Ebstein anomaly review: what’s now,what’s next?

Ebstein anomaly accounts for 1% of all congenital heart disease. It is a right ventricular myopathy with failure of tricuspid valve delamination and highly variable tricuspid valve morphology that usually results in severe regurgitation. It is the only congenital heart lesion that has a range of clinical presentations, from the severely symptomatic neonate to an asymptomatic adult. Neonatal operation has high operative mortality, whereas operation performed beyond infancy and into adulthood has low operative mortality. Late survival and quality of life for hospital survivors are excellent for the majority of patients in all age brackets. Atrial tachyarrhythmias are the most common late complication. There have been more techniques of tricuspid repair reported in the literature than any other congenital or acquired cardiac lesion. This is largely due to the infinite anatomic variability encountered with this anomaly. The cone reconstruction of Ebstein anomaly can achieve near anatomic restoration of the tricuspid valve anatomy. Early and intermediate results with these repairs are promising. Reduced right ventricular function continues to be a challenge for some patients, as is the need for reoperation for recurrent tricuspid regurgitation. The purpose of this article is to outline the current standard of care for diagnosis and treatment of Ebstein anomaly and describe innovative strategies to address poor right ventricular function and associated right-sided heart failure.     

Association of atrial tachyarrhythmias with atrial septal defect,Ebstein’s anomaly and Fontan patients

The number of adults with congenital heart disease is increasing as medical and surgical palliation for congenital heart lesions improves. With this comes long-term complications of congenital heart disease such as the increased risk of atrial tachyarrhythmias. Atrial septal defect, Ebstein’s anomaly and post-Fontan patient subsets are particularly important to focus on due to their unique characteristics and association with atrial tachyarrhythmias. Reviews, randomized controlled trials, and meta-analyses were obtained using electronic search strategies such as Medline and the Cochrane Library. References of electronically obtained studies were then used to obtain additional relevant studies. Sources were deemed relevant if they discussed the relationship between atrial septal defects/Ebstein’s anomaly/Fontan procedure and atrial tachyarrhythmias in respect to incidence, mechanism, recurrence or treatment. Selected sources were then stratified on the basis of quality. Patients in these subsets of congenital heart disease are at increased risk of atrial tachyarrhythmias for a variety of reasons when compared with the general population. It is necessary for pediatric and adult cardiologists alike to understand these differences, as well as their implications in diagnosis and management of such occurrences.     

‘Image-navigated 3-dimensional late gadolinium enhancement cardiovascular magnetic resonance imaging: feasibility and initial clinical results’

Background

Image-navigated 3-dimensional late gadolinium enhancement (iNAV-3D LGE) is an advanced imaging technique that allows for direct respiratory motion correction of the heart. Its feasibility in a routine clinical setting has not been validated.

Methods

Twenty-three consecutive patients referred for cardiovascular magnetic resonance (CMR) examination including late gadolinium enhancement (LGE) imaging were prospectively enrolled. Image-navigated free-breathing 3-dimensional (3D) T1-weighted gradient-echo LGE and two-dimensional (2D LGE) images were acquired in random order on a 1.5 T CMR system. Images were assessed for global, segmental LGE detection and transmural extent. Objective image quality including signal-to-noise (SNR), contrast-to-noise (CNR) and myocardial/blood sharpness were performed.

Results

Interpretable images were obtained in all 2D–LGE and in 22/23 iNAV-3D LGE exams, resulting in a total of 22 datasets and 352 segments. LGE was detected in 5 patients with ischemic pattern, in 7 with non-ischemic pattern, while it was absent in 10 cases. There was an excellent agreement between 2D and 3D data sets with regard to global, segmental LGE detection and transmurality. Blood-myocardium sharpness measurements were also comparable between the two techniques. SNR_blood and CNR_blood-myo was significantly higher for 2D LGE (P?<?0.001, respectively), while SNR_myo was not statistically significant between 2D LGE and iNAV-3D LGE.

Conclusion

Diagnostic performance of iNAV-3D LGE was comparable to 2D LGE in a prospective clinical setting. SNR_blood and CNR_blood-myo was significantly lower in the iNAV-3D LGE group.

     

Which cardiovascular magnetic resonance planes and sequences provide accurate measurements of branch pulmonary artery size in children with right ventricular outflow tract obstruction?

Children with right ventricular outflow tract obstructive (RVOTO) lesions require precise quantification of pulmonary artery (PA) size for proper management of branch PA stenosis. We aimed to determine which cardiovascular magnetic resonance (CMR) sequences and planes correlated best with cardiac catheterization and surgical measurements of branch PA size. Fifty-five children with RVOTO lesions and biventricular circulation underwent CMR prior to; either cardiac catheterization (n = 30) or surgery (n = 25) within a 6 month time frame. CMR sequences included axial black blood, axial, coronal oblique and sagittal oblique cine balanced steady-state free precession (bSSFP), and contrast-enhanced magnetic resonance angiography (MRA) with multiplanar reformatting in axial, coronal oblique, sagittal oblique, and cross-sectional planes. Maximal branch PA and stenosis (if present) diameter were measured. Comparisons of PA size on CMR were made to reference methods: (1) catheterization measurements performed in the anteroposterior plane at maximal expansion, and (2) surgical measurement obtained from a maximal diameter sound which could pass through the lumen. The mean differences (Δ) and intra class correlation (ICC) were used to determine agreement between different modalities. CMR branch PA measurements were compared to the corresponding cardiac catheterization measurements in 30 children (7.6 ± 5.6 years). Reformatted MRA showed better agreement for branch PA measurement (ICC > 0.8) than black blood (ICC 0.4–0.6) and cine sequences (ICC 0.6–0.8). Coronal oblique MRA and maximal cross sectional MRA provided the best correlation of right PA (RPA) size with ICC of 0.9 (Δ ?0.1 ± 2.1 mm and Δ 0.5 ± 2.1 mm). Maximal cross sectional MRA and sagittal oblique MRA provided the best correlate of left PA (LPA) size (Δ 0.1 ± 2.4 and Δ ?0.7 ± 2.4 mm). For stenoses, the best correlations were from coronal oblique MRA of right pulmonary artery (RPA) (Δ ?0.2 ± 0.8 mm, ICC 0.9) and sagittal oblique MRA of left pulmonary artery (LPA) (Δ 0.2 ± 1.1 mm, ICC 0.9). CMR PA measurements were compared to surgical measurements in 25 children (5.4 ± 4.8 years). All MRI sequences demonstrated good agreement (ICC > 0.8) with the best (ICC 0.9) from axial cine bSSFP for both RPA and LPA. Maximal cross sectional and angulated oblique reformatted MRA provide the best correlation to catheterization for measurement of branch PA’s and stenosis diameter. This is likely due to similar angiographic methods based on reformatting techniques that transect the central axis of the arteries. Axial cine bSSFP CMR was the best surgically measured correlate of PA branch size due to this being a measure of stretched diameter. Knowledge of these differences provides more precise PA measurements and may aid catheter or surgical interventions for RVOTO lesions.     

Antithrombin and hypercoagulability in sepsis: insights from thrombelastography?

Antithrombin (AT) has been used for over 25 years to successfully treat disseminated intravascular coagulation (DIC). A four-day AT therapy in patients with DIC in the KyberSept trial has been related to a clear survival benefit in patients not receiving concomitant heparin. Gonano and coworkers performed thrombelastography (TEG) measurements in patients with severe sepsis and clearly showed hypercoagulability, as defined by five TEG parameters, compared to healthy controls. In the AT group they found a trend towards normalization of TEG parameters after treatment, although this did not reach statistical significance. This first clinical evaluation of hypercoagulability during AT treatment could not provide evidence for an attenuation of coagulopathy, an effect that might be due to high inter-individual variability.     

Cardiac magnetic resonance imaging characteristics for the differentiation of athlete’s heart from inherited cardiomyopathies

The International Journal of Cardiovascular Imaging -     

European cardiovascular magnetic resonance (EuroCMR) registry – multi national results from 57 centers in 15 countries

Abstract

Background

The EuroCMR registry sought to evaluate indications, image quality, safety and impact on patient management of clinical routine CMR in a multi-national European setting. Furthermore, interim analysis of the specific protocols should underscore the prognostic potential of CMR.

Methods

Multi-center registry with consecutive enrolment of patients in 57 centers in 15 countries. More than 27000 consecutive patients were enrolled.

Results

The most important indications were risk stratification in suspected CAD/Ischemia (34.2%), workup of myocarditis/cardiomyopathies (32.2%), as well as assessment of viability (14.6%). Image quality was diagnostic in more than 98% of cases. Severe complications occurred in 0.026%, always associated with stress testing. No patient died during or due to CMR. In 61.8% CMR findings impacted on patient management. Importantly, in nearly 8.7% the final diagnosis based on CMR was different to the diagnosis before CMR, leading to a complete change in management. Interim analysis of suspected CAD and risk stratification in HCM specific protocols revealed a low rate of adverse events for suspected CAD patients with normal stress CMR (1.0% per year), and for HCM patients without LGE (2.7% per year).

Conclusion

The most important indications in Europe are risk stratification in suspected CAD/Ischemia, work-up of myocarditis and cardiomyopathies, as well as assessment of viability. CMR imaging is a safe procedure, has diagnostic image quality in more than 98% of cases, and its results have strong impact on patient management. Interim analyses of the specific protocols underscore the prognostic value of clinical routine CMR in CAD and HCM.

Condensed abstract

The EuroCMR registry sought to evaluate indications, image quality, safety and impact on patient management of clinical routine CMR in a multi-national European setting in a large number of cases (n > 27000). Based on our data CMR is frequently performed in European daily clinical routine. The most important indications in Europe are risk stratification in suspected CAD/Ischemia, work-up of myocarditis and cardiomyopathies, as well as assessment of viability. CMR imaging is a safe procedure, has diagnostic image quality in more than 98% of cases, and its results have strong impact on patient management. Interim analyses of the specific protocols underscore the prognostic value of clinical routine CMR in CAD and HCM.     

Colonic Crohn’s disease: value of magnetic resonance colonography for detection and quantification of disease activity

Conventional colonoscopy combined by histological examination, represents the standard for the evaluation of colorectal pathologies and usually is the first examination for the evaluation of patients with suspected or established diagnosis of Crohn’s disease (CD). However, information provided by colonoscopy is limited to mucosal alterations since the technique is unable to evaluate transmural changes or presence of extraluminal complications such as abscesses or fistula. Technological advances in magnetic resonance (MR) raised expectations on the potential role of this imaging modality for evaluation of the gastrointestinal tract based on the high spatial and tissue resolution as well as lack of ionizing radiation. Available evidence indicates that MR colonography (MRC) can be a useful tool as an alternative or complementary to endoscopy for the detection of activity and assessment of severity in colonic CD. In this article, we review the technical aspects of MRC and the spectrum of findings that provide valuable information for the evaluation of colonic CD. Potential applications and limitations of MRC are also discussed.     

Diagnostic value of amygdala volume on structural magnetic resonance imaging in Alzheimer’s disease

BACKGROUNDThe main clinical manifestation of Alzheimer’s disease (AD) is memory loss, which can be accompanied by neuropsychiatric symptoms at different stages of the disease. Amygdala is closely related to emotion and memory. AIMTo evaluate the diagnostic value of amygdala on structural magnetic resonance imaging (sMRI) for AD.METHODSIn this study, 22 patients with AD and 26 controls were enrolled. Their amygdala volumes were measured by sMRI and analyzed using an automatic analysis software. RESULTSThe bilateral amygdala volumes of AD patients were significantly lower than those of the controls and were positively correlated with the hippocampal volumes. Receiver operating characteristic curve analyses showed that the sensitivity of the left and right amygdala volumes in diagnosing AD was 80.8% and 88.5%, respectively. Subgroup analyses showed that amygdala atrophy was more serious in AD patients with neuropsychiatric symptoms, which mainly included irritability (22.73%), sleep difficulties (22.73%), apathy (18.18%), and hallucination (13.64%).CONCLUSIONAmygdala volumes measured by sMRI can be used to diagnose AD, and amygdala atrophy is more serious in patients with neuropsychiatric symptoms.     

The distribution and prognosis of anomalous coronary arteries identified by cardiovascular magnetic resonance: 15?year experience from two tertiary centres

Background

Aberrant coronary arteries represent a diverse group of congenital disorders. Post-mortem studies reveal a high risk of exercise-related sudden cardiac death in those with an anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an inter-arterial course. There is little documentation of lifetime history and long-term follow-up of patients with coronary artery anomalies.

Methods

Patients with anomalous coronary arteries undergoing cardiovascular magnetic resonance over a 15-year period were identified and classified by anatomy and course. Medical records were reviewed for major adverse cardiovascular events (MACE). Revascularisation or myocardial infarction counted only if occurring in the distribution of the anomalous artery.

Results

Consecutive patients with coronary artery anomalies were retrospectively identified (n = 172). Median follow-up time was 4.3 years (IQR 2.5–7.8, maximum 15.6). 116 patients had ACAOS of which 64 (55%) had an inter-arterial course (IAC) and 52 (45%) did not. During follow up 110 ACAOS patients were alive, 5 died and 1 lost to follow-up.ACAOS patients experienced 58 MACE events (5 cardiovascular deaths, 5 PCI, 24 CABG and 24 had myocardial infarction). 47 MACE events occurred in ACAOS with IAC and 11 in those without (p < 0.0001), the statistical difference driven by surgical revascularisation and myocardial infarction.

Conclusions

In life, patients with an anomalous coronary artery originating from the opposite sinus of Valsalva taking an IAC have higher rates of both myocardial infarction and surgical revascularisation during long-term follow up, compared to those without IAC.     

Left ventricular filling pressure by septal and lateral E/e′ equally predict cardiovascular events in the general population

Purpose: There exists no consensus on the site of E/e′ measurement. This study aimed to evaluate the predictive value of septal and lateral E/e′ along with the importance of their intra-individual difference. Methods: In 1775 persons from the general population, peak early diastolic velocity (e′) was obtained by color tissue Doppler at the septal and lateral mitral annular sites. The endpoint was combined of cardiovascular death or admission due to heart failure or acute myocardial infarction. Results: During a median follow-up of 10.9 years, the endpoint occurred in 227 participants. E/e′_septal and E/e′_lateral were equally strong predictors of cardiac events; in age- and sex-adjusted models they did not differ in AUC (septal: 0.8385, lateral: 0.8389; p?=?0.94) or in continuous NRI (p?=?0.84). Models using E/e′_average did not improve AUC or NRI, and the intra-individual difference between sites had no predictive value (p?=?0.79). E/e′_septal was generally higher than E/e′_lateral, thus age- and sex-specific normal values were reported for both sites for a population free of cardiac events during 10 years of follow-up. Conclusions: Septal and lateral E/e′ are equally useful in predicting cardiac events in the general population. Measuring both sites provides no further predictive value than measuring a single site.     

Fetal circulation in left-sided congenital heart disease measured by cardiovascular magnetic resonance: a case–control study

Background

The distribution of blood flow in fetuses with congenital heart disease (CHD) is likely to influence fetal growth, organ development, and postnatal outcome, but has previously been difficult to study. We present the first measurements of the distribution of the fetal circulation in left-sided CHD made using phase contrast cardiac magnetic resonance (CMR).

Methods

Twenty-two fetuses with suspected left-sided CHD and twelve normal controls underwent fetal CMR and echocardiography at a mean of 35 weeks gestation (range 30–39 weeks).

Results

Fetuses with left-sided CHD had a mean combined ventricular output 19% lower than normal controls (p < 0.01). In fetuses with left-sided CHD with pulmonary venous obstruction, pulmonary blood flow was significantly lower than in those with left-sided CHD without pulmonary venous obstruction (p < 0.01). All three fetuses with pulmonary venous obstruction had pulmonary lymphangectasia by fetal CMR and postnatal histology. Fetuses with small but apex forming left ventricles with left ventricular outflow tract or aortic arch obstruction had reduced ascending aortic and foramen ovale flow compared with normals (p < 0.01). Fetuses with left-sided CHD had more variable superior vena caval flows than normal controls (p < 0.05). Six fetuses with CHD had brain weights at or below the 5^th centile for gestational age, while none of the fetuses in the normal control group had brain weights below the 25^th centile.

Conclusions

Measurement of the distribution of the fetal circulation in late gestation left-sided CHD is feasible with CMR. We demonstrated links between fetal blood flow distribution and postnatal course, and examined the relationship between fetal hemodynamics and lung and brain development. CMR enhances our understanding of pathophysiology of the fetal circulation and, with more experience, may help with the planning of perinatal management and fetal counselling.     

Balo’s concentric sclerosis in a patient with spontaneous remission based on magnetic resonance imaging: A case report and review of literature

Balo’s concentric sclerosis (BCS) is a rare monophasic demyelinating disease known as multiple sclerosis subtype and seen as a round lesion with variable hyper and hypo-detoxification layers. Characteristic appearance can be seen as “bulb eye” or “onion bulb”. The initial terminology for this neurological disorder was leukoencephalitis periaxialis concentrica; this is defined as a disease in which the white matter of the brain is destroyed in concentric layers in such a way as to leave the axial cylinders intact. This report presents a case of BCS with spontaneous healing of the patient and a mass lesion with concentric rings adjacent to the left lateral ventricle and the posterior portion of the corpus callosum with peripheral vasogenic edema. The neurological lesion of the patient was similar to the magnetic resonance imaging and clinical findings of the BCS.     

Postoperative diarrhea in Crohn’s disease: Pathogenesis,diagnosis, and therapy

Diarrhea is a frequent symptom in postoperative patients with Crohn’s diseases (CD), and several different mechanisms likely account for postoperative diarrhea in CD. A targeted strategy based on a comprehensive understanding of postoperative diarrhea is helpful for better postoperative recovery.     

Brave Spaces in Discourse About Pregnant Persons’, Women’s,and Children’s Health

Discourse around implicit bias, intersectionality, and inequity in health care requires having conversations that may be uncomfortable. Within this space of discomfort, new understandings can occur. Reflecting on our own biases and assumptions is a moral obligation and a necessity to generate change. Health care settings may be a fitting forum to create and use brave spaces to have these conversations.     

3.0 T cardiovascular magnetic resonance in patients treated with coronary stenting for myocardial infarction: evaluation of short term safety and image quality

Purpose To evaluate safety and image quality of cardiovascular magnetic resonance (CMR) at 3.0 T in patients with coronary stents after myocardial infarction (MI), in comparison to the clinical standard at 1.5 T. Methods Twenty-five patients (21 men; 55 ± 9 years) with first MI treated with primary stenting, underwent 18 scans at 3.0 T and 18 scans at 1.5 T. Twenty-four scans were performed 4 ± 2 days and 12 scans 125 ± 23 days after MI. Cine (steady-state free precession) and late gadolinium-enhanced (LGE, segmented inversion-recovery gradient echo) images were acquired. Patient safety and image artifacts were evaluated, and in 16 patients stent position was assessed during repeat catheterization. Additionally, image quality was scored from 1 (poor quality) to 4 (excellent quality). Results There were no clinical events within 30 days of CMR at 3.0 T or 1.5 T, and no stent migration occurred. At 3.0 T, image quality of cine studies was clinically useful in all, but not sufficient for quantitative analysis in 44% of the scans, due to stent (6/18 scans), flow (7/18 scans) and/or dark band artifacts (8/18 scans). Image quality of LGE images at 3.0 T was not sufficient for quantitative analysis in 53%, and not clinically useful in 12%. At 1.5 T, all cine and LGE images were quantitatively analyzable. Conclusion 3.0 T is safe in the acute and chronic phase after MI treated with primary stenting. Although cine imaging at 3.0 T is suitable for clinical use, quantitative analysis and LGE imaging is less reliable than at 1.5 T. Further optimization of pulse sequences at 3.0 T is essential.