total of 251 patients with median age at repair of 14.6 days met inclusion criteria. Repair was by left thoracotomy in 226 (90%). Follow-up data were available for 186 of 251 patients, with median follow-up time of 5.4 years (range, 0.2 to 15.3 years); 169 (91%) of these patients underwent thoracotomy. There were no early deaths or early reoperations. A proximal transverse arch z-score lower than-4.1 or a distal transverse arch z-score of less than-2.8 was predictive of repair through sternotomy. Only 4 (2%) patients required reintervention (2 patients had balloon angioplasties, 2 had reoperations). Transverse arch hypoplasia was a risk factor for reintervention (p= 0.048), but surgical approach was not (p= 0.35). Late hypertension was identified in only 33 of 186 (18%) patients. Repair of CoA, even with associated transverse arch hypoplasia, by REEEA through left thoracotomy has a low mortality, low reintervention rate, and low incidence of late hypertension.

Aortic coarctation repair through left thoracotomy: results in the modern era.

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. 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. 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. 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.

Cardiac Autonomic Nervous System Activity and Cardiac Function in Children After Coarctation Repair

Coarctation of the aorta (CoA) is one of the most common congenital heart defects. Most patients live into adulthood as a result of improved surgical techniques; however, late complications, including hypertension, recoarctation, and arrhythmias, are common. The autonomic nervous system (ANS) might play a role in the pathology. This study evaluated cardiac ANS activity and cardiac function in children after CoA repair and investigated the relationship between the two. The study participants were 31 children after CoA repair and 62 healthy controls aged between 8 and 18 years. Ambulatory impedance cardiography was used to measure cardiac ANS activity and cardiac output for 24 hours. Transthoracic echocardiography and cardiac magnetic resonance imaging were used to measure cardiac function. No group differences were found in ambulatory cardiac ANS activity. However, ambulatory cardiac output and left ventricular function were significantly decreased in patients compared with controls. Left ventricular function and ambulatory cardiac output are impaired in patients after CoA repair, despite unchanged cardiac ANS activity in this group. These results underscore the importance of clinical follow-up, even in patients without residual stenosis.