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Management of patients with hypertrophic obstructive cardiomyopathy (HOCM) and severe mitral annular calcification, can be challenging. Our cases highlight the importance of addressing all elements contributing to the left ventricular outflow tract (LVOT) obstruction in cases of HOCM: hypertrophic basal interventricular septum, abnormal papillary muscles, systolic anterior motion of the anterior mitral valve leaflet. Addressing the mitral valve repair through the aortotomy, by performing a septal myectomy, papillary muscle realignment, and a resection of aberrant chordae tendineae, allows LVOT obstruction and systolic anterior motion to be successfully corrected. The success of these procedures depended on pre-operative imaging and intra-operative provocation.

Objectives This study sought to assess training volumes and its relationship to learning and identify potential new thresholds for determining expertise. Background Competency-based medical education (CBME) is being rapidly adopted and therefore training programs will need to adapt and identify new and novel methods of defining, measuring, and assessing clinical skills. Methods Consecutive cardiac computed tomography (CT) studies were interpreted independently by trainees and expert readers, and their interpretations (Agatston score, coronary artery disease severity, and Coronary Artery Disease Reporting and Data System) were collected. Kappa agreements were measured between trainees and experts for every 50 consecutive cases. Agreements between trainees and experts were tracked and compared with the agreement between expert readers. Results A total of 36 trainees interpreted 14,432 cardiac CT studies. Agreement between trainees and experts increased with CT case volumes, but trainees learned at different rates. Using a threshold for expertise, skill of measuring coronary calcification was achieved within 50 cases, but expertise for coronary CT angiography appeared to require a mean case volume of 750, comprising 400 abnormal cases. Conclusions Current volume-based training guidelines may be insufficient and higher case volumes may be required. We demonstrate that tracking cardiac CT learners is feasible and that CBME could be incorporated into CT training programs.

Magnetic resonance imaging (MRI) is often considered the gold-standard test for characterizing cardiac as well as noncardiac structure and function. However, many patients with cardiac implantable electronic devices (CIEDs) and/or severe renal dysfunction are unable to undergo this test because of safety concerns. In the past 10 years, newer-generation CIEDs and gadolinium-based contrast agents (GBCAs) as well as coordinated care between imaging and heart rhythm device teams have mitigated risk to patients and improved access to MRI at many hospitals. The purpose of this statement is to review published data on safety of MRI in patients with conditional and nonconditional CIEDs in addition to patient risks from older and newer GBCAs. This statement was developed through multidisciplinary collaboration of pan-Canadian experts after a relevant and independent literature search by the Canadian Agency for Drugs and Technologies in Health. All recommendations align with the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. Key recommendations include: (1) the development of standardized protocols for patients with a CIED undergoing MRI; (2) patients with MRI nonconditional pacemakers and pacemaker dependency should be programmed to asynchronous mode and those with MRI nonconditional transvenous defibrillators should have tachycardia therapies turned off during the scan; and (3) macrocyclic or newer linear GBCAs should be used in preference to older GBCAs because of their better safety profile in patients at higher risk of nephrogenic systemic fibrosis.

Determine in cardiac amyloid (CA) patients, whether cardiac CT derived extracellular volume (ECV) correlates with that obtained by MRI. Perform this correlation with single (SECT) versus dual energy (DECT) CT and evaluate whether a single sample volume ECV-measure was as reliable as a global (16 segment) assessment.CA patients who had undergone a clinical cardiac MRI (CMR) were recruited prospectively. SECT and DECT cardiac scans were performed. Three ECG-triggered prospective SECT scans were acquired: non-contrast, arterial-phase contrast and 5-minute delayed images. A DECT scan was performed at 7 min. Post processing was used to determine ECV. Analyses of SECT or DECT global ECV versus CMR were performed using the Pearson correlation coefficient, Bland Altman analysis and Intraclass correlation coefficient (ICC). Similar analyses were performed to examine the performance of single-segment sampling by SECT or DECT versus CMR.25 patients were recruited, mean age was 80.0 ± 7.1 years, 80 % were male, 21 patients had transthyretin- CA, 4 had light chain- CA. Correlations were close with both SECT or DECT global ECV versus CMR (r = 0.79 and 0.88 respectively, p 0.001 for both). Reliability of both SECT and DECT to assess global ECV in comparison to CMR was good: ICC for SECT was 0.88 (95 % CI 0.73-0.95) and 0.93 (95 % CI 0.82-0.97) for DECT. For single volume sampling techniques: correlations were close with both SECT or DECT versus CMR (r = 0.60 and 0.72 respectively, p 0.01 for both) There was no difference in ICC for SECT (0.74, 95 %CI 0.41-0.88) versus DECT (0.84, 95 % CI 0.63-0.93). Wider confidence intervals were noted for ICC with single versus global CT derived ECV assessment. Mean effective radiation dose was for SECT was 5.49 ± 8.04 mSv and 6.90 ± 3.01 mSv for DECT dual energy CT (p = 0.75).Global ECV values derived by both DECT or SECT correlated with those obtained by CMR and demonstrated good reliability by ICC in a population of CA patients. DECT and SECT single sampling derived ECV values also demonstrated close correlation and good reliability but the ICCs for single sampling had wider confidence intervals than global ECV assessment.

In the 2017 American Heart Association (AHA) Kawasaki disease (KD) guidelines, risk levels (RLs) for long-term management are defined by both maximal and current coronary artery (CA) dimensions normalized as z-scores. We sought to determine the degree to which current recommended practice differs from past actual practice, highlighting areas for knowledge translation efforts. The International KD Registry (IKDR) included 1651 patients with CA aneurysms (z-score > 2.5) from 1999 to 2016. Patients were classified by AHA RL using maximum CA z-score (RL 3 = small, RL 4 = medium, RL 5 = large/giant) and subcategorized based on decreases over time. Medical management provided was compared to recommendations. Low-dose acetylsalicylic acid (ASA) use ranged from 86 (RL 3.1) to 95% (RL 5.1) for RLs where use was “indicated.” Dual antiplatelet therapy (ASA + clopidogrel) use ranged from 16% for RL 5.2 to 9% for RL 5.4. Recommended anticoagulation (warfarin or low molecular weight heparin) use was 65% for RL 5.1, while 12% were on triple therapy (anticoagulation + dual antiplatelet). Optional statin use ranged from 2 to 8% depending on RL. Optional beta-blocker use was 2–25% for RL 5, and 0–5% for RLs 3 and 4 where it is not recommended. Generally, past practice was consistent with the latest AHA guidelines, taking into account the flexible wording of recommendations based on the limited evidence, as well as unmeasured patient-specific factors. In addition to strengthening the overall evidence base, knowledge translation efforts may be needed to address variation in thromboprophylaxis management.

Coronary artery calcification (CAC) on body CT imaging is considered a coincidental finding in cancer patients. In order to determine the significance of CAC in cancer patients we evaluated the prognostic utility of CAC detected on oncology FDG-PET/CT studies. A retrospective study was performed of consecutive FDG-PET/CT studies from January to March 2011. CAC was identified on the CT portion of FDG/PET-CT studies. Chart review documented statin use, the Framingham risk score (FRS) (includes age, diabetes, hypertension, dyslipidemia and smoking), the primary malignancy and metastases. The primary end point was a composite of death and cardiovascular (CV) events (non-fatal myocardial infarction (MI), PCI or coronary artery bypass surgery (CABG)). 266 patients had a median follow up of 41 months (95% CI 31–56 months). CAC was noted in 140 patients. Based on CAC, potentially 84 patients would have had a change in statin prescribing (p

Hypertrophic cardiomyopathy (HCM) is one of the commonest inherited cardiac abnormalities. The disorder is clinically and genetically heterogeneous and is characterized by left ventricular wall thickening that is not explained by abnormal loading conditions. HCM is estimated to affect between 1 in 200 and 1 in 500 people in the general population. In the majority of cases, HCM patients have a relatively benign course; however, if left untreated, this abnormality can lead to sudden cardiac death, especially in young adults and athletes. Therefore, early diagnosis is crucial to help implement the proper management for patients with HCM. In response to the growing need for more HCM centres of excellence in Canada, we developed one such centre at the University of Ottawa Heart Institute from the start of 2018. This centre will help in the early diagnosis and management of HCM patients, especially those with left ventricular outflow tract obstruction who might benefit from myectomy surgery. This paper describes our early experience with surgical myectomy in adult HCM patients between January 2018 and December 2020. We report the results of 27 patients with HCM who underwent myectomy surgery during the study period. All 27 patients survived to discharge, and all were still alive at 6 months postdischarge. Our experience highlights the crucial role that preoperative and perioperative imaging play in the management of this condition, in addition to the vital role of having a committed "heart team" of cardiologists, surgeons, and anesthesiologists.La cardiomyopathie hypertrophique (CMH) est l’une des plus fréquentes anomalies cardiaques héréditaires. L’anomalie qui est cliniquement et génétiquement hétérogène est caractérisée par l’épaississement de la paroi du ventricule gauche qui n’est pas expliqué par des conditions de charge anormales. On estime que la CMH touche entre une personne sur 200 et une personne sur 500 dans la population générale. Dans la plupart des cas de CMH, l’évolution de la maladie est relativement bénigne. Toutefois, si cette anomalie n’est pas traitée, elle peut mener à la mort subite d’origine cardiaque, particulièrement chez les jeunes adultes et les athlètes. Par conséquent, le diagnostic précoce est crucial à la mise en œuvre d’une prise en charge adéquate des patients atteints de CMH. Pour répondre à la nécessité croissante d’un plus grand nombre de centres d’excellence sur la CMH au Canada, nous avons mis en place l’un de ces centres à l’Institut de cardiologie de l’Université d’Ottawa dès le début de 2018. Ce centre contribuera au diagnostic précoce et à la prise en charge des patients atteints de CMH, particulièrement des patients, dont la CMH est associée à une obstruction de la chambre de chasse du ventricule gauche, qui pourraient bénéficier d’une myectomie. Le présent article décrit nos premières expériences de myectomie chez les patients adultes atteints de CMH entre janvier 2018 et décembre 2020. Nous présentons les résultats de 27 patients atteints de CMH qui ont subi une myectomie durant la période étudiée. Les 27 patients ont survécu jusqu’à la sortie de l’hôpital, et étaient tous encore en vie six mois après. Notre expérience démontre le rôle crucial que joue l’imagerie en phase préopératoire et en phase périopératoire lors de la prise en charge de cette maladie, en plus du rôle essentiel de l‘« équipe de cardiologie » dévouée qui est composée de cardiologues, de chirurgiens et d’anesthésistes.

Background While an assessment of the right ventricular (RV) size remains challenging, the entire RV is can be imaged on coronary computed tomography angiography (CCTA) studies. With prospective ECG-triggering, the RV end diastolic volume (RVEDV) cannot be measured; however, the RV mid-diastolic volume (RVMDV) can still be measured accurately from routine CCTA data sets. The objective of this study is to establish normal reference values for RVMDV. Methods Right ventricular mid-diastolic volumes were measured in 4855 consecutive patients undergoing prospectively ECG-triggered coronary CTA. All patients with known cardiac or pulmonary disease (coronary artery disease, myocardial infarction, revascularization, heart failure, pulmonary hypertension, congenital heart disease, valvular heart disease, atrial fibrillation, implantable cardiac defibrillator implantation, cardiac transplant, or cardiac surgery) or smoking history (3313 patients) were excluded. Results 1542 patients were analyzed (mean age 56.4 ± 11.1 years, mean BSA 1.96 ± 0.26 and 47% male). The mean RVMDV for men and women was 168.6 ± 37.6 mL and 117.6 ± 26.4 mL, respectively. Mean BSA-indexed RVMDV was 80.0 ± 15.3 mL/m2 and 64.1 ± 12.2 mL/m2 for men and women, respectively. The presence of hypertension and diabetes did not have an impact on these values. RVMDV and BSA-indexed RVMDV were lower in women and in older individuals. Conclusion Normal reference ranges for RVMDV were established using prospectively ECG-triggered coronary CTA studies. This data can be used to identify patients with abnormal RV volumes and potentially RV dysfunction, adding incremental diagnostic value to routine CCTA studies.

BACKGROUND Left ventricular mass (LVM) is a predictor for adverse cardiovascular outcomes. Coronary atherosclerosis (coronary artery disease [CAD]) and concentric left ventricular (LV) remodeling are linked pathophysiologically by endothelial dysfunction. AIM This study sought to determine the potential association between coronary atherosclerosis and LVM. METHODS A total of 2384 consecutive patients, without structural heart disease or a medical history of CAD, undergoing prospective mid-diastolic electrocardiogram-gated computed tomography coronary angiography were enrolled in the study. LVM and LV mid-diastolic volume were measured using semiautomated software and indexed to body surface area. The average LV mid-diastolic wall thickness and concentricity index (LVM/LV mid-diastolic volume) were calculated. According to the Agatston Score, the patients were divided into 3 groups (Agatston=0, 0.1 to 399.9, ≥400). Similarly, patients were also divided into 4 groups on the basis of the Total Plaque Score (TPS) (0, 1 to 4, 5 to 8, and ≥9). In addition, patients were categorized according to CAD (normal coronaries, nonobstructive CAD, and obstructive stenosis [obstruction >50%]). The association between the different categories of CAD and LV measures was assessed. RESULTS Both left ventricular mass index (LVMi) and the LV concentricity index increased with TPS categories from 55.3±12.1, 57.4±11.7, 60.9±13.6, and 63.7±15.3 g/m2 (P

Background: Abnormalities in computed tomography myocardial perfusion has been associated with coronary artery disease and major adverse cardiovascular events (MACE). We sought to investigate if subendocardial attenuation using coronary computed tomography angiography predicts MACE 30 days postelective noncardiac surgery. Methods: Using a 17-segment model, coronary computed tomography angiography images were analyzed for subendocardial and transmural attenuation and the corresponding blood pool. The segment with the lowest subendocardial attenuation and transmural attenuation were normalized to the segment with the highest subendocardial and transmural attenuation, respectively (SUB normalized , and TRANS normalized , respectively). We evaluated the independent and incremental value of myocardial attenuation to predict the composite of cardiovascular death or nonfatal myocardial infarction. Results: Of a total of 995 coronary CTA VISION (Coronary Computed Tomographic Angiography and Vascular Events in Noncardiac Surgery Patients Cohort Evaluation Study) patients, 735 had available images and complete data for these analyses. Among these patients, 60 had MACE. Based on Revised Cardiovascular Risk Index, 257, 302, 138, and 38 patients had scores of 0, 1, 2, and ≥3, respectively. On coronary computed tomography angiography, 75 patients had normal coronary arteries, 297 patients had nonobstructive coronary artery disease, 264 patients had obstructive disease, and 99 patients had extensive obstructive coronary artery disease. SUB normalized was an independent and incremental predictor of events in the model that included Revised Cardiovascular Risk Index and coronary artery disease severity. Compared with patients in the highest tertile of SUB normalized , patients in the second and first tertiles had an increased hazards ratio for events (2.23 [95% CI, 1.091–4.551] and 2.36 [95% CI, 1.16–4.81], respectively). TRANS normalized , as a continuous variable, was also found to be a predictor of MACE ( P =0.027). Conclusions: Our study demonstrates that SUB normalized and TRANS normalized are independent and incremental predictors of MACE 30 days after elective noncardiac surgery. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT01635309.

Objectives This study sought to: 1) determine if the current training volume guidelines are reasonable for attaining competence for interpreting myocardial perfusion imaging (MPI); and if not, 2) identify potential thresholds for training volumes and competence. Background There is a growing desire to adopt competency-based medical education (CBME). As such, the implementation of CBME will require new and novel methods of defining, measuring, and assessing clinical competence. The potential use of CBME in cardiac imaging has not been well studied. Methods Consecutive MPI studies were interpreted independently by trainees, and expert readers reviewed the same studies. Studies were quantified using summed scores and % left ventricular (LV) ischemia and the kappa agreement between trainee and expert were measured every 50 cases. Agreement for all MPI and abnormal MPI cases was calculated. Results A total of 24 trainees interpreted 9,668 MPI studies over 37 months. Agreement between trainees and expert readers increased with MPI case volumes but at different rates. The threshold for competence was set at 2 SDs below expert interobserver agreement. The average trainee surpassed this threshold for both summed stress score and %LV ischemia after 800 studies and after 400 abnormal MPI studies. Trainees learned at different rates and surpassed the competence threshold after different case volumes. Conclusions The use of CBME within nuclear cardiology appears to be feasible. Our results suggest that current guidelines may be insufficient to ensure competence and would support the need to increase the MPI case volumes. The use of CBME principles would suggest that trainees may achieve competence at different rates and our results suggest a shift in focus from volume-based learning toward target agreement thresholds.

The anomalous aortic origin of the right coronary artery (AAORCA) from the left sinus is a congenital anomaly affecting both the origin and course of the right coronary artery. AAORCA is nowadays easily and increasingly recognized by several cardiac imaging modalities. In most cases, patients remain asymptomatic; however, in some, and especially in young athletes, symptoms start to appear following exertion. A literature review was conducted on the surgical management of AAORCA by searching the Pubmed and Google Scholar databases. The inclusion criteria included manuscripts reporting surgical outcomes of AAORCA for ≥1 of the 3 techniques of interest (unroofing, reimplantation, and coronary artery bypass grafting) and manuscripts written in English and that were published between 2010 and 2020. The surgical management of AAORCA can be done through several techniques, most commonly the unroofing of the intramural segment of the AAORCA, the reimplantation of the native right coronary artery onto the right sinus of the aortic root, and coronary artery bypass grafting with either arterial or venous graft conduits with or without ligation of the proximal right coronary artery. Superiority of one surgical technique has not yet been formally proven because of the rare nature of this condition and the lack of any prospective randomized controlled trial or robust prospective observational studies.

Background Cardiovascular magnetic resonance (CMR) is increasingly used in the evaluation of patients who are potential candidates for implantable cardioverter-defibrillator (ICD) therapy to assess left ventricular (LV) ejection fraction (LVEF), myocardial fibrosis, and etiology of cardiomyopathy. It is unclear whether CMR-derived strain measurements are predictive of appropriate shocks and death among patients who receive an ICD. We evaluated the prognostic value of LV strain parameters on feature-tracking (FT) CMR in patients who underwent subsequent ICD implant for primary or secondary prevention of sudden cardiac death. Methods Consecutive patients from 2 Canadian tertiary care hospitals who underwent ICD implant and had a pre-implant CMR scan were included. Using FT-CMR, a single, blinded, reader measured LV global longitudinal (GLS), circumferential (GCS), and radial (GRS) strain. Cox proportional hazards regression was performed to assess the associations between strain measurements and the primary composite endpoint of all-cause death or appropriate ICD shock that was independently ascertained. Results Of 364 patients (mean 61 years, mean LVEF 32%), 64(17.6%) died and 118(32.4%) reached the primary endpoint over a median follow-up of 62 months. Univariate analyses showed significant associations between GLS, GCS, and GRS and appropriate ICD shocks or death (all p 0.30). Conclusions GLS by FT-CMR is an independent predictor of appropriate shocks or mortality in ICD patients, beyond conventional prognosticators including LVEF. Further study is needed to elucidate the role of LV strain analysis to refine risk stratification in routine assessment of ICD treatment benefit.

Introduction Several case reports or small series have suggested a possible link between mRNA COVID vaccines and the subsequent development of myocarditis and pericarditis. This study is a prospective collection and review of all cases with a myocarditis/pericarditis diagnosis over a 2-month period at an academic medical center. Methods Prospective case series from 1st June 2021 until 31st July 2021. Patients were identified by admission and discharge diagnoses which included myocarditis or pericarditis. Inclusion criteria: in receipt of mRNA vaccine within one month prior to presentation; The CMR protocol included cine imaging, native T1 and T2 mapping, late gadolinium enhancement and post contrast T1 mapping. All CMR studies were read in consensus by two experienced readers. Diagnosis was based on clinical presentation, ECG/echo findings and serial troponins and was confirmed in each case by CMR. Incidence was estimated from total doses of mRNA vaccine administered in the Ottawa region for the matching time-period. This data was obtained from the Public Health Agency of Ottawa. Results 32 patients were identified over the period of interest. Eighteen patients were diagnosed with myocarditis; 12 with myopericarditis; and 2 with pericarditis alone. The median age was 33 years (18-65 years). The sex ratio was 2 females to 29 males. In 5 cases, symptoms developed after only a single dose of mRNA vaccine. In 27 patients, symptoms developed after their second dose of. Median time between vaccine dose and symptoms was 1.5 days (1-26 days). Chest pain was the commonest symptom, but many others were reported. Non-syncopal non-sustained ventricular tachycardia was seen in only a single case. Median LV ejection fraction (EF) was 57% (44-66%). Nine patients had an LVEF below the normal threshold of 55%. Incidence of myopericarditis overall was approximately 10 cases for every 10,000 inoculations. Summary and Conclusions This is the largest series in the literature to clearly relate the temporal relationship between mRNA COVID vaccination, symptoms and CMR findings. In most patients, symptom onset began within the first few days after vaccination with corresponding abnormalities in biomarkers and on ECG. Cardiac MRI confirmed acute myocardial and pericardial changes with the presence of edema demonstrated with both tissue mapping and late gadolinium enhancement. Symptoms settled quickly with standard therapy and patients were discharged within a few days. No major adverse cardiac events and no significant arrythmias were noted during inpatient stay. Further follow up will be required to ascertain the longer-term outcomes of this patient group.

We sought to determine if epicardial adipose tissue (EAT) volume is an independent and incremental prognostic marker of 30-day major adverse cardiovascular events (MACE) after elective noncardiac surgery. The Coronary CTA VISION Study enrolled 995 patients ([1][1]), and the primary outcome was a

Background Obesity is an in independent risk factor for cardiovascular disease. Goal To describe the early LV remodelling pattern in patients with overweight and obesity and structurally normal hearts. Methods Consecutive patients (n = 2374), with structurally normal hearts and BMI ≥ 18.5 kg/m2, undergoing prospective mid-diastolic ECG gated CTCA were selected. Left ventricular mass (LVM) and Left ventricular mid-diastolic volume (LVMDV) were measured. The concentricity index (LVM/LVMDV) were calculated. According to the definitions of the World Health Organization (WHO), the patients were divided into weight categories. Results The mean LVM ± Std. deviation in the subgroups according to WHO classification was 101.68 ± 28.99 g (normal weight), 115.79 ± 29.14 g (overweight), 123.8 ± 33.44 g (class I obesity), 125.85 ± 32.89 g (class II obesity) and 132.45 ± 37.85 g (class III obesity). (p The mean LVMDV progressed with increasing WHO weight category from 112.37 ± 36.46 in patients with normal BMI to 140.26 ± 43.78 in patients with class III obesity. (p The concentricity index was 0.935 ± 0.216 g/ml in patients with normal BMI, 0.979 ± 0.253 g/ml, 1.058 ± 0.635 g/ml, 0.996 ± 0.284 g/ml and 0.9768 ± 0.244 g/ml in patients with BMI categories 25–29.99, 30–34.99, 35–39.99 and ≥40 kg/m2, respectively. Conclusions Our study demonstrates a non-linear (inverse U-shape) relationship between increasing BMI class and concentricity index, reaching its maximum at a BMI of 30–34.99 kg/m2. Further increase in BMI results in LV dilation.

OBJECTIVE: Marfan syndrome is an autosomal-dominant genetic disorder caused by mutations in the fibrillin-1 gene. The condition is a connective tissue disease that frequently involves the cardiovascular system. The existence of a primary cardiomyopathy in Marfan syndrome, however, is controversial. The aims of this study were to investigate the prevalence of left ventricular dysfunction with both transthoracic echocardiography and cardiovascular magnetic resonance (CMR) in a cohort of Marfan syndrome patients and to investigate patterns of myocardial strain across the cohort.METHODS: We used an institutional database to identify all patients with a firm diagnosis of Marfan syndrome based on Ghent criteria. Inclusion required left ventricular ejection fraction (LVEF) to have been measured by both CMR and transthoracic echocardiography within 12 months of each other. Normal LVEF was defined as a value of >55% when measured by CMR. Velocity vector imaging was used to measure left ventricular longitudinal strain patterns by application of feature tracking to cine magnetic resonance images. Results were compared with data from 20 age-matched control subjects.RESULTS: Sixty-nine Marfan syndrome patients met the inclusion criteria. The mean age was 35.4 ± 15.0 years, and 56.5% were male. The mean LVEF was 59.0% ± 7.0% by CMR and 59.1% ± 5.8% by echo. One-fifth of Marfan syndrome patients (15/69; 21.7%) had reduced function with LVEF ≤55% by CMR, but only 5 of these were identified by echo. Furthermore, echo identified 5 Marfan syndrome patients as having reduced LVEF in the presence of a normal LVEF by CMR. Some Marfan syndrome patients had abnormal longitudinal strain patterns even with LVEF within the reference range.CONCLUSIONS: These data provide support for a primary cardiomyopathy in some Marfan syndrome patients. Cardiovascular magnetic resonance is more sensitive than echo for identifying cases with mild systolic dysfunction. Strain analysis may be more sensitive than simple LVEF assessment for identifying at-risk individuals.

The goals of primary prevention in coronary atherosclerosis are to avoid sudden cardiac death, myocardial infarction or the need for revascularization procedures. Successful prevention will rely on accurate identification, effective therapy and monitoring of those at risk. Identification and potential monitoring can be achieved using cardiac computed tomography (CT). Cardiac CT can determine coronary artery calcification (CAC), a useful surrogate of coronary atherosclerosis burden. Cardiac CT can also assess coronary CT angiography (CCTA). CCTA can identify arterial lumen narrowing and highlight mural atherosclerosis hitherto hidden from other anatomical approaches. Herein we consider the role of CCTA and CAC-scoring in subclinical atherosclerosis. We explore the use of these modalities in screening and discuss data that has used CCTA for guiding primary prevention. We examine therapeutic trials using CCTA to determine the effects of plaque-modifying therapies. Finally, we address the role of CCTA and CAC to guide therapy as defined in current primary prevention documents. CCTA has emerged as an essential tool in the detection and management of clinical coronary artery disease. To date, its role in subclinical atherosclerosis is less well defined, yet with modern CT scanners and continued pharmacotherapy development, CCTA is likely to achieve a more prominent place in the primary prevention of coronary atherosclerosis.

Coronary artery calcification (CAC) on body CT imaging is considered a coincidental finding in cancer patients. In order to determine the significance of CAC in cancer patients we evaluated the prognostic utility of CAC detected on oncology FDG-PET/CT studies. A retrospective study was performed of consecutive FDG-PET/CT studies from January to March 2011. CAC was identified on the CT portion of FDG/PET-CT studies. Chart review documented statin use, the Framingham risk score (FRS) (includes age, diabetes, hypertension, dyslipidemia and smoking), the primary malignancy and metastases. The primary end point was a composite of death and cardiovascular (CV) events (non-fatal myocardial infarction (MI), PCI or coronary artery bypass surgery (CABG)). 266 patients had a median follow up of 41 months (95% CI 31-56 months). CAC was noted in 140 patients. Based on CAC, potentially 84 patients would have had a change in statin prescribing (p 0.01). CAC was associated with the primary end point on univariable and multivariable analysis (OR 2.6 (95% CI 1.42-4.77) (p 0.01). On univariable Kaplan-Meier survival analysis, CAC was associated with decreased survival only in the absence of metastases (p 0.01). Cox proportional hazard modelling demonstrated CAC was associated with mortality and cardiac events in patients without metastases, whereas FRS was not (For CAC: HR 1.69 (95% CI 1.22-2.35), p = 0.002). CAC is commonly detected with oncology FDG-PET/CT. In cancer patients CAC was associated with an increased risk of clinical events. CAC reduced survival free time in patients without metastases. CAC might therefore be considered more than a coincidentaloma in patients without metastases.

This study sought to assess training volumes and its relationship to learning and identify potential new thresholds for determining expertise.Competency-based medical education (CBME) is being rapidly adopted and therefore training programs will need to adapt and identify new and novel methods of defining, measuring, and assessing clinical skills.Consecutive cardiac computed tomography (CT) studies were interpreted independently by trainees and expert readers, and their interpretations (Agatston score, coronary artery disease severity, and Coronary Artery Disease Reporting and Data System) were collected. Kappa agreements were measured between trainees and experts for every 50 consecutive cases. Agreements between trainees and experts were tracked and compared with the agreement between expert readers.A total of 36 trainees interpreted 14,432 cardiac CT studies. Agreement between trainees and experts increased with CT case volumes, but trainees learned at different rates. Using a threshold for expertise, skill of measuring coronary calcification was achieved within 50 cases, but expertise for coronary CT angiography appeared to require a mean case volume of 750, comprising 400 abnormal cases.Current volume-based training guidelines may be insufficient and higher case volumes may be required. We demonstrate that tracking cardiac CT learners is feasible and that CBME could be incorporated into CT training programs.

Objective We sought to determine the prognostic value of coronary computed tomography angiography (CCTA) in patients with a history of percutaneous coronary intervention (PCI). Background Although the prognostic value of CCTA has been well studied, its incremental value in patients with previous PCI has not been robustly investigated. Methods Consecutive patients with previous PCI were prospectively enrolled and CCTA images were evaluated for coronary artery disease (CAD) severity. Patients were followed for major adverse cardiovascular events (MACE) which was a composite of cardiac death and non-fatal myocardial infarction. All-cause death was assessed as a secondary endpoint. Results A total of 501 patients were analyzed with a mean follow-up time of 59.5 ± 32.0 months and 52 patients (10.4%) experienced MACE. Multivariable Cox regression analysis showed that CAD severity was a predictor of MACE with 0, 1, 2, and 3 vessel disease having annual rates of 1.3%, 2.2%, 2.2%, and 5.3%, respectively. All-cause death was similar in all categories of CAD. Conclusions In patients with previous PCI, CAD severity as measured with CCTA has independent and incremental prognostic value.

Aims: The impact of anatomical versus functional testing in patients with prior coronary artery bypass surgery (CABG) is poorly defined. We therefore sought to determine the rates of downstream investigations and the attendant healthcare costs in CABG patients undergoing CCTA versus SPECT. Methods and results: 2754 consecutive CABG patients were imaged by SPECT (2163) or CCTA (591). 425 patients (15.4%) underwent downstream testing which was more common in those imaged with CCTA versus SPECT (23.18% vs 13.31% respectively, p

Aims This large prospective cohort study sought to confirm the incremental prognostic value of coronary computed tomographic angiography (CCTA) measured over a prolonged follow-up duration. CCTA has diagnostic and prognostic value but data supporting its long-term prognostic value in a large prospectively recruited cohort with suspected coronary artery disease (CAD) has been limited. Methods and results Consecutive patients (without history of myocardial infarction, revascularization, cardiac transplantation, and congenital heart disease) were prospectively enrolled. CCTA was evaluated for CAD severity, total plaque score (TPS), and left ventricular ejection fraction. Patients were followed for major adverse events (MAE) and major adverse cardiac events (MACE). Over a total of 99 months, 8667 consecutive CCTA patients (mean age = 57.1 ± 11.1 years, 52.9% men) were prospectively enrolled and followed for a mean duration of 7.0 ± 2.6 years. At follow-up, there were a total of 723 MAE, 278 MACE, 547 all-cause deaths, 110 cardiac deaths, and 104 non-fatal myocardial infarction. Patients without coronary atherosclerosis at the time of CCTA had a very low annual event rate for both MAE and MACE (0.45%/year and 0.19%/year, respectively). Both MAE and MACE increased with increasing TPS and severity of CAD. In patients with non-obstructive CAD and who were statin-naive, TPS ≥5 had MACE rates >0.75%/year. Patients with high-risk CAD had an annual MAE and MACE rates of 3.52%/year and 2.58%/year, respectively. Adjusted hazard ratio of the severity of CAD based on multivariable analyses indicated that the prognostic values were incremental. Conclusion CCTA has independent and incremental prognostic value that is durable over time. The absence of coronary atherosclerosis portends an excellent prognosis. Patients with increasing non-obstructive plaque burden have worse prognosis and a TPS threshold ≥5 may identify a population that may benefit from statin therapy.

Purpose To compare left ventricular (LV) and right ventricular (RV) 3.0-T cardiac magnetic resonance (MR) imaging T1 values in Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM) and evaluate the diagnostic value of native T1 values beyond age, sex, and conventional imaging features. Materials and Methods For this prospective study, 30 patients with gene-positive AFD (37% male; mean age ± standard deviation, 45.0 years ± 14.1) and 30 patients with HCM (57% male; mean age, 49.3 years ± 13.5) were prospectively recruited between June 2016 and September 2017 to undergo cardiac MR imaging T1 mapping with a modified Look-Locker inversion recovery (MOLLI) acquisition scheme at 3.0 T (repetition time msec/echo time msec, 280/1.12; section thickness, 8 mm). LV and RV T1 values were evaluated. Statistical analysis included independent samples t test, receiver operating characteristic curve analysis, multivariable logistic regression, and likelihood ratio test. Results Septal LV, global LV, and RV native T1 values were significantly lower in AFD compared with those in HCM (1161 msec ± 47 vs 1296 msec ± 55, respectively [P.001]; 1192 msec ± 52 vs 1268 msec ± 55 [P.001]; and 1221 msec ± 54 vs 1271 msec ± 37 [P = .001], respectively). A septal LV native T1 cutoff point of 1220 msec or lower distinguished AFD from HCM with sensitivity of 97%, specificity of 93%, and accuracy of 95%. Septal LV native T1 values differentiated AFD from HCM after adjustment for age, sex, and conventional imaging features (odds ratio, 0.94; 95% confidence interval: 0.91, 0.98; P =.001). In a nested logistic regression model with age, sex, and conventional imaging features, model fit was significantly improved by the addition of septal LV native T1 values (χ

BACKGROUND There is conflicting data on early left ventricle (LV) remodeling in diabetes mellitus (DM) and hypertension (HTN). This study examines the feasibility of cardiac computed tomography angiography (CCTA) to detect early LV geometric changes in patients with DM and HTN. METHODS Consecutive patients (n = 5,992) who underwent prospective electrocardiography (ECG)-triggered (mid-diastolic) CCTA were screened. Patients with known structural heart disease or known LV dysfunction were excluded. Left ventricular mass (LVM), left ventricular mid-diastolic volume (LVMDV), and LV concentricity (LVM/LVMDV) were measured and indexed to body surface area. RESULTS A total of 4,283 patients were analyzed (mean age 57 ± 10.69 years, female 46.7%). DM, HTN, and HTN + DM were present in 4.1%, 35.8% and 10.6% of patients, respectively. Compared to normal patients, HTN and HTN + DM patients had increased LVM indexed to body surface area (LVMi) (56.87 ± 17.24, 59.26 ± 13.62, and 58.56 ± 13.09, respectively; P < 0.05). There was no difference in LVMi between normal subjects and patients with DM (56.39 ± 11.50, P = 0.617). Concentricity indices were higher in patient with HTN (1.0456 ± 0.417; P < 0.001), DM (1.109 ± 0.638; P = 0.004), and HTN + DM (1.083 ± 0.311, P < 0.001) than normal individuals (0.9671 ± 0.361). There was no overlap of the 95% confidence intervals in the composite of concentricity indices and LVMi between the different groups. CONCLUSIONS CCTA measures of LVM and concentricity index may discriminate patients with HTN and DM before overt structural heart disease.

Objective Although heart failure (HF) is the leading cause of premature death in adult congenital heart disease (ACHD), little population-specific data exist. This study reports early experience from a dedicated, sub-specialty adult congenital heart disease-heart failure (ACHD-HF) clinic, aiming to identify risk factors for adverse outcome. Methods Between 2012 and 2015, 126 patients (57% male) attended the ACHD-HF clinic. Baseline and follow-up data were analysed and compared across 4 anatomical/physiological subgroups: cyanotic ACHD, Fontan circulation (1V), biventricular circulation with a subaortic right ventricle (2V-RV) and biventricular circulation with a subaortic left ventricle (2V-LV). Predictors of the composite primary outcome: death, transplant or ventricular assist device (VAD) were identified using multivariable Cox proportional hazard models. Results Mean age at first visit was 38±13years. Patients were grouped as follows: cyanotic ACHD 10%, 1V 24%, 2V-RV 29% and 2V-LV 37%. During a median follow-up of 1.7 (IQR 0.8–2.9) years, 38 patients (30%) reached the primary outcome. Event-free survival was 89%, 78% and 63% at 1, 2 and 3years. Forty (31.7%) patients experienced 69 HF hospitalisations. Between-group differences were noted for systolic function, valvular regurgitation, pacing prevalence and invasive hemodynamics. Multivariable analysis revealed 2V-RV subgroup ( p =0.001), NYHA class ( p =0.002) B-type natriuretic peptide >164pg/ml ( p =0.003) and sodium p =0.036) as independently associated with death, transplant or VAD. Conclusions Our young ACHD-HF patients experienced high adverse event rates during a short period of follow-up. The prognostic markers identified will aid clinicians to stratify short-term risk and thereby guide advanced HF management decisions in ACHD.

Background Anomalous origin of a coronary artery from the pulmonary artery (PA) is a rare condition that usually presents in childhood. The aim of this study is to describe its characteristics in unrepaired adults. Methods We conducted a retrospective study of 18 patients with left and right anomalous coronary artery from the PA (ALCAPA and ARCAPA) diagnosed in adulthood. Patients ≥18 years old at diagnosis and assessed at least once in our institution were included. Results Median age at diagnosis was 29.5 years [IQR 24.0–48.8]. Presentation mode varied, with two patients (11%) diagnosed following aborted sudden cardiac death. Diagnosis was most frequently made by coronary angiography (44%) and cardiac computed tomography (28%). The mean left ventricle ejection fraction (LVEF) at presentation was 56 ±10% and moderate or severe mitral regurgitation was present in 5 patients. Surgical or interventional repair was performed in 14 patients (78%), 9 of whom (50%) underwent reimplantation. Median follow-up was 8.5 years [IQR 0.98–13.8]. One patient with severe pulmonary hypertension died during the follow-up. Most patients (79%) were in NYHA functional class 1 at last follow-up with a mean LVEF of 53±12%. Conclusions Anomalous origin of a coronary artery from the PA can present in adulthood in various ways. Multimodality imaging allows the diagnosis and assessment of functional impact. Surgical correction of ALCAPA with establishment of a dual coronary pathway was the most frequent management strategy in our cohort. The majority of patients experienced favorable long-term outcomes.

This early feasibility study was designed to obtain in vivo data to confirm assumptions on device loading conditions of the Medtronic Harmony transcatheter pulmonary valve (TPV). Secondary objectives included procedural feasibility, safety, and valve performance. The Harmony TPV was developed for nonsurgical pulmonary valve replacement in non-right ventricle-pulmonary artery conduit patients. The Native Outflow Tract TPV Research Clinical Study was the first study approved under the Food and Drug Administration Early Feasibility Study guidance. Enrollment required that patient anatomy be precisely matched to the single-size Harmony TPV implant, necessitating a rigorous selection process. The study was nonrandomized, prospective, and performed at 3 sites. All patients met standard indications for surgical pulmonary valve replacement. The goal of the screening committee was to match the candidate anatomy to predetermined engineering criteria thought to be predictive of secure Harmony TPV implantation for the single-size device under study. A majority of the screening committee was required to recommend a patient as eligible for implant. A total of 270 patients underwent prescreening cardiac magnetic resonance imaging, 66 were enrolled and received a computed tomography scan (24%), 21 met criteria for implant and were catheterized (8%), and 20 underwent implant. Nineteen of 20 met criteria for implant success. In conclusion, the Medtronic Harmony TPV represents an emerging therapeutic option for patients with complex postoperative right ventricular outflow tract failure. The initial clinical evaluation of this technology was unique, and the highly variable anatomy of this population required careful screening to ensure acceptable device fit.

Objectives This study sought to obtain in vivo data to confirm assumptions on device loading conditions and assess procedural feasibility, safety, and valve performance. Background The Harmony transcatheter pulmonary valve (Medtronic, Minneapolis, Minnesota) was designed for patients with severe pulmonary regurgitation who require pulmonary valve replacement. Methods Three sites participated in this first Food and Drug Administration–approved early feasibility study using an innovative device design to accommodate the complex anatomy of the right ventricular outflow tract. Potentially eligible patients underwent review by a screening committee to determine implant eligibility. Six-month outcomes are reported. Results Between May 2013 and May 2015, 66 subjects were enrolled, and 21 were approved for implant and underwent catheterization; 20 were implanted. Catheterized patients had a median age of 25 years, were predominantly diagnosed with tetralogy of Fallot (95%), had severe pulmonary regurgitation (95%), and had trivial or mild stenosis. The device was delivered in the desired location in 19 of 20 (95%) patients. Proximal migration occurred in 1 patient during delivery system removal. Two devices were surgically explanted. Premature ventricular contractions related to the procedure were reported in 3 patients; 2 were resolved without treatment. One patient had ventricular arrhythmias that required treatment and later were resolved. At 1 month, echocardiography revealed none or trivial pulmonary regurgitation in all and a mean right ventricular outflow tract gradient of 16 ± 8 mm Hg (range 6 to 31 mm Hg). Conclusions In this feasibility study of the Harmony transcatheter pulmonary valve device, there was high procedural success and safety, and favorable acute device performance.

ObjectivesIndividuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population.MethodsWe performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases. We defined paediatric-onset coronary artery anomalies as congenital coronary anomalies and inflammatory arteriopathies of childhood that cause coronary aneurysms. Major cardiovascular events were defined as pulmonary oedema, sustained arrhythmia requiring treatment, stroke, myocardial infarction, cardiac arrest, or death.ResultsA total of 25 surveys were mailed, and 20 were returned (80% response rate). We included 46 articles from the literature, which described cardiovascular outcomes in 82 women (138 pregnancies). These data were amalgamated for a total of 102 women and 194 pregnancies; 59% of women were known to have paediatric-onset coronary artery anomalies before pregnancy. In 23%, the anomaly was unmasked during or shortly after pregnancy. The remainder, 18%, was diagnosed later in life. Major cardiovascular events occurred in 14 women (14%) and included heart failure (n=5, 5%), myocardial infarction (n=7, 7%), maternal death (n=2, 2%), cardiac arrest secondary to ventricular fibrillation (n=1, 1%), and stroke (n=1, 1%). The majority of maternal events (13/14, 93%) occurred in women with no previous diagnosis of coronary disease.ConclusionsWomen with paediatric-onset coronary artery anomalies have a 14% risk of adverse cardiovascular events in pregnancy, indicating the need for careful assessment and close follow-up. Prospective, multicentre studies are required to better define risk and predictors of complications during pregnancy.

We agree with the comments of Dr. Moss and colleagues ([1][1]) regarding the need for greater understanding of the factors that govern high-quality cardiovascular (CV) imaging training. As an initial step, frequent objective assessments of trainees are needed. This allows for monitoring and

Muscular dystrophies (MD) represent a heterogeneous group of rare genetic diseases that often lead to significant weakness due to progressive muscle degeneration. In many forms of MD, cardiac manifestations including heart failure, atrial and ventricular arrhythmias and conduction abnormalities can occur and may be a predominant feature of the disease. Cardiac magnetic resonance (CMR) can assess cardiac anatomy, global and regional ventricular function, volumes and mass as well as presence of myocardial inflammation, infiltration or fibrosis. The role for cardiac MRI has been well-established in a wide range of muscular dystrophies related cardiomyopathies. CMR is a more sensitive technique than echocardiography for early diagnosis of cardiac involvement. It has also great potential to improve the prediction of long-term outcome, particularly the development of heart failure and arrhythmic events; however it still has to be validated by longitudinal studies including large populations. This review will outline the utility of CMR in patients with muscular dystrophies for assessment of myocardial involvement, risk stratification, and in guiding therapeutic management.

Modern cardiology is both a complex business and a team sport. Treatment options have expanded and now extend to patients whose age and degree of sickness might have excluded them in the past. Correct diagnosis and treatment can be challenging, and imaging is central to understanding cardiac pathology and to guiding optimal treatment choices. It is almost impossible for a single physician to have full mastery of all the imaging tools available for diagnosis. Instead, it is incumbent upon hospitals to establish imaging teams which have both overlapping and unique expertise. These may be drawn from the ranks of cardiology, radiology, or nuclear medicine—skill set is of more importance than background specialty. Heart imaging teams, working together in unison, represent an ideal model to facilitate the right test at the right time for the right patient.

Cardiovascular CT is a powerful diagnostic tool for select patients with congenital heart disease (CHD). The improved image quality and decreased radiation dose of newer generation CT scanners have expanded its use in this patient population. This chapter will give a brief overview of the indications, common residual lesions, and proposed scan modifications for the CHD lesions most commonly referred for cardiovascular CT.

Key Points Question Which adverse cardiovascular and noncardiovascular events are most relevant to patients? Findings In this qualitative study of patients with advanced cardiovascular diseases and their caregivers and clinicians, a consensus-based definition of patient-defined adverse cardiovascular and noncardiovascular events (PACE) was reached using a modified Delphi process; the definition included severe stroke necessitating hospitalization for 14 days or more or inpatient rehabilitation, ventilator dependence, new onset or worsening heart failure, nursing home admission, and new onset dialysis. Meaning Given the paucity of patient-centered outcomes in cardiovascular research, the concept of PACE may be applied in future epidemiological and intervention studies to ensure that management of cardiovascular disease is founded on outcomes that are important and relevant to patients, caregivers, and clinicians., This qualitative study attempts to derive patient-defined adverse cardiovascular and noncardiovascular events through a modified Delphi consensus-based process involving panel discussions with patients and their caregivers and clinicians., Importance There is little evidence to support patient-centered outcomes in patients with cardiovascular disease. Objective To derive patient-defined adverse cardiovascular and noncardiovascular events (PACE) through a consensus-based process. Design, Setting, and Participants This pan-Canadian, consensus-based, qualitative study used an iterative Delphi method to achieve consensus within a 35-member panel consisting of patients with cardiovascular diseases and their caregivers and clinicians. The process included 4 rounds of online questionnaires, followed by an in-person final consensus meeting. Data analysis was performed in September 2019. Main Outcomes and Measures Defining PACE as a 5-item composite outcome. Results Thirty-five potential panelists consented to participate, including 11 clinicians (8 men [73%]) and 24 patients and caregivers (13 men [54%]). Twenty-nine (83%), 28 (80%), 26 (74%), and 23 (66%) of the panelists participated in each of respective the online rounds. A shortlist of 11 patient-defined items was further refined at the in-person meeting, which 20 of the panelists attended. The PACE definition that was decided through the consensus process was a composite of severe stroke necessitating hospitalization for 14 days or longer or inpatient rehabilitation, ventilator dependence, new onset or worsening heart failure, nursing home admission, or new onset dialysis. Conclusions and Relevance This study defined PACE as a versatile, patient-centered outcome through a consensus process with input from patients, caregivers, and clinicians. Given the paucity of patient-centered outcomes in cardiovascular research, PACE may be considered as a potential outcome after methodological evaluation of its reliability.

Cardiac allograft vasculopathy (CAV) is a leading cause of mortality after heart transplantation. Noninvasive imaging techniques used in CAV evaluation have important limitations. In a cross-sectional study, we investigated perfusion cardiac magnetic resonance (CMR) imaging to determine an optimal myocardial perfusion reserve index (MPR) cutoff for detecting CAV using receiver operating characteristic curve analysis. We evaluated CMR performance using sensitivity, specificity and likelihood ratio analysis. We included 29 patients (mean 5 ± 4 years after transplant) scheduled for coronary angiography with intravascular ultrasound (IVUS) who completed CMR. CAV was defined as maximal intimal thickness (MIT) >0.5 mm by IVUS of the left anterior descending artery. CAV was evident in 19 patients (70%) on IVUS (mean MIT 0.82 ± 0.42 mm). MPR was significantly lower in patients with MIT ≥0.50 mm (1.35 ± 0.23 vs. 1.71 ± 0.45, p = 0.013). There was moderate inverse correlation between MPR and MIT (r = -0.36, p = 0.075). The optimal MPR cutoff ≤1.68 for predicting CAV showed sensitivity of 100%, specificity of 63%, a negative predictive value of 100%, a positive predictive value of 86% and a positive likelihood ratio of 2.7. An MPR ≤1.68 has high negative predictive value, suggesting its potential as a test to rule out CAV.

The prevalence of congenital coronary anomalies is between 0.3% and 2%, depending on the definition used and the population studied [1,2,3]. Not all congenital coronary anomalies are correlated with negative outcomes. Only anomalous coronaries with an inter-arterial or intramural course, coronary fistulae, and anomalous left coronary artery from the pulmonary artery (ALCAPA) have been associated with an increased rate of cardiac events [4] . Classical definitions of “high-risk features” do not always correlate with necropsy studies [5]. Despite the uncertainty, stakes are high, as coronary anomalies are the secondmost prevalent cause of sudden cardiac death in young athletes [6]. Surgical series report good outcomes with surgical repair, but observational cohorts also report excellent outcomes [7,8]. Guidelines regarding themanagement of coronary anomalies provide indications for surgical repair, but they are different from the management paradigm practiced by most pediatric cardiologists [9,10] .

This is an expert consensus document created to provide information about the current use of cardiovascular computed tomography (CT) in patients of all ages with proven or suspected congenital heart disease (CHD). The discussion and recommendations are based on available literature and the judgment of a diverse group of subspecialists with extensive experience in the use of CT imaging in CHD. The field of CHD CT imaging is evolving rapidly with the availability of new scanner technology. In addition, the prevalence of palliated CHD has increased with marked improvements in patient survival. We believe it is important to review the clinical indications, strengths, limitations, and risks of cardiovascular CT in this patient population. This is the first of two complementary documents. It will concentrate on the disease entities and circumstances in which CT may be used. The second document will focus on recommendations for the technical performance of cardiovascular CT in patients with CHD. Successful cardiovascular CT imaging of CHD requires an in depth understanding of the core teaching elements of both cardiology and radiology. The ability to perform and interpret high quality congenital cardiovascular CT in a clinical context requires focused time and effort regardless of the previous background of the cardiac imager. This is reflected by a writing committee that consists of pediatric and adult radiologists and cardiologists, all whom have extensive experience in performing CT in this patient population. Cardiovascular CT is complementary to other imaging modalities and its optimal use will be in centers where all diagnostic modalities are available. The choice of modality for an individual patient should be determined by age, diagnosis, clinical condition, clinical question and patient preference.(1-4) Use of CT in CHD should be reserved for situations in which it is expected to provide unique diagnostic information for the individual patient or clinical indication, and/or less risk than other modalities. This multi-disciplinary document is intended to guide the optimal selection of CHD patients for cardiovascular CT. The goals of this document apply to both pediatric and adult CHD patients and are to.

There are now more adults alive with congenital heart disease than children (Marelli et al. 2007), and many will have ongoing problems despite surgical repair. Surgery is never a cure—merely a fix—and in some cases directly introduces new problems that were not present previously (Celermajer and Greaves 2002). ACHD patients therefore generally require lifelong follow-up in specialized institutions where the technical and clinical expertise exists to recognize and deal with issues as they arise.

Anomalies of the coronary arteries are paradoxically amongst the simplest and most difficult of subjects to get to grips with in congenital heart disease. This chapter reviews some of the commoner congenital anomalies encountered and discusses technical tips and tricks for optimal CMR imaging.

Background In many cardiac units, aortic valve-sparing operations have become the preferred surgical procedure to treat aortic root aneurysm in patients with Marfan syndrome, based on relatively short-term outcomes. Objectives This study examined the long-term outcomes of aortic valve-sparing operations in patients with Marfan syndrome. Methods All patients with Marfan syndrome operated on for aortic root aneurysm from 1988 through 2012 were followed prospectively for a median of 10 years. Follow-up was 100% complete. Time-to-event analyses were calculated using the Kaplan-Meier method with log-rank test for comparisons. Results A total of 146 patients with Marfan syndrome had aortic valve-sparing operations. Reimplantation of the aortic valve was performed in 121 and remodeling of the aortic root was performed in 25 patients. Mean age was 35.7 ± 11.4 years and two-thirds were men. Nine patients had acute, 2 had chronic type A, and 3 had chronic type B aortic dissections before surgery. There were 1 operative and 6 late deaths, 5 caused by complications of dissections. Mortality rate at 15 years was 6.8 ± 2.9%, higher than the general population matched for age and sex. Five patients required reoperation on the aortic valve: 2 for endocarditis and 3 for aortic insufficiency. Three patients developed severe, 4 moderate, and 3 mild-to-moderate aortic insufficiency. Rate of aortic insufficiency at 15 years was 7.9 ± 3.3%, lower after reimplantation than remodeling. Nine patients developed new distal aortic dissections during follow-up. Rate of dissection at 15 years was 16.5 ± 3.4%. Conclusions Aortic valve-sparing operations in patients with Marfan syndrome were associated with low rates of valve-related complications in long-term follow-up. Residual and new aortic dissections were the leading cause of death.

Purpose To evaluate the value of cardiac magnetic resonance imaging (MRI)–based measurements of inferior vena cava (IVC) cross-sectional area in the diagnosis of pericardial constriction. Methods Patients who had undergone cardiac MRI for evaluation of clinically suspected pericardial constriction were identified retrospectively. The diagnosis of pericardial constriction was established by clinical history, echocardiography, cardiac catheterization, intraoperative findings, and/or histopathology. Cross-sectional areas of the suprahepatic IVC and descending aorta were measured on a single axial steady-state free-precession (SSFP) image at the level of the esophageal hiatus in end-systole. Logistic regression and receiver-operating curve (ROC) analyses were performed. Results Thirty-six patients were included; 50% (n = 18) had pericardial constriction. Mean age was 53.9 ± 15.3 years, and 72% (n = 26) were male. IVC area, ratio of IVC to aortic area, pericardial thickness, and presence of respirophasic septal shift were all significantly different between patients with constriction and those without ( P < .001 for all). IVC to aortic area ratio had the highest odds ratio for the prediction of constriction (1070, 95% confidence interval [8.0-143051], P = .005). ROC analysis illustrated that IVC to aortic area ratio discriminated between those with and without constriction with an area under the curve of 0.96 (95% confidence interval [0.91-1.00]). Conclusions In patients referred for cardiac MRI assessment of suspected pericardial constriction, measurement of suprahepatic IVC cross-sectional area may be useful in confirming the diagnosis of constriction when used in combination with other imaging findings, including pericardial thickness and respirophasic septal shift.