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.

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.

Coarctation of Aorta (CoA) is a congenital disease consisting of a narrowing that obstructs the systemic blood flow. This proof-of-concept study aimed to develop a framework for automatically and robustly personalizing aortic hemodynamic computations for the assessment of pre- and post-intervention CoA patients from 3D rotational angiography (3DRA) data.

We propose a framework that combines hemodynamic modelling and machine learning (ML) based techniques, and rely on 3DRA data for non-invasive pressure computation in CoA patients. The key features of our framework are a parameter estimation method for calibrating inlet and outlet boundary conditions, and regional mechanical wall properties, to ensure that the computational results match the patient-specific measurements, and an improved ML based pressure drop model capable of predicting the instantaneous pressure drop for a wide range of flow conditions and anatomical CoA variations.

We evaluated the framework by investigating 6 patient datasets, under pre- and post-operative setting, and, since all calibration procedures converged successfully, the proposed approach is deemed robust. We compared the peak-to-peak and the cycle-averaged pressure drop computed using the reduced-order hemodynamic model with the catheter based measurements, before and after virtual and actual stenting. The mean absolute error for the peak-to-peak pressure drop, which is the most relevant measure for clinical decision making, was 2.98 mmHg for the pre- and 2.11 mmHg for the post-operative setting. Moreover, the proposed method is computationally efficient: the average execution time was of only [Formula: see text] minutes on a standard hardware configuration.

The use of 3DRA for hemodynamic modelling could allow for a complete hemodynamic assessment, as well as virtual interventions or surgeries and predictive modeling. However, before such an approach can be used routinely, significant advancements are required for automating the workflow.

Coarctation of the aorta (CoA) typically requires repair, but re-interventions and vascular complications occur, particularly with associated defects like bicuspid aortic valve (BAV). Magnetic resonance imaging (MRI) may identify anatomic and hemodynamic factors contributing to clinical complications.

To investigate 4D flow MRI characteristics in pediatric CoA to determine parameters for long-term clinical surveillance.

Retrospective.

CoA (n = 21), CoA with BAV (n = 24), BAV alone (n = 29), and healthy control (n = 25).

A 1.5 T, 3D CE IR FLASH MRA, 4D flow MRI using 3D time resolved PC-MRI with velocity encoding.

Thoracic aorta diameters were measured from 3D CE-MRA. Peak systolic velocities and wall shear stress were calculated and flow patterns were visualized throughout the thoracic aorta using 4D flow. Repair characteristics, re-interventions, and need for anti-hypertensive medications were recorded.

Descriptive statistics, ANOVA with post hoc t-testing and Bonferroni correction, Kruskal-Wallis H, intraclass correlation coefficient, Fleiss' kappa.

Patients with CoA with or without repair had smaller transverse arch diameters compared to BAV alone and control cohorts (P < 0.05), higher peak systolic flow velocities and wall shear stress compared to controls in the transverse arch and descending aorta (P < 0.05), and flow derangements in the descending aorta. The most common CoA repairs were extended end-to-end anastomosis (n = 22/45, 48.9%, age at repair 1 ± 2 years, seven re-interventions) and stent/interposition graft placement (n = 10/45, 22.2%, age at repair 12 ± 3 years, one re-intervention). Anti-hypertensive medications were prescribed to 33.3% (n = 15/45) of CoA and 34.4% of BAV alone patients (n = 10/29).

Despite repair, CoA alters hemodynamics and flow patterns in the transverse arch and descending aorta. These findings may contribute to vascular remodeling and secondary complications. 4D flow MRI may be valuable in risk stratification, treatment selection and postintervention assessment. Long-term, prospective studies are warranted to correlate patient and MRI factors with clinical outcomes.

3 TECHNICAL EFFICACY: Stage 3.

Congenital heart disease (CHD) affects approximately one million people in the USA with the number increasing by 5% each year. Patients are usually both diagnosed and treated in infancy, however many of them may have subclinical CHD that remains undiagnosed until late adulthood. Patients with complex CHD tend to be symptomatic and are diagnosed at a younger age than those with a single defect. CHDs can be divided into three categories, including cardiac, great vessels and coronary artery anomalies. Recent advances in computed tomography (CT) technology with faster acquisition time and improved spatial resolution allow for detailed evaluation of cardiac morphology and function. The concomitant increased utilization of CT has simultaneously led to more sensitive detection and more thorough diagnosis of CHD. Recognition of and understanding the imaging attributes specific to each anomaly is important for radiologists in order to make a correct and definite diagnosis. This article reviews the spectrum of CHDs, which persist into adulthood that may be encountered by radiologists on CT.

Coarctation of the aorta (CoA) is a relatively common congenital cardiac defect often causing few symptoms and therefore can be challenging to diagnose. The hallmark finding on physical examination is upper extremity hypertension, and for this reason, CoA should be considered in any young hypertensive patient, justifying measurement of lower extremity blood pressure at least once in these individuals. The presence of a significant pressure gradient between the arms and legs is highly suggestive of the diagnosis. Early diagnosis and treatment are important as long-term data consistently demonstrate that patients with CoA have a reduced life expectancy and increased risk of cardiovascular complications. Surgical repair has traditionally been the mainstay of therapy for correction, although advances in endovascular technology with covered stents or stent grafts permit nonsurgical approaches for the management of older children and adults with native CoA and complications. Persistent hypertension and vascular dysfunction can lead to an increased risk of coronary disease, which, remains the greatest cause of long-term mortality. Thus, blood pressure control and periodic reassessment with transthoracic echocardiography and three-dimensional imaging (computed tomography or cardiac magnetic resonance) for should be performed regularly as cardiovascular complications may occur decades after the intervention.

Aortic coarctation is a local narrowing of the aortic lumen, which is located at the level of the isthmus in 95% of patients. Aortic coarctation accounts for 5 to 8% of all congenital heart diseases. It may have an acute presentation in the form of heart failure in the neonate or may be discovered incidentally in adult because of severe treatment-resistant hypertension. Ultrasound may reveal the presence of aortic coarctation during the antenatal period. In this situation, associated abnormalities should be investigated (including karyotype), because they influence prognosis and indicates whether or not the birth should occur in a center with pediatric cardiology expertise. Postnatally, ultrasound and chest radiography are the basic imaging work-up. Computed tomography is often the second line imaging investigation in infants and young children for whom magnetic resonance imaging fails to confirm the diagnosis. Magnetic resonance imaging with cardiac synchronization is the preferred imaging tool in the post-treatment period. Aortic coarctation may be treated surgically or by endovascular techniques. Potential complications should be searched for using ultrasound and magnetic resonance imaging.

We investigated the relationship between pulse pressure (PP)--a surrogate marker of arterial stiffness-and activity of the renin-angiotensin-aldosterone system (RAAS) in adult patients with repaired coarctation and normal left ventricular (LV) function. A total of 114 patients (44 (26-74) years, 13 (0.1-40) years at repair) and 20 healthy controls were examined with 24-h ambulatory blood pressure monitoring, echocardiography, vasoactive hormone levels and magnetic resonance of the thoracic aorta. Forty-one patients (36%) were taking antihypertensives (28 RAAS inhibitors). Fifty-one had mean 24-h blood pressures >130/80 mm Hg. Hypertension was not associated with age at repair (P=0.257). Patients had higher PP and LV mass compared with controls (52±11 vs. 45±5 mm Hg and 221±71 vs. 154±55 g, respectively; both P<0.05). Differences were more pronounced in the presence of recoarctation, but independently of RAA levels. Even normotensive patients had higher LV mass than controls. LV mass and recoarctation were correlated with PP levels. In conclusion, adult patients with repaired coarctation have increased PP and LV mass compared with controls. PP increased with increasing recoarctation. Hypertension was present also in the absence of recoarctation. These changes could not be explained by abnormal activation of the RAAS.

Coarctation of aorta (CoA) is a narrowing of the aorta leading to a pressure gradient (ΔP) across the coarctation, increased afterload and reduced peripheral perfusion pressures. Indication to invasive treatment is based on values of maximal (systolic) trans-coarctation ΔP. A computational fluid dynamic (CFD) approach is herein presented for the non-invasive haemodynamic assessment of ΔP across CoA. Patient-specific CFD simulations were created from contrast-enhanced computed tomography (CT) and appropriate flow boundary conditions. Computed ΔP was validated with invasive intravascular trans-CoA pressure measurements. Haemodynamic indices, including pressure loss coefficient (PLc), time-averaged wall shear stress (TAWSS) and oscillatory shear index (OSI), were also quantified. CFD-estimated ΔP values were comparable to the invasive ones. Moreover, the aorta proximal to CoA was exposed to altered TAWSS and OSI suggesting hypertension. PLc was found as a further geometric marker of CoA severity. Finally, CFD-estimated ΔP confirmed a significant reduction after percutaneous balloon dilatation and stenting of the CoA in one patient (e.g. from ΔP∼52 mmHg to ΔP∼3 mmHg). The validation of the ΔP computations with catheterisation measurements suggests that CFD simulation, based on CT-derived anatomical data, is a useful tool to readily quantify CoA severity.

Aortic pathology can be more complex to understand on imaging than is initially appreciated. There are a number of imaging modalities that provide excellent assessment of aortic pathology and enable the accurate monitoring of disease. This review discusses the imaging of the most common disease processes that affect the aorta in adults, with the primary focus being on CT and MRI.

Systemic hypertension is a highly prevalent potentially modifiable cardiovascular risk factor. Imaging plays an important role in the diagnosis of underlying causes for hypertension, in assessing cardiovascular complications of hypertension, and in understanding the pathophysiology of the disease process. Cardiovascular magnetic resonance (CMR) provides accurate and reproducible measures of ventricular volumes, mass, function and haemodynamics as well as uniquely allowing tissue characterization of diffuse and focal fibrosis. In addition, CMR is well suited for exclusion of common secondary causes for hypertension. We review the current and emerging clinical and research applications of CMR in hypertension.

Over the last 10 years, the development of newer pulse sequences and applications in new clinical areas has enabled cardiovascular magnetic resonance to emerge as a powerful tool for the physicians to both diagnose and guide treatments of various cardiac pathologies. The greatest strengths of cardiovascular magnetic resonance include the assessment of ischemia and viability, evaluation of nonischemic cardiomyopathies, including myocarditis, pericardial disease, congenital heart disease, and tissue characterization of cardiac masses.

The objective of this study was to assess late morbidity after repair of aortic coarctation and its association with residual aortic arch obstruction.

This is an observational cohort study of 133 patients who underwent surgical repair during 1965-1985. Echocardiography, bicycle exercise testing, 24-hour ambulatory blood pressure monitoring, and magnetic resonance imaging/computerized tomography scan of the thoracic aorta were performed. The setting of this study was a tertiary referral center.

Among 156 survivors, 133 (84 men) accepted study participation. Median age (range) was 10 (0.1-40) years at repair and 44 (26-74) years at follow-up.

Outcome measures used are prevalence of previous cardiovascular reinterventions, current cardiac and valvular function, exercise capacity, blood pressure levels at rest and during exercise, and presence of recurrent or residual aortic arch obstruction and/or aortic aneurysms.

Thirty-five had undergone cardiovascular reinterventions. Sixteen had an aortic and three had a mitral valve prosthesis; 117 had a native aortic valve that was bicuspid in 63 and dysfunctional in 45. Ejection fraction was below 50% in 16. On exercise, performance was reduced in 37 and hypertension was induced in 47. Fifty-eight had elevated blood pressures and further 17 received antihypertensives. The ascending aorta was aneurysmal in 28 and the distal arch in five. The presence of a bicuspid aortic valve was significantly associated with valve regurgitation and ascending aortic ectasia. Fifty-eight of 121 patients had minimal aortic arch diameters between 46% and 79% of the diaphragmatic aortic diameter, indicating moderate/mild recoarctation. This was associated with elevated blood pressures and use of antihypertensive medication, but not with hypertension in unmedicated patients or with echocardiographic or exercise parameters. Only five patients had normal study findings, were normotensive, and without reinterventions after coarctation repair.

Cure by repair of aortic coarctation is rare; heart diseases, aortopathy, and hypertension are common. Morbidity is only weakly associated with mild/moderate recoarctation.

PURPOSE: Late morbidity of surgically repaired coarctation of the aorta includes early cardiovascular and cerebrovascular disease, shortened life expectancy, abnormal vasomodulator response, hypertension and exercise-induced hypertension in the absence of recurrent coarctation. Observational studies have linked patterns of arch remodeling (Gothic, Crenel, and Romanesque) to late morbidity, with Gothic arches having the highest incidence. We evaluated flow in native and surgically repaired aortic arches to correlate respective hemodynamic indices with incidence of late morbidity. METHODS: Three dimensional reconstructions of each remodeled arch were created from an anatomic stack of magnetic resonance (MR) images. A structured mesh core with a boundary layer was generated. Computational fluid dynamic (CFD) analysis was performed assuming peak flow conditions with a uniform velocity profile and unsteady turbulent flow. Wall shear stress (WSS), pressure and velocity data were extracted. RESULTS: The region of maximum WSS was located in the mid-transverse arch for the Crenel, Romanesque and Native arches. Peak WSS was located in the isthmus of the Gothic model. Variations in descending aorta flow patterns were also observed among the models. CONCLUSION: The location of peak WSS is a primary difference among the models tested, and may have clinical relevance. Specifically, the Gothic arch had a unique location of peak WSS with flow disorganization in the descending aorta. Our results suggest that varied patterns and locations of WSS resulting from abnormal arch remodeling may exhibit a primary effect on clinical vascular dysfunction.

Guidelines recommend screening cardiovascular magnetic resonance (Sc-CMR) imaging for all patients after coarctation of the aorta repair, although there are limited data verifying its clinical utility. Therefore, we sought to assess the value of Sc-CMR in detecting aortic complications and at-risk abnormalities after coarctation of the aorta repair and to identify significant risk factors. We reviewed 76 patients (mean age 31 ± 10 years), including 40 with symptomatically indicated CMR (Sx-CMR) and 36 with Sc-CMR studies. CMR angiograms were evaluated for aortic abnormalities. Recoarctation was defined as residual narrowing/descending aorta at the diaphragm ≤0.5 (at risk ≤0.75), ascending aorta aneurysm as maximum ascending cross-sectional area/height ≥10 (at risk ≥5), and descending aorta aneurysm as maximum descending diameter/descending aorta at the diaphragm ≥1.5 (at risk ≥1.25). Aortic complications or abnormalities were found in 45 patients (59%). No patient met criteria for recoarctation (at risk 10 Sx-CMR vs 5 Sc-CMR). Significant risk factors included heart failure symptoms and female gender (p <0.05). One patient (Sc-CMR) had ascending aneurysm (at risk 17 Sx-CMR vs 8 Sc-CMR). Time from repair was a significant predictor (p <0.05). There were 10 patients (6 Sx-CMR vs 4 Sc-CMR) with descending aneurysm (at risk 8 Sx-CMR vs 7 Sc-CMR). Cardiovascular symptoms, hypertension, and echocardiogram were not predictive. In conclusion, >50% of patients undergoing Sc-CMR had aortic abnormalities, which was not significantly different from those undergoing Sx-CMR. In particular, Sc-CMR identified descending aorta aneurysms that were not predicted by clinical parameters or echocardiogram.

Coarctation of the aorta is the discrete narrowing of the proximal descending aorta and is the sixth most common lesion in congenital heart disease. Repair of the coarctation can relieve the obstruction, but recurrent coarctation and future aneurysm formation can occur, and a heightened risk of vascular disease is present. This review focuses on advances in the management of native and previously treated coarctation and provides insights into future vascular risk.

Coarctation of the aorta is associated with other left heart obstructive lesions, and advances in the genetic basis of these conditions have been made. Recurrent coarctation and aneurysm formation are common after surgical and endovascular repair of coarctation of the aorta. Endovascular treatment is an acceptable alternative to surgical repair of native and recurrent coarctation. Covered stents and stent grafts can be used to treat arch complications with a low risk of complications. In spite of repair of the obstruction, hypertension persists and appears to be multifactorial due to a variety of factors, including endothelial dysfunction, aortic stiffness, altered arch morphology and increased ventricular stiffness.

People with previously repaired coarctation of the aorta require long-term surveillance for local complications with aortic imaging and surveillance and management of hypertension to prevent vascular disease.