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There is limited data on the prognostic implications of residual mild coarctation (RMC) in patients with repaired native coarctation of the aorta (CoA). To explore the association of RMC with mid-term comorbidities in post-interventional patients, and the predictive value of the residual pressure gradient. The authors retrospectively analyzed 79 native CoA patients who received successful intervention at our hospital between October 2010 and June 2023. The outcomes of the study were late arterial hypertension (either raised blood pressure or commencement of hypotensive medications) only in normotensive patients at early follow-up and the composite mid-term comorbidities including new-onset aortic injury, re-stenosis, and re-intervention. At a median follow-up of 60 months, late hypertension and mid-term comorbidities occurred in 16 (28.1%) and nine (11.4%) patients, respectively. Multivariate Cox proportional hazard regression analysis identified invasive peak systolic CoA pressure gradient (PSPG) as the best independent predictor of both outcomes. The maximally selected rank statistics indicated 10 mm Hg as the best PSPG cut-off value for predicting late hypertension. Compared to patients with PSPG < 11 mm Hg, the cumulative event rates of both outcomes were higher in those with PSPG ≥ 11 mm Hg (log-rank test, p < .001 for both endpoints). PSPG ≥ 11 mm Hg was proved to be the independent predictor of late hypertension with a significantly increased risk. In patients with non-surgical CoA repair, the post-interventional RMC and PSPG ≥11 mm Hg are important predictors of clinical comorbidities at mid-term follow-up., (© 2024 The Author(s). The Journal of Clinical Hypertension published by Wiley Periodicals LLC.)

Introduction and Objectives: Stent implantation is the preferred treatment in older children and adults with aortic coarctation (CoA). We aimed to determine the incidence of very late events after CoA stenting., Methods: We analyzed a cohort of CoA patients who underwent stent implantation at our center between 1993 and 2018. Patients were periodically followed up in outpatient clinics, including computed tomography (CT) and fluoroscopy assessment., Results: A total of 167 patients with CT and fluoroscopy data were included: 83 (49.7%) were aged ≤ 12 years and 46 (28%) were female. The mean clinical follow-up time was 17±8 (range 4-30) years and the mean time to CT/fluoroscopy was 11±7 years. Aortic aneurysm was present in 13% and was associated with the PALMAZ stent (OR, 3.09; 95%CI, 1.11-9.49; P=.036) and the stented length (OR, 0.94; 95%CI, 0.89-0.99; P=.039). Stent fracture was frequent (34%), but was not related to the presence of aneurysm. Stent fracture was associated with young age (OR, 3.57; 95%CI, 1.54-8.33; P=.003), male sex (OR, 4.00; 95%CI, 1.51-12.5, P=.008) and inversely with the PALMAZ stent (OR, 0.29; 95%CI, 0.12-0.67, P=.005). Reintervention was lower in adults (10%), mainly related to aneurysms. Those treated when aged ≤ 12 years had higher reintervention rates (43%) due to recoarctation somatic growth., Conclusions: This long-term follow-up study of CoA patients treated with stenting revealed a significant incidence of late events. Reintervention rates were higher in patients treated at younger ages. Periodic imaging surveillance appears to be advisable., (Copyright © 2023 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.)

Competing Interests: Declaration of competing interest The authors have no competing interests to declare.

Background: Aortic aneurysm is common in patients with coarctation of aorta (COA), but it is unclear whether the risk of aortic aneurysms is due to COA or related to the presence of other risk factors such as bicuspid aortic valve (BAV) and hypertension., Objectives: The purpose of this study was to assess the relationship among COA, BAV, and thoracic aortic aneurysms., Methods: A total of 867 patients with COA (COA group) were matched 1:1:1 to 867 patients with isolated BAV (BAV group) and 867 patients without structural heart disease (SHD) (no-SHD group). The COA group was further subdivided into a COA+BAV subgroup (n = 304 [35%]), and COA with tricuspid aortic valve (TAV) (COA+TAV subgroup [n = 563 (65%)]). Aortic dimensions were assessed at baseline and at 3, 5, and 7 years., Results: Compared with the no-SHD group, the COA+BAV subgroup had larger aortic root diameter (37 mm [Q1-Q3: 30-43 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001) and mid ascending aorta dimeter (34 mm [Q1-Q3: 29-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P = 0.008). Similarly, the BAV group had larger aortic root diameter (37 mm [Q1-Q3: 30-42 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001), and mid ascending aorta dimeter (35 mm [Q1-Q3: 30-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P < 0.001). Compared with the COA+TAV subgroup, the COA+BAV subgroup and BAV group were associated with larger aortic root and mid ascending aorta diameter at baseline and follow-up. The risk of acute aortic complications was low in all groups., Conclusions: These findings suggest that BAV (and not COA) was associated with ascending thoracic aorta dimensions, and that patients with COA+TAV were not at a greater risk of developing ascending aortic aneurysms as compared with patients without SHD., Competing Interests: Funding Support and Author Disclosures The MACHD registry is supported by the Al-Bahar Research grant. Dr Egbe is supported by National Heart, Lung, and Blood Institute grants (R01 HL158517, R01 HL160761, and R01 HL162830). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Prenatal diagnosis (PND) of aortic coarctation (AoCo) has been associated with a significant improvement in early results, but there is limited information on the long-term cardiovascular outcome., Methods: We studied 103 patients with simple AoCo, operated in the neonatal period, with a median follow-up of 8,5 years (2 to 23,7 years), with 47% followed for over 10 years. PND was made in 35%. The primary aim was to determine the short and long-term cardiovascular impact of PND of AoCo., Results: Neonates with PND had less preoperative neonatal complications, with only 2,8% incidence of a composite preoperative severe morbidity course, compared to 28% in the postnatal group. PND patients underwent surgery 8 days earlier and had a shorter length of stay in ICU. PND did not impact the incidence of post-operative complications. On the long-term, prevalence of hypertension, left ventricular hypertrophy and rate of recoarctation were not influenced by PND. The PND group had mean 24 h diastolic BP 9 mmHg lower and mean daytime diastolic BP 11 mmHg lower. In the final multivariable model, PND was the single independent variable correlating with daytime diastolic BP., Conclusion: PND of AoCo effectively leads to a better pre-operative course with less pre-operative morbidity. We found no significant differences in immediate post-operative cardiovascular outcomes. A better initial course of patients with PND does not have a major long-term impact on cardiovascular outcomes, nevertheless, at late follow-up PND patients had lower diastolic BP values on ambulatory monitoring, which may have an impact on long-term cardiovascular risk., Competing Interests: Declaration of Competing Interest The authors report no relationships that could be construed as a conflict of interest., (Copyright © 2023. Published by Elsevier B.V.)

Coarctation of aorta (CoA) is a common congenital anomaly which portends patients to early diastolic and systolic heart failure. In this retrospective cohort study, we aimed to evaluate the impact of CoA on heart failure hospitalization. Using the national inpatient sample, the study compared the outcomes of heart failure hospitalization between patients with and without CoA. We noted increasing prevalence of CoA related heart failure admissions over the last decade. Heart failure patients with CoA were younger (mean age 57 vs 71.6 years, P < 0.001), had a longer length of stay (7.4 vs 5.4 days, P < 0.001), and a higher incidence of cardiogenic shock (6.5% vs 2.1%, P = 0.001). However, there was no statistically significant difference in in-hospital mortality (OR 1.45, 95% CI: 0.58, 3.62, P = 0.421) between both groups. These findings demonstrate that CoA increase healthcare resource utilization in patients admitted with heart failure without any significant increase in in-hospital mortality., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Inc. All rights reserved.)

The lack of United States population-based data on Turner syndrome limits assessments of prevalence and associated characteristics for this sex chromosome abnormality. Therefore, we collated 2000-2017 data from seven birth defects surveillance programs within the National Birth Defects Prevention Network. We estimated the prevalence of karyotype-confirmed Turner syndrome diagnosed within the first year of life. We also calculated the proportion of cases with commonly ascertained birth defects, assessed associations with maternal and infant characteristics using prevalence ratios (PR) with 95% confidence intervals (CI), and estimated survival probability. The prevalence of Turner syndrome of any pregnancy outcome was 3.2 per 10,000 female live births (95% CI = 3.0-3.3, program range: 1.0-10.4), and 1.9 for live birth and stillbirth (≥20 weeks gestation) cases (95% CI = 1.8-2.1, program range: 0.2-3.9). Prevalence was lowest among cases born to non-Hispanic Black women compared to non-Hispanic White women (PR = 0.5, 95% CI = 0.4-0.6). Coarctation of the aorta was the most common defect (11.6% of cases), and across the cohort, individuals without hypoplastic left heart had a five-year survival probability of 94.6%. The findings from this population-based study may inform surveillance practices, prenatal counseling, and diagnosis. We also identified racial and ethnic disparities in prevalence, an observation that warrants further investigation., (© 2023 Wiley Periodicals LLC.)

Turner syndrome (TS) is a chromosomal disorder that affects 25-50 per 100,000 live born females. Patients with TS face a heavy burden of cardiovascular disease (congenital and acquired) with an increased risk of mortality and morbidity compared to the general population. Cardiovascular diseases are a major cause of death in females with TS. Approximately 50% of TS patients have a congenital heart abnormality, with a high incidence of bicuspid aortic valve, coarctation of the aorta and generalised arteriopathy. Frequently, females with TS have systemic hypertension, which is also a risk factor for progressive cardiac dysfunction and aortopathy. This paper aims to provide an overview of the cardiovascular assessment, management and follow up strategies in this high-risk population., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: We reviewed our center's experience with neonatal and infant hypoplastic aortic arch, unassociated with intracardiac malformations, and investigated changes in prenatal detection rates over time for those requiring therapeutic procedures., Methods: We identified all prenatal diagnoses of hypoplastic aortic arch with situs solitus, unassociated with intracardiac malformations, made in Nevada between May 2017 and April 2022. In addition, we identified all those 0-180 days old, with prenatal care, that underwent a surgical or interventional cardiac catheterization aortic arch procedure, whether prenatally or postnatally diagnosed. We excluded those with ventricular septal defects, functionally univentricular hearts, interrupted aortic arches, or any associated malformation requiring an additional surgical or interventional procedure ≤6 months old. Additionally, we calculated prenatal detection rates for those undergoing a surgical or interventional catheterization procedure for each of the 5 years., Results: We identified 107 patients prenatally and postnatally. Of the 107 patients, 56 (34 prenatally diagnosed and 22 postnatally diagnosed) underwent an aortic arch procedure, and 51 additionally prenatally diagnosed, live-born infants did not undergo a procedure. Of the 56 procedures, 2 were by interventional catheterization, and 54 underwent a surgical repair. Prenatal detection for those undergoing a procedure statistically significantly increased over the 5 years from 38% to 82%, rho = 0.95 (p = .04)., Conclusions: Currently in Nevada, our prenatal detection rate is >80% in the general population for those between 0 and 6 months old who require a therapeutic procedure for aortic arch obstruction without intracardiac malformations., (© 2022 Wiley Periodicals LLC.)

Objective: The purpose of this study was to evaluate our institution's 16-year arterial switch operation (ASO) experience and to determine early and late mortality and late morbidity, as well as the need for reoperation and catheter intervention, and finally, to explore risk factors for late complications and reintervention., Methods: The clinical data of 185 transposition of the great arteries (TGA) patients who received ASO treatment in our center from January 2006 to January 2022 were continuously included for retrospective study., Results: There were 13 early deaths (7.03%), 5 late deaths (3.01%), and 6 lost to follow-up. The median follow-up time for the 166 hospitalized survivors was 88.5 (2190) months. Moderate or above new aortic valve regurgitation (NAR; in this article, NAR represents moderate or greater reflux unless otherwise specified) occurred in 19 cases (11.45%), and aortic root dilation (ARD) occurred in 28 cases (16.87%). Late right ventricular outflow tract obstruction (RVOTO) occurred in 33 cases (19.88%). There were 18 patients (10.84%) who underwent late re-intervention, and the most common indication for intervention was RVOTO, followed by recurrent aortic coarctation in patients undergoing concurrent arch repair and NAR or ARD. Receiver operating characteristics analysis found that NAR had the strongest predictive power for ARD, followed by RVOTO, followed by bicuspid native pulmonary valve (BPV), and aorto-pulmonary diameter mismatch (APDMM) was the weakest. Multivariate analysis showed that APDMM, previous pulmonary artery banding (PAB), and mild NAR at discharge were independent risk factors for late NAR and ARD. Low surgical weight was an independent risk factor specific to NAR, and BPV was an independent risk factor specific to ARD. Older surgical age and ARD were independent risk factors for late RVOTO. Older surgical age, operation before 2014, late RVOTO, and late ARD were independent risk factors for late intervention. No reintervention events for coronary dysfunction were found in the late stage, but one patient occurred myocardial infarction due to coronary embolism after reoperation., Conclusion: Early and late survival rates after ASO in TGA patients have been remarkably improved in recent decades. Increased rates of NAR, ARD, recurrent coarctation of the aorta, and RVOTO as children age are major future outcomes of concern and may imply more late reinterventions. Careful follow-up of neo-aortic valve and root function is imperative, especially in patients with APDMM, previous PAB, mild NAR at discharge, low surgical weight, and BPV structures., (© 2022 Wiley Periodicals LLC.)

The objective of this study was to define the frequency of the bovine aortic arch in patients with coarctation using the tomographic studies. This is a descriptive retrospective study involving analysis of reports of computed tomographic angiography done at the Prince Sultan Cardiac center for 700 children with congenital heart diseases over a span of about 10 years from April 1, 2008, to August 1, 2018. Cases with coarctation of aorta were chosen to determine the frequency of the bovine aortic arch using the tomographic studies. From a total of 700 cases which underwent computed tomographic angiography, 117 (16.71%) were diagnosed with coarctation and 19 (2.71%) had bovine arch. Among the 117 patients with coarctation, the total number of patients with bovine arch was 7 patients representing 5.98%, while in patients without coarctation we found a total number of bovine arches of 12 out of 583 patients representing 2.06%. Patients having coarctation with normal branching pattern were at a slight increased incidence of atrial septal defect, ventricular septal defect and anomalous pulmonary venous return than patients having coarctation with bovine arch. In the face of increased incidence of bovine aortic arch in patients with coarctation, we recommend multislice computed tomographic angiography as a noninvasive and potentially safe and accurate method to precisely delineate the branching pattern of the arch of aorta in patients with coarctation before the interventional procedures and surgeries., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objective: Patients with previously repaired aortic coarctation (CoA) are at risk of developing late surgical complications. Many patients with CoA also have a bicuspid aortic valve (BAV). We sought to determine in patients with repaired CoA whether the presence of BAV is associated with more cardiovascular reinterventions during follow-up., Methods: Adults with previously repaired simple CoA were recruited from our Adult Congenital Heart Disease database (Sydney, Australia). The incidence of complications relating to the 'CoA-site' (descending aortic aneurysm or dissection, or recoarctation) and the 'AV/AscAo' (aortic valve or ascending aortic pathology) that required intervention was compared between those with BAV ('CoA-BAV') and without BAV ('CoA-only')., Results: Of 146 patients with repaired CoA, 101 (69%) had BAV. Age at CoA repair was similar (median 6.0 (IQR 0.5-14.0) years vs 5.0 (IQR 0.5-11.0) years, p=0.44), as was the distribution of repair types, with end-to-end repair the most common in both groups (45.9% vs 45.6%). At a median of 28 years following initial repair, a significantly higher proportion of patients with CoA-BAV required cardiovascular reintervention (45.5% vs 20.0%, p=0.003). Whereas 'CoA-site' complications were more common than 'AV/AscAo' complications in patients with CoA only (13.3% and 0%, respectively), patients with CoA-BAV had a high prevalence of both 'CoA-site' as well as 'AV/AscAo' complications (19.8% and 21.8%, respectively). Overall survival was similar (p=0.42)., Conclusion: In adults with repaired CoA, patients with CoA-BAV are more than twice as likely to require cardiovascular reintervention by early-to-mid-adult life compared with those with CoA alone. Despite this, no difference in survival outcomes was observed., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: Aortic coarctation is the most frequent structural anomaly out of congenital heart diseases. This congenital defect is an important cause of death worldwide. We sought to determine the prevalence of aortic coarctation in Colombia and whether new policies have had an impact on its diagnosis., Methods: In this study information from the Bogotá birth defect surveillance program during the years 2001-2018 from 63 hospitals was used. 537,026 live births of any weight and stillbirths of any weight were analyzed. The information was stored in a database on the servers of the Health Secretariat and the Pontificia Universidad Javeriana. We analyzed the presence of aortic coarctation according to the newborn's sex, weight, size, mother's age, and gestational age at the time of birth and when coarctation is accompanied by other types of congenital malformations., Results: The prevalence of aortic coarctation in Bogotá during the years 2001-2018 found in this study was 1.25 in 10,000 live births. We also found that prevalence of aortic coarctation in Bogotá changes throughout the years having a significant increase in the year 2018 with 6.57 cases in 10,000 live births., Conclusions: This prevalence is higher than the one found in a study with data from 2001 to 2014, which suggests an improvement in the country's epidemiological surveillance and medical training. However, the prevalence found in Bogotá is still lower compared to the prevalence worldwide and from other continents, the prevalence for Latinamerica was significantly lower as compared to those in Asia, Europe, and United States so we emphasize the importance of continuing with improvements, such as standardizing screening methods and sensitivity of said methods in a local scale as well as a continental scale.

Background: The relationship between office vs. ambulatory blood pressure (BP) indices are well-studied in patients with essential hypertension and based on these data, it is known that the average 24-h ambulatory BP is typically lower than office BP. However, emerging data show that office SBP underestimates arterial afterload in patients with coarctation of aorta (COA), and a minimal increase in stroke volume during low-intensity exercise results in an exaggerated rise in SBP as compared to those with essential hypertension. We hypothesized that COA patients will have higher ambulatory SBP and a higher prevalence of masked hypertension compared to patients with essential hypertension., Methods: Case-control study of 118 COA patients and 118 patients with essential hypertension matched by age, sex, BMI and office SBP., Results: Although both groups had similar office SBP (132 ± 17 mmHg) by design, the COA group had paradoxical increases in 24-h ambulatory SBP (135 ± 14 vs. 126 ± 13; P < 0.001) and daytime ambulatory SBP (142 ± 16 vs. 130 ± 13; P < 0.001), and less nocturnal dipping (-3 ± 5 vs. -9 ± 4; P < 0.001). The COA group also had a higher prevalence of masked hypertension [36 (31%) vs 14 (12%); P < 0.001), and worse arterial function indices., Conclusion: These findings underscore the potential limitations of relying on office SBP for screening/monitoring of hypertension in COA and potential pitfalls in extrapolating essential hypertension guidelines recommendations to the treatment of COA. It also provides the rationale for further studies to determine if pharmacologic BP interventions guided by ambulatory BP data will improve clinical outcomes in the COA population., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Chronic elevation of left heart filling pressure causes pulmonary vascular remodeling, pulmonary hypertension, and right heart dysfunction. Although diastolic dysfunction is relatively common in patients with coarctation of aorta, there are limited data about the prevalence and prognostic implications of pulmonary hypertension and right heart dysfunction in this population. The purpose of the study was to assess right heart function and hemodynamics in patients with coarctation of aorta and to determine the relationship between right heart indices and cardiovascular events defined as heart failure hospitalization, heart transplant, or cardiovascular death., Methods: Right heart structure, function, and hemodynamics were assessed with these indices: right atrial volume, right atrial pressure, right atrial reservoir strain, right ventricular global longitudinal strain, right ventricular end-diastolic area, right ventricular systolic pressure, and tricuspid regurgitation severity. Right heart hemodynamic score, range 0 to 5, was generated based on the correlation between the right heart indices and cardiovascular events, using half of the cohort (derivation cohort, n=411), and then tested on the validation cohort (n=410). The goodness of fit and discrimination power was compared using C statistics and risk score., Results: The median follow-up in the derivation cohort was 8.2 (4.0-11.1) years, and 59 (14%) patients had cardiovascular events during this period. Right heart hemodynamic score was independently associated with cardiovascular events (hazard ratio, 1.64 [95% CI, 1.38-2.17]) for every unit increase in right heart hemodynamic score after adjustment for clinical and echocardiographic indices (C statistic, 0.718 [95% CI, 0.682-0.746]). The right heart hemodynamic score was also independently associated with cardiovascular events in the validation cohort (C statistic, 0.711 [95% CI, 0.679-0.741]). The C statistic difference (0.007 [95% CI, 0.014-0.022]) and risk score (0.86 [95% CI, 0.54-1.17]) suggest a good model fit., Conclusions: The current study underscores the prognostic importance of right heart dysfunction in patients with coarctation of aorta and suggests that right heart indices should be used for risks stratification in this population.

Background The 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 Results Using 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]). Conclusions This 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.

Background: Aortic coarctation (AoCoa) is a congenital stenosis of aorta, which often co-exists with other congenital heart defects. Many studies have shown the importance of regular follow-up in these patients however there is scarcity of knowledge relating to the impact of left ventricle inflow lesions (LVIT) and left ventricle outflow track lesions (LVOT). The aim of this study is to evaluate the impact of isolated AoCoa with LVIT and/or LVOT on haemodynamic, morbidity and mortality., Methods: We have retrospectively analysed clinical data of all adult AoCoa patients who underwent echocardiography between 2010-2018 in our centre. Outcome measures included death, number of hospitalisations for cardiac causes, development of cardiac arrhythmia, new prescription of HF medication., Results: A total of 406 AoCoa patients were included and were followed for a median 4.2 years. At baseline, 38% patients had AoCoa alone, 54% patients had LVOT, 3% patients had LVIT, and 5% patients had mixed LVIT and LVOT, including patients with Shone syndrome. Patients with mixed LVIT and LVOT had the highest mortality of the four groups and the highest heart failure-related morbidity. Moreover, they were the most prone to have a higher indexed LA volume compared to patients with no LVOT (p=0.0001). During follow-up, 13 patients died, of which 21% patients were from the mixed LVIT and LVOT group., Conclusions: AoCoa patients with a combination of LVIT and LVOT including Shone complex are associated with a significantly higher morbidity and mortality compared to AoCoa alone., (Copyright © 2021. Published by Elsevier B.V.)

Background: Left ventricular non-compaction has been associated with heart failure, arrhythmia, thromboembolism and sudden death. The prevalence of non-compaction in patients with coarctation of the aorta and its clinical significance remains unknown, although obstructive left heart disease is common in patients with non-compaction. We sought to evaluate the prevalence of left ventricular non-compaction in patients with repaired aortic coarctation as well as its effect on left ventricular size and systolic function., Methods and Results: In total, 268 patients (Mean age 26 (inter-quartile range 21-37) years, 63% male) undergoing cardiac magnetic resonance imaging for clinical follow-up were included from three tertiary centres for adult congenital heart disease. Clinical data was obtained from medical records and correlated with ventricular volumes and function. Left ventricular non-compaction was defined as a diastolic non-compacted:compacted dimension ratio >2.3 in the worst affected segment on a long-axis view. Left ventricular non-compaction was present in 8.2% of patients with repaired coarctation. Left ventricular end-diastolic volumes and stroke volumes were significantly higher in patients with non-compaction compared to those without. There were no significant differences in ventricular mass or ejection fraction in these two groups., Conclusions: Left ventricular non-compaction is relatively common in patients with repaired coarctation of the aorta and correlates with increased left ventricular end-diastolic volumes.

Turner syndrome (TS) is a rare disease (ORPHA #881) which affects about 50 in 100 000 newborn girls. Their karyotype shows a complete or partial loss of the second X chromosome. In TS, congenital cardiovascular malformations, such as bicuspid aortic valves and aortic coarctation are frequent, affecting 20-30% and 7-18% of the TS population, respectively. The morbidity and mortality of these patients are high and related to the presence of hypertension and/or aortic dilatation (40%), inducing aortic dissection. European guidelines published in 2017 have indicated how to monitor patients using magnetic resonance imaging (MRI) and/or echography. Different studies have shown that a cardiovascular lifelong follow-up is necessary and therefore education of patients with TS and their families represents a major issue. This review will present recent data concerning the progression of aortic diameters as well as current molecular knowledge of the cardiovascular system in patients with TS., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Background: Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients., Methods and Results: From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83])., Conclusions: Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure.

Background: Bicuspid aortic valve(BAV) is common. Some studies suggest that all BAV patients require screening for intracranial aneurysm(IA) in order to prevent sub-arachnoid hemorrhage(SAH). Aortic coarctation(CoA) carries high-risk of both IA and SAH. Using a nationally-representative population, we assessed the frequency of IA and SAH in admissions with BAV-without-CoA versus admissions with CoA(with or without BAV)., Methods: Between 2000 and 2016, adult admissions with a primary/secondary diagnosis of BAV and/or CoA were identified using the National Inpatient Sample. Admissions with traumatic SAH and inter-hospital transfers were excluded. Outcomes were frequency of IA and SAH, and in-hospital mortality in BAV-without-CoA versus CoA., Results: In this 17-year period, 254,675 admissions met inclusion criteria and 236,930(93.0%) had BAV-without-CoA. BAV-with-CoA was present in 2846(1.1%) and isolated-CoA in 14,899(5.9%), for a total of 17,745(7%) with CoA. IA was noted in 405 admissions(0.2%) overall, BAV-without-CoA versus CoA having 293(0.1%) versus 112(0.6%), p < 0.001. SAH was noted in 910 admissions(0.4%) overall, with BAV-without-CoA versus CoA having 760(0.3%) versus 150(0.9%), p < 0.001., Conclusions: In this study, BAV-without-CoA admissions had 0.1%(6-times lower than CoA) and 0.3%(3-times lower that CoA) IA and SAH, respectively, which is comparable to the general population. This suggests that BAV-without-CoA patients likely do not require routine surveillance for IA., Competing Interests: Declaration of Competing Interest None., (Copyright © 2021. Published by Elsevier B.V.)

Importance: The prevalence and characteristics of bicuspid aortic valve (BAV) are mainly reported from selected cohorts. BAV is associated with aortopathy, but it is unclear if it represents a fetal developmental defect or is secondary to abnormal valve dynamics., Objective: To determine the prevalence of BAV and BAV subtypes and to describe the associated aortopathy in a large, population-based cohort of newborns., Design, Setting, and Participants: The Copenhagen Baby Heart Study was a cross-sectional, population-based study open to all newborns born in Copenhagen between April 1, 2016, and October 31, 2018. Newborns with BAV were matched 1:2 to newborns with a tricuspid aortic valve (non-BAV group) on sex, singleton/twin pregnancy, gestational age, weight, and age at time of examination., Exposures: Transthoracic echocardiography within 60 days after birth., Main Outcomes and Measures: Primary outcome was BAV prevalence and types, ie, number of raphes and spatial orientation of raphes or cusps (no raphes), according to the classification system of Sievers and Schmidtke (classified as type 0, 1, or 2, with numbers indicating the number of raphes). Secondary outcome was valve function and BAV-associated aortopathy, defined as aortic diameter z score of 3 or greater or coarctation., Results: In total, 25 556 newborns (51.7% male; mean age, 12 [SD, 8] days) underwent echocardiography. BAV was diagnosed in 196 newborns (prevalence, 0.77% [95% CI, 0.67%-0.88%]), with male-female ratio 2.1:1. BAV was classified as type 0 in 17 newborns (8.7% [95% CI, 5.5%-13.5%]), type 1 in 178 (90.8% [95% CI, 86.0%-94.1%]) (147 [75.0% {95% CI, 68.5%-80.5%}] right-left coronary raphe, 27 [13.8% {95% CI, 9.6%-19.3%}] right coronary-noncoronary raphe, 4 [2.0% {95% CI, 0.8%-5.1%}] left coronary-noncoronary raphe), and type 2 in 1 (0.5% [95% CI, 0.1%-2.8%]). Aortic regurgitation was more prevalent in newborns with BAV (n = 29 [14.7%]) than in those without BAV (1.3%) (absolute % difference, 13.4% [95% CI, 7.8%-18.9%]; P < .001). Newborns with BAV had higher flow velocities across the valve (0.67 [95% CI, 0.65-0.69] m/s vs 0.61 [95% CI, 0.60-0.62] m/s; mean difference, 0.06 m/s [95% CI, 0-0.1]) and larger aortic root and tubular ascending aortic diameters than those without BAV (10.7 [95% CI, 10.7-10.9] mm vs 10.3 [95% CI, 10.2-10.4] mm; mean difference, 0.43 mm [95% CI, 0.2-0.6 mm] and 9.8 [95% CI, 9.6-10.0] mm vs 9.4 [95% CI, 9.3-9.5] mm; mean difference, 0.46 mm [95% CI, 0.30-0.70], respectively) (P < .001 for all). Aortopathy was seen in 65 newborns (33.2%) with BAV (62 with aortic z score ≥3; 3 with coarctation)., Conclusions and Relevance: Among newborns in Copenhagen, the prevalence of BAV was 0.77%. Aortopathy was common in newborns with BAV, suggesting that it also represents a fetal malformation.

Background: A 10% prevalence of intracranial aneurysms in patients with coarctation of the aorta has been described in a few studies. Our objective is to describe the rate of intracranial aneurysm detection in patients with coarctation of the aorta in the current era. We hypothesise that, with earlier detection and coarctation of the aorta intervention, the rate of intracranial aneurysm is lower than previously reported and screening imaging may only be warranted in older patients or patients with certain risk factors., Methods: This is a retrospective study of 102 patients aged 13 years and older with coarctation who underwent brain computed tomography angiography, magnetic resonance imaging (MRI), or magnetic resonance angiography between January, 2000 and February, 2018., Results: The median age of coarctation repair was 4.4 months (2 days-47 years) and the initial repair was primarily surgical (90.2%). There were 11 former smokers, 4 current smokers, and 13 patients with ongoing hypertension. Imaging modalities included computed tomography angiography (13.7%), MRI (41.2%), and magnetic resonance angiography (46.1%), performed at a median age of 33.3 years, 22.4 years, and 25 years, respectively. There were 42 studies performed for screening, 48 studies performed for neurologic symptoms, and 12 studies performed for both screening and symptoms. There were no intracranial aneurysms detected in this study., Conclusions: These results suggest that the rate of intracranial aneurysms may be lower than previously reported and larger studies should explore the risk of intracranial aneurysms in coarctation of the aorta in the current era.

Objective: We sought to describe patient characteristics associated with prolonged post-operative length of stay in a contemporary cohort of infants who underwent isolated repair of aortic coarctation., Methods: We reviewed patients less than 1 year of age who underwent isolated repair of aortic coarctation at our institution from 2009 to 2016. Prolonged post-operative length of stay was defined as length of stay within the upper tertile for the cohort. Bivariate and multi-variable analyses were performed to determine independent risk factors for prolonged length of stay., Results: We reviewed 95 consecutive patients who underwent isolated repair of aortic coarctation, of whom 71 were neonates at the time of diagnosis. The median post-operative length of stay was 6.5 days. The upper tertile for post-operative length of stay was greater than 10 days; 32 patients within this tertile and 1 patient who died at 8.5 days after surgery were analysed as having prolonged post-operative length of stay. In a multi-variable analysis, pre-maturity (odds ratio: 3.5, 95% confidence interval: 1.2, 10.7), genetic anomalies (odds ratio: 4.7, 95% confidence interval: 1.2, 18), absence of pre-operative oral feeding (odds ratio: 7.4, 95% confidence interval: 2.4, 22.3), and 12-hour vasoactive-ventilation-renal score greater than 25 (odds ratio: 7.4, 95% confidence interval: 1.9, 29) were independently associated with prolonged length of stay., Conclusions: In neonates and infants who underwent isolated repair of aortic coarctation, pre-maturity, genetic anomalies, lack of pre-operative oral feedings, and 12-hour vasoactive-ventilation-renal score more than 25 were independent risk factors for prolonged post-operative length of stay. Further study on the relationship between pre-operative oral feedings and post-operative length of stay should be pursued.

Background: This study examines the contemporary medium- and long-term outcomes of endovascular repair of aortic coarctation in the adult., Methods: We reviewed the clinical and imaging data of 56 consecutive adult patients with aortic coarctation who underwent endovascular repair at the Mazankowski Alberta Heart Institute, Edmonton, Alberta, Canada, from 2003 to 2018., Results: There were 20 (35.7%) female and 36 (64.3%) male patients (including 9 re-intervention cases) with a mean age of 33.6 ± 13.6 years. Thirty-seven (66.1%) were treated with balloon-expandable covered stent and 12 (21.4%) were treated with balloon-expandable bare-metal stent. Pressure gradients decreased from baseline level of 27.99 ± 12.75 (8-70) mm Hg to 5.33 ± 4.42 (0-17.5) mm Hg following the procedure. There were 2 (3.6%) procedure related complications (aortic dissection [n = 1] and stent malposition [n = 1]). During a median (Q1 - Q3) follow up of 5.36 (2.28-7.58) years, 2 deaths (4.2%) and 9 (19%) re-interventions occurred, and the overall survival was 95.8%., Conclusion: Percutaneous coarctoplasty, with either covered or bare metal stents, is a safe and durable option for aortic coarctation repair with excellent long-term survival., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Patients 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 Results: We 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., Conclusions: Neurological 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., (Copyright © 2020. Published by Elsevier B.V.)

Objective: Coarctation of the aorta can be treated surgically or with balloon angioplasty. The objective of our study was to describe the results after percutaneous balloon angioplasty with or without stent implantation for coarctation of the aorta and establish the incidence of recovery during follow-up., Method: Cohort study. 89 patients of any age where included in a follow up period of nine years., Results: Of the 89 patients included in the study, 69.0% were male. The mean follow-up for all participants was 33.66 months. 32.5% of the patients had a stent implanted during the angioplasty procedure; of which 24.1% suffered recoarctation during follow-up. In the group without stent implantation, 36.6% suffered recoarctation. There was not significant difference in the survival curves of the two groups (p = 0.899)., Conclusions: Stent implantation during balloon angioplasty to treat aortic coarctation did not influence in the incidence of aortic recoarctation; but factors such as preangioplasty arterial hypertension and the final angioplasty gradient > 20 mmHg is associated with aortic recoarctation., (Copyright: © 2021 Permanyer.)

Infants with aortic coarctation may present with left ventricular (LV) dysfunction which may complicate the postoperative course and lead to increased healthcare costs. We aimed to define the prevalence of moderate to severe left ventricular (LV) systolic dysfunction, evaluate time to recovery, and compare health care costs. Single-center retrospective cohort study at a tertiary care hospital was conducted. Infants < 6 months old at diagnosis with aortic coarctation were identified using surgical codes for coarctation repair between January 2010 and May 2018. Moderate to severe dysfunction was defined as ejection fraction (EF) < 40%. Of 160 infants studied, 18 (11%) had moderate to severe LV dysfunction at presentation. Compared to those with better LV function, infants with moderate to severe LV dysfunction were older at presentation (12 vs. 6 days, p = 0.004), had more postoperative cardiac intensive care unit (ICU) days (5 vs. 3, p < 0.001), and more ventilator days (3.5 vs. 1, p < 0.001). The median time to normal LV EF (≥ 55%) was 6 days postoperatively (range 1-230 days). Infants presenting with moderate to severe LV dysfunction had higher index hospitalization costs ($90,560 vs. $59,968, p = 0.02), but no difference in cost of medical follow-up for the first year following discharge ($3,078 vs. $2,568, p = 0.46). In the current era, > 10% of infants with coarctation present with moderate to severe LV dysfunction that typically recovers. Those with moderate to severe dysfunction had longer duration of mechanical ventilation and postoperative cardiac ICU stays, likely driving higher costs of index hospitalization.

Objectives: Prenatal diagnosis of coarctation of the aorta (CoA) is associated with reduced mortality and morbidity, however, accurate prenatal prediction remains challenging. To date, studies have used retrospective measurements of the outflow tracts to evaluate their potential to predict CoA. Our primary objective was to evaluate prospectively acquired measurements of the outflow tracts in fetuses with prenatally suspected CoA. A secondary aim was to report the postnatal prevalence of bicuspid aortic valve in this cohort., Methods: Pregnancies with suspicion of isolated CoA and with a minimum of 6 months' postnatal follow-up available were identified from the cardiac database of a tertiary fetal cardiology center in the UK, between January 2002 and December 2017. Measurement of the aortic valve, pulmonary valve, distal transverse aortic arch (DTAA) and arterial duct (AD) diameters were undertaken routinely in fetuses with suspected CoA during the study period. Z-scores were computed using published reference ranges based on > 7000 fetuses from our own unit., Results: Of 149 pregnancies with prenatally suspected CoA included in the study, CoA was confirmed within 6 months after birth in 77/149 (51.7%) cases. DTAA diameter Z-score and the Z-score of second-trimester DTAA/AD diameter ratio were smaller in fetuses with postnatally confirmed CoA than those in false-positive cases (-2.8 vs -1.9; P = 0.039 and -3.13 vs -2.61; P = 0.005, respectively). Multiple regression analysis demonstrated that the Z-scores of DTAA and AD diameters were the only significant predictors of postnatal CoA (P = 0.001). Bicuspid aortic valve was identified in 30% of the false-positive cases., Conclusions: Measurement of DTAA and AD diameter Z-scores can be used to ascertain risk for postnatal CoA in a selected cohort. The high incidence of bicuspid aortic valve in false-positive cases merits further study with respect to both etiology and longer-term significance. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.)

Background: This study aimed to evaluate hemodynamic phenotypes and prevalence of left ventricular hypertrophy in children after coarctation repair with right arm and leg blood pressure difference < 20 mmHg. Secondary objectives were analysis of effects of age at intervention, residual gradient across the descending aorta, and type of correction., Methods: Blood pressure status and left ventricular hypertrophy were diagnosed according to European Society of Hypertension 2016 guidelines., Results: Of 90 patients with a median age 12.5 (8.9-15.8) years, 8.5 (6.0-11.8) years after coarctation repair who were included, 42 (46.7%) were hypertensive. Isolated systolic hypertension dominated among 29 hypertensive patients with uncontrolled or masked hypertension (25 of 29; 86.2%). Of the 48 patients with office normotension, 14.6% (7) had masked hypertension, 8.3% (4) had ambulatory prehypertension, and 54.2% (26) were truly normotensive. Left ventricular hypertrophy was diagnosed in 29 patients (32.2%), including 14 of 42 (33.3%) hypertensive and 15 of 48 (31.3%) normotensive patients. The peak systolic gradient across the descending aorta was greater in hypertensive subjects (33.3 ± 12.7 mmHg) compared with normotensive subjects (25 ± 8.2 mmHg, p = 0.0008). Surgical correction was performed earlier than percutaneous intervention (p < 0.0001) and dominated in 40 of 48 (83.3%) normotensive versus 24 of 42 (57.1%) hypertensive patients (p = 0.006)., Conclusions: Arterial hypertension with isolated systolic hypertension as the dominant phenotype and left ventricular hypertrophy are prevalent even after successful coarctation repair. Coarctation correction from the age of 9 years and older was associated with a higher prevalence of hypertension.

Objectives: To evaluate short- and middle-term outcomes after transcatheter patent ductus arteriosus (TC-PDA) closure in small infants, specifically device-related left pulmonary artery (LPA) stenosis and aortic coarctation, risk factors, and changes over time., Background: Recent studies have demonstrated successful transcatheter PDA (TC-PDA) closure in small infants. LPA stenosis and aortic coarctation have been seen after TC-PDA, but it is not clear whether device-related LPA/aortic obstruction persists., Methods: A single-center retrospective study of infants ≤4 kg who underwent TC-PDA closure from February 1, 2007 to September 1, 2018 was performed, evaluating the incidence and risk factors for LPA stenosis and coarctation., Results: Forty-four patients underwent successful TC-PDA with Amplatzer Vascular Plug II (AVPII; n = 30), Amplatzer Duct Occluder II-Additional Sizes (n = 10), Amplatzer Duct Occluder I (n = 3), and coil-filled AVPI (n = 1) devices, all via an antegrade approach. Median birthweight and procedural weight were 890 g (range: 490-3,250) and 2.8 kg (range: 1.2-4.0), respectively. Median follow-up was 0.7 years (range: 2 days-7 years). Thirty-eight patients had post-procedure echocardiograms assessing LPA/aortic obstruction. Of those, 17 had LPA flow acceleration/stenosis (≥1.5 m/s), which improved or resolved in all patients with available follow-up; 3 developed mild coarctation (>2 m/s), which improved in the two with more than short-term follow-up; 4 developed mild flow acceleration (1.5-2 m/s) in the descending aorta, which resolved in three and increased in one (2.4 m/s). Flow acceleration in the LPA was associated with younger procedural age, larger PDA minimal diameter, and placement of a device other than the AVPII. There was no device-related mortality or need for reintervention., Conclusion: TC-PDA in small infants is effective, without significant complications. Device-related LPA/aortic obstruction can improve with time/growth., (© 2020 Wiley Periodicals, Inc.)

Objective: Cardiovascular morbidity is high in patients with coarctation of aorta even after successful repair. This study aimed to assess the frequency of late hypertension and the relationship between ambulatory hypertension and cardiovascular target organ damage in children and adolescents after early and successful repair of coarctation of the aorta., Methods: Twenty-five children and adolescents (mean age 13.5 ± 3.43 years) with repaired coarctation of the aorta (median age at repair 4 months, arm-leg gradient <20 mmHg) and 16 healthy controls were included. Office and ambulatory blood pressure, pulse wave velocity, and left ventricular mass index were assessed., Results: Both day- and night-time systolic blood pressure standard deviation score and left ventricular mass index were significantly higher in patients compared to controls (p ≤ 0.001 for all), whereas pulse wave velocity did not differ. The prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy were 40, 28, and 24%, respectively. Left ventricular mass index was higher in patients with sustained hypertension, masked hypertension, and normotension compared to controls (p < 0.05). In multivariate analysis, higher night-time systolic blood pressure standard deviation score was the only independent predictor of left ventricular mass index., Conclusion: The present study reveals a high prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy in children and adolescents with coarctation of the aorta even after early and successful repair. Ambulatory blood pressure monitoring should be considered to diagnose hypertension. All coarctation of aorta patients should be followed up lifelong and encouraged to establish a healthy lifestyle starting from childhood.

Objectives: (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., Methods: 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., Results: 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., Conclusion: 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., (© 2020 John Wiley & Sons, Ltd.)

Objective: To determine if there is an association between aortic coarctation (CoA) and intraventricular hemorrhage (IVH) in low birth weight (LBW) infants that may justify earlier intervention. While there is an increasing number of reports of successful early CoA intervention in LBW infants, there are no data to justify this approach. Material and methods: Retrospective review of the University Health System Consortium Clinical Data Base/Resource Manager, a national hospital discharge database. LBW (≤2 kg) infants, with and without IVH and isolated CoA were identified; IVH was stratified into low (grade 1 or 2) and high (grade 3 or 4) severity. Odds ratios were calculated for any, low and high-grade IVH with CoA. Results: Forty-six thousand and twenty LBW infants were identified; 3716 (8.1%) had IVH, 3001 (81%) with low, and 724 (19%) with high severity. Sixty-four infants had CoA, 13 had associated IVH (10 with low and three with high severity). The odds ratio for any IVH with CoA was 2.91 (95% CI 1.58-5.35), low severity 2.77 (95% CI 1.41-5.46) and high severity 3.45 (95% CI 1.07-11.07). Conclusions: In conclusion, this large retrospective database review found that LBW infants with CoA may be at increased risk of IVH. Further study is needed to determine if earlier catheter- or surgical-based intervention for CoA could reduce the risk of IVH in this population.

Objective: We aimed to investigate the combined effects of arterial hypertension, bicuspid aortic valve disease (BAVD) and age on the distensibility of the ascending and descending aortas in patients with aortic coarctation., Design: Cross-sectional study., Setting: The study was conducted at two university medical centres, located in Berlin and London., Participants: A total of 121 patients with aortic coarctation (ages 1-71 years) underwent cardiac MRI, echocardiography and blood pressure measurements., Outcome Measures: Cross-sectional diameters of the ascending and descending aortas were assessed to compute aortic area distensibility. Findings were compared with age-specific reference values. The study complied with the Strengthening the Reporting of Observational Studies in Epidemiology statement and reporting guidelines., Results: Impaired distensibility (below fifth percentile) was seen in 37% of all patients with coarctation in the ascending aorta and in 43% in the descending aorta. BAVD (43%) and arterial hypertension (72%) were present across all ages. In patients >10 years distensibility impairment of the ascending aorta was predominantly associated with BAVD (OR 3.1, 95% CI 1.33 to 7.22, p=0.009). Distensibility impairment of the descending aorta was predominantly associated with arterial hypertension (OR 2.8, 95% CI 1.08 to 7.2, p=0.033) and was most pronounced in patients with uncontrolled hypertension despite antihypertensive treatment., Conclusion: From early adolescence on, both arterial hypertension and BAVD have a major impact on aortic distensibility. Their specific effects differ in strength and localisation (descending vs ascending aorta). Moreover, adequate blood pressure control is associated with improved distensibility. These findings could contribute to the understanding of cardiovascular complications and the management of patients with aortic coarctation., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background Because of the increasing numbers of congenital patients surviving into adulthood, early diagnosis and prevention of acquired cardiovascular disease is reasonable. The aim of this study was to detect diagnostic subgroups of adults with congenital heart disease (ACHD) that have increased carotid intima-media thickness (cIMT), a subclinical marker of cardiovascular damage. Methods and Results This study enrolled 831 ACHD patients (392 women, aged 38.8±11.7 years) from May 2015 to February 2019 at their regular outpatient visit. Far wall cIMT was measured using a semiautomatic ultrasound system at 4 angles. Age, sex, height, weight, blood pressure, smoking status, and antihypertensive medication were registered and entered in a multiple linear regression model to compare diagnostic subgroups to 191 healthy controls (111 women, aged 36.7±13.5 years). There were no significant differences in cIMT of ACHD (0.538±0.086 mm) compared with healthy controls (0.541±0.083 mm; P =0.649) after adjusting for the aforementioned covariates. Only patients with coarctation of the aorta showed significantly higher cIMT values (0.592±0.075 mm; P <0.001) compared with healthy controls. In addition, ACHD patients who were men ( P =0.032), older ( P <0.001), and were prescribed antihypertensive medications ( P =0.003) were all found to have thicker cIMT values. Conclusions Overall, we determined that within the ACHD cohort, only those patients with a history of coarctation have higher cIMT values. To better determine the mechanism of abnormal vasculature, further basic research is needed.

Background: PHACE syndrome is a rare inborn condition characterized by large facial hemangiomas and variable malformations of the arterial system, heart, central nervous system, and eyes. According to Orphanet estimates, the prevalence is <1.0 per million. Data from Europe are limited to small case series, and there are no population-based data available., Objectives: We conducted the present study to provide population-based estimates of the disease prevalence of PHACE syndrome in children in Germany, Switzerland, and Austria. We compared these first systematic data on PHACE syndrome from Europe to published data from the PHACE Syndrome International Clinical Registry and Genetic Repository (USA). Clinical features in our cohort with PHACE syndrome were assessed in detail, including the need for early supportive measures., Methods: We used a population-based approach by means of a previously well-established network of child neurologists from Germany, Switzerland, and Austria ("ESNEK") to identify potential patients. The patients' guardians and child neurologists were asked to fill in questionnaires developed in collaboration with the International PHACE Registry., Results: We identified 19 patients with PHACE syndrome. Estimated prevalence rates were 6.5 per million in Switzerland, 0.59 per million in Germany, and 0.65 per million in Austria. A subset of 10 patients from Germany and Switzerland participated in our study, providing detailed clinical assessment (median age: 2.5 years; 9 females, 1 male). Cerebrovascular involvement was frequent (80%). Facial hemangioma extent correlated significantly with the number of organs involved (p = 0.011). In 9 out of 10 patients, facial hemangiomas were treated successfully with oral propranolol. Baseline demographic data as well as the rate of cerebrovascular and cardiovascular anomalies were in line with those from the US International PHACE Registry and other published PHACE cohorts., Conclusions: Our study provides population-based estimates for PHACE syndrome in 3 German-speaking countries. The data from Switzerland indicate that PHACE syndrome may be more prevalent than demonstrated by previous reports. Underreporting of PHACE syndrome in Germany and Austria likely accounts for the differences in prevalence rates. The clinical observation of a potential association between the size of facial hemangioma and extent of organ involvement warrants further investigation., (© 2020 S. Karger AG, Basel.)

Exercise-induced hypertension is a predictor of cardiovascular events in patients with coarctation of aorta (COA). However, it is unclear whether mild COA diagnosis is an independent risk factor of exercise-induced hypertension. We hypothesized that for every unit increase in exercise, patients with COA (without hemodynamically significant coarctation) will have a higher rise in systolic blood pressure (SBP) compared with matched controls. One hundred forty-nine patients with COA (aortic coarctation peak velocity <2 m/s) who underwent exercise testing were matched 1:1 to controls using propensity score method based on age, sex, body mass index, hypertension diagnosis, and SBP at rest. We compared exercise-induced change in SBP between patients with COA and controls and also assessed the correlation between Doppler-derived aortic vascular function indices (effective arterial elastance index and total arterial compliance index) and exercise-induced changes in SBP. Compared with controls, patients with COA had a greater change in SBP per unit metabolic equivalent (β=2.86; 95% CI, 1.96-4.77 versus 1.07, 95% CI, -0.15 to 1.75; P =0.018) and per unit oxygen pulse (β=4.57; 95% CI, 2.97-7.12 versus 1.45, 95% CI, -0.79 to 2.09, P <0.001). There was a correlation between SBP peak -SBP rest and elastance index ( r =0.38, P =0.032) and between SBP peak -SBP rest and total arterial compliance index ( r =-0.51, P =0.001), suggesting an association between vascular dysfunction and exercise-induced BP changes. Patients with COA, without significant obstruction, had higher exercise-induced changes in SBP after adjustment for other risk factors for hypertension. Considering the already known prognostic importance of exercise-induced hypertension, the current study highlights the potential role of exercise testing for risk stratification of patients with mild COA.

Objectives: Coarctation of the aorta (CoA) is rarely diagnosed and treated in adults and nowadays mostly treated with percutaneous techniques. The objective of this study is to report the long-term outcomes and health-related quality of life in a unique cohort of patients treated with an open surgical repair for their primary adult CoA., Methods: Ninety adult patients underwent primary surgical repair of CoA at our tertiary referral centre between 1961 and 2008 when the treatment strategy for adult CoA was exclusively surgical., Results: The median age at surgery was 24 years (interquartile range 20-36 years), and 39 patients (43%) were asymptomatic at presentation. CoA was located paraductally in most patients (64%), and bicuspid aortic valve was present in 39 (43%) patients. Surgical reconstruction of CoA with an end-to-end anastomosis was performed in majority of the patients (57%). Overall, in-hospital mortality occurred in 1 patient (1%). There was no in-hospital stroke, spinal cord ischaemia, renal replacement therapy or respiratory failure. The cumulative survival was 97.7%, 89.5%, 82.6%, 70.9% and 61.4% at 10, 20, 30, 40 and 50 years, respectively. Thirty-one patients (34%) required an additional cardiac surgery during follow-up. The majority of patients (77%) suffered from refractory hypertension even after decades of surgery. Compared with the matched Dutch population, patients reported a lower social functioning, mental health, vitality and general health with a higher body pain., Conclusions: Patients with native adult CoA have low in-hospital morbidity and mortality when treated with an open surgical reconstruction. However, refractory hypertension and impaired quality of life remain important challenges during follow-up., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: 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)., Methods: 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., Results: 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., Conclusion: 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.

Objectives: The aim of the present study was to evaluate procedural risk factors, incidence and timing of reintervention because of recurrent aortic coarctation in children., Methods: The study cohort consisted of 304 patients with isolated coarctation: 251 underwent surgery and 53 were treated percutaneously (40 balloon angioplasty, 13 stent) at the Helsinki Children's Hospital in 2000-2012. Characteristics, intervention and reintervention data were retrospectively collected from clinical records until 2014 (median follow-up 7.9 years). Age- and sex-matched comparisons between the treatment groups were performed in 86 patients (surgery n = 43, percutaneous n = 43)., Results: Forty of the 251 (16%) patients after surgery, 9/40 (23%) patients after balloon angioplasty and 4/13 (31%) patients after stent placement underwent a reintervention after a median time of 3.4, 11.7 and 19.5 months (P < 0.05), respectively. In the surgery group, all reinterventions occurred in children operated on ≤12 months of age and were related to lower body weight and smaller dimensions of the aorta. In the balloon angioplasty group, a higher post-procedure systolic arm-leg blood pressure gradient was associated with reintervention. After stent placement, three-fourths of the reinterventions were performed in a planned postinterventional catheterization. In the age- and sex-matched comparisons (median 5,7 years, range 0,5-17,6), post-procedure blood pressure gradients were higher (mean 10 vs 4 mmHg, P = 0.03), and reinterventions were more common (28%, 95% confidence interval 17-43 vs 2%, 95% confidence interval 0-12) in the percutaneous group compared to the surgery group., Conclusions: Reinterventions after surgery in neonates were relatively common. In older children, percutaneous treatment carried a higher risk of reinterventions, which were mainly related to residual coarctation after primary treatment., (© The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Coarctation of the aorta is known to present with hypertension in older patients; we reviewed our experience and assessed the outcome of hypertension following surgical correction., Methods: From April 2004 to date, 43 patients above the age of 12 underwent coarctation of the aorta repair. The mean age was 20.4 + 9.7 years (maximum 56 years); 21 (48.8%) were older than 18 years and 28 (65.1%) were men. Thirty (69.8%) patients had hypertension. Fourteen (32.6%) had a bicuspid aortic valve; 11 (25.6%) had patent ductus arteriosus; 6 (14%) had myxomatous mitral valve; 4 (9.3%) had ascending aortic aneurysms; and 2 (4.7%) had descending aneurysms., Results: Surgical correction included resection and interposition of a tube graft in 31 (72.1%), an end-to-end anastomosis in 6 (14%) and patch aortoplasty in 3 (7%). Three (7%) patients required an extra-anatomical bypass: 1 had a long segment coarctation of the aorta, and 2 had a Bentall procedure with an ascending-to-descending aortic bypass. Staged procedures were done for concomitant disease in 4 (9.3%). There was 1 death: a 56-year-old woman died of refractory ventricular fibrillation during surgery. Thirty (69.8%) patients were discharged with antihypertensive medication. At a follow-up of 2.8 ± 2.2 years (maximum 9.2 years), the number of hypertensive patients decreased (17/36; 47.2%) (P = 0.042). Univariable predictors for persistence of hypertension revealed the use of an interpositional tube graft for repair (odds ratio 13.855, confidence interval 0.000-0.001; P = 0.001) as an indicator, whereas there were no independent predictors for persistence of hypertension., Conclusions: Surgical intervention is warranted irrespective of age and helps correct and control hypertension better; however, significant numbers of patients still require antihypertensive medication and regular monitoring. Intervention using an interposition tube graft may affect the prevalence of hypertension.

Coronary computed tomography (CT) angiography is often performed in adults with coarctation of the aorta (CoA) for anatomic assessment. As this population ages, assessment of atherosclerotic cardiovascular disease burden is important. Thus, quantitative and qualitative coronary artery calcium (CAC) scores were assessed for patients with CoA ≥16 years of age, who were seen at a referral center. CoA patients had either coronary CT angiography or chest CT with interpretable coronary information performed for clinical indications (follow-up, preoperative, or for symptoms) from 2004 to 2017. Qualitative CAC was determined based on low-dose CT and lung cancer screening protocols. Quantitative CAC scores were compared with an age- and gender-matched control cohort of patients chosen from an emergency department database of patients who received coronary CT angiography for chest pain evaluation. Atherosclerotic cardiovascular disease 10-year predicted risk scores were calculated for both cohorts. Out of 131 patients with CoA (mean age 46.1 ± 15.3 years), 22 patients (17%) had multivessel atherosclerotic disease on qualitative assessment. In the subgroup of patients ≥40 years, those with CoA were more likely to have a quantitative CAC score ≥400 compared with those without CoA (14% vs 4%, p = 0.02). Median atherosclerotic cardiovascular disease risk score was 8% (interquartile range 2% to 12%) for CoA patients ≥40 years, and 5% (interquartile range 2% to 9%) for patient without CoA ≥40 years. In conclusion, we determined that CoA patients have subclinical atherosclerosis identifiable on CT in high rates when compared with patients without CoA. Atherosclerotic cardiovascular disease should be assessed in these patients for prevention and treatment., (Copyright © 2018. Published by Elsevier Inc.)

Adults with repaired coarctation of the aorta (CoA) suffer reduced long-term survival compared with the general population, in part due to coronary artery disease (CAD). There is conflicting evidence as to whether or not CoA is an independent risk factor for CAD. The primary aim was to determine if CoA is independently associated with premature myocardial infarction (MI) in the contemporary era. The secondary aim was to determine if CoA is independently associated with early coronary intervention. In a cross-sectional study using the National Inpatient Sample database from 2005 to 2014, we compared the age at MI and the age at coronary intervention (coronary artery bypass grafting or percutaneous coronary intervention, in the absence of MI diagnosis) in patients with and without CoA using weighted linear regression. Among 5,472,416 observations with a primary diagnosis of MI, 174 had a diagnosis of CoA. Patients with CoA had MI 7.2 years younger than those without CoA, after adjusting for potential confounders (95% CI -11.3, -3.1, p = 0.001). Among 3,631,718 patients without a diagnosis of MI who underwent coronary artery bypass grafting or percutaneous coronary intervention, 279 had a diagnosis of CoA. Patients with CoA who underwent coronary intervention were 15.6 years younger than those without CoA, after adjusting for potential confounders (95% CI -18.3, -12.9, p < 0.001). In conclusion, patients with CoA have MI at a slightly younger age and undergo coronary intervention at a significantly younger age than those without CoA in the contemporary era. Our findings support continued close surveillance for and treatment of modifiable risk factors for CAD., (Copyright © 2018. Published by Elsevier Inc.)

Methods: This is a retrospective study including 47 patients with isthmic coarctation (CoA) diagnosed in ante et postnatal périod, hospitalized in pediatric cardiology departement of la Rabta Hospital-Tunisia during the period from 2000 to 2017., Results: They were 36 girls and 11 boys with an average age of 14 days. The diagnosis of CoA was suspected during the anténatalperiod in eight cases. In postnatal period heart failure was observed in (38.5%), abolition of femoral pulse (74%), a tension asymetry was found in all patients. Antenatal echocardiography suspected indicators of fetal coarctation especially ventriculo-arterial asymmetryechocardiography confirmed the diagnosis of CoA ,The ejection fraction was impaired in 31% of the cases.The abnormalities associated with coarctation were dominated by the patent ductus arteriosus (68%), atrial septal defect (55.5%), aortic hypoplasia, bicuspidia in respectively in 34% and 31% coarctation syndrome in (23.4%). Prostaglandin wasneccessary in (89.3%). forty two patients were operated with good immédiaterésults. the early postoperative mortality was 12.5%. In the long term, we deplored two late deaths, six cases of recoarctation with a follow-up of 14 months treated by percutaneous angioplasty with a result considered good in all cases., Conclusion: Coarctation of the aorta in neonates is a special form of aorticcoarctaion that differs from the child and adult forms in clinical, echocardiographic, and therapeutic characteristics . His clinical diagnosis must be early, if possible in antenatal , to asssure optimal management.

Background: Recoarctation (reCoA) of the aorta is a common complication afer the Norwood procedure. Untreated, it can lead to failure of the systemic ventricle and death. The main goal of the study is to define risk factors of reCoA after the Norwood procedure in hypoplastic left heart syndrome (HLHS)., Methods: We retrospectively analyzed the pre-, intra- and postoperative data of 96 successive patients who underwent the Norwood procedure between 2007 and 2011. In case of reCoA balloon angioplasty was performed. We analyzed and compared the data of the patients with reCoA and without reCoA using the StatSo STATISTICA TM 10 software., Results: ReCoA was noted in 23 patients (33.3%). This complication was diagnosed 95.1 days (49-156 days) on the average a er the Norwood procedure. Balloon angioplasty successfully allowed for decreasing the mean gradient across the site of the narrowing from the average 27.5 mmHg to the average 9.7 mmHg (p = 0.008) and enlarged the neo-isthmus by the average of 2 mm (p <0.05). The risks factors seemed to be the diameter of the ascending aorta OR = 7.82 (p = 0.001), atresia of the mitral valve OR = 7.00 (p = 0.003) and atresia of the aortic valve - OR = 6.22 (p = 0.002)., Conclusion: Balloon angioplasty seems to be an effective intervention in case of reCoA. A low diameter of the native ascending aorta (<=3mm) and the presence of atresia of the mitral and/or aortic valve should intensify the vigilance of a cardiologist in the search for signs of reCoA of the aorta.

Background: In roughly half of patients with coarctation of the aorta (CoA), the aorta may be enlarged. It is uncertain whether enlargement is independent of aortic valve morphology. We sought to compare aortic size in CoA with a tricuspid valve (TAV) to those with bicuspid aortic valve (BAV)., Methods: Sixty-eight CoA patients and 20 healthy controls with prior cardiac magnetic resonance (CMR) imaging were included. CMR was retrospectively reanalyzed to measure aortic root and mid-ascending aorta. The maximum aortic diameter was compared between CoA with TAV, CoA with BAV, and control groups., Results: CoA with TAV patients (n=27) had smaller aortic root diameters than CoA with BAV (n=41) (32±4.9 vs. 37±5.8mm, p=0.001), despite being older (40 vs. 32years, p=0.01). Similarly, TAV CoA patients had a smaller mid-ascending aortic diameter (28±4.5 vs. 33±6.9mm, p=0.019) than BAV patients. TAV CoA was similar to controls in all metrics. Twenty-four patients (35%) with CoA had dilated aortas (>37mm), of which 79% had BAV. A history of hypertension did not predict larger aortic root or mid-ascending aortic dimensions., Conclusions: In patients with CoA, TAV is associated with smaller aortic size compared to those with BAV, and similar to healthy controls. Aortic size in CoA is independent of hypertension. Therefore, aortopathy associated with BAV is likely a reflection of the BAV phenotype rather than CoA or its physiologic effects. This distinction may have implications for the frequency and types of monitoring and treatment of CoA patients., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Introduction and Objectives: Dilatation of the ascending aorta associated with bicuspid aortic valve is a major cause of morbidity and mortality in adults. The main objective was to recognize the aortic involvement in children, its characteristics and risk factors., Methods: Aortic measures of all pediatric patients with bicuspid aortic valve followed in a tertiary pediatric hospital between 1997 and 2015 were retrospectively taken. Patients with syndromes associated with aortic dilatation were excluded (n=17)., Results: Two hundred and six patients were included, 67.9% males. The commonest opening pattern was horizontal: 137 (66.7%). Half of the patients (101) had a history of surgical aortic coarctation, 46 (22.3%) had≥moderate aortic valve stenosis and 13 (6%) had≥moderate aortic insufficiency. Mean follow-up time was 6.1 (4.9) years; diagnosis of aortic dilatation was made during the first year of follow-up. Progression of the dilatation of the ascending aorta was noted in 17.1%, and of the aortic root in 2.5%. More than one-third (80/206) had aortic dilatation (z-score>2). The ascending aorta was exclusively affected in 70/80 patients, with sparing of the aortic root. In the multivariate analysis, patients with dilatation of the ascending aorta were associated with absence of coarctation (P=.001) and vertical opening pattern (P=.007)., Conclusions: Pediatric patients with bicuspid aortic valve warrant medical follow-up for the frequent association with valve impairment and/or dilatation of the ascending aorta., (Copyright © 2017 Elsevier España, S.L.U. All rights reserved.)