Coarctation of Aorta With Tricuspid Aortic Valve Is Not Associated With Ascending Aortic Aneurysm.

BACKGROUND: Aortic aneurysm is common in patients with coarctation of aorta (COA), but it is unclear whether the risk of aortic aneurysms is due to COA or related to the presence of other risk factors such as bicuspid aortic valve (BAV) and hypertension. OBJECTIVES: The purpose of this study was to assess the relationship among COA, BAV, and thoracic aortic aneurysms. METHODS: A total of 867 patients with COA (COA group) were matched 1:1:1 to 867 patients with isolated BAV (BAV group) and 867 patients without structural heart disease (SHD) (no-SHD group). The COA group was further subdivided into a COA+BAV subgroup (n = 304 [35%]), and COA with tricuspid aortic valve (TAV) (COA+TAV subgroup [n = 563 (65%)]). Aortic dimensions were assessed at baseline and at 3, 5, and 7 years. RESULTS: Compared with the no-SHD group, the COA+BAV subgroup had larger aortic root diameter (37 mm [Q1-Q3: 30-43 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001) and mid ascending aorta dimeter (34 mm [Q1-Q3: 29-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P = 0.008). Similarly, the BAV group had larger aortic root diameter (37 mm [Q1-Q3: 30-42 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001), and mid ascending aorta dimeter (35 mm [Q1-Q3: 30-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P < 0.001). Compared with the COA+TAV subgroup, the COA+BAV subgroup and BAV group were associated with larger aortic root and mid ascending aorta diameter at baseline and follow-up. The risk of acute aortic complications was low in all groups. CONCLUSIONS: These findings suggest that BAV (and not COA) was associated with ascending thoracic aorta dimensions, and that patients with COA+TAV were not at a greater risk of developing ascending aortic aneurysms as compared with patients without SHD.

Surgically induced aortic coarctation in a neonatal porcine model allows for longitudinal assessment of cardiovascular changes.

Noncritical aortic coarctation (COA) typically presents beyond early childhood with hypertension. Correction of COA does not ensure a return to normal cardiovascular health, but the mechanisms are poorly understood. Therefore, we developed a porcine COA model to study the secondary cardiovascular changes. Eight male neonatal piglets (4 sham, 4 COA) underwent left posterolateral thoracotomy with descending aorta (DAO) mobilization. COA was created via a 1-cm longitudinal DAO incision with suture closure, plication, and placement and an 8-mm external band. All animals had cardiac catheterization at 6 (11-13 kg), 12 (26-31 kg), and 20 (67-70 kg) wk of age. Aortic luminal diameters were similar along the thoracic aorta, except for the COA region [6.4 mm COA vs. 17.3 mm sham at 20 wk (P < 0.001)]. Collateral flow could be seen as early as 6 wk. COA peak systolic pressure gradient was 20 mmHg at 6 wk and persisted through 20 wk increasing to 40 mmHg with dobutamine. Pulse pressures distal to the COA were diminished at 12 and 20 wk. This model addresses many limitations of prior COA models including neonatal creation at an expected anatomic position with intimal injury and vessel sizes similar to humans.NEW & NOTEWORTHY A neonatal model of aortic coarctation was developed in a porcine model using a readily reproducible method of aortic plication and external wrap placement. This model addresses the limitations of existing models including neonatal stenosis creation, appropriate anatomic location of the stenosis, and intimal injury creation and mimics human somatic growth. Pigs met American Heart Association (AHA) criteria for consideration of intervention, and the stenoses were graded as moderate to severe.

Role of Computed Tomography Angiography in the Short-Term Follow-up of Aortic Coarctation Repair.

INTRODUCTION: Coarctation of the aorta (CoA) is a narrowing of the thoracic aorta that often manifests as discrete stenosis but may be tortuous or in long segment. The study aimed to evaluate pre and post-surgical aspects of pediatric patients submitted to CoA surgical correction and to identify possible predisposing factors for aortic recoarctation. METHODS: Twenty-five patients were divided into groups according to presence (N=8) or absence (N=17) of recoarctation after surgical correction of CoA and evaluated according to clinical-demographic profile, vascular characteristics via computed angiotomography (CAT), and other pathological conditions. RESULTS: Majority of males (64%), ≥ 15 days old (76%), ≥ 2.5 kg (80%). There was similarity between groups with and without recoarctation regarding sex (male: 87% vs. 53%; P=0.277), age (≥ 15 days: 62.5 vs. 82%; P=0.505), and weight (≥ 2.5 kg: 87.5 vs. 76.5; P=0,492). Altered values of aortic root/Valsalva diameter, proximal transverse arch, and distal isthmus, and normal values for aorta prevailed in preoperative CAT. Normal values for the aortic root/Valsalva sinus diameter were observed with and without recoarctation, the same for both groups regarding ascending and descending aorta in postoperative CAT. No significant difference for altered values of proximal transverse arch and alteration in distal isthmus was observed. CONCLUSION: No predictive risk for recoarctation was observed. CTA proved to be important in CoA diagnosis and management, since CoA is mainly related with altered diameter of aortic root/sinus of Valsalva and proximal and distal aortic arch/isthmus, however, it failed to show predictive risk for recoarctation.

Results of a hybrid approach for high risk term newborn patients with interrupted aortic arch (IAA) with left ventricular outflow tract obstruction.

This study aims to share the results of critically ill newborn cases with interrupted aortic arch (IAA) and Left ventricular outflow tract (LVOT) obstruction (LVOTO) who underwent the hybrid approach, which consists of bilateral pulmonary artery banding and/or patent ductus arteriosus stenting, as first-line treatment. This retrospective study includes the results of high-risk term newborns whom we applied a hybrid approach due to IAA and LVOTO in our clinic between January 1, 2021 and December 31, 2021. The demographic characteristics, hybrid approach methods and results of the cases were evaluated. Nine cases underwent hybrid approach during the study period. The mean age and weight at interventions were 7 days (3-16 days) and 3280 g (2700-4300 g). Six of the patients were diagnosed with type B IAA, 2 with type A, and one with type C. LVOTO was present in 7 patients. The success rate for the procedures was 100%. No patients died during the procedure or within the first 5 days after the procedure or from reasons related to the procedure. The median length of the hospital stay after stent placement was 28 days (22-35 days) for discharged patients. Three patients died in interstage period, and 6 patients underwent total corrective surgery after a median of 7 months (4-10 months). The average LVOT diameter was increased from 3.1 mm to 4.8 mm before total repair surgery. The hybrid approach should be kept in mind for treating high risk newborns with IAA with LVOTO and high-risk newborns who are not suitable for single stage total corrective surgery.

Association of Prenatally Diagnosed Isolated Single Left Superior Vena Cava and Postnatal Development of Coarctation of the Aorta.

To report the prevalence of coarctation of the aorta (CoA) in fetuses with single left superior vena cava (SL-SVC) and to evaluate changes in echocardiographic measurements. Additionally, to report the prevalence of associated malformations. Retrospective observational study of fetuses diagnosed with SL-SVC between 2012 and 2021 at a tertiary fetal cardiology unit. In fetuses without intracardiac abnormalities, Z-scores of the ventricles, great arteries, and Doppler flow patterns are reported. We identified 47 fetuses with SL-SVC of which 8/47 (17%) had abnormal intracardiac anatomy. One fetus was lost to follow-up. Of those with normal intracardiac anatomy and postnatal follow-up (38), karyotype abnormalities were confirmed in 2/38 (5%) and ECA in 8/38 (21%). 33/38 were live-born. None developed CoA postnatally. Paired analysis of Z-scores between early and late scans of 24 fetuses showed that diameters of the right heart structures and Doppler flows of tricuspid valve increased significantly during pregnancy, while the left heart structures and flow patterns did not change. The median risk of CoA did not change between the early and the late scan. We did not observe CoA in this cohort. A degree of ventricular asymmetry was present, but this was due to right heart dominance rather than hypoplasia of left heart structures. This likely reflects redistribution of blood and does not appear to confer increased risk of CoA. Predictive models of the postnatal development of CoA which set the dimensions of right and left heart structures in relation might not be applicable in this situation.

Medium-Term Outcomes of Stent Therapy for Aortic Coarctation in Children Under 30 kg with New Generation Low-Profile Stents: A Follow-Up Study of a Single Centre Experience.

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.

Non-invasive Estimation of Pressure Drop Across Aortic Coarctations: Validation of 0D and 3D Computational Models with In Vivo Measurements.

Blood pressure gradient ( Δ P ) across an aortic coarctation (CoA) is an important measurement to diagnose CoA severity and gauge treatment efficacy. Invasive cardiac catheterization is currently the gold-standard method for measuring blood pressure. The objective of this study was to evaluate the accuracy of Δ P estimates derived non-invasively using patient-specific 0D and 3D deformable wall simulations. Medical imaging and routine clinical measurements were used to create patient-specific models of patients with CoA (N = 17). 0D simulations were performed first and used to tune boundary conditions and initialize 3D simulations. Δ P across the CoA estimated using both 0D and 3D simulations were compared to invasive catheter-based pressure measurements for validation. The 0D simulations were extremely efficient ( ∼ 15 s computation time) compared to 3D simulations ( ∼ 30 h computation time on a cluster). However, the 0D Δ P estimates, unsurprisingly, had larger mean errors when compared to catheterization than 3D estimates (12.1 ± 9.9 mmHg vs 5.3 ± 5.4 mmHg). In particular, the 0D model performance degraded in cases where the CoA was adjacent to a bifurcation. The 0D model classified patients with severe CoA requiring intervention (defined as Δ P ≥ 20 mmHg) with 76% accuracy and 3D simulations improved this to 88%. Overall, a combined approach, using 0D models to efficiently tune and launch 3D models, offers the best combination of speed and accuracy for non-invasive classification of CoA severity.

Coral reef aorta: a rare form of obstruction of the ascending aorta in adolescent with aortopathy- case report.

BACKGROUND: Supra aortic obstruction in children is uncommon and is seen in certain unique conditions. While intraluminal obstruction due to heavy calcification is seen in older populations, it is not described in pediatric populations. The coral reef aorta is a rare and distinct calcifying disease causing luminal obstruction of the suprarenal aorta in adults. The definition of this diagnosis relies entirely on the unique aspects and consistency of the lesions, which are rock-hard, irregular, gritty plaques with a white luminal surface resembling a coral reef. However, no such case has been described in children. CASE PRESENTATION: We present an adolescent boy who presented with a heavily calcified ascending aortic lesion associated with aortopathy and hypertension, 12 years after an aortic coarctation repair. The investigations included echocardiography, magnetic resonance and computer-tomographic imaging. A 3-D model was printed in order to visualize and plan surgical steps in advance for safe placement of clamps and defining the extent of resection. In addition, it provided an idea about tissue quality, thickness, spatial relationship, and orientation in relation to surrounding structures. Successful resection and replacement of the diseased segment of the aorta were achieved on cardiopulmonary bypass support. Post-operative recovery was uneventful, and at 6-month follow-up, the patient is doing well. In this report, various aspects of such lesions have been discussed, including clinical presentations, complications, planning and conduct of a safe cardiopulmonary bypass, and precautions during surgery for a successful outcome. CONCLUSION: Complicated obstructive aortic lesions in children require careful assessment, appropriate advanced imaging, and the use of 3-D printing technology in order to plan and perform safe and effective surgical management. The etiology of severe calcified aorta in children may be related to metabolic factors, previous surgery, use of a homograft, or an inflammatory process. However, it has yet to be proven.

A Meta-Analysis for Postoperative Alternations of Aortic Coarctation.

OBJECTIVE: To investigate postoperative vascular changes of patients with coarctation of the aorta (CoA). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Impact of left ventricular hemodynamic forces in adult patients with treated aortic coarctation and preserved left ventricular systolic function.

BACKGROUND: The LV myocardial strain and hemodynamic forces (HDFs) are innovative markers of LV function. Aortic coarctation is safely repaired in infancy; however, mortality and morbidity remain increased in later life. The study investigated the role of left ventricular myocardial deformation and HDFs in asymptomatic patients who underwent successful aortic coarctation repair. METHODS: Clinical and echocardiographic data were analyzed from 42 repaired CoA, 32 ± 20 years after surgery, 2D echocardiographic global longitudinal strain (GLS), circumferential strain (GCS) and HDFs were determined. CoA patients were compared with 42 patients affected by blood hypertension and 84 healthy controls; all matched for age and gender. RESULTS: All groups had normal LV ejection fraction (LVEF), dimensions, and volumes. CoA patients showed a significantly higher rate of LV mass indexed (p < .001) and left atrial volumes indexed (p < .001). LV myocardial and endocardial global longitudinal and circumferential strain were decreased in CoA patients (p < .001, p < .001; p = .032 and p < .001, respectively). HDF parameters such as LV longitudinal force, LV systolic longitudinal force and LV impulse (LVim) were uniformly reduced (p = .006, p = .001, and p = .001, respectively). LV myocardial strain and HDF parameter values were independently associated with hospitalization for heart failure on univariable Cox regression analysis. CONCLUSION: Despite preserved LVEF, patients with CoA had lower LV myocardial strain and HDF parameters values, independently associated with hospitalization for heart failure.

An Undiagnosed Shone Complex in a 52-Year-Old Female: A Case Report.

Shone complex (SC) is a rare congenital heart disease characterized by four obstructive anomalies, including parachute mitral valve (PMV), left atrial supra-valvular ring, subaortic stenosis, and coarctation of the aorta. Typically, SC manifests early in life. However, we encountered a 52-year-old female with a history of hypertension diagnosed at 26 years and left-sided weakness poststroke. She presented with worsening dyspnea and palpitations, prompting a thorough investigation. Echocardiography revealed a heavily calcified bicuspid aortic valve with severe aortic stenosis and parachute mitral valve with severe mitral stenosis and preserved ejection fraction, raising suspicions regarding the presence of SC. Cardiac catheterization, aortic-angiography, and noncontrast chest computed tomography (CT) revealed abrupt occlusion of the postductal aorta, giving a picture of aortic coarctation with well-established collateral vessels including prominent right and left internal mammary arteries. So, she was diagnosed with an incomplete SC at the age of 52. Shone complex is a rare congenital heart disease that typically presents in early childhood, but late presentations due to misdiagnosis or incomplete work up are possible. This case emphasizes the rarity of late presentations of SC and highlights the importance of early diagnosis and intervention to improve outcomes. An incomplete SC should be considered in adult patients presenting with left-sided obstructive lesions.

Diagnostic accuracy of prenatal ultrasound in coarctation of aorta: systematic review and individual participant data meta-analysis.

OBJECTIVE: To determine the diagnostic accuracy of prenatal ultrasound in detecting coarctation of the aorta (CoA). METHODS: An individual participant data meta-analysis was performed to report on the strength of association and diagnostic accuracy of different ultrasound signs in detecting CoA prenatally. MEDLINE, EMBASE and CINAHL were searched for studies published between January 2000 and November 2021. Inclusion criteria were fetuses with suspected isolated CoA, defined as ventricular and/or great vessel disproportion with right dominance on ultrasound assessment. Individual participant-level data were obtained by two leading teams. PRISMA-IPD and PRISMA-DTA guidelines were used for extracting data, and the QUADAS-2 tool was used for assessing quality and applicability. The reference standard was CoA, defined as narrowing of the aortic arch, diagnosed after birth. The most commonly evaluated parameters on ultrasound, both in B-mode and on Doppler, constituted the index test. Summary estimates of sensitivity, specificity, diagnostic odds ratio (DOR) and likelihood ratios were computed using the hierarchical summary receiver-operating-characteristics model. RESULTS: The initial search yielded 72 studies, of which 25 met the inclusion criteria. Seventeen studies (640 fetuses) were included. On random-effects logistic regression analysis, tricuspid valve/mitral valve diameter ratio > 1.4 and > 1.6, aortic isthmus/arterial duct diameter ratio < 0.7, hypoplastic aortic arch (all P < 0.001), aortic isthmus diameter Z-score of < -2 in the sagittal (P = 0.003) and three-vessel-and-trachea (P < 0.001) views, pulmonary artery/ascending aorta diameter ratio > 1.4 (P = 0.048) and bidirectional flow at the foramen ovale (P = 0.012) were independently associated with CoA. Redundant foramen ovale was inversely associated with CoA (P = 0.037). Regarding diagnostic accuracy, tricuspid valve/mitral valve diameter ratio > 1.4 had a sensitivity of 72.6% (95% CI, 48.2-88.3%), specificity of 65.4% (95% CI, 46.9-80.2%) and DOR of 5.02 (95% CI, 1.82-13.9). The sensitivity and specificity values were, respectively, 75.0% (95% CI, 61.1-86.0%) and 39.7% (95% CI, 27.0-53.4%) for pulmonary artery/ascending aorta diameter ratio > 1.4, 47.8% (95% CI, 14.6-83.0%) and 87.6% (95% CI, 27.3-99.3%) for aortic isthmus diameter Z-score of < -2 in the sagittal view and 74.1% (95% CI, 58.0-85.6%) and 62.0% (95% CI, 41.6-78.9%) for aortic isthmus diameter Z-score of < -2 in the three-vessel-and-trachea view. Hypoplastic aortic arch had a sensitivity of 70.0% (95% CI, 42.0-88.6%), specificity of 91.3% (95% CI, 78.6-96.8%) and DOR of 24.9 (95% CI, 6.18-100). The diagnostic yield of prenatal ultrasound in detecting CoA did not change significantly when considering multiple categorical parameters. Five of the 11 evaluated continuous parameters were independently associated with CoA (all P < 0.001) but all had low-to-moderate diagnostic yield. CONCLUSIONS: Several prenatal ultrasound parameters are associated with an increased risk for postnatal CoA. However, diagnostic accuracy is only moderate, even when combinations of parameters are considered. © 2024 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Multicenter Study of Long-Term Outcomes and Quality of Life in PHACE Syndrome after Age 10.

OBJECTIVE: To characterize long-term outcomes of PHACE syndrome. STUDY DESIGN: Multicenter study with cross-sectional interviews and chart review of individuals with definite PHACE syndrome ≥10 years of age. Data from charts were collected across multiple PHACE-related topics. Data not available in charts were collected from patients directly. Likert scales were used to assess the impact of specific findings. Patient-Reported Outcomes Measurement Information System (PROMIS) scales were used to assess quality of life domains. RESULTS: A total of 104/153 (68%) individuals contacted participated in the study at a median of 14 years of age (range 10-77 years). There were infantile hemangioma (IH) residua in 94.1%. Approximately one-half had received laser treatment for residual IH, and the majority (89.5%) of participants were satisfied or very satisfied with the appearance. Neurocognitive manifestations were common including headaches/migraines (72.1%), participant-reported learning differences (45.1%), and need for individualized education plans (39.4%). Cerebrovascular arteriopathy was present in 91.3%, with progression identified in 20/68 (29.4%) of those with available follow-up imaging reports. Among these, 6/68 (8.8%) developed moyamoya vasculopathy or progressive stenoocclusion, leading to isolated circulation at or above the level of the circle of Willis. Despite the prevalence of cerebrovascular arteriopathy, the proportion of those with ischemic stroke was low (2/104; 1.9%). PROMIS global health scores were lower than population norms by at least 1 SD. CONCLUSIONS: PHACE syndrome is associated with long-term, mild to severe morbidities including IH residua, headaches, learning differences, and progressive arteriopathy. Primary and specialty follow-up care is critical for PHACE patients into adulthood.

Fetal echocardiographic features associated with bicuspid aortic valve.

BACKGROUND: Prenatal diagnosis of bicuspid aortic valve is challenging. Bicuspid aortic valve is often associated with aortic dilation. METHODS: Fetuses with postnatally confirmed bicuspid aortic valve were gestational age-matched with normal controls. Complex lesions were excluded. Aortic valve and arch measurements by two blinded investigators were compared. RESULTS: We identified 27 cases and 27 controls. Estimated fetal weight percentile was lower in cases than controls. Seven cases had one or more significant lesions including perimembranous ventricular septal defects (n = 2), isolated annular hypoplasia (n = 2), and/or arch hypoplasia/coarctation (n = 4). Fetuses with bicuspid aortic valves had significantly smaller median z-scores of the aortic annulus (-1.60 versus -0.53, p < 0.001) and root (-1.10 versus -0.53, p = 0.040), and larger ratios of root to annulus (1.32 versus 1.21, p < 0.001), sinotubular junction to annulus (1.07 versus 0.99, p < 0.001), ascending aorta to annulus (1.29 versus 1.18, p < 0.001), and transverse aorta to annulus (1.04 versus 0.96, p = 0.023). Leaflets were "doming" in 11 cases (41%) and 0 controls (p = 0.010), "thickened" in 10 cases (37%) and 0 controls (p = 0.002). We noted similar findings in the subgroup without significant additional cardiac defects. CONCLUSIONS: The appearance of doming or thickened aortic valve leaflets on fetal echocardiogram is associated with bicuspid aortic valve. Compared to controls, fetuses with bicuspid aortic valve had smaller aortic annulus sizes (possibly related to smaller fetal size) without proportionally smaller aortic measurements, resulting in larger aortic dimension to annulus ratios. Despite inherent challenges of diagnosing bicuspid aortic valve prenatally, these findings may increase suspicion and prompt appropriate postnatal follow-up.

Diagnostic and Prognostic Role of Left Ventricular Strain Imaging in Adults with Coarctation of aorta.

The relative diagnostic and prognostic performance of left ventricular (LV) global longitudinal strain (LVGLS) compared with LV ejection fraction (LVEF) and the role of LVGLS for detecting the early stages of LV systolic dysfunction in adults with repaired coarctation of the aorta are unknown. This study aimed to address these knowledge gaps. We used a retrospective cohort study of adults with repaired coarctation of the aorta who underwent transthoracic echocardiogram (2003 to 2020). LV systolic function was assessed using LVEF (derived from volumetric analysis) and LVGLS (derived from speckle-tracking echocardiography). Of the 795 patients (age 36 ± 14 years), the mean LVEF and LVGLS were 62 ± 11% and 21 ± 4%, respectively. The prevalence of LV systolic dysfunction was higher when assessed using LVGLS than using LVEF (20% vs 6%, p <0.001). Of 795 patients, 94 (12%) patients died, of which 75 (9%) died from cardiovascular causes. LVGLS provided more robust prognostic power in predicting the all-cause mortality than LVEF, as evidenced by a higher C-statistic (0.743, 95% confidence interval 0.730 to 0.755 vs 0.782, 95% confidence interval 0.771 to 0.792, p <0.001). Furthermore, patients with normal LVEF in the setting of reduced LVGLS had a higher risk of all-cause mortality (than patients with normal LVGLS and LVEF) and were at risk for a temporal decrease in LVEF during follow-up. These findings suggest that the use of LVGLS for risk stratification can help identify high-risk patients and provide opportunities for interventions, which would, in turn, improve clinical outcomes. Further studies are required to empirically test these postulates.

Pre-operative Imaging of Critical Coarctation of the Aorta: Impact on Surgical Approach and Outcomes.

Computed tomographic angiography (CTA) has been increasingly used for the evaluation of infants with aortic arch hypoplasia and coarctation of the aorta. The goals of this study were to compare echocardiographic and CTA findings in critical coarctation of the aorta, to evaluate each modality's influence on surgical approach for repair and determine if pre-operative measurements or surgical approach are associated with residual lesions/re-interventions. This was a single-center retrospective cohort study that included 85 neonates and infants who underwent repair of coarctation/arch hypoplasia by three months of age. Two groups were compared: patients with pre-operative echocardiograms only and patients with both echocardiogram and CTA evaluations. 44 (52%) patients received an echocardiogram and CTA, and 41 (48%) patients received an echocardiogram only. Patients in the CTA + echo group had smaller mitral valve and ascending aorta measurements (p = 0.01). When comparing CTA to echocardiogram measurements, the aortic valve annulus, ascending aorta, proximal and distal transverse arch, and isthmus were smaller on echo (p < 0.01). A smaller aortic valve annulus and aortic root as well as thoracotomy approach were associated with residual gradients/re-intervention (p < 0.01). Our study found that patients who underwent CTA preoperatively had smaller left-sided structures. Aortic measurements were smaller on echocardiogram when compared to CTA. Smaller left-sided structures proximal to the aortic arch and thoracotomy predicted the development of residual lesions/re-intervention. CTA is useful in the surgical planning for neonates with arch hypoplasia/coarctation and may help risk stratify for residual lesions/re-intervention.