Imaging findings of multisystem inflammatory syndrome in children associated with COVID-19.

BACKGROUND: A hyperinflammatory immune-mediated shock syndrome has been recognised in children exposed to the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the virus that causes coronavirus disease 2019 (COVID-19). OBJECTIVE: To describe typical imaging findings in children with multisystem inflammatory syndrome associated with COVID-19. MATERIALS AND METHODS: During the first wave of the COVID-19 pandemic, imaging studies and clinical data from children treated for multisystem inflammatory syndrome were collected from multiple centres. Standardised case templates including demographic, biochemical and imaging information were completed by participating centres and reviewed by paediatric radiologists and paediatricians. RESULTS: We included 37 children (21 boys; median age 8.0 years). Polymerase chain reaction (PCR) testing was positive for SARS-CoV-2 in 15/37 (41%) children and immunoglobulins in 13/19 children (68%). Common clinical presentations were fever (100%), abdominal pain (68%), rash (54%), conjunctivitis (38%) and cough (32%). Thirty-three children (89%) showed laboratory or imaging findings of cardiac involvement. Thirty of the 37 children (81%) required admission to the intensive care unit, with good recovery in all cases. Chest radiographs demonstrated cardiomegaly in 54% and signs of pulmonary venous hypertension/congestion in 73%. The most common chest CT abnormalities were ground-glass and interstitial opacities (83%), airspace consolidation (58%), pleural effusion (58%) and bronchial wall thickening (42%). Echocardiography revealed impaired cardiac function in half of cases (51%) and coronary artery abnormalities in 14%. Cardiac MRI showed myocardial oedema in 58%, pericardial effusion in 42% and decreased left ventricular function in 25%. Twenty children required imaging for abdominal symptoms, the commonest abnormalities being free fluid (71%) and terminal ileum wall thickening (57%). Twelve children underwent brain imaging, showing abnormalities in two cases. CONCLUSION: Children with multisystem inflammatory syndrome showed pulmonary, cardiac, abdominal and brain imaging findings, reflecting the multisystem inflammatory disease. Awareness of the imaging features of this disease is important for early diagnosis and treatment.

Lung T1 mapping magnetic resonance imaging in the assessment of pulmonary disease in children with cystic fibrosis: a pilot study.

BACKGROUND: Assessment tools for early cystic fibrosis (CF) lung disease are limited. Detecting early pulmonary disease is crucial to increasing life expectancy by starting interventions to slow the progression of the pulmonary disease with the many treatment options available. OBJECTIVE: To compare the utility of lung T1-mapping MRI with ultrashort echo time (UTE) MRI in children with cystic fibrosis in detecting early stage lung disease and monitoring pulmonary exacerbations. MATERIALS AND METHODS: We performed a prospective study in 16 children between September 2017 and January 2018. In Phase 1, we compared five CF patients with normal spirometry (mean 11.2 years) to five age- and gender-matched healthy volunteers. In Phase 2, we longitudinally evaluated six CF patients (median 11 years) in acute pulmonary exacerbation. All children had non-contrast lung T1-mapping and UTE MRI and spirometry testing. We compared the mean normalized T1 value and percentage lung volume without T1 value in CF patients and healthy subjects in Phase 1 and during treatment in Phase 2. We also performed cystic fibrosis MRI scoring. We evaluated differences in continuous variables using standard statistical tests. RESULTS: In Phase 1, mean normalized T1 values of the lung were significantly lower in CF patients in comparison to healthy controls (P=0.02) except in the right lower lobe (P=0.29). The percentage lung volume without T1 value was also significantly higher in CF patients (P=0.006). UTE MRI showed no significant differences between CF patients and healthy volunteers (P=0.11). In Phase 2, excluding one outlier case who developed systemic disease in the course of treatment, the whole-lung T1 value increased (P=0.001) and perfusion scoring improved (P=0.02) following therapy. We observed no other significant changes in the MRI scoring. CONCLUSION: Lung T1-mapping MRI can detect early regional pulmonary CF disease in children and might be helpful in the assessment of acute pulmonary exacerbations.

Thoracic imaging of coronavirus disease 2019 (COVID-19) in children: a series of 91 cases.

BACKGROUND: Pulmonary infection with SARS-CoV-2 virus (severe acute respiratory syndrome coronavirus 2; COVID-19) has rapidly spread worldwide to become a global pandemic. OBJECTIVE: To collect paediatric COVID-19 cases worldwide and to summarize both clinical and imaging findings in children who tested positive on polymerase chain reaction testing for SARS-CoV-2. MATERIALS AND METHODS: Data were collected by completion of a standardised case report form submitted to the office of the European Society of Paediatric Radiology from March 12 to April 8, 2020. Chest imaging findings in children younger than 18 years old who tested positive on polymerase chain reaction testing for SARS-CoV-2 were included. Representative imaging studies were evaluated by multiple senior paediatric radiologists from this group with expertise in paediatric chest imaging. RESULTS: Ninety-one children were included (49 males; median age: 6.1 years, interquartile range: 1.0 to 13.0 years, range: 9 days-17 years). Most had mild symptoms, mostly fever and cough, and one-third had coexisting medical conditions. Eleven percent of children presented with severe symptoms and required intensive unit care. Chest radiographs were available in 89% of patients and 10% of them were normal. Abnormal chest radiographs showed mainly perihilar bronchial wall thickening (58%) and/or airspace consolidation (35%). Computed tomography (CT) scans were available in 26% of cases, with the most common abnormality being ground glass opacities (88%) and/or airspace consolidation (58%). Tree in bud opacities were seen in 6 of 24 CTs (25%). Lung ultrasound and chest magnetic resonance imaging were rarely utilized. CONCLUSION: It seems unnecessary to perform chest imaging in children to diagnose COVID-19. Chest radiography can be used in symptomatic children to assess airway infection or pneumonia. CT should be reserved for when there is clinical concern to assess for possible complications, especially in children with coexisting medical conditions.

Differences in Pulmonary and Systemic Flow Measurements by Cardiac Magnetic Resonance vs Cardiac Catheterization and Relation to Collateral Flow in Single Ventricle Patients.

Both cardiac magnetic resonance (CMR) and cardiac catheterization (cath) may assess patients with single ventricle physiology prior to stage II or Fontan palliation. However, development of significant aortopulmonary collaterals may invalidate assumptions of the Fick method. We compared CMR and cath flow measurements and evaluated the relation to collateral flow. This single-center study included all pre-stage II and pre-Fontan patients between 2010 and 2017 with CMR and cath within 1 month. Pulmonary (Qp) and systemic flow (Qs) by cath were calculated by Fick method. CMR Qp was calculated by total pulmonary venous flow, and Qs by total vena caval flow. Collateral flow by CMR was the difference of pulmonary vein and pulmonary artery flow. In 26 studies (16 pre-stage II and 10 pre-Fontan) in 21 patients, collateral flow was higher in pre-Fontan patients (1.8 ± 0.6 vs 0.9 ± 0.8 L/min/m2, p = 0.01). Overall, CMR and cath had good agreement for Qs and Qp:Qs, with moderate correlation (r = 0.44, p = 0.02 for Qs, r = 0.48, p = 0.02 for Qp:Qs). In pre-Fontan but not in pre-stage II patients, CMR had higher Qp (mean difference - 1.71 L/min/m2) and Qp:Qs (mean difference - 0.36). The underestimation of cath Qp correlated with amount of collateral flow (r = - 0.47, p = 0.02). Neither cath nor CMR flow measurements correlated with outcomes in this small cohort. In conclusion, collaterals lead to systematically higher Qp and Qp:Qs measurements by CMR vs cath in single ventricle patients. Measurements may not be used interchangeably, with potential clinical significance in estimating pulmonary vascular resistance. Further study is necessary to evaluate possible relation to clinical outcomes.

Differential Myocardial Mechanics in Volume and Pressure Loaded Right Ventricles Demonstrated by Cardiac Magnetic Resonance.

In patients with D-looped transposition of the great arteries (D-TGA) status post atrial switch operation, the systemic right ventricle (RV) shifts to predominantly circumferential (CS) rather than longitudinal strain (LS), which may represent adaptation or dysfunction. We aimed to evaluate myocardial mechanics in pressure loaded, volume-loaded, and normal RVs by cardiac magnetic resonance (CMR). Patients with D-TGA post atrial switch operation with CMR from 2008 to 2015 were matched 1:1 for age and RV ejection fraction (EF) with repaired tetralogy of Fallot (TOF) patients (volume-loaded RVs), and 1:1 for age with control patients. RV free wall LS and CS were measured using feature tracking software (TomTec, Unterscleissheim, Germany). A total of 32 D-TGA (median age 32 years, 56% male), 32 TOF, and 32 control patients were included. D-TGA patients had less dilatation than TOF patients (125 ± 35 ml/m2 vs. 149 ± 44 ml/m2, p = 0.02) and lower RVEF than controls (42.9 ± 7.7% vs. 56.3 ± 5.6%, p < 0.0001). RV LS was similar in D-TGA and TOF ( - 13.2 ± 4.5% vs. - 14.5 ± 5.9%, p = 0.32), both decreased compared to controls. However, CS in D-TGA was higher than controls ( - 14.1 ± 4.1% vs. - 11.4 ± 4.4%, p = 0.01), with a higher CS:LS ratio (1.2 ± 0.7 vs. 0.6 ± 0.3, p < 0.0001), while CS in TOF and controls did not differ. RVEF in D-TGA correlated closely with CS (r = - 0.85, p < 0.0001) but not LS (r = 0.10, p = 0.58). I n conclusion, CMR can differentiate strain patterns in pressure- and volume-loaded RVs, with decreased LS in both conditions, while systemic RVs compensate with supra-normal CS. CS may be a more clinically relevant measure of RV function in this population.

Predictors of missed appointments in patients referred for congenital or pediatric cardiac magnetic resonance.

BACKGROUND: Congenital cardiac magnetic resonance is a limited resource because of scanner and physician availability. Missed appointments decrease scheduling efficiency, have financial implications and represent missed care opportunities. OBJECTIVE: To characterize the rate of missed appointments and identify modifiable predictors. MATERIALS AND METHODS: This single-center retrospective study included all patients with outpatient congenital or pediatric cardiac MR appointments from Jan. 1, 2014, through Dec. 31, 2015. We identified missed appointments (no-shows or same-day cancellations) from the electronic medical record. We obtained demographic and clinical factors from the medical record and assessed socioeconomic factors by U.S. Census block data by patient ZIP code. Statistically significant variables (P<0.05) were included into a multivariable analysis. RESULTS: Of 795 outpatients (median age 18.5 years, interquartile range 13.4-27.1 years) referred for congenital cardiac MR, a total of 91 patients (11.4%) missed appointments; 28 (3.5%) missed multiple appointments. Reason for missed appointment could be identified in only 38 patients (42%), but of these, 28 (74%) were preventable or could have been identified prior to the appointment. In multivariable analysis, independent predictors of missed appointments were referral by a non-cardiologist (adjusted odds ratio [AOR] 5.8, P=0.0002), referral for research (AOR 3.6, P=0.01), having public insurance (AOR 2.1, P=0.004), and having scheduled cardiac MR from November to April (AOR 1.8, P=0.01). CONCLUSION: Demographic factors can identify patients at higher risk for missing appointments. These data may inform initiatives to limit missed appointments, such as targeted education of referring providers and patients. Further data are needed to evaluate the efficacy of potential interventions.

CT for Assessment of Thrombosis and Pulmonary Embolism in Multiple Stages of Single-Ventricle Palliation: Challenges and Suggested Protocols.

The total cavopulmonary connection (TCPC), or Fontan procedure, diverts systemic venous blood directly into the pulmonary arteries and is the palliative surgery of choice for patients with a wide variety of congenital heart diseases with single-ventricle physiologic characteristics. Pulmonary embolism and thrombosis are known complications and are among the major causes of morbidity and mortality in patients after TCPC. Magnetic resonance (MR) imaging is usually performed for postoperative evaluation of patients after single-ventricle repair; however, screening for thrombosis or embolism with MR imaging is not always feasible because of the emergent nature of the clinical presentation or because of artifacts from metallic devices or coils. Computed tomographic (CT) angiography is an effective method for diagnosing pulmonary embolism in children. However, because of altered hemodynamics after single-ventricle palliation, there are unique challenges in achieving optimal opacification of the pulmonary arteries and Fontan circuit that can result in nondiagnostic CT angiographic studies or erroneous image interpretation. Radiologists should be familiar with the multiple stages of single-ventricle palliation, understand the technique for performing pulmonary CT angiography at each stage, and recognize common pitfalls in obtaining and interpreting pulmonary CT angiographic images in patients who have undergone single-ventricle repair. Online supplemental material is available for this article. (©)RSNA, 2016.

Use of a 1.0 Tesla open scanner for evaluation of pediatric and congenital heart disease: a retrospective cohort study.

BACKGROUND: Open cardiovascular magnetic resonance (CMR) scanners offer the potential for imaging patients with claustrophobia or large body size, but at a lower 1.0 Tesla magnetic field. This study aimed to evaluate the efficacy of open CMR for evaluation of pediatric and congenital heart disease. METHODS: This retrospective, cross-sectional study included all patients ≤18 years old or with congenital heart disease who underwent CMR on an open 1.0 Tesla scanner at two centers from 2012-2014. Indications for CMR and clinical questions were extracted from the medical record. Studies were qualitatively graded for image quality and diagnostic utility. In a subset of 25 patients, signal-to-noise (SNR) and contrast-to-noise (CNR) ratios were compared to size- and diagnosis-matched patients with CMR on a 1.5 Tesla scanner. RESULTS: A total of 65 patients (median 17.3 years old, 60% male) were included. Congenital heart disease was present in 32 (50%), with tetralogy of Fallot and bicuspid aortic valve the most common diagnoses. Open CMR was used due to scheduling/equipment issues in 51 (80%), claustrophobia in 7 (11%), and patient size in 3 (5%); 4 patients with claustrophobia had failed CMR on a different scanner, but completed the study on open CMR without sedation. All patients had good or excellent image quality on black blood, phase contrast, magnetic resonance angiography, and late gadolinium enhancement imaging. There was below average image quality in 3/63 (5%) patients with cine images, and 4/15 (27%) patients with coronary artery imaging. SNR and CNR were decreased in cine and magnetic resonance angiography images compared to 1.5 Tesla. The clinical question was answered adequately in all but 2 patients; 1 patient with a Fontan had artifact from an embolization coil limiting RV volume analysis, and in 1 patient the right coronary artery origin was not well seen. CONCLUSIONS: Open 1.0 Tesla scanners can effectively evaluate pediatric and congenital heart disease, including patients with claustrophobia and larger body size. Despite minor artifacts and differences in SNR and CNR, the majority of clinical questions can be answered adequately, with some limitations with coronary artery imaging. Further evaluation is necessary to optimize protocols and image quality.

MRI of pediatric cardiac masses.

OBJECTIVE: The purpose of this article is to describe the characteristic cardiac MRI features of primary and secondary cardiac tumors, including differentiation from masslike lesions, such as thrombus or focal myocardial hypertrophy. CONCLUSION: The frequency and type of cardiac tumors in children differ from those in adults. Although transthoracic echocardiography is the initial imaging technique of choice for evaluation of cardiac tumors, cardiac MRI is an important complementary modality for characterization of the mass and effect on cardiac function.

Real-Time Strain-Encoding Cardiovascular MRI for Assessment of Regional Heart Function in Tetralogy of Fallot Patients.

BACKGROUND: Tetralogy of Fallot (ToF) is the most common form of cyanotic congenital heart disease, where right ventricular (RV) function is an important determinant of subsequent intervention. OBJECTIVE: In this study, we evaluate the feasibility of fast strain-encoding (fastSENC; a one-heartbeat sequence) magnetic resonance imaging (MRI) for assessing regional cardiac function in ToF. METHOD: FastSENC was implemented to characterize regional circumferential (Ecc) and longitudinal (Ell) strains in the left ventricle (LV) and RV in post-repair ToF. Data analysis was conducted to compare strain measurements in the RV to those in the LV, as well as to those generated by the MRI Tissue-Tracking (MRI-TT) technique, and to assess the relationship between strain and ejection fraction (EF). RESULTS: Despite normal LVEF (55±8.5%), RVEF was borderline (46±6.4%), but significantly lower than LVEF. RV strains (RV-Ell=-20.2±2.9%, RV-Ecc=-15.7±6.4%) were less than LV strains (LV-Ell=-21.7±3.7%, LV-Ecc=-18.3±4.7%), and Ell was the dominant strain component. Strain differences between fastSENC and MRI-TT were less significant in RV than in LV. There existed moderate and weak correlations for RV-Ecc and RV-Ell, respectively, against RVEF. Compared to LV strain, RV strain showed regional heterogeneity with a trend for reduced strain from the inferior to anterior regions. Inter-ventricular strain delay was larger for Ell (64±47ms) compared to Ecc (36±40ms), reflecting a trend for contraction dyssynchrony. CONCLUSION: FastSENC allows for characterizing subclinical regional RV dysfunction in ToF. Due to its sensitivity for evaluating regional myocardial contractility patterns and real-time imaging capability without the need for breath-holding, fastSENC makes it more suitable for evaluating RV function in ToF.

Evaluation with cardiovascular MR imaging of baffles and conduits used in palliation or repair of congenital heart disease.

A wide array of baffles and conduits are used in repair or palliation of congenital heart disease, which is the most common major birth defect, often with complex redirection of blood flow to achieve a more stable physiology. Cardiovascular magnetic resonance (CMR) imaging is an increasingly used modality for noninvasive assessment of anatomy and physiology both before and after surgical intervention, with highly reproducible measurements of ventricular size and function, quantification of valvular insufficiency and flow volumes, and excellent delineation of intracardiac and extracardiac anatomy. The authors review the indications, appearances on CMR images, and potential complications of various cardiovascular baffles and conduits: Mustard and Senning procedures, venoatrial baffles, intraventricular baffles, ventriculoarterial conduits, and baffles and conduits used in functional single-ventricle palliation. CMR imaging offers the most complete evaluation of single-ventricle anatomy and physiology, demonstrating the anatomy of venous pathways and pulmonary arteries and quantifying systemic ventricular size and systolic function, differential pulmonary blood flow, ratio of pulmonary to systemic blood flow, and aortopulmonary collateral flow. Anatomic and physiologic considerations are discussed, and suggested CMR imaging protocols and practical advice for performing and interpreting CMR studies are provided. The diversity and complexity of baffles and conduits complicates performance and interpretation of studies in this population, but a fundamental understanding of the goals of the procedure, postoperative physiology, and potential complications allows targeted imaging and precise reporting of clinically significant findings. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.323115096/-/DC1.

MRI of congenital urethroperineal fistula.

We present the MRI features of a congenital urethroperineal fistula diagnosed in a 12-year-old boy being evaluated after a single urinary tract infection. This diagnosis was initially suggested by voiding cystourethrogram and confirmed by MRI. Imaging revealed an abnormal fluid-filled tract arising from the posterior urethra and tracking to the perineal skin surface that increased in size during micturition. Surgical resection and histopathological evaluation of the abnormal tract confirmed the diagnosis of congenital urethroperineal fistula. MRI played important roles in confirming the diagnosis and assisting surgical planning.

Biatrial Drainage of the Right Superior Vena Cava: Imaging Findings.

Biatrial drainage of the right superior vena cava (SVC) is a rare form of interatrial shunting that can have substantial clinical consequences. Cross-sectional imaging techniques (CT and MRI) are well suited for evaluation and surgical planning. This review article focuses on the embryologic development, hemodynamics, and imaging features to enable a timely diagnosis. Biatrial drainage of the right SVC has important clinical implications, and knowledge of its imaging appearance and hemodynamics is essential in diagnosis and treatment planning. © RSNA, 2020.

3D-printed, externally-implanted, bioresorbable airway splints for severe tracheobronchomalacia.

OBJECTIVES/HYPOTHESIS: To report the clinical safety and efficacy of three-dimensional (3D)-printed, patient-specific, bioresorbable airway splints in a cohort of critically ill children with severe tracheobronchomalacia. STUDY DESIGN: Case series. METHODS: From 2012 to 2018, 15 subjects received 29 splints on their trachea, right and/or left mainstem bronchi. The median age at implantation was 8 months (range, 3-25 months). Nine children were female. Five subjects had a history of extracorporeal membrane oxygenation (ECMO), and 11 required continuous sedation, six of whom required paralytics to maintain adequate ventilation. Thirteen were chronically hospitalized, unable to be discharged, and seven were hospitalized their entire lives. At the time of splint implantation, one subject required ECMO, one required positive airway pressure, and 13 subjects were tracheostomy and ventilator dependent, requiring a median positive end-expiratory pressure (PEEP) of 14 cm H2 O (range, 6-20 cm H2 0). Outcomes collected included level of respiratory support, disposition, and splint-related complications. RESULTS: At the time of discharge from our institution, at a median of 28 days postimplantation (range, 10-56 days), the subject on ECMO was weaned from extracorporeal support, and the subjects who were ventilated via tracheostomy had a median change in PEEP (discharge-baseline) of -2.5 cm H2 O (range, -15 to 2 cm H2 O, P = .022). At median follow-up of 8.5 months (range, 0.3-77 months), all but one of the 12 surviving subjects lives at home. Of the 11 survivors who were tracheostomy dependent preoperatively, one is decannulated, one uses a speaking valve, six use a ventilator exclusively at night, and three remain ventilator dependent. CONCLUSIONS: This case series demonstrates the initial clinical efficacy of the 3D-printed bioresorbable airway splint device in a cohort of critically ill children with severe tracheobronchomalacia. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:1763-1771, 2019.

Optimized cardiac magnetic resonance imaging inversion recovery sequence for metal artifact reduction and accurate myocardial scar assessment in patients with cardiac implantable electronic devices.

Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) is the gold standard for imaging myocardial viability. An important application of LGE CMR is the assessment of the location and extent of the myocardial scar in patients with ventricular tachycardia (VT), which allows for more accurate identification of the ablation targets. However, a large percentage of patients with VT have cardiac implantable electronic devices (CIEDs), which is a relative contraindication for cardiac magnetic resonance imaging due to safety and image artifact concerns. Previous studies showed that these patients can be safely scanned on 1.5 T scanners provided that an adequate imaging protocol is adopted. Nevertheless, imaging patients with a CIED result in metal artifacts due to the strong frequency off-resonance effects near the device; therefore, the spins in the surrounding myocardium are not completely inverted, and thus give rise to hyperintensity artifacts. These artifacts obscure the myocardial scar tissue and limit the ability to study the correlation between the myocardial scar structure and the electro-anatomical map during catheter ablation. In this study, we developed a modified inversion recovery technique to alleviate the CIED-induced metal artifacts and improve the diagnostic image quality of LGE images in patients with CIEDs without increasing scan time or requiring additional hardware. The developed technique was tested in phantom experiments and in vivo scans, which showed its capability for suppressing the hyperintensity artifacts without compromising myocardium nulling in the resulting LGE images.

Relation of Increased Epicardial Fat After Fontan Palliation to Cardiac Output and Systemic Ventricular Ejection Fraction.

Epicardial fat produces multiple proinflammatory cytokines and is associated with adverse cardiovascular events. Inflammation and resultant endothelial dysfunction may play a role in progressive myocardial dysfunction among adults with single ventricle physiology after Fontan palliation, but the potential impact of increased epicardial fat volume (EFV) has not been studied. This study sought to determine if there is greater EFV in Fontan patients compared with a group of repaired tetralogy of Fallot (rTOF) patients. We retrospectively measured EFV manually on cardiac magnetic resonance imaging in Fontan patients, ≥15 years, and 1:1 age, sex, and body mass index-matched patients with rTOF. EFV was indexed to body surface area. A random subset of studies was re-measured to assess intra- and interobserver reliability. Fontan patients (n = 63, median age 21.6 years, 51% male, mean body mass index 24.2 ± 5.6 kg/m2) had a larger indexed EFV compared with matched rTOF patients (75.3 ± 29.2 ml/m2 vs 60.0 ± 19.9 ml/m2, p = 0.001). In Fontan patients, indexed EFV was inversely correlated with ventricular ejection fraction (r = -0.26, p = 0.04) and cardiac index (r = -0.33, p = 0.01). Intra- and interobserver reliabilities of the indexed EFV measurements in both groups were excellent (intraclass correlation coefficient ranges from 0.93 to 0.97). In conclusion, indexed EFV is higher in Fontan patients compared with patients with rTOF and is associated with lower ventricular ejection fraction and cardiac index. Increased EFV could play a role in the failing Fontan circulation, but longitudinal studies are necessary to establish any causative role.

Interpretation and Reporting of Coronary Arteries in Transposition of the Great Arteries: Cross-sectional Imaging Perspective.

Knowledge of coronary artery nomenclature in transposition of the great arteries is essential, given the increasing population of adults with congenital heart disease and greater utilization of imaging in these patients. This article reviews appropriate terminology for describing coronary artery anatomy, commonly encountered coronary artery patterns, and postoperative coronary complications in the setting of transposition of the great arteries.

Differential Impact of Pulmonary Regurgitation on Patients With Surgically Repaired Pulmonary Stenosis Versus Tetralogy of Fallot.

Patients with repaired pulmonary stenosis (PS) or tetralogy of Fallot (TOF) both develop pulmonary regurgitation (PR) leading to right ventricular (RV) dilatation and dysfunction. We aimed to characterize differential effects of chronic PR in these populations. Patients with surgically repaired PS were matched 1:2 by age and PR fraction with patients with TOF. Patients with previous pulmonary valve replacement were excluded. Cardiovascular magnetic resonance data were compared; peak longitudinal and circumferential systolic strain by feature tracking were compared to evaluate differential contribution of the RV sinus and outflow tract, respectively. PS (n = 24, 41 ± 13 years old) and TOF (n = 47, 39 ± 13 years old) patients did not differ in RV end-diastolic volume (153 ± 45 vs 154 ± 45 ml/m(2), p = 0.99) or diastolic function. However, patients with PS had preserved RV ejection fraction (54.3 ± 4.4% vs 48.0 ± 7.1%, p <0.0001). Differences were greater in RV circumferential (-15.8 ± 3.3 vs -11.8 ± 3.4, p <0.0001) than longitudinal strain (-18.0 ± 3.8 vs -15.9 ± 3.8, p = 0.04), with particular decrease in the infundibulum (-17.4 ± 7.5 vs -6.8 ± 6.3, p <0.0001). Late gadolinium enhancement in the RV outflow tract was more frequent in patients with TOF (70.2% vs 45.8%, p = 0.001). In conclusion, surgical repair of PS leads to similar RV dilatation and diastolic dysfunction compared to patients with TOF, but differential effects on ventricular systolic function, largely related to differences in the outflow tract. With different patterns of scarring and ventricular remodeling, further study is needed to clarify whether criteria for pulmonary valve replacement in patients with PS should differ from those with TOF.