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Most cardiac diseases in the newborn are caused by structural abnormalities developed in utero. With few exceptions, palliative and definitive treatments require cardiac surgery. The diagnosis and management decisions regarding uncomplicated lesions, such as atrial septal defect, ventricular septal defect, patent ductus arteriosus, and tetralogy of Fallot, can be accomplished by echocardiography alone. Abnormalities beyond the sonographic window, complex 3-dimensional lesions, and detailed functional information require additional imaging. In the past, this was fulfilled by catheter angiography, but today much of the information can be obtained from noninvasive computed tomography angiography and magnetic resonance imaging. This article discusses the design and application of these imaging techniques to the newborn, with emphasis on safety, efficacy, and image quality. Understanding the capabilities and limitations of these techniques is crucial for making rational choices among imaging options based on sound risk and benefit considerations. Important examples of congenital heart lesions have been illustrated with 3-dimensional reconstruction from computed tomography and magnetic resonance images.

With improvements in surgical and medical management, patients with congenital heart disease (CHD) are often living well into adulthood. MRI provides critical data for diagnosis and monitoring of these patients, yielding information on cardiac anatomy, blood flow, and cardiac function. Though historically these exams have been complex and lengthy, four-dimensional (4D) flow is emerging as a single fast technique for comprehensive assessment of CHD. The 4D flow consists of a volumetric time-resolved acquisition that is gated to the cardiac cycle, providing a time-varying vector field of blood flow as well as registered anatomic images. In this article, we provide an overview of MRI evaluation of congenital heart disease by means of example of three relatively common representative conditions: tetralogy of Fallot, aortic coarctation, and anomalous pulmonary venous drainage. Then 4D flow data acquisition, data correction, and postprocessing techniques are reviewed. We conclude with several examples that highlight the comprehensive nature of the evaluation of congenital heart disease with 4D flow.

PURPOSE: The purpose of this study was to determine the prognostic significance of cardiac magnetic resonance imaging (MRI) findings in patients with apical hypertrophic cardiomyopathy (HCM). MATERIALS AND METHODS: Cardiac MRI studies of 93 consecutive patients with apical HCM were retrospectively evaluated. Quantification of late gadolinium enhancement (LGE) was determined and expressed as a percentage of total left ventricular (LV) myocardial mass (%LGE). Morphologic features including presence of apical aneurysm, right ventricular hypertrophy, and LV thrombus were also assessed. Clinical data were collected during follow-up to assess for occurrence of major adverse events, defined as: heart failure, stroke, appropriate automatic implantable cardioverter defibrillator discharge, sustained ventricular tachycardia, aborted sudden cardiac death, and/or all-cause death. RESULTS: The mean age of the patients was 54.9±13.8 years, and 72.0% (n=67) were male. LGE, right ventricular hypertrophy, apical aneurysm, and LV thrombus were identified in 69.4%, 25.8%, 18.3%, and 4.3%, respectively. Mean %LGE was 10.8%±11.1%. Over 2.4±1.7 years of follow-up, 14 subjects (15.1%) experienced a major adverse event (event rate, 6.3%/y): heart failure (6.5%), stroke (6.5%), appropriate automatic implantable cardioverter defibrillator discharge (2.2%), sustained ventricular tachycardia (2.2%), aborted sudden cardiac death (1.1%), and all-cause death (0.0%). Presence of apical aneurysm and extent of LGE were significant predictors of major adverse events [odds ratio (OR) 4.6, P=0.015; and OR 1.4/5% LGE, P=0.030, respectively]. Patients with both apical aneurysm and >5% LGE were at highest risk for major adverse events (OR 6.7, P=0.004) and had shortest event-free survival (P=0.001). CONCLUSIONS: Within our population of apical HCM patients, the extent of LGE and the presence of an apical aneurysm identified by cardiac MRI were both significant predictors of major adverse clinical events.

Background Calculation of cardiovascular magnetic resonance (CMR) extracellular volume (ECV) requires input of hematocrit, which may not be readily available. The purpose of this study was to evaluate the diagnostic accuracy of ECV calculated using various noninvasive measures of hematocrit compared to ECV calculated with input of laboratory hematocrit as the reference standard. Methods One hundred twenty three subjects (47.7 ± 14.1 years; 42% male) were prospectively recruited for CMR T1 mapping between August 2016 and April 2017. Laboratory hematocrit was assessed by venipuncture. Noninvasive hematocrit was assessed with a point-of-care (POC) device (Pronto-7® Pulse CO-Oximeter®, Masimo Personal Health, Irvine, California, USA) and by synthetic derivation based on the relationship with blood pool T1 values. Left ventricular ECV was calculated with input of laboratory hematocrit (Lab-ECV), POC hematocrit (POC-ECV), and synthetic hematocrit (synthetic-ECV), respectively. Statistical analysis included Wilcoxon signed-rank test, Bland-Altman analysis, receiver-operating curve analysis and intra-class correlation (ICC). Results There was no significant difference between Lab-ECV and POC-ECV (27.1 ± 4.7% vs. 27.3 ± 4.8%, p = 0.106), with minimal bias and modest precision (bias − 0.18%, 95%CI [− 2.85, 2.49]). There was no significant difference between Lab-ECV and synthetic-ECV (26.7 ± 4.4% vs. 26.5 ± 4.3%, p = 0.084) in subjects imaged at 1.5 T, although bias was slightly higher and limits of agreement were wider (bias 0.23%, 95%CI [− 2.82, 3.27]). For discrimination of abnormal Lab-ECV ≥30%, POC-ECV had good diagnostic performance (sensitivity 85%, specificity 96%, accuracy 94%, and AUC 0.902) and synthetic-ECV had moderate diagnostic performance (sensitivity 71%, specificity 98%, accuracy 93%, and AUC 0.849). POC-ECV had excellent test-retest (ICC 0.994, 95%CI[0.987, 0.997]) and inter-observer agreement (ICC 0.974, 95%CI[0.929, 0.991]). Conclusions Myocardial ECV can be accurately and reproducibly calculated with input of hematocrit measured using a noninvasive POC device, potentially overcoming an important barrier to implementation of ECV. Further evaluation of synthetic ECV is required prior to clinical implementation.

Aims Our aims were to explore cardiac magnetic resonance quantification of myocardial extracellular volume (ECV) in adults with repaired tetralogy of Fallot (rTOF) when compared with healthy controls and to investigate the association between ECV and major adverse cardiovascular outcomes. Methods and results We prospectively recruited adults with rTOF (n = 44, 59% male, 32.9 ± 13.6 years) and evaluated right ventricular (RV) and left ventricular (LV) ECV by pre/post-gadolinium T1 measurements (modified Look-Locker inversion recovery technique) on a 1.5-T Siemens scanner compared with the healthy controls (n = 10, 50% male, 31.5 ± 4.4 years). The primary end point was a composite of death, out-of-hospital cardiac arrest, heart failure (HF) requiring admission for escalation of therapy, or haemodynamically significant ventricular tachycardia (VT) (lasting >30 s and/or resulting in invasive therapy). The association between ECV and adverse events was assessed using Cox proportional hazard models [median follow-up 236 days, interquartile range (IQR) 38-342]. RVECV was higher in patients compared with the controls (31.5 ± 5.4% vs. 26.3 ± 2.1%, P = 0.027). The following major adverse events occurred (n = 9, 21%): death (n = 1), out-of-hospital cardiac arrest (n = 1), HF (n = 1), and VT (n = 6). RVECV was higher among those with an adverse event compared to those without (35.0 ± 5.5% vs. 29.6 ± 4.5%, P = 0.014) and was associated with increased risk for adverse events [hazard ratio 1.13, 95% confidence interval (1.01-1.28); P = 0.037]. LVECV was not associated with adverse events (P = 0.667). Conclusion Increased RVECV is associated with adverse cardiovascular events in adults with rTOF. These results may lead to further studies exploring the potential role for RVECV in risk stratification and targeted therapeutic interventions in this population.

The purpose of this study was to compare combined PET/MRI with PET/CT and cardiac MRI in the evaluation of cardiac sarcoidosis and myocarditis.Ten patients (4 men and 6 women; 56.1 ± 9.6 years old) were prospectively enrolled for evaluation of suspected cardiac sarcoidosis or myocarditis. Written informed consent was obtained. Following administration of 9.9 ± 0.9 mCi F-FDG, patients underwent standard cardiac PET/CT followed by combined PET/MRI using a simultaneous 3-T scanner. Cardiac MRI sequences included ECG-triggered cine SSFP, T2-weighted, and late gadolinium-enhanced imaging. Myocardial involvement was assessed with separate analysis of combined PET/MRI, PET/CT, and cardiac MRI data using dedicated postprocessing software. Estimates of radiation dose were derived from the applied doses of F-FDG and CT protocol parameters.Imaging was acquired with a delay from F-FDG injection of 90.2 ± 27.4 minutes for PET/CT and 207.7 ± 40.3 minutes for PET/MRI. Total scan time for PET/MRI was significantly longer than for PET/CT (81.4 ± 14.8 vs 12.0 minutes, P0.001). Total effective radiation dose was significantly lower for PET/MRI compared with PET/CT (6.9 ± 0.6 vs 8.2 ± 1.1 mSv, P = 0.007). There was no significant difference in the number of positive cases identified between combined PET/MRI (n = 10 [100%]), PET/CT (n = 6 [60%]), and cardiac MRI (n = 8 [80%]), P = 0.091.Simultaneous cardiac PET/MRI is feasible in the evaluation of cardiac sarcoidosis and myocarditis achieving diagnostic image quality.

Purpose To determine the cumulative effective dose (CED) of radiation from medical imaging and intervention in patients with hereditary hemorrhagic telangiectasia (HHT) who have pulmonary arteriovenous malformations and to identify clinical factors associated with exposure to high levels of radiation. Methods All patients with at least 1 pulmonary arteriovenous malformation were identified from the dedicated patient database of a tertiary HHT referral centre. Computerized imaging and electronic patient records were systematically examined to identify all imaging studies performed from 1989-2010. The effective dose was determined for each study, and CED was calculated retrospectively. Results Among 246 patients (mean age, 53 years; 62.2% women) with a total of 2065 patient-years, 3309 procedures that involved ionizing radiation were performed. CED ranged from 0.2-307.6 mSv, with a mean of 51.7 mSv. CED exceeded 100 mSv in 26 patients (11%). Interventional procedures and computed tomography (CT) were the greatest contributors, which accounted for 51% and 46% of the total CED, respectively. Factors associated with high cumulative exposure were epistaxis (odds ratio 2.7 [95% confidence interval, 1.1-6.3]; P = .02), HHT-related gastrointestinal bleeding (odds ratio 2.0 [95% confidence interval, 1.0-3.8]; P = .04) and number of patient-years ( P < .0001). Conclusions Patients with HHT are exposed to a significant cumulative radiation dose from diagnostic and therapeutic interventions. Identifiable subsets of patients are at increased risk. A proportion of patients receive doses at levels that are associated with harm. Imaging indications and doses should be optimized to reduce radiation exposure in this population.

The diagnosis of pericardial constriction remains challenging.We sought to evaluate the predictive value of cardiovascular CT-based measurements of inferior vena cava (IVC) parameters in the diagnosis of pericardial constriction.Forty-two consecutive patients referred for assessment of pericardial constriction by 64-slice CT were evaluated. The diagnosis of pericardial constriction was confirmed by clinical history, echocardiography, cardiac catheterization, intraoperative findings, histopathology, or a combination. Diameter and cross-sectional area of the suprahepatic IVC and cross-sectional area of the aorta were measured on a single-axial CT image at the level of the esophageal hiatus. Maximum pericardial thickness was measured. Logistic regression and receiver operating curve analyses were performed.Twenty-two patients had pericardial constriction. Mean age of the 42 patients was 57.1 ± 16.4 years, 57.1% were men. IVC diameter, IVC area, the ratio of IVC to aortic area, and pericardial thickness were all significantly greater in patients with constriction than in patients without (P.05 for all). IVC-to-aortic area ratio had the highest odds ratio (51; 95% CI, 2.8-922) for the prediction of constriction and remained a significant predictor in multivariable analysis. In nested models, IVC-to-aortic area ratio had incremental value over pericardial thickness for the diagnosis of constriction. IVC-to-aortic area ratio discriminated between patients with and without constriction with an area under the curve of 0.88 on receiver operating curve analysis, with a value ≥ 1.6 having a sensitivity and specificity of 95% and 76%, respectively. Interobserver agreement for IVC-to-aortic area ratio was excellent (intraclass correlation coefficient, 0.98).Assessment of IVC-to-aortic area ratio on CT aids with the diagnosis of pericardial constriction and has independent and incremental value over pericardial thickness alone.

Background Test-retest reproducibility is of utmost importance in follow-up of right ventricular (RV) volumes and function; optimal slice orientation though is not yet known. We compared test-retest reproducibility and intra-/inter-observer variability of right ventricular (RV) volumes and function assessed with short-axis and transverse cardiovascular magnetic resonance (CMR). Methods Eighteen volunteers underwent cine CMR for RV assessment obtaining ventricular coverage in short-axis and transverse slice orientation. Additional 2D phase contrast flow imaging of the main pulmonary artery (MPA) was performed. After complete repositioning repeat acquisitions were performed. Data sets were contoured by two blinded observers. Statistical analysis included Student’s t-test, Bland-Altman plots, intra-class correlation coefficient (ICC) and 2-way ANOVA, SEM and minimal detectable difference calculations. Results Heart rates (65.0 ± 7.4 vs. 67.6 ± 9.9 bpm; P = 0.1) and MPA flow (89.8 ± 16.6 vs. 87.2 ± 14.9 mL; P = 0.1) did not differ between imaging sessions. EDV and ESV demonstrated an inter-study bias of 0.4 %[−9.5 %,10.3 %] and 2.1 %[−12.3 %,16.4 %] for short-axis and 1.1 %[−7.3 %,9.4 %] and 0.8 %[−16.0 %,17.6 %] for transverse orientation, respectively. There was no significant interaction between imaging orientation and interstudy reproducibility (p = 0.395–0.824), intra-observer variability (p = 0.726–0.862) or inter-observer variability (p = 0.447–0.706) by 2-way ANOVA. Inter-observer agreement by ICC was greater for short axis versus transverse orientation for all parameters (0.769–0.986 vs. 0.625–0.983, respectively). Minimal detectable differences for short axis and transverse orientations were 10.1 mL/11.5 mL for EDV, 8.3 mL/8.4 mL for ESV and 4.1 % vs. 4.7 % for EF, respectively. Conclusion Short-axis and transverse orientation both provide reliable and reproducible measures for follow-up of RV volumes and global function. Therefore, additional transverse SSFP cine CMR may not necessarily be required if performed for the sole purpose of quantitative volumetric RV assessment.

Fifth arch anomalies are rare and complex and frequently misdiagnosed or mistaken for other entities. We report a double arch vascular ring that is thought to consist of right fourth arch and left fifth arch components, a previously undescribed persistent fifth arch variant. The currently recognized spectrum and classification of fifth arch vascular anomalies are expanded along with illustrative images to justify the proposed changes. Reviewing and expanding the classification of fifth arch anomalies to include a double arch ring variant will promote recognition, correct diagnosis and appropriate management of these anomalies.

Three-dimensional datasets acquired using computed tomography and magnetic resonance imaging are ideally suited for characterization of the aortic root. These modalities offer different advantages and limitations, which must be weighed according to the clinical context. This article provides an overview of current aortic root imaging, highlighting normal anatomy, pathologic conditions, imaging techniques, measurement thresholds, relevant surgical procedures, postoperative complications and potential imaging pitfalls. Patients with a range of clinical conditions are predisposed to aortic root disease, including Marfan syndrome, bicuspid aortic valve, vascular Ehlers-Danlos syndrome, and Loeys-Dietz syndrome. Various surgical techniques may be used to repair the aortic root, including placement of a composite valve graft, such as the Bentall and Cabrol procedures; placement of an aortic root graft with preservation of the native valve, such as the Yacoub and David techniques; and implantation of a biologic graft, such as a homograft, autograft, or xenograft. Potential imaging pitfalls in the postoperative period include mimickers of pathologic processes such as felt pledgets, graft folds, and nonabsorbable hemostatic agents. Postoperative complications that may be encountered include pseudoaneurysms, infection, and dehiscence. Radiologists should be familiar with normal aortic root anatomy, surgical procedures, and postoperative complications, to accurately interpret pre- and postoperative imaging performed for evaluation of the aortic root. Online supplemental material is available for this article.

To compare the precision and interobserver agreement of ventricular volume, function, and mass quantification by 3D time-resolved (4D) flow MRI relative to cine steady-state free precession (SSFP).With Institutional Research Board approval, informed consent, and HIPAA compliance, 22 consecutive patients with congenital heart disease (CHD) (10 males, 6.4 ± 4.8 years) referred for 3T ferumoxytol-enhanced cardiac MRI were prospectively recruited. Complete ventricular coverage with standard 2D short-axis cine SSFP and whole chest coverage with axial 4D flow were obtained. Two blinded radiologists independently segmented images for left ventricular (LV) and right ventricular (RV) myocardium at end systole (ES) and end diastole (ED). Statistical analysis included linear regression, analysis of variance (ANOVA), Bland-Altman (BA) analysis, and intraclass correlation (ICC).Significant positive correlations were found between 4D flow and SSFP for ventricular volumes (r = 0.808-0.972, P0.001), ejection fraction (EF) (r = 0.900-928, P0.001), and mass (r = 0.884-0.934, P0.001). BA relative limits of agreement for both ventricles were between -52% to 34% for volumes, -29% to 27% for EF, and -41% to 48% for mass, with wider limits of agreement for the RV compared to the LV. There was no significant difference between techniques with respect to mean square difference of ED-ES mass for either LV (F = 2.05, P = 0.159) or RV (F = 0.625, P = 0.434). Interobserver agreement was moderate to good with both 4D flow (ICC 0.523-0.993) and SSFP (ICC 0.619-0.982), with overlapping confidence intervals.Quantification of ventricular volume, function, and mass can be accomplished with 4D flow MRI with precision and interobserver agreement comparable to that of cine SSFP. J. Magn. Reson. Imaging 2016;44:383-392.

Purpose The purpose of this pilot study was to evaluate the safety and efficacy of preoperative computed tomography (CT)-guided percutaneous microcoil lung nodule localization without pleural marking compared with the established technique with pleural marking. Materials and methods Sixty-three consecutive patients (66.7% female, mean age 61.6±11.4 y) with 64 lung nodules resected between October 2008 and January 2014 were retrospectively evaluated. Of the nodules, 29.7% (n=19) had microcoil deployment with pleural marking (control group) and 70.3% (n=45) had microcoil deployment without pleural marking (pilot group). Clinical, pathologic, and imaging characteristics, radiation dose, CT procedure and operating room time, and complete resection and complication rates were compared between the pilot and control groups. Results There was no significant difference in nodule size (P=0.552) or distance from the pleural surface (P=0.222) between the pilot and control groups. However, mean procedure duration (53.6±18.3 vs. 72.8±25.3 min, P=0.001) and total effective radiation dose (5.1±2.6 vs. 7.1±4.9 mSv, P=0.039) were significantly lower in the pilot group compared with the control group. CT procedure-related complications (P=0.483) [including pneumothoraces (P=0.769) and pulmonary hemorrhage (P=1.000)], operating room time (P=0.926), complete resection rates (P=0.520), intraoperative complications (P=0.549), and postoperative complications (P=1.000) were similar between the pilot and control groups. Conclusions Preoperative CT-guided lung nodule microcoil localization performed without visceral pleural marking appears to decrease the CT procedure time and radiation dose while maintaining equivalent complete resection rates and procedural and surgical complications, when compared with microcoil localization performed with pleural marking.

Objective:We sought to determine whether patients or their families could identify adverse events in the emergency department (ED), to characterize patient reports of errors and to compare patient reports to events recorded by health care providers.Methods:This was a prospective cohort study in a quaternary care inner city teaching hospital with approximately 40 000 annual visits. ED patients were recruited for participation in a standardized interview within 24 hours of ED discharge and a follow-up interview 3–7 days after discharge. Responses regarding events were tabulated and compared with physician and nurse notations in the medical record and hospital event reporting system.Results:Of 292 eligible patients, 201 (69%) were interviewed within 24 hours of ED discharge, and 143 (71% of interviewees) underwent a follow-up interview 3–7 days after discharge. Interviewees did not differ from the base ED population in terms of age, sex or language. Analysis of patient interviews identified 10 adverse events (5% incident rate; 95% confidence interval [CI] 2.41%–8.96%), 8 near misses (4% incident rate; 95% CI 1.73%–7.69%) and no medical errors. Of the 10 adverse events, 6 (60%) were characterized as preventable (2 raters; κ = 0.78, standard error [SE] 0.20; 95% CI 0.39–1.00;p= 0.01). Adverse events were primarily related to delayed or inadequate analgesia. Only 4 out of 8 (50%) near misses were intercepted by hospital personnel. The secondary interview elicited 2 out of 10 adverse events and 3 out of 8 near misses that had not been identified in the primary interview. No designation (0 out of 10) of an adverse event was recorded in the ED medical record or in the confidential hospital event reporting system.Conclusion:ED patients can identify adverse events affecting their care. Moreover, many of these events are not recorded in the medical record. Engaging patients and their family members in identification of errors may enhance patient safety.

With improvements in surgical and medical management, patients with congenital heart disease (CHD) are often living well into adulthood. MRI provides critical data for diagnosis and monitoring of these patients, yielding information on cardiac anatomy, blood flow, and cardiac function. Though historically these exams have been complex and lengthy, four-dimensional (4D) flow is emerging as a single fast technique for comprehensive assessment of CHD. The 4D flow consists of a volumetric time-resolved acquisition that is gated to the cardiac cycle, providing a time-varying vector field of blood flow as well as registered anatomic images. In this article, we provide an overview of MRI evaluation of congenital heart disease by means of example of three relatively common representative conditions: tetralogy of Fallot, aortic coarctation, and anomalous pulmonary venous drainage. Then 4D flow data acquisition, data correction, and postprocessing techniques are reviewed. We conclude with several examples that highlight the comprehensive nature of the evaluation of congenital heart disease with 4D flow.

To investigate four-dimensional (4D) phase-contrast (PC) magnetic resonance (MR) in the evaluation of intracardiac shunts by simultaneous assessment of pulmonary (QP) and systemic (QS) flows in a pilot study and to compare results to through-plane two-dimensional (2D) PC MR.Institutional review board approval and written informed consent were obtained. Nineteen patients with suspected intracardiac shunts underwent cardiac MR at 1.5T. Assessments of QP and QS were performed using free-breathing retrospectively gated 2D PC gradient recalled echo (GRE; 1.6 × 1.6 × 5 mm(3)) imaging with one-dimensional through-plane velocity encoding gradient (venc = 150 cm/s) in consecutive measurements for the main pulmonary artery (MPA) and ascending aorta (AA), respectively. A prospectively triggered 4D PC GRE technique (2.4 × 1.8 × 3 mm(3)) with three orthogonal venc directions was also used with volume coverage of both MPA and AA.QP and QS assessed by 4D PC correlated with 2D PC acquisitions (r = 0.92 and r = 0.67 respectively; P .0001 for both) but demonstrated significant underestimation of individual flow volumes (-21.9 ± 12.2 mL; P .0001 and -10.7 ± 13.1 mL; P = .0023, respectively). Calculated QP:QS ratios demonstrated high correlation (r = 0.78; P .0001) and no significant differences between 4D PC and 2D PC acquisitions (-0.09 ± 0.24, P = .14). Image acquisition times for 2D PC assessment of QP and QS were 2.98 ± 0.52 and 2.84 ± 0.50 minutes, respectively (P = .038), whereas time to acquire 4D PC images was significantly longer, 18.75 ± 4.58 minutes (P .001).Four-dimensional PC MR imaging allows for accurate assessment of QP:QS ratios in the evaluation of intracardiac shunts while absolute flow volumes demonstrate offsets. Further refinement of the technique with improvement in acquisition times may be required before widespread clinical implementation.

The purpose of this study is to examine the effect of different iron chelation regimens on the distribution of myocardial iron in patients with transfusion-dependent anemias. Institutional review board approval was obtained. Patients treated with iron chelation therapy who had undergone baseline and 1-year follow-up cardiac T2* MR studies in a four-year period were identified retrospectively. One hundred and eight patients (44 % male, mean age 31.6 ± 9.7 years) were included. The interventricular septum on three short-axis slices (basal, mid and apical) was divided into anterior and inferior regions of interest for T2* analysis. Cardiac iron concentration (CIC) was calculated from T2* values. Statistical analysis included analysis of variance and paired t-test, using Bonferroni adjustment in all pairwise comparisons. At baseline, T2* measurements varied significantly across all six regions (p < 0.001): lowest in the mid anteroseptum (mean 22.3 ± 10.1 ms) and highest in the apical inferoseptum (mean 26.2 ± 12.8 ms). At follow-up, T2* and CIC values improved significantly in all segments [mean change of 3.78 ms (95 % CI (2.93, 4.62), p < 0.001) and 0.23 mg/g (95 % CI (0.16, 0.29), p < 0.001), respectively]. Change in T2* values varied significantly between segments (p < 0.001) with greatest improvement in the apical inferoseptum [4.26 ms, 95 % CI (2.42, 6.11)] and least improvement in the basal anteroseptum [2.95 ms, 95 % CI (1.37, 4.54)]. The largest improvement in T2* values was noted in patients treated with deferiprone [4.96 ms, 95 % CI (2.34, 7.58)]. There was a statistically significant difference in improvement in CIC values between chelation regimens (p = 0.016). This is the first study to report heterogeneity in response to iron chelating drugs with variable segmental changes in T2* values.

We report the case of a 42-year-old patient with hypertrophic cardiomyopathy (HCM) who presented to the emergency department with severe shortness of breath one week following uneventful cesarean delivery. Thoracic CT ruled out pulmonary embolus and confirmed pulmonary edema. Asymmetric interventricular septal thickening was clearly identified, demonstrating that the heart may be evaluated even on a non-ECG gated study. Acute pulmonary edema in the postpartum period is an unusual clinical presentation of HCM.

Background The diagnosis of pericardial constriction (PC) remains challenging and cardiac magnetic resonance (CMR) is increasingly used as a diagnostic tool. The objective of this study was to evaluate CMR findings for the prediction of subsequent surgical pericardiectomy. Methods CMR studies of 36 patients referred to assess for PC were evaluated retrospectively. Patients were divided into two groups depending on whether they subsequently had their pericardium stripped (n=18) or did not (n=18). IVC and aortic areas were determined by manual contouring on a single axial-SSFP image in maximum systole at the level of the esophageal hiatus. The ratio of IVC to aortic (I:A) area was calculated. Cross-sectional areas were indexed to body surface area (BSA). Quantitative data was assessed with a two-sample t-test and qualitative data was assessed with Fisher’s exact test. A logistic regression model was used to determine the predictive probability of surgical pericardiectomy based on CMR features. Odds ratios (ORs) were calculated and receiver operating characteristic (ROC) analysis was performed. Results Mean age of patients was 53.9±15.3 years, 72% (n=26) male, with no significant difference in mean age between the two groups (p=0.429). In patients with constriction, the underlying etiology was idiopathic (39%, n=7), infectious (28%, n=5), post-surgical (17%, n=3), connective-tissue disease (11%, n=2), and post-radiation (6%, n=1). IVC area, indexed IVC area, I:A ratio, pericardial thickness, RV area and indexed RV area were significantly different in patients who underwent pericardiectomy compared to those who did not (Table 1). Pericardiectomy was significantly associated with pericardial enhancement (p=0.011) as well as septal bounce (p

Objective:We sought to characterize patients who are referred from the emergency department (ED) to specialty clinics but do not complete the referral, and to identify reasons for their failure to follow up.Methods:A prospective cohort study was carried out over 3 months of patients who were discharged from the ED of a teaching hospital with referral to internal medicine, cardiology or neurology clinics, but who did not complete the referral. Information on demographics, barriers to care and reasons for not completing the referral was obtained through a standardized telephone interview.Results:Of 171 ED referrals, 42 (24.6%) were not completed. Interviews were completed for 71.4% (30 patients). Of the nonattenders, 80% were functional in English and most had high school (73.1%) or university (60.7%) education. Virtually all (93.0%) interviewees could get to hospital by themselves or have someone take them. Only 42.9% (12 patients) understood why the emergency physician (EP) requested consultation, and 42.9% (12 patients) described EP instructions as poor or fair. Primary reasons for noncompletion of consult were patient choice (46.7%, 95% confidence interval [CI] 27.1%–66.2%), physical or social barriers (13.3%, 95% CI 0.0%–27.2%), communication failure (20%, 95% CI 4.0%–36.0%) and consultant's refusal of the consultation (20% [95% CI 4.0%–36.0%]). All consultant refusals were from one internal medicine clinic, representing 42% (8/19) of ED referrals to that clinic. None of the 6 patients interviewed who were declined consultation was aware that their consultation had been refused.Conclusion:Patients discharged by the EP with referral to specialty clinics frequently do not complete the consultation. Causes for failure to follow up relate to patient decision, inadequate or poorly understood discharge information, and system factors. Institutional audits of patients who fail to complete follow-up may reveal unanticipated barriers to care.

Multimodality Imaging in the Preoperative Diagnosis of Lipomatous Hypertrophy Kate Hanneman, MD, Laura Jimenez-Juan, MD, Anish Kirpalani, MD, Andrew T. Yan, MD, and Kim A. Connelly, MBBS, PhD a Department of Medical Imaging, St Michael’s Hospital, University of Toronto, Toronto, Ontario, Canada b Department of Medical Imaging, University Health Network, University of Toronto, Toronto, Ontario, Canada

Background Although it is known that Anderson-Fabry Disease (AFD) can mimic the morphologic manifestations of hypertrophic cardiomyopathy (HCM) on echocardiography, there is a lack of cardiovascular magnetic resonance (CMR) literature on this. There is limited information in the published literature on the distribution of myocardial fibrosis in patients with AFD, with scar reported principally in the basal inferolateral midwall. Methods All patients with confirmed AFD undergoing CMR at our center were included. Left ventricular (LV) volumes, wall thicknesses and scar were analyzed offline. Patients were categorized into 4 groups: 1) no wall thickening; 2) concentric hypertrophy; 3) asymmetric septal hypertrophy (ASH); and 4) apical hypertrophy. Charts were reviewed for clinical information. Results Thirty-nine patients were included (20 males [51 %], median age 45.2 years [range 22.3–64.4]). Almost half (17/39) had concentric wall thickening. Almost half (17/39) had pathologic LV scar; three quarters of these (13/17) had typical inferolateral midwall scar. A quarter (9/39) had both concentric wall thickening and typical inferolateral scar. A subgroup with ASH and apical hypertrophy (n = 5) had greater maximum wall thickness, total LV scar, apical scar and mid-ventricular scar than those with concentric hypertrophy (n = 17, p < 0.05). Patients with elevated LVMI had more overall arrhythmia (p = 0.007) more ventricular arrhythmia (p = 0.007) and sustained ventricular tachycardia (p = 0.008). Conclusions Concentric thickening and inferolateral mid-myocardial scar are the most common manifestations of AFD, but the spectrum includes cases morphologically identical to apical and ASH subtypes of HCM and these have more apical and mid-ventricular LV scar. Significant LVH is associated with ventricular arrhythmia. Electronic supplementary material The online version of this article (doi:10.1186/s12968-016-0233-6) contains supplementary material, which is available to authorized users.