Quantification of systemic-to-pulmonary collateral flow in univentricular physiology with 4D flow MRI.

PURPOSE: Systemic-to-pulmonary collateral flow is a well-recognised phenomenon in patients with single ventricle physiology, but remains difficult to quantify. The aim was to compare the reported formula's that have been used for calculation of systemic-to-pulmonary-collateral flow to assess their consistency and to quantify systemic-to-pulmonary collateral flow in patients with a Glenn and/or Fontan circulation using four-dimensional flow MRI (4D flow MR). METHODS: Retrospective case-control study of Glenn and Fontan patients who had a 4D flow MR study. Flows were measured at the ascending aorta, left and right pulmonary arteries, left and right pulmonary veins, and both caval veins. Systemic-to-pulmonary collateral flow was calculated using two formulas: 1) pulmonary veins - pulmonary arteries and 2) ascending aorta - caval veins. Anatomical identification of collaterals was performed using the 4D MR image set. RESULTS: Fourteen patients (n = 11 Fontan, n = 3 Glenn) were included (age 26 [22-30] years). Systemic-to-pulmonary collateral flow was significantly higher in the patients than the controls (n = 10, age 31.2 [15.1-38.4] years) with both formulas: 0.28 [0.09-0.5] versus 0.04 [-0.66-0.21] l/min/m2 (p = 0.036, formula 1) and 0.67 [0.24-0.88] versus -0.07 [-0.16-0.08] l/min/m2 (p < 0.001, formula 2). In patients, systemic-to-pulmonary collateral flow differed significantly between formulas 1 and 2 (13% versus 26% of aortic flow, p = 0.038). In seven patients, veno-venous collaterals were detected and no aortopulmonary collaterals were visualised. CONCLUSION: 4D flow MR is able to detect increased systemic-to-pulmonary collateral flow and visualise collaterals vessels in Glenn and Fontan patients. However, the amount of systemic-to-pulmonary collateral flow varies with the formula employed. Therefore, further research is necessary before it could be applied in clinical care.

Pediatric cardiothoracic vasculitis: multimodality imaging review.

The pediatric vasculitides are a relatively uncommon and heterogeneous group of disorders characterized by vessel inflammation, often with cardiothoracic involvement. Diagnosis and monitoring are often clinically challenging because of the nonspecific symptoms and laboratory markers. Thus, imaging has assumed increasing importance for early detection of disease activity, extent and complications as well as long-term monitoring pre- and post-treatment. Herein, we review the major pediatric vasculitides with frequent chest manifestations, including Takayasu arteritis, Kawasaki disease, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, Behçet disease and potential mimics. We highlight key clinical features and management considerations, emphasizing the central role of imaging.

Aorto-iliac/right leg arterial thrombosis necessitating limb amputation, pulmonary arterial, intracardiac, and ilio-caval venous thrombosis in a 40-year-old with COVID-19.

We describe a 40-year-old man with severe COVID-19 requiring mechanical ventilation who developed aorto-bi-iliac arterial, right lower extremity arterial, intracardiac, pulmonary arterial and ilio-caval venous thromboses and required right lower extremity amputation for acute limb ischemia. This unique case illustrates COVID-19-associated thrombotic complications occurring at multiple, different sites in the cardiovascular system of a single infected patient.

Cost-effectiveness analysis of magnetic resonance-guided focused ultrasound ablation for palliation of refractory painful bone metastases.

OBJECTIVE: The aim of this study was to determine if magnetic resonance-guided focused ultrasound (MRgFUS) is cost-effective compared with medication, for refractory pain from bone metastases in the United States. METHODS: We constructed a Markov state transition model using TreeAge Pro software (TreeAge Software, Inc., Williamstown, MA, USA) to model costs, outcomes, and the cost-effectiveness of a treatment strategy using MRgFUS for palliative treatment of painful bone metastases compared with a Medication Only strategy (Figure 1). Model transition state probabilities, costs (in 2018 US$), and effectiveness data (quality-adjusted life-years [QALYs]) were derived from available literature, local expert opinion, and reimbursement patterns at two U.S. tertiary academic medical centers actively performing MRgFUS. Costs and QALYs, discounted at three percent per year, were accumulated each month over a 24-month time horizon. One-way and probabilistic sensitivity analyses were performed. RESULTS: In the base-case analysis, the MRgFUS treatment strategy costs an additional $11,863 over the 2-year time horizon to accumulate additional 0.22 QALYs, equal to a $54,160/QALY ICER, thus making MRgFUS the preferred strategy. One-way sensitivity analyses demonstrate that for the base-case analysis, the crossover point at which Medication Only would instead become the preferred strategy is $23,341 per treatment. Probabilistic sensitivity analyses demonstrate that 67 percent of model iterations supported the conclusion of the base case. CONCLUSIONS: Our model demonstrates that MRgFUS is cost-effective compared with Medication Only for palliation of painful bone metastases for patients with medically refractory metastatic bone pain across a range of sensitivity analyses.

Machine learning for endoleak detection after endovascular aortic repair.

Diagnosis of endoleak following endovascular aortic repair (EVAR) relies on manual review of multi-slice CT angiography (CTA) by physicians which is a tedious and time-consuming process that is susceptible to error. We evaluate the use of a deep neural network for the detection of endoleak on CTA for post-EVAR patients using a novel data efficient training approach. 50 CTAs and 20 CTAs with and without endoleak respectively were identified based on gold standard interpretation by a cardiovascular subspecialty radiologist. The Endoleak Augmentor, a custom designed augmentation method, provided robust training for the machine learning (ML) model. Predicted segmentation maps underwent post-processing to determine the presence of endoleak. The model was tested against 3 blinded general radiologists and 1 blinded subspecialist using a held-out subset (10 positive endoleak CTAs, 10 control CTAs). Model accuracy, precision and recall for endoleak diagnosis were 95%, 90% and 100% relative to reference subspecialist interpretation (AUC = 0.99). Accuracy, precision and recall was 70/70/70% for generalist1, 50/50/90% for generalist2, and 90/83/100% for generalist3. The blinded subspecialist had concordant interpretations for all test cases compared with the reference. In conclusion, our ML-based approach has similar performance for endoleak diagnosis relative to subspecialists and superior performance compared with generalists.

Image-based scaling laws for somatic growth and pulmonary artery morphometry from infancy to adulthood.

Pulmonary artery (PA) morphometry has been extensively explored in adults, with particular focus on intra-acinar arteries. However, scaling law relationships for length and diameter of extensive preacinar PAs by age have not been previously reported for in vivo human data. To understand preacinar PA growth spanning children to adults, we performed morphometric analyses of all PAs visible in the computed tomography (CT) and magnetic resonance (MR) images from a healthy subject cohort [n = 16; age: 1-51 yr; body surface area (BSA): 0.49-2.01 m2]. Subject-specific anatomic PA models were constructed from CT and MR images, and morphometric information-diameter, length, tortuosity, bifurcation angle, and connectivity-was extracted and sorted into diameter-defined Strahler orders. Validation of Murray's law, describing optimal scaling exponents of radii for branching vessels, was performed to determine how closely PAs conform to this classical relationship. Using regression analyses of vessel diameters and lengths against orders and patient metrics (BSA, age, height), we found that diameters increased exponentially with order and allometrically with patient metrics. Length increased allometrically with patient metrics, albeit weakly. The average tortuosity index of all vessels was 0.026 ± 0.024, average bifurcation angle was 28.2 ± 15.1°, and average Murray's law exponent was 2.92 ± 1.07. We report a set of scaling laws for vessel diameter and length, along with other morphometric information. These provide an initial understanding of healthy structural preacinar PA development with age, which can be used for computational modeling studies and comparison with diseased PA anatomy.NEW & NOTEWORTHY Pulmonary artery (PA) morphometry studies to date have focused primarily on large arteries and intra-acinar arteries in either adults or children, neglecting preacinar arteries in both populations. Our study is the first to quantify in vivo preacinar PA morphometry changes spanning infants to adults. For preacinar arteries > 1 mm in diameter, we identify scaling laws for vessel diameters and lengths with patient metrics of growth and establish a healthy PA morphometry baseline for most preacinar PAs.

Sex differences in patients with repaired tetralogy of Fallot support a tailored approach for males and females: a cardiac magnetic resonance study.

Purpose Substantial differences between sexes exist with respect to cardiovascular diseases, including congenital heart disease. Nevertheless, clinical decisions in the long-term follow-up of patients with repaired tetralogy of Fallot (rTOF) are currently based on unisex thresholds for cardiac magnetic resonance (CMR) measurements. This study aimed to assess whether sex differences exist in cardiac adaptation to hemodynamic loading conditions in patients with rTOF. Methods and Results This cross-sectional, two-center, combined pediatric and adult cohort included 320 rTOF patients (163 males, 51%) who underwent routine CMR. Despite similar age (median and interquartile range [m + IQR] 23.4 [15.2-34.4] years), surgical history, and hemodynamic loading, males with rTOF demonstrated higher biventricular CMR-derived volumes and masses, indexed for body surface area, compared to females (e.g. m + IQR right ventricular (RV) end-diastolic volume: males 123 [100-151] mL/m2, females 114 [94-131] mL/m2, P = 0.007). Sex-specific Z-scores of biventricular volumes and masses were similar for males and females. RV volumes and masses correlated with hemodynamic loading, but these relations did not differ between sexes. Biventricular ejection fraction (EF) appeared to be lower in male patients, compared to female patients (e.g. m + IQR RVEF: males 48 [43-54]%, females 52 [46-57]%, P < 0.001). Conclusion Indexed ventricular volumes and masses are higher in males with rTOF, compared to females, similar to the healthy population. RV hypertrophy and dilatation correlated to loading conditions similarly for both sexes. However, under comparable loading conditions, males demonstrated more severe functional impairment. These results indicate that sex-differences should no longer be ignored in treatment strategies, including timing of pulmonary valve replacement.

Accuracy of a novel stress echocardiography pattern for myocardial bridging in patients with angina and no obstructive coronary artery disease - A retrospective and prospective cohort study.

BACKGROUND: Myocardial bridge (MB) may cause angina in patients with no obstructive coronary artery disease (CAD). We previously reported a novel stress echocardiography (SE) pattern of focal septal buckling with apical sparing in the end-systolic to early-diastolic phase that is associated with the presence of an MB. We evaluated the diagnostic accuracy of this pattern, and prospectively validated our results. METHODS: The retrospective cohort included 158 patients with angina who underwent both SE and coronary CT angiography (CCTA). The validation cohort included 37 patients who underwent CCTA in the emergency department for angina, and prospectively underwent SE. CCTA was used as a reference standard for the presence/absence of an MB, and also confirmed no obstructive CAD. RESULTS: In the retrospective cohort, an MB was present in 107 (67.7%). The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were 91.6%, 70.6%, 86.7% and 80%, respectively. On logistic regression, focal septal buckling and Duke treadmill score were associated with an MB. In the validation cohort, an MB was present in 31 (84%). The sensitivity, specificity PPV and NPV were 90.3%, 83.3%, 96.5% and 62.5%, respectively. On logistic regression, focal septal buckling was associated with an MB. CONCLUSION: Presence of focal septal buckling with apical sparing on SE is an accurate predictor of an MB in patients with angina and no obstructive CAD. This pattern can reliably be used to screen patients who may benefit from advanced non-invasive/invasive testing for an MB as a cause of their angina.

Fluid-structure interaction simulations of patient-specific aortic dissection.

Credible computational fluid dynamic (CFD) simulations of aortic dissection are challenging, because the defining parallel flow channels-the true and the false lumen-are separated from each other by a more or less mobile dissection membrane, which is made up of a delaminated portion of the elastic aortic wall. We present a comprehensive numerical framework for CFD simulations of aortic dissection, which captures the complex interplay between physiologic deformation, flow, pressures, and time-averaged wall shear stress (TAWSS) in a patient-specific model. Our numerical model includes (1) two-way fluid-structure interaction (FSI) to describe the dynamic deformation of the vessel wall and dissection flap; (2) prestress and (3) external tissue support of the structural domain to avoid unphysiologic dilation of the aortic wall and stretching of the dissection flap; (4) tethering of the aorta by intercostal and lumbar arteries to restrict translatory motion of the aorta; and a (5) independently defined elastic modulus for the dissection flap and the outer vessel wall to account for their different material properties. The patient-specific aortic geometry is derived from computed tomography angiography (CTA). Three-dimensional phase contrast magnetic resonance imaging (4D flow MRI) and the patient's blood pressure are used to inform physiologically realistic, patient-specific boundary conditions. Our simulations closely capture the cyclical deformation of the dissection membrane, with flow simulations in good agreement with 4D flow MRI. We demonstrate that decreasing flap stiffness from [Formula: see text] to [Formula: see text] kPa (a) increases the displacement of the dissection flap from 1.4 to 13.4 mm, (b) decreases the surface area of TAWSS by a factor of 2.3, (c) decreases the mean pressure difference between true lumen and false lumen by a factor of 0.63, and (d) decreases the true lumen flow rate by up to 20% in the abdominal aorta. We conclude that the mobility of the dissection flap substantially influences local hemodynamics and therefore needs to be accounted for in patient-specific simulations of aortic dissection. Further research to accurately measure flap stiffness and its local variations could help advance future CFD applications.

4D flow vs. 2D cardiac MRI for the evaluation of pulmonary regurgitation and ventricular volume in repaired tetralogy of Fallot: a retrospective case control study.

Lengthy exams and breath-holding limit the use of pediatric cardiac MRI (CMR). 3D time-resolved flow MRI (4DF) is a free-breathing, single-sequence exam that obtains magnitude (anatomic) and phase contrast (PC) data. We compare the accuracy of gadobenate dimeglumine-enhanced 4DF on a 1.5 T magnet to 2D CMR in children with repaired tetralogy of Fallot (rTOF) to measure pulmonary net flow (PNF) as a reflection of pulmonary regurgitation, forward flow (FF) and ventricular volumetry. Thirty-four consecutive cases were included. 2D PCs were obtained at the valve level. Using 4DF, we measured PNF at the valve and at the main and branch pulmonary arteries. PNF measured at the valve by 4DF demonstrated the strongest correlation (r = 0.87, p < 0.001) and lowest mean difference (3.5 ± 9.4 mL/beat) to aortic net flow (ANF). Semilunar FF and stroke volume of the respective ventricle demonstrated moderate-strong correlation by 4DF (r = 0.66-0.81, p < 0.001) and strong correlation by 2D (r = 0.81-0.84, p < 0.001) with similar correlations and mean differences between techniques (p > 0.05). Ventricular volumes correlated strongly between 2D and 4DF (r = 0.75-0.96, p < 0.001), though 4DF overestimated right ventricle volumes by 11.8-19.2 mL/beat. Inter-rater reliability was excellent for 2D and 4DF volumetry (ICC = 0.91-0.99). Ejection fraction moderately correlated (r = 0.60-0.75, p < 0.001) with better reliability by 4DF (ICC: 0.80-0.85) than 2D (ICC: 0.69-0.89). 4DF exams were shorter than 2D (9 vs. 71 min, p < 0.001). 4DF provides highly reproducible and accurate measurements of flow with slight overestimation of RV volumes compared to 2D in pediatric rTOF. 4DF offers important advantages in this population with long-term monitoring needs.

Assessment of airway abnormalities in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals.

BACKGROUND: Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/MAPCAs) are at risk for post-operative respiratory complications after undergoing unifocalisation surgery. Thus, we assessed and further defined the incidence of airway abnormalities in our series of over 500 children with TOF/MAPCAs as determined by direct laryngoscopy, chest computed tomography (CT), and/or bronchoscopy. METHODS: The medical records of all patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery from March, 2002 to June, 2018 were reviewed. Anaesthesia records, peri-operative bronchoscopy, and/or chest CT reports were reviewed to assess for diagnoses of abnormal or difficult airway. Associations between chromosomal anomalies and airway abnormalities - difficult anaesthetic airway, bronchoscopy, and/or CT findings - were defined. RESULTS: Of the 564 patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery at our institution, 211 (37%) had a documented chromosome 22q11 microdeletion and 28 (5%) had a difficult airway/intubation reported at the time of surgery. Chest CT and/or peri-operative bronchoscopy were performed in 234 (41%) of these patients. Abnormalities related to malacia or compression were common. In total 35 patients had both CT and bronchoscopy within 3 months of each other, with concordant findings in 32 (91%) and partially concordant findings in the other 3. CONCLUSION: This is the largest series of detailed airway findings (direct laryngoscopy, CT, and bronchoscopy) in TOF/MAPCAS patients. Although these findings are specific to an at-risk population for airway abnormalities, they support the utility of CT and /or bronchoscopy in detecting airway abnormalities in patients with TOF/MAPCAs.

Feasibility and utility of dual-energy chest CTA for preoperative planning in pediatric pulmonary artery reconstruction.

The purpose of this study was to assess in pediatric pulmonary artery (PA) reconstruction candidates the feasibility and added utility of preoperative chest computed tomography angiography (CTA) using dual-energy technique, from which perfused blood volume (PBV)/iodine maps can be generated as a surrogate of pulmonary perfusion. Pediatric PA reconstruction patients were prospectively recruited for a new dose-neutral dual-energy CTA protocol. For each case, the severity of anatomic PA obstruction was graded by two pediatric cardiovascular radiologists in consensus using a modified Qanadli index. PBV maps were qualitatively reviewed and auto-segmented using Siemens syngo.via software. Associations between Qanadli scores and PBV were assessed with Spearman correlation (r) and ROC analysis. Effective radiation doses were estimated from dose-length product and ICRP 103 k-factors, using cubic Hermite spline interpolation. 19 patients were recruited with mean (SD) age of 6.0 (5.1), 11 (57.9%) female, 11 (73.7%) anesthetized. Higher QS correlated with lower PBV, both on a whole lung (r = - 0.54, p < 0.001) and lobar (r = - 0.50, p < 0.001) basis. The lung with lowest absolute PBV was predictive of the lung with highest Qanadli score, with AUC of 0.70 (95% CI 0.47-0.93). Qualitatively, PBV maps were heterogeneous, corresponding to multifocal PA stenoses, with decreased iodine content in areas of most severe obstruction. In conclusion, dual-energy chest CTA is feasible for pediatric PA reconstruction candidates. PBV maps show deficits in regions of more severe anatomic obstruction and may serve as a novel biomarker in this population.

Outcomes After Aortopulmonary Window for Hypoplastic Pulmonary Arteries and Dual-Supply Collaterals.

BACKGROUND: Our institutional approach to tetralogy of Fallot with major aortopulmonary collateral arteries (MAPCAs) emphasizes early unifocalization and complete repair (CR). In the small subset of patients with dual-supply MAPCAs and confluent but hypoplastic central pulmonary arteries (PAs), our surgical approach is early creation of an aortopulmonary window (APW) to promote PA growth. Factors associated with successful progression to CR and mid-term outcomes have not been assessed. METHODS: Clinical data were reviewed. PA diameters were measured offline from angiograms prior to APW and on follow-up catheterization >1 month after APW but prior to any additional surgical interventions. RESULTS: From November 2001 to March 2018, 352 patients with tetralogy of Fallot/MAPCAs underwent initial surgery at our center, 40 of whom had a simple APW with or without ligation of MAPCAs as the first procedure (median age, 1.4 months). All PA diameters increased significantly on follow-up angiography. Ultimately, 35 patients underwent CR after APW. Nine of these patients (26%) underwent intermediate palliative operation between 5 and 39 months (median, 8 months) after APW. There were no early deaths. The cumulative incidence of CR was 65% 1 year post-APW and 87% at 3 years. Repaired patients were followed for a median of 4.2 years after repair; the median PA:aortic pressure ratio was 0.39 (range, 0.22 to 0.74). CONCLUSIONS: Most patients with tetralogy of Fallot/MAPCAs and hypoplastic but normally arborizing PAs and dual-supply MAPCAs are able to undergo CR with low right ventricular pressure after APW early in life. Long-term outcomes were good, with acceptable PA pressures in most patients.

Evolution of hemodynamic forces in the pulmonary tree with progressively worsening pulmonary arterial hypertension in pediatric patients.

Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling resulting in right ventricular (RV) dysfunction and ultimately RV failure. Mechanical stimuli acting on the vessel walls of the full pulmonary tree have not previously been comprehensively characterized. The goal of this study is to characterize wall shear stress (WSS) and strain in pediatric PAH patients at different stages of disease severity using computational patient-specific modeling. Computed tomography, magnetic resonance imaging and right heart catheterization data were collected and assimilated into pulmonary artery (PA) models for patients with and without PAH. Patients were grouped in three disease severity groups (control, moderate and severe) based on clinical evaluations. A finite element solver was employed to quantify hemodynamics and wall strains. To estimate WSS in the distal small PAs with diameters ranging from 50 to 500 [Formula: see text], a morphometric tree model was created, with inputs coming from outlets of the 3D model. WSS in the proximal PAs decreased with disease severity (control 20.5 vs. moderate 15.8 vs. severe 6.3 [Formula: see text], [Formula: see text]). Oscillatory shear index increased in the main pulmonary artery (MPA) with disease severity (0.13 vs. 0.13 vs. 0.2, [Formula: see text]). Wall strains measured by the first invariant of Green strain tensor decreased with disease severity (0.16 vs. 0.12 vs. 0.11, [Formula: see text]). Mean WSS for the distal PAs between 100 and 500 [Formula: see text] significantly increased with disease severity (20 vs. 52 vs. 116 [Formula: see text], [Formula: see text]). In conclusion, 3D flow simulations showed that WSS is significantly decreased in the MPA with disease while the mathematical morphometric model suggested increased WSS in the distal small vessels. Computational models can reveal mechanical stimuli acting on vessel walls that may inform patient risk stratification and flow shear experiments.

Risk of cardiac tachyarrhythmia in patients with repaired tetralogy of Fallot: a multicenter cardiac MRI based study.

Cardiac tachyarrhythmias are the leading cause of morbidity and mortality in patients with repaired tetralogy of Fallot (TOF). We evaluated risk factors for sustained ventricular tachyarrhythmia (VT) and atrial tachyarrhythmia (ATA) in these patients. Patients (n = 319) who underwent cardiac magnetic resonance (CMR) imaging at two tertiary centers between 2007 and 2016 were assessed. Potential risk markers, based on history, cardiac magnetic resonance imaging (CMR), electrocardiography (ECG) and echocardiography, were analyzed for prediction of the primary endpoint of VT, and the secondary endpoint of ATA. During a follow-up of 3.5 (0.9-6.1) years, 20 (6.3%) patients reached the primary endpoint, and 30 (9.4%) the secondary endpoint. Multivariable cox hazards regression identified right ventricular (RV) end-diastolic volume (Hazard ratio [HR] 2.03, per 10 ml/m2 increase; p = 0.02), RV end-systolic volume (HR 3.04, per 10 ml/m2 increase; p = 0.04), RV mass (HR 1.88, per 10 g/m2 increase; p = 0.02), and RV ejection fraction (HR 6.06, per 10% decrease; p = 0.02) derived from CMR to be independent risk factors of VT. In addition, QRS-duration (HR 1.70, per 10 ms increase; p = 0.001) and body mass index (BMI: HR 1.8, per 5 kg/m2 increase; p = 0.02) were independent markers of VT. Older age at TOF repair (HR 1.33, per 2 months increase; p = 0.03) and BMI (HR 1.76, per 5 kg/m2 increase; p < 0.001) independently predicted ATA. RV systolic dysfunction, hypertrophy and dilatation on CMR, together with QRS prolongation, and obesity are predictive of VT in TOF patients. Older age at TOF repair and obesity were associated with the occurrence of ATA.

Repair of Untreated Older Patients With Tetralogy of Fallot With Major Aortopulmonary Collaterals.

BACKGROUND: Our programmatic approach to tetralogy of Fallot with major aortopulmonary collaterals emphasizes single-stage unifocalization with complete intracardiac repair during infancy. Little is known about suitability for complete repair in patients beyond infancy. We sought to analyze outcomes of our approach in older patients with previously untreated tetralogy of Fallot with major aortopulmonary collaterals. METHODS: Any patient with this lesion not treated before 2 years of age referred to our center from 2002 to 2017 met inclusion criteria. RESULTS: Of 33 patients, 32 were out-of-state (64% international) referrals, and 33% (n = 11) were older than 9 years, had polycythemia, or at least 1 high pressure collateral (>25 mm Hg). Complete repair was achieved in 94% (n = 31) of patients, 82% (n = 27) in one stage and 12% (n = 4) after unifocalization to a central shunt. The median right ventricular-to-aortic pressure ratio was 0.31 after the operation and 0.37 at follow-up. At a median of 4.8 years after repair, 9 patients (19%) underwent reintervention, including 5 conduit and 7 branch pulmonary artery interventions. Three patients also underwent aortic valve replacement. CONCLUSIONS: In this selected cohort of older patients with previously unoperated tetralogy of Fallot with major aortopulmonary collaterals, outcomes were comparable with infants undergoing treatment according to our approach. These findings support the notion that patients who are either born in low-resource settings or present to health care providers beyond infancy should be considered candidates and evaluated for complete repair.

Computational simulation of postoperative pulmonary flow distribution in Alagille patients with peripheral pulmonary artery stenosis.

BACKGROUND: Up to 90% of individuals with Alagille syndrome have congenital heart diseases. Peripheral pulmonary artery stenosis (PPS), resulting in right ventricular hypertension and pulmonary flow disparity, is one of the most common abnormalities, yet the hemodynamic effects are ill-defined, and optimal patient management and treatment strategies are not well established. The purpose of this pilot study is to use recently refined computational simulation in the setting of multiple surgical strategies, to examine the influence of pulmonary artery reconstruction on hemodynamics in this population. MATERIALS AND METHODS: Based on computed tomography angiography and cardiac catheterization data, preoperative pulmonary artery models were constructed for 4 patients with Alagille syndrome with PPS (all male, age range: 0.6-2.9 years), and flow simulations with deformable walls were performed. Surgeon directed virtual surgery, mimicking the surgical procedure, was then performed to derive postoperative models. Postoperative simulation-derived hemodynamics and blood flow distribution were then compared with the clinical results. RESULTS: Simulations confirmed substantial resistance, resulting from preoperative severe ostial stenoses, and the use of newly developed adaptive outflow boundary conditions led to excellent agreement with in vivo measurements. Relief of PPS decreased pulmonary artery pressures and improved pulmonary flow distribution both in vivo and in silico with good correlation. CONCLUSIONS: Using adaptive outflow boundary conditions, computational simulations can estimate postoperative overall pulmonary flow distribution in patients with Alagille syndrome after pulmonary artery reconstruction. Obstruction relief along with pulmonary artery vasodilation determines postoperative pulmonary flow distribution and newer methods can incorporate these physiologic changes. Evolving blood flow simulations may be useful in surgical or transcatheter planning and in understanding the complex interplay among various obstructions in patients with peripheral pulmonary stenosis.

Myocardial Bridges on Coronary Computed Tomography Angiography　- Correlation With Intravascular Ultrasound and Fractional Flow Reserve.

BACKGROUND: Myocardial bridges (MB) are commonly seen on coronary CT angiography (CCTA) in asymptomatic individuals, but in patients with recurrent typical angina symptoms, yet no obstructive coronary artery disease (CAD), evaluation of their potential hemodynamic significance is clinically relevant. The aim of this study was to compare CCTA to invasive coronary angiography (ICA), including intravascular ultrasound (IVUS), to confirm MB morphology and estimate their functional significance in symptomatic patients.Methods and Results:We retrospectively identified 59 patients from our clinical databases between 2009 and 2014 in whom the suspicion for MB was raised by symptoms of recurrent typical angina in the absence of significant obstructive CAD on ICA. All patients underwent CCTA, ICA and IVUS. MB length and depth by CCTA agreed well with length (0.6±23.7 mm) and depth (CT coverage) as seen on IVUS. The product of CT length and depth (CT coverage), (MB muscle index (MMI)), ≥31 predicted an abnormal diastolic fractional flow reserve (dFFR) ≤0.76 with a sensitivity and specificity of 74% and 62% respectively (area under the curve=0.722). CONCLUSIONS: In patients with recurrent symptoms of typical angina yet no obstructive CAD, clinicians should consider dynamic ischemia from an MB in the differential diagnosis. The product of length and depth (i.e., MMI) by CCTA may provide some non-invasive insight into the hemodynamic significance of a myocardial bridge, as compared with invasive assessment with dFFR.

Surgical Repair of 115 Patients With Anomalous Aortic Origin of a Coronary Artery From a Single Institution.

OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) has been associated with myocardial ischemia and sudden death. The past decade has provided important insights into the natural history and typical patterns of presentation. However, there are also a number of unresolved controversies regarding the indications for surgery and the efficacy of that surgery. The purpose of this study was to review our surgical experience with AAOCA in 115 patients at a single institution. DESIGN: One hundred and fifteen patients have undergone surgical repair of AAOCA at our institution. There were 82 males and 33 females, and the median age at surgery was 16 years. Fifty-nine patients had preoperative symptoms of myocardial ischemia, including 56 with exertional chest pain or syncope and 3 sudden death events. Twenty-four patients had associated congenital heart defects. Seven patients had an associated myocardial bridge. RESULTS: Surgical repair was accomplished by unroofing of an intramural coronary in 86, reimplantation in 9, and pulmonary artery translocation in 20. There has been no early or late mortality. Fifty-seven (97%) of the 59 symptomatic patients have been free of any cardiac symptoms postoperatively. Two patients had recurrent symptoms and underwent reoperation (one had revision of the initial repair and one had repair of a myocardial bridge). CONCLUSIONS: Surgical repair of AAOCA can be safely performed and is highly efficacious in relieving symptoms of myocardial ischemia. The two "surgical failures" in this series had an anatomic basis and underscore the need to reassess both the proximal and distal anatomy in these patients.

CT-defined phenotype of pulmonary artery stenoses in Alagille syndrome.

BACKGROUND: Alagille syndrome is a rare disorder commonly associated with pulmonary artery stenosis. Studies exist discussing the cardiovascular sequela but no consistent phenotype, or pattern of pulmonary artery stenosis, has been described. OBJECTIVE: The objective of this study was to characterize the distribution and severity of pulmonary artery stenosis in patients with Alagille syndrome based on computed tomography angiography. MATERIALS AND METHODS: A retrospective chart review identified patients with Alagille syndrome who had undergone CT angiography. Pulmonary trunk (MPA), left main pulmonary artery (LPA) and right main pulmonary artery (RPA) diameters in Alagille patients were compared with those from matched control subjects. Stenoses at lobar and segmental pulmonary arteries were categorized as: Grade 1 (<33% stenosis), Grade 2 (33-66% stenosis) or Grade 3 (>66% stenosis). Involvement among the different lung regions was then compared. RESULTS: Fifteen patients ages 6 months to 17 years were identified; one had surgical augmentation of the central pulmonary arteries and was excluded from the central (main, right and left) pulmonary artery analysis. The proximal LPA and RPA, but not the MPA, were significantly smaller than those of the control subjects (P<0.01). The proximal LPA was significantly smaller than the proximal RPA (P<0.01) in the Alagille group (0.55 LPA:RPA ratio). Within the Alagille group, 75% of the lobar and segmental branches showed mild or no stenoses (Grade 1), 17% showed moderate stenosis (Grade 2) and 8% showed severe stenosis (Grade 3). While not statistically significant, the right lung demonstrated a greater percentage of Grades 2 and 3 stenoses (28%, right vs. 20% left, P=0.1). The right middle and lingula lobes of both lungs showed more Grade 2 and 3 stenoses (33% upper/middle vs. 18% lower, P<0.01). CONCLUSION: We describe a common pattern pulmonary artery stenosis in Alagille patients consisting of severe proximal LPA stenosis, heavy involvement of the lobar and segmental branches (more often right than left), and a greater involvement of the upper lobes. Knowledge of this phenotypic pattern can help in the diagnosis of Alagille syndrome in patients presenting with pulmonary artery stenosis.