Coarctation of the aorta (CoA) is one of the most common types of congenital heart disease. Unfortunately, there is a high prevalence of hypertension and late cardiovascular mortality in patients with CoA despite successful repair. The growing impact of acquired cardiovascular disease remains a significant concern as the adult congenital heart disease population continues to rapidly expand and age. This review aims to explore (1) the determinants of vascular health and atherosclerosis including endothelial dysfunction and vascular wall abnormalities, (2) the prevalence of atherosclerosis and associated sequelae in repaired CoA including coronary artery disease, coronary artery calcium, aortic calcium, stroke, and peripheral artery disease, and (3) the contributing factors specific to CoA. This review aims to guide optimization of long-term cardiovascular health to ultimately reduce mortality and morbidity in this young high-risk population.

Late hypertension (HTN) after coarctation of the aorta (CoA) repair contributes to higher morbidity and mortality. An association between transverse aortic arch (TAA) hypoplasia and HTN has been found, but its relationship with surgical strategy is unclear. We studied the association between late HTN and initial surgical strategy pertaining to the TAA.

We retrospectively reviewed patients who underwent surgical repair of CoA during infancy with at least 10 years of follow-up, excluding those with atypical coarctation, major associated heart defects, and residual isthmic narrowing. TAA diameter z-score immediately postrepair was measured as a marker of surgical strategy. Systemic HTN at latest follow-up was assessed using standard criteria.

A total of 130 patients underwent surgical repair of CoA (76% via thoracotomy, 24% via sternotomy) with resection and end-to-end anastomosis (62%), extended end-to-end anastomosis (30%), subclavian flap (5%), or arch repair with patch (4%), at a median age of 14 days (interquartile range [IQR], 7-62 days). The median postrepair TAA diameter z-score was -2.04 (IQR, -2.69 to 1.24). At a mean follow-up of 17.3 years, 43 of the 130 patients (33%) developed HTN. After controlling for age at repair, sex, and presence of a genetic syndrome, HTN was not associated with immediate postrepair TAA diameter z-score (P = .41), type of surgical incision (P = .99), or type of surgical repair (P = .66).

In patients undergoing surgical repair of CoA during infancy, late HTN was not associated with immediate postrepair TAA size or surgical strategy pertaining to the TAA. These results suggest that factors other than surgical strategy, such as differential growth of the TAA during childhood, may be important.

Restenosis occurs commonly after aortic coarctation (CoA) repair, usually requiring treatment by balloon dilation. Its effect on physical exercise performance is not documented. A retrospective analysis of exercise testing and echocardiographic assessment was performed in children after CoA repair. A group without restenosis (= group 1) and a group with recoarctation, treated by single balloon dilation (= group 2) were compared by matching for age and gender at a 2:1 ratio. Group 1 included 40 children and group 2 comprised 21 children, with mean age of 12.3 ± 2.3y and 11.9 ± 2.9y (p = 0.536) at evaluation. Group 2 children were younger at CoA repair (group 1: median 32.0d (11.0-188.0)-group 2: 9.0d (5.5-19.0), p = 0.011). Arterial hypertension was frequently noticed (group 1: 23(57.5%)-group 2: 7(33.3%), p = 0.106). Echocardiography revealed comparable cardiac function properties, showing more left ventricular (LV) hypertrophy in all CoA patients compared to their peers (Posterior wall thickness z-value: 1.03 ± 1.65, p < 0.001)). Exercise results were generally comparable, despite small differences in favor of group 1 (predicted % peak VO2 = group 1: 95.2 ± 21.4%-group 2: 82.3 ± 10.5%, p = 0.024; peak VO2/kg = group 1: 38.9 ± 9.5 ml/min/kg-group 2: 33.8 ± 6.9 ml/min/kg, p = 0.034; VO2/WR slope = group 1: 12.6 ± 2.0 ml/min/watt-group 2: 11.3 ± 1.7 ml/min/watt, p = 0.018). Compared to uncomplicated coarctation patients, children developing a surgical restenosis after CoA repair, and treated effectively by balloon dilation, had lower-but still acceptable-oxygen uptake during exercise. As intrinsic cardiovascular function characteristics were similar, further study to evaluate the impact of non-disease-specific factors is mandatory.

Pediatric hypertension (HTN) is a significant, growing health concern worldwide and also in Korea. Diagnosis, evaluation, and treatment of HTN in Korean children and adolescents are uncertain due to limitations in using the current international guidelines, since the recommendations by the American Academy of Pediatrics (AAP) and European Society of Hypertension (ESH) guidelines differ. Furthermore, these are guidelines for Western youth, who are racially and ethnically different from Koreans. In addition, reference blood pressure values for all pediatric age groups, which are essential for the diagnosis of HTN according to these two guidelines, are absent in Korea. Therefore, HTN guidelines for Korean children and adolescents should be established. The Korean Working Group of Pediatric Hypertension established clinical guidelines for the diagnosis, evaluation, and management of HTN in Korean children and adolescents. These guidelines were based on reported clinical evidence, expert recommendations, and AAP and ESH guidelines. The characteristics of Korean youth and the Korean medical and insurance system were considered during the establishment of the guidelines. By providing recommendations suitable for Korean youth, these guidelines will help in the prevention and management of childhood HTN, thus relieving the burden of cardiovascular disease in adulthood in Korea.

Pulmonary hypertension (pH) and secondary right ventricle dysfunction is present in 20 % of adults with coarctation of aorta (COA) based on echocardiographic studies. There are limited data about invasive hemodynamic characterization of PH in COA. The purpose of this study was to delineate the clinical features, hemodynamics, and outcomes of PH in COA.

Retrospective cohort study of adults with repaired COA that underwent right heart catheterization (RHC). PH was defined as pulmonary artery (PA) mean pressure > 20 mmHg, and PH was classified as isolated precapillary PH and combined pre/postcapillary PH.

Of 99 COA patients that underwent RHC, 57 (58 %) had PH. Of the patients with PH, 14 (25 %) had isolated precapillary PH while 43 (75 %) had postcapillary PH with or without precapillary disease. The correlates of PH were PA compliance (adjusted OR 0.79, 95 % CI 0.71-0.86 per 1 ml/mmHg), left atrial reservoir strain (adjusted OR 0.95, 95 % CI 0921-0.98 per 1 %), and atrial fibrillation (adjusted OR 2.18, 95 % CI 1.20-13.5). Higher PA mean pressure was associated with risk of cardiovascular events (adjusted HR 1.04, 95 % CI 1.02-1.06 per 1 mmHg) and all-cause mortality (adjusted HR 1.05, 95 % CI 1.02-1.08 per 1 mmHg).

PH was present in over half of adults with COA referred for RHC, and one-quarter of the patients with PH presented with isolated precapillary PH suggesting an underlying PA vascular dysfunction as a contributing mechanism. Further studies are required to determine optimal therapies and strategies for prevention and treatment of PH in this population.

Of 99 adults with repaired coarctation of aorta (COA) that underwent right heart catheterization, 57 % had pulmonary hypertension (pH). Of the patients with PH, 25 % had isolated precapillary PH while 75 % had combined pre/postcapillary PH. The correlates of PH were pulmonary artery (PA) compliance, left atrial reservoir strain and atrial fibrillation. PH was associated with cardiovascular events and all-cause mortality. These data suggest PA vascular dysfunction in addition to left-sided heart disease as potential etiologies for PH in this population. Further studies are required to determine optimal therapies and strategies for prevention and treatment of PH in this population.

Aortic valve disease is common in adults with coarctation of aorta. However, no systematic comparative analyses have been performed of the clinical course of aortic valve disease for male vs female patients in this population. The purpose of this study was to compare cardiac remodelling, onset of symptoms, and incidence of aortic valve replacement (AVR) for male vs female patients.

A retrospective study was conducted of adults with repaired coarctation of aorta and ≥ moderate aortic stenosis and/or aortic regurgitation. Cardiac remodelling (left ventricular [LV], left atrial, right ventricular [RV], and right atrial structure and function) and symptomatic and/or functional class were determined at the baseline encounter. Development of new-onset symptoms and the incidence of AVR were ascertained for the period from baseline to last encounter.

We identified 214 patients (121 male [57%], 93 female [43%]). Although both groups had a similar aortic valve gradient, aortic valve area indexed to body surface area, aortic regurgitation severity, and functional status at baseline, female patients had more LV concentric hypertrophy and remodelling, left atrial hypertension and dysfunction, elevated RV systolic pressure, and RV systolic dysfunction. Of 151 patients without symptoms at baseline,102 (72%) developed symptoms. Female sex was independently associated with new-onset symptoms (adjusted hazard ratio 1.14, [95% confidence interval 1.05-1.23]). Of 214 patients, 191 (89%) underwent AVR. Female sex was not associated with AVR upon multivariable analysis. However, LV concentric hypertrophy and remodelling (both of which were more common in female patients) were associated with new-onset symptoms and AVR.

Female patients, compared to male patients, had more-advanced cardiac remodelling, and more-rapid onset of symptoms, but a similar risk of AVR.

Background: The utilization of hemodynamic parameters, whose estimation is often cumbersome, can fasten diagnostics and decision-making related to congenital heart diseases. The main goal of this study is to investigate the relationship between hemodynamic and morphometric features of the thoracic aorta and to construct corresponding predictive models. Methods: Multi-slice spiral computed tomography images of the aortas of patients with coarctation diagnoses and patients without cardiac or vascular diseases were evaluated to obtain numerical models of the aorta and branches of the aortic arch. Hemodynamic characteristics were estimated in key subdomains of the aorta and three branches using computational fluid dynamics methods. The key morphometric features (diameters) were calculated at locations in proximity to the domains, where hemodynamic characteristics are evaluated. Results: The functional dependencies for velocities and pressure on the corresponding diameters have been fitted, and a metamodel has been constructed employing the predicted values from these models. Conclusions: The metamodel demonstrated high accuracy in classifying aortas into their respective types, thereby confirming the adequacy of the predicted hemodynamic characteristics by morphometric characteristics. The proposed methodology is applicable to other heart diseases without fundamental changes.

Objectives: The configuration of the aortic arch, particularly a Gothic arch shape, in individuals with corrected coarctation of the aorta (CoA) has been associated with a decreased systolic wave amplitude across the arch, which could potentially impair renal perfusion and elevate the risk of arterial hypertension. This study aims to explore the relationship between the morphological characteristics of the aortic arch and their impact on renal perfusion in patients with CoA. Methods: Seventy-one subjects with corrected CoA underwent continuous 24 h ambulatory blood pressure monitoring, computed tomography to assess the aortic arch, and renal perfusion scanning. Subjects were stratified into three groups based on the height-to-width (H/W) ratio of their aortic arch: Group 1 with a H/W ratio of <0.65, Group 2 with a H/W ratio between 0.65 and 0.85, and Group 3 with a H/W ratio of >0.85. Results: Groups 1 and 2 (53,78% and 62.63%) presented with a higher hypertension prevalence of elevated blood pressure than Group 3 (38.89%). Notable variations were observed among the subjects in the time to peak perfusion (Tmax) in the left kidney across the groups. Group 1 showed a median Tmax at 0.27, Group 2 at 0.13, and Group 3 at -0.38 (p-value = 0.079). The differences in Tmax for the right kidney followed a similar trend but were not statistically significant (Group 1 at 0.61, Group 2 at 0.22, and Group 3 at 0.11; p-value = 0.229). Conclusions: This study suggests that variations in the aortic arch morphology might not significantly influence renal perfusion in CoA patients. This indicates the potential adaptability of the renal blood flow, which appears to compensate for reduced perfusion, thus minimizing adverse effects on the kidney function. This adaptability suggests an inherent physiological resilience, emphasizing the need for further targeted research to understand the specific interactions and impacts on treatment strategies for CoA.

This study aimed to investigate the safety and efficacy of transverse aortic arch stenting and evaluate the course of hypertension and the act of arch stenting on systemic hypertension.

The transverse aortic arch stenting procedures between January 2007 and May 2023 were retrospectively analyzed. Detailed procedure information, technical aspects, pressure measurements, angiographic data, balloons and stents used, complications, and immediate results were examined. Early and mid-term results were assessed.

Eighteen patients (10 males and 8 females; mean age: 14.5±5.3 years; range, 4 to 23 years) were included in the study, all of whom were hypertensive before the procedure. The mean weight was 56.8±19.6 kg. In seven patients, the stent struts had to be dilated due to the stent causing jailing at the entrance of nearby arch vessels. After stenting, there was a significant increase in arch diameter and a decrease in ascending aorta pressure and the pressure gradient across the aorta. There were no early mortality or major complications. Late migration of the stent was observed in one patient. Three patients became normotensive immediately after the intervention, and five became drug-free during the follow-up. The requirement for dual antihypertensive therapy was significantly reduced.

Residual transverse arch lesions may contribute to the persistence of systemic hypertension after coarctation treatment. Transverse arch stent implantation can be performed safely with favorable outcomes, facilitating better blood pressure control. However, it should be noted that these patients remain at risk for lifelong hypertension and should be closely monitored in this regard.

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.

The purpose of this study was to assess the relationship among COA, BAV, and thoracic aortic aneurysms.

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.

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.

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.

We previously reported short-term outcomes for stenting of aortic coarctation (CoA) (native or re-coarctation) with newer generation low-profile stents (Valeo, Formula, and Begraft stents) in children under 30 kg. We present here the medium-term outcomes of this procedure. Retrospective review of patients weighing under 30 kg who had percutaneous stent treatments for coarctation between 2012 and 2021 was performed. Clinical and procedural data were collected; 19 patients were included. The median age at the time of procedure was 5.1 [4.1-6.4] years and median weight 21.0 [17.3-22.3] kg. One patient had a history of re-coarctation. Thirteen (68%) patients were on anti-hypertensives pre-procedure. Different types of stents were used (14 Valeo™, 4 Formula® 535, 1 BeGraft), which can all be dilated to 18 mm or larger. One patient required a 9 F sheath, all others required a 7 F sheath. The narrowest diameter in the aorta increased from a median of 3.5 [3.0-4.5] to 9.4 [8.9-9.8] mm, p < 0.001; there was a reduction in the median pressure gradient across the coarctation from 35.0 [30.0-43.0] to 5.0 [0-10.0] mmHg, p < 0.001. There were no intra-procedural complications. Follow-up was for a median of 56.0 [13.0-65.0] months. Five (26%) of patients underwent re-intervention after a median time frame of 40.0 [39.5-52.0] months; four had balloon dilation, one had repeat stent implantation. Five (26%) patients were on anti-hypertensive agent(s) post-intervention. Our single centre experience demonstrates that percutaneous stenting for coarctation of aorta in children under 30 kg, with low-profile stents, had no significant complications during the median follow-up time of 56 months. This study demonstrated that the procedure is safe and effective for short and medium-term therapy in this group of patients with a 26% re-intervention rate. A quarter of patients remained on anti-hypertensive medication post stenting, emphasizing the importance of long-term follow-up.

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.

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.

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.

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.

Left atrial (LA) dysfunction and atrial fibrillation are also relatively common in adults with coarctation of aorta (COA), and the severity of LA dysfunction is associated with a higher risk of atrial fibrillation in this population. The purpose of this study was to determine whether LA function improved after COA repair (LA reverse remodelling), and the relationship between LA reverse remodelling and atrial fibrillation.

Retrospective cohort study of adults undergoing COA repair (2003-20). LA reservoir strain was assessed pre intervention and 12-24 months post intervention, using speckle tracking echocardiography. Incident atrial fibrillation was assessed from COA repair to last follow-up. Of 261 adults who underwent COA repair [age 37 ± 13 years; males 148 (57%)], 124 (47%) and 137 (53%) presented with native vs. recurrent COA, respectively. Of 261 patients, 231 (82%) and 48 (18%) underwent surgical and transcatheter COA repair, respectively. The LA reservoir strain increased from 32 ± 8% (pre intervention) to 39 ± 7% (post intervention), yielding a relative increase of 21 ± 5%. Older age [β ± standard error (SE) -0.16 ± 0.09 per 5 years, P = 0.02], higher systolic blood pressure (β ± SE -0.12 ± 0.04 per 5 mmHg, P = 0.005), and higher residual COA mean gradient (β ± SE -0.17 ± 0.06 per 5 mmHg, P = 0.002) post intervention were associated with less LA reverse remodelling, after adjustment for sex, hypertension diagnosis, and left ventricular indices. LA reverse remodelling (hazard ratio 0.97, 95% confidence interval 0.96-0.98 per 1% increase from pre-intervention LA function, P = 0.006) was associated with a lower risk of atrial fibrillation after adjustment for age, sex, pre-intervention LA reservoir strain, and history of atrial fibrillation.

COA repair resulted in improved LA function and decreased risk for atrial fibrillation, especially in patients without residual hypertension or significant residual COA gradient.

Hypertension affects ∼1% to 3% of newborns in the NICU. However, the identification and management of hypertension can be challenging because of the lack of data-driven diagnostic criteria and management guidelines. In this review, we summarize the most recent approaches to diagnosis, evaluation, and treatment of hypertension in neonates and infants. We also identify common clinical conditions in neonates in whom hypertension occurs, such as renal vascular and parenchymal disease, bronchopulmonary dysplasia, and cardiac conditions, and address specific considerations for the evaluation and treatment of hypertension in those conditions. Finally, we discuss the importance of ongoing monitoring and long-term follow-up of infants diagnosed with hypertension.

Rates of successful surgical repair and life expectancy for patients with congenital heart disease have increased dramatically in recent decades. Thanks to advances in diagnosis, treatment, and follow-up care, an ever-increasing number of individuals with congenital heart disease are reaching advanced age. The exposure to cardiovascular risk factors during their lifetime is modifying the outlook and late clinical trajectory of adult congenital heart disease (ACHD). Their disease burden is shifting from congenital to acquired, primarily atherosclerotic cardiovascular disease (ASCVD) with worrisome consequences. In addition, the complex background of ACHD often curbs appropriate preventive strategies by general practitioners or adult cardiologists. Comprehensive guidance for the prevention and management of acquired heart disease in ACHD patients is currently not available, as this topic has not been covered by the European Society of Cardiology (ESC) guidelines on cardiovascular disease prevention or the ESC guidelines for the management of ACHD. In this document, a state-of-the-art overview of acquired heart disease in ACHD patients and guidance on ASCVD prevention for both ACHD specialists and non-ACHD cardiologists are provided. The aim is to provide a clinical consensus statement to foster the development of a sustainable strategy for the prevention of ASCVD in a practical and simple-to-follow way in this ever-growing cardiovascular cohort, thus reducing their cardiovascular burden.

The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes).

A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate.

Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.

While coarctation of the aorta varies greatly in both severity and age at presentation, all patients are at increased risk of hypertension both before and after repair. Despite advances in knowledge about genetic etiologies, pathophysiologic mechanisms, and optimal repair strategies, patients with repaired coarctation of the aorta remain at increased risk of acquired cardiovascular disease. The aims of this review are to describe the management of coarctation of the aorta at all ages before and after repair, highlight pathophysiologic mechanisms of hypertension, and review long-term follow-up considerations.

Owing to the great advances in the care for children with congenital heart disease by paediatric cardiac surgeons and cardiologists, there are ever increasing numbers of patients with congenital heart disease who reach adult life. At some stage during the late teenage years or soon after, these patients 'transition' from paediatric cardiac care to surveillance by cardiologists who look after adults. Many such specialists, however, are more familiar with commoner acquired heart problems such as coronary disease, heart failure, and arrhythmia in structurally normal hearts and less familiar with congenital heart disease. For this reason, international guidelines have suggested that the care of young adults with congenital heart disease take place in designated specialist adult congenital heart disease centres. It remains very important, however, for general cardiologists to have a good understanding of many aspects of adult congenital heart disease, including common pitfalls to avoid and, importantly, when to refer on, to a specialist centre. To help healthcare providers across the spectrum of cardiology practice to address common themes in adult congenital heart disease, this state-of-the-art review provides a series of case vignettes to illustrate frequent diagnostic problems that we have seen in our tertiary-level adult congenital heart disease centres, which are sometimes encountered in general cardiology settings. These include commonly 'missed' diagnoses, or errors with diagnosis or management, in these often very complex patients.

Data on the evolution of exercise capacity in adults with repaired coarctation of the aorta (CoA) are scarce. We aimed to investigate the evolution and change of measures of exercise capacity obtained by cardiopulmonary exercise testing (CPET) in adults with repaired CoA.

Patients 16 years of age and older with CoA, who performed at least two maximal CPETs in our institution, were included in the study. The first and last available tests were used for comparative statistical analysis of common exercise variables.

Sixty patients (43 men) performed serial maximal CPET. Mean age at first assessment was 30 ± 10 years (range 17-68). Mean time between first and last assessment was 3.5 years (range 1-7). Mean peak VO2 was 85.6 ± 20.4% of the predicted value at the initial test, and 87.0 ± 20.5% at the final test (p = 0.294). There were no significant differences in the mean values of oxygen pulse, VO2 at anaerobic threshold, systolic and diastolic blood pressures and peak heart rate between the two assessments. There was a slightly higher VE/VCO2 slope at the final test (p = 0.047). Higher age and Borg scale were found to be related with a decline in percent-predicted peak VO2 from initial to final assessment.

In adults with repaired CoA, we found no significant change in peak VO2 during a mean follow-up of 3.5 years, yet a small increase in VE/VCO2 slope was observed. Higher age was predictive for a decline in percent-predicted peak VO2, starting in the third decade of life.

Coarctation of the aorta (CoA) is a common congenital cardiovascular malformation, and improvements in the diagnostic process for surgical decision-making are important. We sought to compare the diagnostic accuracy of transthoracic echocardiography (TTE) with computed tomographic angiography (CTA) to diagnose CoA.

We retrospectively reviewed 197 cases of CoA diagnosed by TTE and CTA and confirmed at surgery from July 2009 to August 2019.

The surgical findings confirmed that 19 patients (9.6%) had isolated CoA and 178 (90.4%) had CoA combined with other congenital cardiovascular malformations. The diagnostic accuracy of CoA by CTA was significantly higher than that of TTE (χ2 = 6.52, p = 0.01). In contrast, the diagnostic accuracy of TTE for associated cardiovascular malformations of CoA was significantly higher than that of CTA (χ2 = 15.36, p < 0.0001). Infants and young children had more preductal type of CoA, and PDA was the most frequent cardiovascular lesion associated with CoA. The pressure gradient was significantly decreased after the first operation, similar at 6 months, 1 year, and 3 years follow-ups by TTE.

CTA is more accurate as a clinical tool for diagnosing CoA; however, TTE with color Doppler can better identify associated congenital cardiovascular malformations. Therefore, combining TTE and CTA would benefit clinical evaluation and management in patients suspected of CoA. TTE was valuable for post-operation follow-up and clinical management.

Aortic coarctation (AoCo) leads to long-term sequelae that may impair heart function. Data regarding new echocardiographic function parameters such as atrial strain, in affected patients, are scarce. This study aims to describe these parameters in AoCo patients and define their association with severity measures. 53 AoCo patients and 31 healthy controls, aged 12-40 years, were evaluated. Effectively corrected AoCo (cAoCo) was defined as aortic trans-isthmic corrected Doppler gradient (Dgrad) ≤ 20 mmHg (n = 36), and recoarctation (rAoCo) as Dgrad > 20 mmHg (n = 17). Dependent variables were: E/E'; atrial reservoir strain (Ares); and atrial conduit strain (Acd). T-tests/Mann-Whitney U tests were used to compare these among groups. Multivariable regression was used to test correlation with systolic blood pressure (SBP), indexed LV mass (ILVM), Dgrad, and the ratio between the narrowest diameter of aortic arch and aorta at diaphragm level (Aoratio). In cAoCo and rAoCo patients, E/E' was higher (p < 0.001), Ares, and Acd were lower (p < 0.001 for both) comparing with controls. Acd was higher in cAoCo than rAoCo (p = 0.045). Higher Ares was associated with higher Aoratio (p = 0.002), and lower Acd with higher Dgrad (0.014). EF and GLS were not different among groups. Young patients with effectively corrected aortic coarctation have persistent changes in diastolic function parameters (E/E' and atrial strain), and these are affected by anatomical sequelae. These patients' physiology is closer to patients with recoarctation, than to healthy individuals. This provides rationale for a stronger prevention, and treatment, of arterial dysfunction and high left ventricular afterload in these patients.

This review highlights the major changes reflected in the 2022 American Heart Association (AHA) Scientific Statement on Ambulatory Blood Pressure Monitoring (ABPM) in Children and Adolescents with a specific focus on the newly defined phenotypes of hypertension and their epidemiology and associated outcomes.

The 2022 AHA guidelines' most notable changes include the following: (1) alignment of blood pressure (BP) thresholds with the 2017 American Academy of Pediatrics (AAP) clinical practice guidelines, 2017 American College of Cardiology (ACC)/AHA hypertension guidelines, and 2016 European Society of Hypertension (ESH) pediatric recommendations; (2) expansion of the use of ABPM to diagnose and phenotype pediatric hypertension in all pediatric patients; (3) removal of BP loads from diagnostic criteria; and (4) simplified classification of new hypertension phenotypes to prognosticate risks and guide clinical management. Recent studies suggest that utilizing the 2022 AHA pediatric ABPM guidelines will increase the prevalence of pediatric ambulatory hypertension, especially for wake ambulatory hypertension in older, taller males and for nocturnal hypertension in both males and females ≥ 8 years of age. The new definitions simplify the ambulatory hypertension criteria to include only the elements most predictive of future health outcomes, increase the sensitivity of BP thresholds in alignment with recent data and other guidelines, and thus make hypertension diagnoses more clinically meaningful. This guideline will also aid in the transition of adolescents and young adults to adult medical care. Further studies will be necessary to study ambulatory BP norms in a more diverse pediatric population and evaluate the impact of these guidelines on prevalence and future outcomes.

Although, hypertension is common in adults with repaired coarctation of aorta (COA), there are no data about on-treatment blood pressure (BP), and its relationship to outcomes in this population. The purpose of this study was to determine the relationship between on-treatment BP and cardiovascular mortality in adults with repaired COA.

Retrospective study of adults with repaired COA on antihypertensive therapy (n = 461, age 39 ± 11). All BP measurements obtained within the first 3 years were averaged to determine the on-treatment BP, and the patients were stratified into BP quartiles using the cut-off points from the guidelines.

Being in the upper systolic BP (SBP) quartiles (SBP 120-129, 130-139 and ≥140) was associated with higher risk of cardiovascular mortality (HR 1.05, 95%CI 1.01-1.07, HR 1.12, 95%CI 1.04 to 1.15 and HR 1.39, 95%CI 1.13 to 1.59), as compared to being the lowest SBP quartile. We observed a 7% increase in the risk of cardiovascular mortality for every 5 mmHg increase in SBP, and a 4% increase in risk of cardiovascular mortality for every 5 mmHg increase in DBP.

Collectively, these data suggest that even s less severe form of hypertension SBP (120-129 mmHg) was not benign, and perhaps should be considered for antihypertensive therapy. A randomized controlled clinical trial is required to determine whether this group of patients (SBP 120 to 129 mmHg) would benefit from antihypertensive therapy, and to determine the optimal type and intensity of antihypertensive therapy in this population.

The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes).

A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate. Structure: Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.

The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes).

A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate.

Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.

Risk stratification is challenging in adults with repaired coarctation of aorta (COA) because of the complex interaction of multiple hemodynamic factors and differences in left ventricular adaptation to these factors. The H2FPEF score was originally developed for differentiating between heart failure with preserved ejection fraction and noncardiac dyspnea, but it has been shown to be useful for prognostication in other cardiovascular pathologies.

The purpose of this study was to assess the prognostic role of the H2FPEF score in adults with repaired COA.

This is a retrospective cohort study of adults with repaired COA at the Mayo Clinic (2003-2019). The H2FPEF score was calculated at baseline and at 5-year follow-up. Cardiovascular events (heart failure hospitalization, transplant, or cardiovascular death) were ascertained from medical records.

We identified 712 patients (age 33 years [range 21-45 years]; 419 [59%] males). The baseline H2FPEF score was 2.2 ± 1.4. There was a temporal increase in the H2FPEF score at 5 years (ΔH2FPEF score 0.34 ± 0.11) due to the increase in the prevalence of hypertension, obesity, and high filling pressures. The H2FPEF score correlated with left atrial volume (r = 0.73, P < 0.001), right atrial volume (r = 0.41, P < 0.001), right ventricular fractional area change (r = -0.46, P < 0.001), and left ventricular e' (r = -0.52, P < 0.001). Both the baseline H2FPEF score and ΔH2FPEF score were independently associated with cardiovascular events.

These results suggest that the H2FPEF score can be used for prognostication in patients with COA. The temporal increase in the H2FPEF score was due to factors such as hypertension, obesity, and high filling pressures, and hence, it provides potential therapeutic targets to improve outcomes in this population.

Stent implantation for aortic coarctation (CoA) is associated with anatomical and hemodynamic advantages. Although the anatomy is corrected after the intervention, patients often still suffer from hypertension during the follow-up. Its risk factors remain unclear. There are only a few studies on the long-term follow-up of hypertension after stenting. This study aimed to investigate the factors influencing systemic hypertension after CoA by transcatheter stent placement.

A total of 82 patients (native CoA: 37; men: 53) who underwent stent implantation for CoA at a median age of 123 months (range, 40 days-44 years) between January 2004 and September 2012 were included in this study. Patients with native or recurrent CoA after surgery, simple CoA or complex CoA associated with other cardiac anomalies were recruited in this study. Only stents that were expandable to adult size were implanted. After the initial stent implantation, the patients were followed up for 76.7 months (range, 6-151 months). Those who met the indications for a re-intervention underwent a balloon dilatation or stent implantation to relieve restenosis and alleviate the hypertension caused by restenosis. Patients with hypertension were treated with anti-hypertensive agents.

In the initial intervention, 87 stents were implanted in 82 patients. Seventy-two patients (87.8%) were diagnosed with hypertension before treatment, but only 23 (28.0%) patients had hypertension at the last follow-up, which is a number significantly lower than that before the intervention (P<0.001). Cox regression analysis showed that age at stent implantation was significantly correlated with the incidence of hypertension, which indicates that the younger the patient receiving the intervention, the lower the incidence of hypertension. Moreover, the incidence of hypertension in follow-up is related to the diagnosis. It was higher in the patients with simple CoA than those with complex CoA. There was no significant correlation between native CoA and recurrent CoA.

The incidence of hypertension after CoA stent implantation was significantly lower than that before the intervention. Age at stent implantation is an essential influencing factor for paradoxical hypertension after CoA stent implantation. Complex CoA in patients was related to a significantly lower prevalence of follow-up hypertension.

There are several studies reporting the outcomes of hypoplastic aortic arch and aortic coarctation repair with combination of techniques. However, only few studies reported of aortic arch and coarctation repair using a homograft patch through sternotomy and circulatory arrest with retrograde cerebral perfusion. We report our experience and outcomes of this cohort of neonates and infants.

We performed retrospective data collection for all neonates and infants who underwent aortic arch reconstruction between 2015 and 2020 at our institute. Data are presented as median and inter-quartile range (IQR).

The cohort included 76 patients: 49 were males (64.5%). Median age at operation was 16 days (IQR 9-43.25 days). Median weight was 3.5 kg (IQR 3.10-4 kg). There was no 30 days mortality. Three patients died in hospital after 30 days (3.95%), neurological adverse events occurred in only one patient (1.32%) and recurrent laryngeal nerve injury was noted in four patients (5.26%). Only three patients required the support of extracorporeal membrane oxygenation (ECMO) with a median ECMO run of 4 days. Median follow-up was 35 months (IQR 18.9-46.4 months); 5 years survival was 93.42% (n = 71). The rate of re-intervention on the aortic arch was 9.21% (n = 7).

Our experience shows excellent outcomes in repairing aortic arch hypoplasia with homograft patch under moderate to deep hypothermia with low in-hospital and 5 years mortality rates.

Coarctation of the Aorta (CoA) was assumed to be one of the congenital heart defects not associated with major long-term sequels. Meanwhile, it is known that there are long-term cardiovascular consequences. This study investigates the functional outcome measures in children with CoA.

77 children (40.3% girls, 13.1 ± 3.3 years) with CoA were examined for their functional outcome measures and compared to healthy controls (CG). Carotid Intima-Media wall thickness (cIMT) was measured by ultrasound of the common carotid artery. In addition, Health-related Physical Fitness (HrPF) was assessed by five tasks of the FITNESSGRAM® and health-related quality of life (HrQoL) was analyzed with a self-report questionnaire (KINDL-R).

After adjustment for age and sex and in comparison to the CG, the CoA patients showed structural changes in cIMT (CoA: 0.480 ± 0.043 mm vs CG: 0.465 ± 0.033 mm; p = 0.002) and significantly lower HrPF (z-score -0.46 ± 0.7; p < 0.001; 32nd percentile). HrQoL in children with CoA was significantly better in comparison to CG (p = 0.020).

Early onset of structural changes of the cIMT in children with CoA could be shown. These structural changes in combination with hypertension, which often is associated with CoA, should be the focus of structured follow-up during childhood. The children with CoA showed impaired HrPF in comparison, where the promotion of physical activity should be the key factor for improvement. Encouragingly they showed better HrQoL.

Current management of isolated CoA, localized narrowing of the aortic arch in the absence of other congenital heart disease, is a success story with improved prenatal diagnosis, high survival and improved understanding of long-term complication. Isolated CoA has heterogenous presentations, complex etiologic mechanisms, and progressive pathophysiologic changes that influence outcome. End-to-end or extended end-to-end anastomosis are the favored surgical approaches for isolated CoA in infants and transcatheter intervention is favored for children and adults. Primary stent placement is the procedure of choice in larger children and adults. Most adults with treated isolated CoA thrive, have normal daily activities, and undergo successful childbirth. Fetal echocardiography is the cornerstone of prenatal counseling and genetic testing is recommended. Advanced 3D imaging identifies aortic complications and myocardial dysfunction and guides individualized therapies including re-intervention. Adult CHD program enrollment is recommended. Longer follow-up data are needed to determine the frequency and severity of aneurysm formation, myocardial dysfunction, and whether childhood lifestyle modifications reduce late-onset complications.

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.

Case-control study of 118 COA patients and 118 patients with essential hypertension matched by age, sex, BMI and office SBP.

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.

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.

Aortic coarctation in the adult is usually associated with chronic systemic hypertension, which leads to the sequelae of congestive heart failure, vascular dysfunction and decreased lifespan. Open and endovascular treatment modalities both provide excellent procedural outcomes with minimal mortality and morbidity, but a structured algorithm for workup and periprocedural decision making is not well established. We outline our heart team decision making approach along with our institution's experience treating this condition.

We retrospectively reviewed twenty-four consecutive adult patients treated for aortic coarctation since 2010 at a single center. Outcomes of interest included mortality, treatment approach, device used and post-procedure hypertension status. We describe our protocol for work-up and intervention decision making. We explain our rationale for recommending treatment and the approach, open or endovascular, using existing literature and our experience.

Procedural success rate was 100%, and there were no 30-day, one-year or five-year mortalities, whether the approach was open or endovascular. At last contact, 32% of patients were normotensive and no longer taking blood pressure medications. Several patients presented with complex problems as a result of commonly described complications of prior open or endovascular repair, and we describe our approach to the management of these difficult cases.

Even at a high-volume heart and vascular hospital, aortic coarctation is an uncommon presentation in adult patients. Our experience suggests that excellent outcomes are obtained by discussing each patient among a multidisciplinary heart team and developing a work-up and treatment protocol to guide selection of interventional modality.