"In-patient echocardiography utilization post repair of congenital heart disease. Analysis of data from the Pediatric Health Information System from 2010 to 2019".

BACKGROUND: Echocardiography is a key diagnostic tool for medical decision-making following congenital heart surgery. Overall utilisation of echocardiography for specific congenital heart lesions following cardiac surgery has not previously been reported. This study aims to assess echocardiogram utilisation following the surgical repair of CHD to describe the variation in use across centres and provide clinical benchmarks. METHODS: All patients < 18 years of age undergoing surgical repair of CHD were identified from the Pediatric Health Information System from 2010 to 2019. Surgeries were grouped based on their Risk Adjustment for Congenital Heart Surgery-1 scores. Detailed billing data were used to assess the frequency/cost of post-operative echocardiograms, phase of hospital care, and hospital length of stay. RESULTS: In total, 37,238 surgical encounters were identified for inclusion across 48 centres. Higher Risk Adjustment for Congenital Heart Surgery scores were associated with an increased median number of post-operative echocardiograms (2 versus 4 in Risk Adjustment for Congenital Heart Surgery score 1 versus 6, p < 0.001), and longer median post-operative length of stay (3 days versus 31 days in Risk Adjustment for Congenital Heart Surgery score 1 versus 6, p < 0.001). After accounting for surgical complexity, there was significant variability in echocardiogram utilisation across centres (median daily echocardiogram utilisation range 0.2/day-0.6/day, p < 0.001). There is no difference in the proportion of patients with high surgical complexity (Risk Adjustment for Congenital Heart Surgery ≥ 4) between centres with high versus low echocardiogram utilisation (p = 0.44). CONCLUSIONS: Increasing surgical complexity is associated with longer post-operative length of stay and increased utilisation of echocardiography. There is wide variability in echocardiography resource utilisation across centres, even when accounting for surgical complexity.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for centers performing pediatric heart surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Desenlaces maternos y neonatales en gestantes con cardiopatía con evaluación única vs. evaluación semiestructurada por un equipo cardio-obstétrico / Maternal and neonatal outcomes in pregnant women with heart disease with single evaluation vs. semi-structured evaluation by a cardio-obstetric team

Resumen Introducción: Las enfermedades cardiovasculares en las gestantes son desafiantes, con alta morbimortalidad materna y perinatal, por lo que se recomienda un equipo cardio-obstétrico para su atención. Aun así, pocos datos evalúan el impacto de estos equipos. Por lo tanto, el presente estudio tiene como objetivo comparar los resultados obstétricos, maternos y neonatales del seguimiento semiestructurado (SSE) en una clínica cardio-obstétrica con respecto a un seguimiento usual o seguimiento no estructurado (SNE) en gestantes con enfermedad cardiaca. Métodos: Se realizó un registro prospectivo de gestantes con cardiopatías. Se compararon las pacientes con SSE por un equipo cardio-obstétrico, contra aquellas con evaluación única o SNE. Se calculó el riesgo de eventos según la clasificación de la Organización Mundial de la Salud modificado (OMSm) y la escala del Cardiac Disease in Pregnancy Study II (CARPREG-II) y se evaluaron los desenlaces cardiacos, obstétricos y neonatales. Resultados: Se evaluaron 168 pacientes, 37 con SSE y 131 con evaluación única (SNE). Los principales diagnósticos fueron cardiopatía congénita, arritmias y valvulopatías. La media del CARPREG-II en pacientes de SNE fue 2.48 (DE: 2.3) y en pacientes de SSE fue 3.37 (DE: 2.45; p = 0.041). La media de la OMSm en pacientes de SNE fue 2.1 (DE: 1.6) y con SSE fue 2.65 (DE: 0.95; p = 0.0052). No hubo diferencias significativas en los desenlaces cardiacos primarios (13.8% en SNE vs. 5.4% en SSE; p = 0.134), cardiacos secundarios (5.3 en SNE vs. 2.7 en SSE; p = 0.410), obstétricos (10% en SNE vs. 16.2% en SSE; p = 0.253) y neonatales (35.9% en SNE y 40.5% en SSE; p = 0.486) a pesar de que las pacientes con SSE tenían un riesgo mayor que las pacientes con SNE según las escalas de la OMSm y el CARPREG-II. Conclusiones: En gestantes con cardiopatía, un SSE comparado con un SNE por un equipo cardio-obstétrico no mostró diferencias estadísticamente significativas en los desenlaces cardiovasculares, obstétricos y neonatales, a pesar de que las pacientes con SSE tenían un riesgo significativamente más alto de desenlaces adversos por las escalas de la OMSm y el CARPREG-II. Esto sugiere que el SSE logra al menos equiparar los desenlaces a pesar del mayor riesgo de eventos adversos que tenían las pacientes de este grupo.

Abstract Introduction: Cardiovascular diseases in pregnant women are challenging, with high maternal and perinatal morbidity and mortality, so a cardio-obstetric team is recommended for their care. Even so, little data evaluates the impact of these teams. Therefore, the present study aims to compare the obstetric, maternal, and neonatal outcomes of semi-structured follow-up (SSF) in a Cardio-obstetric clinic concerning regular or unstructured follow-up (USF) in pregnant women with heart disease. Methods: A prospective registry of pregnant women with heart disease was carried out. Patients with SSF by a cardio-obstetric team were compared with those with single evaluation or USF. The risk of events was calculated according to the modified World Health Organization (mWHO) classification and the CARPREG-II scale, and cardiac, obstetric, and neonatal outcomes were evaluated. Results: One hundred sixty-eight patients were evaluated, 37 with SSF and 131 with single evaluation (USF). The primary diagnoses were congenital heart disease, arrhythmias, and valve disease. The average CARPREG-II in USF patients was 2.48 (SD 2.3); in SSF patients, it was 3.37 (SD 2.45; p = 0.041). The average of the mWHO in patients with USF was 2.1 (SD 1.6), and with SSF, it was 2.65 (SD 0.95; p = 0.0052). There were no significant differences in primary cardiac outcomes (13.8% in USF vs. 5.4% in SSF; p = 0.134), secondary cardiac (5.3% in USF vs. 2.7% in SSF; p = 0.410), obstetric (10% in USF vs. 16.2% in SSF; p = 0.253) and neonatal (35.9% in USF and 40.5% in SSF; p = 0.486) even though patients with SSF had a higher risk than patients with USF according to the mWHO and CARPREG-II scales. Conclusions: In pregnant women with heart disease, an SSF compared with a USF by a cardio-obstetric team did not show statistically significant differences in cardiovascular, obstetric, and neonatal outcomes. However, patients with SSF had a significantly higher risk of adverse outcomes due to the mWHO and CARPREG-II scales. This result suggests that the SSF achieves at least equal outcomes despite the higher risk of adverse events that patients in this group had.

Machine Learning to Predict Interstage Mortality Following Single Ventricle Palliation: A NPC-QIC Database Analysis.

There is high risk of mortality between stage I and stage II palliation of single ventricle heart disease. This study aimed to leverage advanced machine learning algorithms to optimize risk-prediction models and identify features most predictive of interstage mortality. This study utilized retrospective data from the National Pediatric Cardiology Quality Improvement Collaborative and included all patients who underwent stage I palliation and survived to hospital discharge (2008-2019). Multiple machine learning models were evaluated, including logistic regression, random forest, gradient boosting trees, extreme gradient boost trees, and light gradient boosting machines. A total of 3267 patients were included with 208 (6.4%) interstage deaths. Machine learning models were trained on 180 clinical features. Digoxin use at discharge was the most influential factor resulting in a lower risk of interstage mortality (p < 0.0001). Stage I surgery with Blalock-Taussig-Thomas shunt portended higher risk than Sano conduit (7.8% vs 4.4%, p = 0.0002). Non-modifiable risk factors identified with increased risk of interstage mortality included female sex, lower gestational age, and lower birth weight. Post-operative risk factors included the requirement of unplanned catheterization and more severe atrioventricular valve insufficiency at discharge. Light gradient boosting machines demonstrated the best performance with an area under the receiver operative characteristic curve of 0.642. Advanced machine learning algorithms highlight a number of modifiable and non-modifiable risk factors for interstage mortality following stage I palliation. However, model performance remains modest, suggesting the presence of unmeasured confounders that contribute to interstage risk.

Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study.

BACKGROUND: After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria. OBJECTIVES: The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information. METHODS: CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis. RESULTS: Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis. CONCLUSIONS: CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease: A Report of the American College of Cardiology Solution Set Oversight Committee and Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography.

The American College of Cardiology (ACC) collaborated with the American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and the Society of Pediatric Echocardiography to develop Appropriate Use Criteria (AUC) for multimodality imaging during the follow-up care of patients with congenital heart disease (CHD). This is the first AUC to address cardiac imaging in adult and pediatric patients with established CHD. A number of common patient scenarios (also termed "indications") and associated assumptions and definitions were developed using guidelines, clinical trial data, and expert opinion in the field of CHD.1 The indications relate primarily to evaluation before and after cardiac surgery or catheter-based intervention, and they address routine surveillance as well as evaluation of new-onset signs or symptoms. The writing group developed 324 clinical indications, which they separated into 19 tables according to the type of cardiac lesion. Noninvasive cardiac imaging modalities that could potentially be used for these indications were incorporated into the tables, resulting in a total of 1,035 unique scenarios. These scenarios were presented to a separate, independent panel for rating, with each being scored on a scale of 1 to 9, with 1 to 3 categorized as "Rarely Appropriate," 4 to 6 as "May Be Appropriate," and 7 to 9 as "Appropriate." Forty-four percent of the scenarios were rated as Appropriate, 39% as May Be Appropriate, and 17% as Rarely Appropriate. This AUC document will provide guidance to clinicians in the care of patients with established CHD by identifying the reasonable imaging modality options available for evaluation and surveillance of such patients. It will also serve as an educational and quality improvement tool to identify patterns of care and reduce the number of Rarely Appropriate tests in clinical practice.

Design, manufacture and initial tests of a drug-eluting coronary stent / Diseño, fabricación y pruebas iniciales de una endoprótesis coronaria liberadora de fármaco

Abstract Introduction: A drug-eluting coronary stent is being developed at the National Institute of Cardiology of Mexico for the treatment of ischemic heart disease. Objective: To establish the best animal model for the tests, to show the advances in the drug-eluting stent prototype, to assess two drugs’ antiproliferative activity and histological results. Method: Smooth muscle cell culture tests were performed in order to assess sirolimus and paclitaxel antiproliferative properties. The drugs were encapsulated inside the polymeric matrix of the stents. Rabbits and pigs were used as animal models. Results: Sirolimus and paclitaxel showed an inhibitory effect, which was higher for the latter. Infrared spectroscopy and light and optical microscopy showed that the drug/polymer layer properly adhered to the stent. At a four-week follow-up, both animal models showed satisfactory clinical evolution and adequate histological response, although the porcine model was shown to be more suitable for future protocols. Conclusions: Preliminary tests of the drug-eluting stent provided bases for the development of a study protocol with an adequate number of pigs and with clinical angiographic and histopathological three-month follow-up.

Resumen Introducción: En el Instituto Nacional de Cardiología de México se desarrolla una endoprótesis (stent) coronaria liberadora de fármacos para el tratamiento de la cardiopatía isquémica. Objetivo: Establecer el mejor modelo animal para las pruebas, mostrar los avances en el prototipo del stent liberador de fármacos, evaluar la actividad antiproliferativa de dos fármacos y los resultados histológicos. Método: Se realizaron cultivos de células de músculo liso para evaluar las propiedades antiproliferativas de sirolimus y paclitaxel. Los fármacos fueron encapsulados en el interior de la matriz polimérica de los stents. Se emplearon conejos y cerdos como modelos animales. Resultados: Sirolimus y paclitaxel mostraron efecto inhibitorio, mayor en el segundo. La espectroscopia infrarroja y la microscopia óptica y electrónica mostraron que la capa del polímero con el fármaco se adhería adecuadamente al stent. A las cuatro semanas de seguimiento, ambos modelos animales mostraron evolución clínica satisfactoria y adecuada respuesta histológica, si bien el modelo porcino resultó más conveniente para protocolos futuros. Conclusiones: Las pruebas preliminares del stent liberador de fármaco brindó bases para desarrollar el protocolo con un número adecuado en cerdos y con seguimiento clínico angiográfico e histopatológico a tres meses.

Design and manufacture of a coronary stent INC-1 and initial tests in experimental animal model / Diseño y fabricación del stent coronario INC-1y pruebas iniciales en animales de experimentación

Abstract In this paper, we describe our coronary stent (INC-1) design and development, the way that we found the specific characteristics needed for our device including biophysics aspects, design, finite element testing, manufacturing, and mechanic trials, we submitted and animal models experiences. The stent platform was cobalt-chromium L605 (Co-Cr), with struts thickness of 80 μm, balloon expandable. We placed the coronary stent INC-1 on a rabbit and dog models so we can evaluate efficacy and security of the device in relationship to its biomechanical properties including navigation capacity, traceability, symmetric expansion, and safety, as well as endothelial attachment, thrombogenicity, and lack of involvement of secondary branches in these models. We succeeded in efficacy and safety of the device after fluoroscopy-guided implant proving excellent capacity of release system, traceability, fluoroscopic visualization, symmetric expansion, and complete endothelial attach. Furthermore, we obtained a good post-implant balloon withdrawal, functional integrity, and no vascular complications. We observed adequate clinical evolution 3 weeks after the stent implantation.

Resumen En esta publicación se describen el diseño y el desarrollo de un stent coronario (INC-1), la forma en que se determinaron las características biofísicas deseadas, el diseño y las pruebas computacionales por elemento finito, su fabricación, así como las pruebas mecánicas a las que se sometió el prototipo y por último las primeras experiencias en modelos animales. La plataforma del stent desarrollado fue de cobalto y cromo L605 (Co-Cr), con struts de 80 μm de grosor, expandido por balón. La colocación del stent coronario INC-1 se realizó en un modelo de conejo y uno canino con el objetivo de evaluar el éxito técnico y de seguridad del stent en relación con las propiedades biomecánicas (capacidad de navegación, maniobrabilidad, expansión simétrica del stent) y de seguridad (fijación a la pared vascular y ausencia de compromiso de ramas secundarias y formación de trombos en el momento de la colocación del stent en el modelo animal). Se obtuvo éxito técnico y de seguridad del stent después del implante guiado por fluoroscopia, lo que demostró adecuada capacidad del sistema liberador, excelente maniobrabilidad, adecuada visualización fluoroscópica, expansión simétrica y fijación completa a la pared vascular, capacidad para retirar el balón desinflado después del implante, e integridad funcional y sin complicaciones vasculares. La evolución clínica fue favorable a tres semanas del seguimiento planeado.

Sedated Echocardiograms Better Characterize Branch Pulmonary Arteries Following Bidirectional Glenn Palliation with Minimal Risk of Adverse Events.

Patients post-bidirectional Glenn (BDG) operation are at risk of left and right pulmonary artery (LPA and RPA) hypoplasia. Transthoracic echocardiograms (TTE) in active children can miss essential elements of anatomy. Procedural sedation improves image quality but increases risk of adverse events. We hypothesized that echocardiograms performed with sedation in patients post-BDG would improve visualization of branch pulmonary arteries with minimal adverse events. Patients post-BDG between 2007-2016 were identified. Exclusion criteria were > 12 months of age, absence of complete TTE before discharge, death before discharge, conversion to shunt physiology, and prolonged post-operative course > 7 weeks. Of 254 post-BDG patients, 153 met inclusion/exclusion criteria. TTE reports were reviewed for visualization of LPA/RPA and hypoplasia of LPA/RPA. Blinded assessment of image quality was performed (scale of 1[poor] to 5[excellent]). Pertinent clinical data were recorded. Pearson's chi-squared and Wilcoxon Rank Sum tests used for statistical analysis. The median age at surgery and hospital stay were 4.8 months and 10 days. Twenty-three patients underwent sedated TTE (15%). Sedated TTE significantly improved visualization of the RPA (100% vs 82%, p = 0.029) and LPA, though this did not reach statistical significance (100% vs 91%, p = 0.129). Sedated TTEs has significantly better image quality (median of 4 vs 3, p < 0.001). There were no serious adverse events due to sedation. Sedated TTE early post-BDG is safe, improves visualization of the RPA and LPA, and improves overall image quality. Routine sedated TTE in these patients should be considered. Implications for long-term outcome need to be further analyzed.

Human pilot study of bare-metal stent: INC-01

ABSTRACT Background: The cost of performing a percutaneous coronary intervention is considerably high for the patient as well as for health systems, which have promoted the development of local technology to help meet the need for these devices. Methods: The INC-01 bare-metal stent was developed at the National Institute of Cardiology in Mexico City and was first implanted on porcine models with technical success in 100% of the evaluated parameters. Presentation of Cases: We present the first three cases of patients with ischemic heart disease, to whom the INC-01 bare-metal stent was implanted. Intracoronary ultrasonography was performed post-stent implantation, showing all the characteristics of implant success during evaluation and clinical follow-up. Conclusions: Angiography and intracoronary ultrasound were carried out demonstrating that the INC-01 bare-metal stent has physical, biological, and histological characteristics similar to those found in commercial metallic stents.

The Role of Matrix Metalloproteinases and Tissue Inhibitors of Metalloproteinases in Duchenne Muscular Dystrophy Cardiomyopathy.

BACKGROUND: Cardiomyopathy is the leading cause of death in Duchenne muscular dystrophy (DMD). Standard cardiac biomarkers are poor indicators of DMD cardiovascular disease. Matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs) regulate collagen turnover. Given the cardiac fibrosis seen in DMD, we hypothesized that MMPs and TIMPs correlate with severity of DMD cardiomyopathy. METHODS AND RESULTS: Prospectively enrolled DMD subjects (n = 42) underwent cardiac magnetic resonance imaging for function and late gadolinium enhancement (LGE), including LGE severity from 0 (no LGE) to 4 (severe). Serum from DMD and healthy male control subjects (n = 15) analyzed for MMPs 1, 2, 3, 7, 9, and 10 and TIMPs 1-4. MMP1, MMP7, and MMP10 were higher in DMD than in control (respectively, median 5080 pg/mL vs 2120 pg/mL [P = .007], 2170 pg/mL vs 1420 pg/mL [P < .001], and 216 pg/mL vs 140pg/mL [P = .040]); TIMP4 was lower in DMD (124 pg/mL vs 263 pg/mL; P = .046). Within DMD, MMP7 correlated inversely with left ventricular ejection fraction (r = -0.40; P = .012) and directly with strain (r = 0.54; P = .001) and LGE severity (r = 0.47; P = .003). MMP7 was higher in DMD patients with LGE compared with those without LGE and control subjects (P < .001). CONCLUSIONS: Multiple MMPs are elevated in DMD compared with control subjects. MMP7 is related to DMD cardiac dysfunction and myocardial fibrosis, possibly through remodeling of the extracellular matrix.

Influence of Aortic Stiffness on Aortic-Root Growth Rate and Outcome in Patients With the Marfan Syndrome.

The Pediatric Heart Network randomized trial of atenolol versus losartan in the Marfan syndrome showed no treatment differences in the rates of aortic-root growth or clinical outcomes. In this report we present treatment effects on aortic stiffness and determine whether baseline aortic stiffness predicts aortic-root growth and clinical outcomes. Echocardiograms at 0, 6, 12, 24, and 36 months from 608 subjects (6 months to 25 years) who met original Ghent criteria and had a maximum aortic-root z-score (ARz) >3 were centrally reviewed. Stiffness index (SI) and elastic modulus (EM) were calculated for aortic root and ascending aorta. Data were analyzed using multivariable mixed effects modeling and Cox regression. Heart rate-corrected aortic-root SI over 3 years decreased with atenolol but did not change with losartan (-0.298 ± 0.139 vs 0.141 ± 0.139/year, p = 0.01). In the entire cohort, above-median aortic-root SI (>9.1) and EM (>618 mm Hg) predicted a smaller annual decrease in ARz (p ≤0.001). Upper-quartile aortic-root EM (>914 mm Hg) predicted the composite outcome of aortic-root surgery, dissection, or death (hazard ratio 2.17, 95% confidence interval 1.02 to 4.63, p = 0.04). Crude 3-year event rates were 10.4% versus 3.2% for higher versus lower EM groups. In conclusion, atenolol was associated with a decrease in aortic-root SI, whereas losartan was not. Higher baseline aortic-root SI and EM were associated with a smaller decrease in ARz and increased risk for clinical outcomes. These data suggest that noninvasive aortic stiffness measures may identify patients at higher risk of progressive aortic enlargement and adverse clinical outcomes, potentially allowing for closer monitoring and more aggressive therapy.