Coarctation Of The Aorta Patients Research Articles

Reverse cardiac remodeling induced by endovascular treatment of coarctation of the aorta

Abstract Background Previously, we have showed the 3-year clinical safety and efficacy of balloon expandable versus self-expandable stents in native coarctation of the aorta (CoA) in a randomized clinical trial (RCT). Very few prospective studies have addressed the changes of echocardiographic parameters in mid to long-term follow up of CoA patients treated endovascularly. Purpose We aimed to demonstrate the improvement of echocardiographic parameters among patients undergoing coarctoplasty with stent based on core laboratory-confirmed baseline and 3-year follow up transthoracic echocardiographic (TTE) images. Methods In the index RCT, adult patients with de novo native CoA were randomized into two groups based on stent types. All randomized patients received the 3-year structural follow-up including aortic CT angiography and TTE. The current prospective cohort substudy, included all patients with available baseline and three-year core laboratory TTEs. Results Of 92 patients initially randomized, 71 participated in the 3-year structural follow up. No baseline core lab images were available in 39 patients, and thus, 32 patients (a median age of 32 (24.8 – 37.5) years, (14 women [43.8%]) were included in this subgroup study. One out of the 32 patients presented with recoarctation which was successfully treated before the 3-year evaluation. Peak systolic gradient and end-systolic velocity of the coarctation stenting site were 17.38 ± 6.55 mmHg and 0.93 ± 0.24 m/s respectively in the 3-year follow-up. Septal E’ and lateral E’ velocities were improved significantly (8.6 ± 1.73 cm/s to 9.35 ± 2 cm/s, P = 0.02 and 12.74 ± 2.96 cm/s to 13.92 ± 2.44 cm/s, P = 0.03, respectively). In addition, septal E/E’ ratio (10.84 ± 2.83 to 9.21 ± 3.17, P = 0.02) and lateral E/E’ ratio (7.39 ± 2.24 to 6.29 ± 1.97, P = 0.02) showed significant improvement. Significant reduction in LV mass [160 (130–203) g to 142 (IQR= 121–172) g, P = 0.001], and septal and posterior LV thicknesses were also demonstrated (0.9 (0.8–1.0) cm to 0.81 (0.77–0.93) cm, P = 0.004) and 0.92 ± 0.14 cm to 0.85 ±0.09 cm, P = 0.01, respectively). Twelve (37.5%) patients had baseline LVH which was normalized in 9 (75%). Lastly, 12 (41.3%) of our population had diastolic dysfunction at baseline study which significantly decreased to 4 (13.7%) in the three-year follow-up evaluation. Conclusion In the present core laboratory-based study, coarctoplasty with stent has favorable effects on diastolic function and LV hypertrophy during midterm follow-up in adult population with de novo native aortic coarctation treated with stent.

Prognostic implications of residual mild coarctation gradient after interventional repair.

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 60months, 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 10mm Hg as the best PSPG cut-off value for predicting late hypertension. Compared to patients with PSPG<11mm Hg, the cumulative event rates of both outcomes were higher in those with PSPG≥11mm Hg (log-rank test, p<.001 for both endpoints). PSPG≥11mmHg 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 ≥11mm Hg are important predictors of clinical comorbidities at mid-term follow-up.

Exploring precipitants of re-coarctation in coarctation of the aorta patients

Exploring precipitants of re-coarctation in coarctation of the aorta patients

A Novel Diastolic Doppler Index Less Affected by Aortic Arch Anomalies Co-existing with Coarctation.

Severity assessment for coarctation of the aorta (CoA) is challenging due to concomitant morphological anomalies (complex CoA) and inaccurate Doppler-based indices. Promising diagnostic performance has been reported for the continuous flow pressure gradient (CFPG), but it has not been studied in complex CoA. Our objective was to characterize the effect of complex CoA and associated hemodynamics on CFPG in a clinical cohort. Retrospective analysis identified discrete juxtaductal (n = 25) and complex CoA (n = 43; transverse arch and/or isthmus hypoplasia) patients with arm-leg systolic blood pressure gradients (BPG) within 24 h of echocardiography for comparison to BPG by conventional Doppler indices (simplified Bernoulli equation and modified forms correcting for proximal kinetic energy and/or recovered pressure). Results were interpreted using the current CoA guideline (BPG ≥ 20 mmHg) to compare diagnostic performance indicators including receiver operating characteristic curves, sensitivity, specificity, and diagnostic accuracy, among others. Echocardiography Z-scored aortic diameters were applied with computational simulations from a preclinical CoA model to understand aspects of the CFPG driving performance differences. Diagnostic performance was substantially reduced from discrete to complex CoA for conventional Doppler indices calculated from patient data, and by hypoplasia and/or long segment stenosis in simulations. In contrast, diagnostic indicators for the CFPG only modestly dropped for complex vs discrete CoA. Simulations revealed differences in performance due to inclusion of the Doppler velocity index and diastolic pressure half-time in the CFPG calculation. CFPG is less affected by aortic arch anomalies co-existing with CoA when compared to conventional Doppler indices.

Deep learning based assessment of hemodynamics in the coarctation of the aorta: comparison of bidirectional recurrent and convolutional neural networks.

The utilization of numerical methods, such as computational fluid dynamics (CFD), has been widely established for modeling patient-specific hemodynamics based on medical imaging data. Hemodynamics assessment plays a crucial role in treatment decisions for the coarctation of the aorta (CoA), a congenital heart disease, with the pressure drop (PD) being a crucial biomarker for CoA treatment decisions. However, implementing CFD methods in the clinical environment remains challenging due to their computational cost and the requirement for expert knowledge. This study proposes a deep learning approach to mitigate the computational need and produce fast results. Building upon a previous proof-of-concept study, we compared the effects of two different artificial neural network (ANN) architectures trained on data with different dimensionalities, both capable of predicting hemodynamic parameters in CoA patients: a one-dimensional bidirectional recurrent neural network (1D BRNN) and a three-dimensional convolutional neural network (3D CNN). The performance was evaluated by median point-wise root mean square error (RMSE) for pressures along the centerline in 18 test cases, which were not included in a training cohort. We found that the 3D CNN (median RMSE of 3.23mmHg) outperforms the 1D BRNN (median RMSE of 4.25mmHg). In contrast, the 1D BRNN is more precise in PD prediction, with a lower standard deviation of the error (±7.03mmHg) compared to the 3D CNN (±8.91mmHg). The differences between both ANNs are not statistically significant, suggesting that compressing the 3D aorta hemodynamics into a 1D centerline representation does not result in the loss of valuable information when training ANN models. Additionally, we evaluated the utility of the synthetic geometries of the aortas with CoA generated by using a statistical shape model (SSM), as well as the impact of aortic arch geometry (gothic arch shape) on the model's training. The results show that incorporating a synthetic cohort obtained through the SSM of the clinical cohort does not significantly increase the model's accuracy, indicating that the synthetic cohort generation might be oversimplified. Furthermore, our study reveals that selecting training cases based on aortic arch shape (gothic versus non-gothic) does not improve ANN performance for test cases sharing the same shape.

A Meta-Analysis for Postoperative Alternations of Aortic Coarctation.

To investigate postoperative vascular changes of patients with coarctation of the aorta (CoA). Literature review of updated articles was performed in June 2023 through the following databases: PubMed, Web of Science, EMBASE, Crohrane Library, CNKI and Wanfang database. All the case-control studies regarding the postoperative changes of vascular structure and function in patients with CoA were analyzed. A total of 596 articles from the above databases were initially identified, with 10 articles being selected for meta-analysis. The analysis showed that weighted mean difference (WMD) of carotid intima-media thickness (cIMT) was 0.07 (95% CI = 0.01~0.13, p < 0.01) and WMD of flow mediated dilation (FMD) was -4.36 (95% CI = -7.49~-1.24, p < 0.01), respectively. The postoperative cIMT of CoA patients was higher than that of the control group, but the postoperative FMD was lower than that of the control group. The operation on CoA patients ameliorates anatomical deformity in the vascular structures. However, intima-media thickening and endothelial malfunction remain as the key postoperative issues.

High prevalence of pre-eclampsia in women with coarctation of the aorta.

The aim was to study pregnancy outcomes in women with coarctation of the aorta (CoA) and associations to hypertensive disorders of pregnancy. Maternal morbidity and mortality are higher in women with heart disease and pre-eclampsia. Chronic hypertension, frequently encountered in CoA, is a risk factor for pre-eclampsia. Clinical data from the National Unit for Pregnancy and Heart Disease database was reviewed for pregnant women with CoA from 2008 to 2021. The primary outcome was hypertensive pregnancy disorders. The secondary outcomes were other cardiovascular, obstetric, and foetal complications. Seventy-six patients were included, with a total of 87 pregnancies. Seventeen (20%) patients were treated for chronic hypertension before pregnancy. Fifteen (20%) patients developed pre-eclampsia, and 5 (7%) had pregnancy-induced hypertension. Major adverse cardiac events developed in four (5%) patients, with no maternal or foetal mortality. Maternal age at first pregnancy [odds ratio (OR) 1.37], body mass index before first pregnancy (OR 1.77), and using acetylsalicylic acid from the first trimester (OR 0.22) were statistically significantly associated with pre-eclampsia. At follow-up (median) 8 years after pregnancy, 29 (38%) patients had anti-hypertensive treatment, an increase of 16% compared to pre-pregnancy. Five (7%) patients had progression of aorta ascendens dilatation to >40 mm, seven (9%) had an upper to lower systolic blood pressure gradient >20 mmHg, and six (8%) had received CoA re-intervention. Pre-eclampsia occurred in 20% of women with CoA in their first pregnancy. All pre-eclamptic patients received adequate anti-hypertensive treatment. All CoA patients were provided multi-disciplinary management, including cardiologic follow-up, to optimize maternal-foetal outcomes.

Arrhythmic and thromboembolic outcomes in adults with coarctation of the aorta

BackgroundAdults with congenital heart disease (ACHD) experience a high prevalence of atrial arrhythmia (AA) and thromboembolic cerebrovascular complications. However, data on AA and associated long-term outcomes are limited in ACHD patients with coarctation of the aorta (CoA). ObjectivesThis study aimed to characterize the prevalence and risk factors for AA and thromboembolic complications in adults with CoA. MethodsWe conducted a retrospective cohort study in a tertiary ACHD care center and included consecutive CoA patients older than 18 years old with more than one year of follow-up. ResultsTwo hundred seventy patients with CoA were followed for 7.2 ± 3.95 years. The mean age was 35.3 ± 11.1 and 55.2% were male. Patients had a mean of 2.1 ± 1.8 cardiovascular surgical or transcatheter procedures. Thirty-five patients (13%) had AA. Ten subjects (3.8%) had a thromboembolic cerebrovascular event, of which four (1.4%) had AA. In univariate analysis, age (p = 0.005) and total intracardiac interventions (p = 0.007) were associated with the presence of AA. Age (p = 0.021), history of heart failure (p = 0.022), and dyslipidemia (p = 0.019) were associated with thromboembolism. In multivariate analysis, age (p < 0.001) and intracardiac interventions (p = 0.007) were associated with AA. ConclusionsThe rate of AA is higher in adults with CoA than in the general population but lower than in other ACHD. Increasing age and intracardiac interventions were associated with AA. The rate of thromboembolic events was low. Some traditional risk factors for stroke may apply. Larger studies are needed to validate predictors for stroke in this population.

Temporal evolution of mechanical stimuli from vascular remodeling in response to the severity and duration of aortic coarctation in a preclinical model

Coarctation of the aorta (CoA) is one of the most common congenital cardiovascular diseases. CoA patients frequently undergo surgical repair, but hypertension (HTN) is still common. The current treatment guideline has revealed irreversible changes in structure and function, yet revised severity guidelines have not been proposed. Our objective was to quantify temporal alterations in mechanical stimuli and changes in arterial geometry in response to the range of CoA severities and durations (i.e. age of treatment) seen clinically. Rabbits were exposed to CoA resulting in peak-to-peak blood pressure gradient (BPGpp) severities of ≤ 10, 10–20, and ≥ 20 mmHg for a duration of ~ 1, 3, or 20 weeks using permanent, dissolvable, and rapidly dissolvable sutures. Elastic moduli and thickness were estimated from imaging and longitudinal fluid–structure interaction (FSI) simulations were conducted at different ages using geometries and boundary conditions from experimentally measured data. Mechanical stimuli were characterized including blood flow velocity patterns, wall tension, and radial strain. Experimental results show vascular alternations including thickening and stiffening proximal to the coarctation with increasing severity and/or duration of CoA. FSI simulations indicate wall tension in the proximal region increases markedly with coarctation severity. Importantly, even mild CoA induced stimuli for remodeling that exceeds values seen in adulthood if not treated early and using a BPGpp lower than the current clinical threshold. The findings are aligned with observations from other species and provide some guidance for the values of mechanical stimuli that could be used to predict the likelihood of HTN in human patients with CoA.

Submaximal Exercise Response is Associated with Future Hypertension in Patients with Coarctation of the Aorta.

Hypertension (HTN) is common in patients with a history of coarctation of the aorta (CoA) and remains underrecognized and undertreated. Studies in the non-coarctation otherwise healthy adult population have correlated an exaggerated blood pressure response during mild to moderate exercise with subsequent diagnosis of HTN. The goal of this study was to determine if blood pressure response to submaximal exercise in normotensive CoA patients correlated with development of HTN.Retrospective chart review was performed in individuals ≥ 13 years old with CoA and no diagnosis of HTN at time of cardiopulmonary exercise testing (CPET). Systolic blood pressure (SBP) during CPET at rest, submax 1 (stage 1 Bruce or minute 2 bicycle ramp), submax 2 (stage 2 Bruce or minute 4 bicycle ramp), and peak were recorded. The primary composite outcome was HTN diagnosis or initiation of anti-hypertensive medications at follow up.There were 177 patients (53% female, median age 18.5 years), of whom 38 patients (21%) met composite outcome during a median follow up of 46 months. Men were more likely to develop hypertension. Age at repair and age at CPET were not significant covariates. At each stage of CPET, SBP was significantly higher in those who met the composite outcome. Submax 2 SBP ≥ 145 mmHg was 75% sensitive, 71% specific in males and 67% sensitive, 76% specific in females for development of composite outcome.Our study shows an exaggerated SBP response to submaximal exercise may portend an increased risk of developing hypertension during short- to mid-term follow up.

Surgical Correction of Aortic Coarctation with Left Subclavian Artery and Proximal Descending Thoracic Aorta Aneurysm with moderate Left Ventricular Dysfunction in an adult Female- a case report

In its simplest form, coarctation of the aorta (CoA) refers to congenital narrowing of the thoracic aorta. Usually, a significant pressure gradient between the arms and legs is suggestive of the diagnosis. With the advancement of aortic imaging in our country many CoA patients are diagnosed at an adult age, but the management still carries a challenge. We reported a case of 30-year-old lady with CoA with an aortic aneurysm involving proximal left subclavian artery with proximal DTA and LV dysfunction. J Bangladesh Coll Phys Surg 2023; 41: 93-97

Abstract 12157: 4d Flow Mri Demonstrates Hemodynamic Normalization After Coarctation Repair in Pediatric Patients

Background: Coarctation of the aorta (COA) is a focal aortic narrowing, and intervention is required for patients with severe hemodynamic impairment. Following intervention, patients remain at risk for long-term re-coarctation. We used 4D flow MRI to assess aortic hemodynamics pre- and post- COA repair in children compared to age matched healthy controls. Methods: Aortic 4D flow MRI was performed in 5 pediatric COA patients (baseline age 6-14 yrs, 1F) pre- and post COA repair (4 native COA, 1 re-intervention) and 5 age-matched controls (ages 9-17 yrs, 2 female). Peak systolic velocities were quantified with velocity maximum intensity projections (MIP) ROIs in the ascending aorta (AAo), aortic arch, distal to the coarctation, and descending aorta (DAo) (Figure 1A). For flow quantification, nine 2D planes were placed along the aorta. Regional net flow was determined by averaging the 2D plane flows by region. The ratio between DAo to AAo (DAo/AAo) net flow was used to assess collateral flow. Results: Velocities were generally higher and more consistent across the aorta following surgery (Figure 1A). Plots highlighting changes in DAo/AAo flow ratio are shown in Figure 1B. Compared to controls, DAo/AAo flow ratio was significantly lower prior to COA repair (0.38±0.11 vs 0.61±0.05, p = 0.0004), indicating flow obstruction at the COA and presence of substantial collateral flow. Post COA repair DAo/AAo flow ratio was similar compared to age matched controls (0.59±0.06 vs 0.61±0.05, p = 0.51), indicating normalization of aortic flow. Patient 2, who underwent COA repair via stent placement showed the greatest improvement and normalization of flow consistency (DAo/AAo) across the aorta after intervention. Conclusion: A ortic 4D flow MRI shows normalization of aortic flow velocities, decreased collateral flow following COA repair, and has potential for long term surveillance and quantification of pre- and post-COA repair hemodynamic changes in children.

Cerebral Infarction Following Correction of Aortic Coarctation Surgery in Children

Background: Coarctation of the aorta (CoA) is a congenital heart defect. Due to the narrowing of the descending aorta, blood flow mainly reduces after the stenosis, and CoA can occur at any region in the thoracic and abdominal aorta. Cardiac surgeons and cardiologists are familiar with postoperative complications of CoA; however, there are also some other complications that have not been reported to date. Case Presentation: The present study investigated three cases of CoA undergoing reconstructive surgery. Nevertheless, a couple of days after the surgery, they manifested symptoms suspected of cerebral infarction. Ischemic infarction was observed after performing brain computed tomography. Additionally, we discuss possible pathophysiology and reasons that can lead to this problem. Conclusions: In this case report, we presented three cases of CoA patients who underwent reconstructive surgery and manifested cerebral infarction as an adverse effect of the reconstructive surgery.

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

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

The effect of coarctation degrees on wall shear stress indices

Coarctation of the aorta (CoA) is a congenital tightening of the proximal descending aorta. Flow quantification can be immensely valuable for an early and accurate diagnosis. However, there is a lack of appropriate diagnostic approaches for a variety of cardiovascular diseases, such as CoA. An accurate understanding of the disease depends on measurements of the global haemodynamics (criteria for heart function) and also the local haemodynamics (detailed data on the dynamics of blood flow). Playing a significant role in clinical processes, wall shear stress (WSS) cannot be measured clinically; thus, computation tools are needed to give an insight into this crucial haemodynamic parameter. In the present study, in order to enable the progress of non-invasive approaches that quantify global and local haemodynamics for different CoA severities, innovative computational blueprint simulations that include fluid–solid interaction models are developed. Since there is no clear approach for managing the CoA regarding its severity, this study proposes the use of WSS indices and pressure gradient to better establish a framework for treatment procedures in CoA patients with different severities. This provides a platform for improving CoA therapy on a patient-specific level, in which physicians can perform treatment methods based on WSS indices on top of using a mere experience. Results show how severe CoA affects the aorta in comparison to the milder cases, which can give the medical community valuable information before and after any intervention.

AUTOMATING THE IDENTIFICATION AND EVALUATION OF THE POSITION OF AXIAL TRIRADIUS ON PALM PRINT: AN APPROACH TO EARLY DETECTION OF CONGENITAL HEART DISEASES

In this paper, we propose a novel method to detect Congenital Heart Diseases (CHDs) using digital palm images. An Axial triradius is one of the features of palm whose position can be used to detect CHDs. In palm print image, axial triradius is identified using a pattern matching algorithm which is a well-known algorithm in image processing. Along with axial triradius, two more triradii are identified. One triradius is located near the little finger and another one located near the index finger. The location of all these three triradii is obtained. Two vectors are drawn from axial triradius, one vector towards left triradius and another vector towards right triradius. The angle at axial triradius is calculated. The angle obtained is used to detect CHDs. Here template matching method has been proposed to identify triradii on palmprint images. Using this approach CHDs such as Fallot’s Tetralogy (FT), Atrial Septal Defect (ASD), Ventricular Septal Defect (VSD) and Coarctation of the Aorta (COA) can be detected. This paper deals with the angle at axial triradius which is calculated for two different people to determine the disease. Out of these two, one person is clinically diagnosed as suffering from FT and another person is healthy. The proposed method can predict that the person is suffering from TF. The proposed method has been used to detect the angle at axial triradius by taking palm images of 100 FT patients, 100 ASD patients, 100 VSD patients, 100 COA patients and 100 healthy people. The result obtained has proved the correctness of the results of previous research works to a certain extent. The present approach allows the automation towards the identification of triradii and evaluation of the position of axial triradius along with the results of its application on some sample images.

Abstract 13170: Bicuspid Aortic Valve Exacerbates the Ascending Aorta Aortopathy but Not Hypertension in Repaired Coarctation of the Aorta

Introduction: Coarctation of the aorta (COA) is a generalized vascular disease, associated with reduced aortic distensibility and a higher prevalence of systemic hypertension. 60-85% of COA patients have a bicuspid aortic valve (BAV). BAV is independently associated with lower aortic distensibility, but not hypertension (HTN). It is not known if the presence of a BAV accentuates the aortopathy and hypertension in CoA patients. Hypothesis: COA patients with a BAV have a stiffer aorta than those with a tricuspid aortic valve (TAV). Methods: We retrospectively identified COA patients with and without BAV in whom the cardiac MRI (CMR) data was available from 2005-2019 and calculated aortic distensibility at the ascending (AAO) and descending aorta (DAO). Patients with residual COA were excluded. Results: Among 215 COA patients (median age 25.3 years, 56% male), 67% of patients had a BAV. BAV and TAV groups were similar to the current age, gender, and modality of COA treatment. As expected, BAV patients had larger AAO size (median diameter z -score 1.4 versus 0.1, p&lt;0.001). Median AAO distensibility z-score was significantly lower in the BAV group (-1.2 versus -0.7; p=0.014) but DAO distensibility was similar in BAV and TAV patients. The prevalence of HTN was not different between BAV (32%) and TAV groups (36%, p = 0.56). On multivariable analysis, HTN was not associated with BAV or AAO distensibility but was associated with lower DAO distensibility (p=0.01). Conclusions: In young adults with adequately treated COA, the BAV group had lower AAO distensibility and a larger AAO compared with the TAV group. However, DAO distensibility and the prevalence of hypertension were similar in BAV and TAV patients. These results suggest that although the presence of a BAV in COA exacerbates the AAO aortopathy in a localized fashion, it does not exacerbate the more generalized vascular dysfunction and associated HTN.

Risk of myocardial infarction in middle aged and older patients with coarctation of the aorta

Abstract Introduction Previous case-control studies have compared the risk of coronary artery disease/myocardial infarction (MI) in patients with coarctation of the aorta (CoA) with other congenital heart disease diagnoses, however, these studies have only included younger patients in their 20s and 30s, not older patients. As the atherosclerotic burden is increasing with increasing age, it is important to study the risk of MI in older patients (from early middle age and older) with CoA. Purpose The aim of our study was to investigate the risk of MI in older patients (≥40 years) with CoA, and to compare this risk with the risk in patients of the same age with ventricular septal defects (VSD), the most common congenital heart condition. Methods We used data from the Swedish National Patient Registry (NPR) to identify all patients alive at 40 years of age with a diagnosis of CoA or VSD, born during the period 1930–1970. The follow-up through the NPR and the Cause-specific Death Registry started in January 1970 and went on until December 2017. Results Altogether 1204 patients with CoA and 2079 patients with VSD were identified in the registers, and in total, 97 (8.1%) patients with CoA and 162 (7.8%) patients with VSD developed an MI during follow up. Mean follow up time was 20.6±10.3 years in CoA patients and 21.3±10.9 in the VSD group. The risk of MI was similar in CoA compared with VSD patients (HR 1.1, 95% confidence interval 0.9–1.5, p=0.3) Median age at MI was similar in CoA patients and in VSD patients; in CoA 59.8 years (range 40.1–87.5), in VSD 61.2 (range 40.2–87.5), p=0.3. Hypertension (diagnosed before MI or within a year after MI) was more common in CoA patients with an MI (58.8%) compared with VSD patients (37.7%), however, prevalence of diabetes mellitus and hyperlipidemia was similar in both patient groups (18.6% and 21.6% in CoA respectively, compared to 17.9% and 22.8% in VSD group). After adjustment for hypertension, diabetes mellitus and hyperlipidemia, the risk of MI remained similar in patients with CoA and VSD (hazard ratio 1.1, 95% confidence interval 0.8–1.4, p=0.5). Conclusion In this large nationwide study, we found that in older patients with CoA (median age at MI 60 years, range 40–88 years) the risk of MI was not increased and similar to that of patients with VSD with similar age at MI. Adjustment for hypertension, diabetes mellitus and hyperlipidemia did not modify this finding which suggests that patients with CoA do not have an increased risk of MI, compared to patients with VSD. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): This work was funded by the Swedish state under an agreement between the Swedish government and county councils, the ALF agreement (Grant number: 236611) and the Swedish Heart-Lung Foundation (Grant Number: 20090724).

Changes in inflammation and oxidative stress signalling pathways in coarcted aorta triggered by bicuspid aortic valve and growth in young children

Neonates with coarctation of the aorta (CoA) combined with a bicuspid aortic valve (BAV) show significant structural differences compared to neonatal CoA patients with a normal tricuspid aortic valve (TAV). These effects are likely to change over time in response to growth. This study investigated proteomic differences between coarcted aortic tissue of BAV and TAV patients in children older than one month. Aortic tissue just proximal to the coarctation site was collected from 10 children (BAV; n=6, 1.9±1.7 years, TAV; n=4, 1.7±1.5 years, (mean ± SEM, P=0.92.) Tissue were snap frozen, proteins extracted, and the extracts used for proteomic and phosphoproteomic analysis using Tandem Mass Tag (TMT) analysis. A total of 1811 protein and 76 phosphoprotein accession numbers were detected, of which 40 proteins and 6 phosphoproteins were significantly differentially expressed between BAV and TAV patients. Several canonical pathways involved in inflammation demonstrated enriched protein expression, including acute phase response signalling, EIF2 signalling and macrophage production of IL12 and reactive oxygen species. Acute phase response signalling also demonstrated enriched phosphoprotein expression, as did Th17 activation. Other pathways with significantly enriched protein expression include degradation of superoxide radicals and several pathways involved in apoptosis. This work suggests that BAV CoA patients older than one month have an altered proteome consistent with changes in inflammation, apoptosis and oxidative stress compared to TAV CoA patients of the same age. There is no evidence of structural differences, suggesting the pathology associated with BAV evolves with age in paediatric CoA patients.

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

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