Computed tomography and magnetic resonance imaging of vascular rings and other things: a pictorial review.

Compression of the airway, esophagus or both by aortic and pulmonary vessels can be caused by a variety of anatomical situations. Vascular rings are the most commonly encountered entity; however, compression can also occur from less common anomalies such as a left pulmonary artery sling or innominate artery compression. Vascular rings and other vascular compression abnormalities can be challenging to visualize and image and often require advanced imaging by CT or MRI to better understand the cause and extent of compression. Atretic vascular structures, such as the ligamentum arteriosum or atretic arch, play a key role in creating a vascular ring and do not enhance with contrast agent in a typical fashion. Despite these imaging challenges, classic and useful signs can indicate the presence or absence of a vascular ring or compression.

Correction to: Aortic dilatation in children with mild to moderate chronic kidney disease.

The original version of this article unfortunately contained a mistake.

MRI detection of occult venous anomalies in a patient with Williams syndrome: a case report.

Williams syndrome is a multisystem, congenital disorder which is commonly associated with arterial stenoses: supravalvar aortic stenosis and peripheral pulmonary artery stenosis. Venous abnormalities have not been previously reported in children with Williams syndrome. We present a case of a 3-year-old girl with Williams syndrome and diffuse venous ectasia as detected by MRI.

Aortic dilatation in children with mild to moderate chronic kidney disease.

BACKGROUND: Children with mild to moderate chronic kidney disease are at an increased risk for cardiovascular sequelae, the leading cause of death in children with end-stage renal disease. We aimed to establish the prevalence of aortic dilatation, a newly recognized cardiovascular sequelae of renal disease, within a cohort of pediatric patients with mild to moderate kidney disease. METHODS: A total of 501 children enrolled in the Chronic Kidney Disease in Children study contributed imaging data between April 2011 and February 2015. Aortic dilatation was defined as a dimension exceeding a z-score of 2 at any of three locations: aortic root, sinotubular junction, or the ascending aorta. RESULTS: At baseline echocardiographic evaluation, 30 (6%) children were identified to have aortic dilatation in at least one of the three locations. Multivariate analysis demonstrated an increased odds ratio for the presence of aortic dilatation associated with the following variables: high diastolic blood pressure z-scores, low weight z-score, and low body mass index z-score. Presense of protein energy wasting (modified definition, OR 2.41, 95%CI 1.23, 4.70) was the strongest independent predictor of aortic dilatation. CONCLUSION: In conclusion, aortic dilatation does occur early in the course of chronic kidney disease and associates with markers of poor nutrition. Future studies should continue to evaluate these risk factors longitudinally as the kidney disease progresses.

Establishment of Specialized Clinical Cardiovascular Genetics Programs: Recognizing the Need and Meeting Standards: A Scientific Statement From the American Heart Association.

Cardiovascular genetics is a rapidly evolving subspecialty within cardiovascular medicine, and its growth is attributed to advances in genome sequencing and genetic testing and the expanding understanding of the genetic basis of multiple cardiac conditions, including arrhythmias (channelopathies), heart failure (cardiomyopathies), lipid disorders, cardiac complications of neuromuscular conditions, and vascular disease, including aortopathies. There have also been great advances in clinical diagnostic methods, as well as in therapies to ameliorate symptoms, slow progression of disease, and mitigate the risk of adverse outcomes. Emerging challenges include interpretation of genetic test results and the evaluation, counseling, and management of genetically at-risk family members who have inherited pathogenic variants but do not yet manifest disease. With these advances and challenges, there is a need for specialized programs combining both cardiovascular medicine and genetics expertise. The integration of clinical cardiovascular findings, including those obtained from physical examination, imaging, and functional assessment, with genetic information allows for improved diagnosis, prognostication, and cascade family testing to identify and to manage risk, and in some cases to provide genotype-specific therapy. This emerging subspecialty may ultimately require a new cardiovascular subspecialist, the genetic cardiologist, equipped with these combined skills, to permit interpretation of genetic variation within the context of phenotype and to extend the utility of genetic testing. This scientific statement outlines current best practices for delivering cardiovascular genetic evaluation and care in both the pediatric and the adult settings, with a focus on team member expertise and conditions that most benefit from genetic evaluation.

Comparison of Native T1, Strain, and Traditional Measures of Cardiovascular Structure and Function by Cardiac Magnetic Resonance Imaging in Patients With Anderson-Fabry Disease.

Cardiovascular magnetic resonance imaging (CMR) has emerged as a powerful tool to illuminate cardiovascular pathology in Anderson-Fabry disease (AFD); however, further study is required to develop clinically useful monitoring paradigms. The objective of this study was to retrospectively evaluate strain, native septal T1 values, and standard CMR measurements in a cohort of AFD patients to characterize useful measures of cardiovascular dysfunction that may be derived from a CMR platform. Eighteen patients were identified (n = 8 males) and divided according to presence or absence of left ventricular hypertrophy (LVH). Biometric data were gathered and native T1 and strain values were measured for all patients. Patients with LVH were older and had significantly lower native T1 measured at the apical septal (893 ± 78 vs 1044 ± 217 ms, p = 0.035), midventricular septal (864 ± 76 vs 988 ± 67 ms, p = 0.016), and basal septal (867 ± 58 vs 1027 ± 84 ms, p = 0.006) regions. Circumferential strain was more positive in patients with LVH (-13.5% ± 5.0% vs -18.7% ± 2.7%, p = 0.042), but longitudinal strain was not significantly different between groups. Patients with LVH had higher stroke volumes (114.5 ± 9.7 vs 96.7 ± 17.8 ml, p = 0.050), but other standard CMR measures were not significantly different. In conclusion, AFD patients with LVH have reduced native T1 and more positive circumferential strain compared to those without. The basal septum may be an appropriate region for standard measure of native T1 in this population.

Neonatal Enterovirus Myocarditis With Severe Dystrophic Calcification: Novel Treatment With Pocapavir.

Dystrophic myocardial calcification occurs in the setting of myocardial injury and normal serum calcium. We present a case of a neonate with prominent dystrophic calcification and severe left ventricular systolic dysfunction in the setting of enterovirus myocarditis. These findings are superbly illustrated by multiple imaging modalities. The patient was treated with the novel antiviral, pocapavir, in addition to a standard heart failure regimen. The dystrophic calcification persisted but the left ventricle remodeled significantly. To our knowledge, this is the first reported use of pocapavir for this indication. The literature regarding enterovirus myocarditis and pocapavir is briefly reviewed.

Arrhythmia and Clinical Cardiac Findings in Children With Anderson-Fabry Disease.

Anderson-Fabry Disease (AFD) is a lysosomal storage disorder that results in progressive cardiovascular hypertrophy, scarring, and arrhythmia burden; yet, the early cardiac phenotype of AFD is still poorly defined. To further characterize early cardiac features in AFD, we evaluated electrocardiographic and clinical findings contained in a local cohort of pediatric AFD patients and arrhythmia data in children enrolled in the Fabry Registry. Twenty-six local patients aged <18 years were identified (average age 9.7 ± 3.8 years, n = 12 males). Sinus bradycardia was the most frequent rhythm abnormality (23%), followed by ectopic atrial rhythm (12%) and premature atrial contractions (8%). No PR, QRS, or QTc intervals were prolonged. First-degree atrioventricular block developed in 1 female during follow-up. Chest pain (35%) and palpitations (23%) were highly prevalent complaints in clinical follow-up and did not differ significantly between genders. Structural findings included aortic root dilation in 3 patients and concurrent aortic insufficiency in 1. Among 593 patients aged < 18 years with electrocardiographic data identified in the Fabry Registry, sinus bradycardia, defined as heart rate <60 beats per minute per registry guidelines, was the most common arrhythmia (12.3%). In conclusion, clinical findings and subtle abnormalities of conduction, rhythm, and structure point toward a heterogeneous inception of Fabry cardiomyopathy. Bradycardia, common in adults, is frequent even among children with AFD. Given the potential for early initiation of enzyme replacement therapy to reduce cardiovascular morbidity, continued work to develop paradigms of therapy and longitudinal cardiovascular surveillance is warranted.

Utility of Echocardiography in the Assessment of Left Ventricular Diastolic Function and Restrictive Physiology in Children and Young Adults with Restrictive Cardiomyopathy: A Comparative Echocardiography-Catheterization Study.

The aim of the study is to determine the utility of echocardiography in the assessment of diastolic function in children and young adults with restrictive cardiomyopathy (RCM). RCM is a rare disease with high mortality requiring frequent surveillance. Accurate, noninvasive echocardiographic measures of diastolic function may reduce the need for invasive catheterization. Single-center, prospective, observational study of pediatric and young adult RCM patients undergoing assessment of diastolic parameters by simultaneous transthoracic echocardiogram (TTE) and invasive catheterization. Twenty-one studies in 15 subjects [median (IQR) = 13.8 years (7.0-19.2), 60% female] were acquired with median left ventricular end-diastolic pressure (LVEDP) 21 (IQR 18-25) mmHg. TTE parameters of diastolic function, including pulmonary vein A wave duration (r s  = 0.79) and indexed left atrial volume (r s  = 0.49), demonstrated significant positive correlation, while mitral valve A (r s  = -0.44), lateral e' (r s  = -0.61) and lateral a' (r s  = -0.61) velocities showed significant negative correlation with LVEDP. Lateral a' velocity (≤0.042 m/s) and pulmonary vein A wave duration (≥156 m/s) both had sensitivity and specificity ≥80% for LVEDP ≥ 20 mmHg. In pediatric and young adult patients with RCM, lateral a' velocity and pulmonary vein A wave duration predicted elevated LVEDP with high sensitivity and specificity; however, due to technical limitations the latter was reliably measured in 12/21 patients. These noninvasive parameters may have utility in identifying patients that require further assessment with invasive testing. These findings require validation in a multicenter prospective cohort prior to widespread clinical implementation.

Unexpectedly low left ventricular voltage on ECG in hypertrophic cardiomyopathy.

OBJECTIVE: While late gadolinium enhancement (LGE) in paediatric patients with hypertrophic cardiomyopathy (HCM) is reported as similar to adults, the relationship between LGE and ECG findings in paediatric patients is unknown. We sought to evaluate the relationship between LGE on cardiac MRI and LV precordial voltage on ECG. METHODS: This was a retrospective analysis of paediatric patients with HCM aged 9-21 years with cardiac MRI and ECG completed within 60 days of each other. Demographic, MRI and ECG data were compared between patients with and without LGE. Maximal diastolic septal thickness, septal to free wall ratio and LGE presence were compared with LV precordial voltage (SV1, RV6 and SV1+RV6). RESULTS: This study included 37 patients (33 male). Mean age was 15.8±2.8 years. Mean maximal LV diastolic septal thickness was 22.1±7.9 mm. Mean septal to free wall ratio was 2.4±1.6 mm. LGE was present in 18 patients, with 16 isolated to the ventricular septum. Comparing patients with and without LGE, there was no difference in age (p=0.2) or body surface area (p=0.9). However, the presence of LGE was associated with significantly increased septal thickness (p=0.03), yet decreased voltages in SV1 (p=0.005), RV6 (p=0.005) and SV1+RV6 (p=0.002) despite increased septal dimensions. CONCLUSIONS: A significant inverse relationship exists between LGE presence and LV precordial voltage in this population. Unexpectedly low LV precordial voltages in patients with HCM may serve as a clinical surrogate marker for myocardial fibrosis and potential loss of viable myocardial tissue.

The use of a Berlin Heart EXCOR LVAD in a child receiving chemotherapy for Castleman's disease.

We present the unique case of a pediatric patient who received chemotherapy for a diagnosis of CD, while mechanically supported with a Berlin EXCOR LVAD secondary to restrictive cardiomyopathy. A four-yr-old previously healthy male with restrictive cardiomyopathy required MCS after cardiac arrest but was diagnosed with multicentric CD, a non-malignant lymphoproliferative disorder fueled by excessive IL-6 production. Treatment with IL-6 blockade (tocilizumab) every two wk and methylprednisolone had no effect on his lymph nodes or cardiac function while on temporary RotaFlow. A Berlin LVAD was placed for treatment with rituximab, COP, vincristine, and methylprednisolone. After three courses of chemotherapy, his inflammatory markers normalized and his lymphadenopathy decreased but cardiac function remained severely depressed. He tolerated chemotherapy on the Berlin but required frequent titrations of his anti-coagulation regimen and he did suffer a hemorrhagic stroke. His clinical status improved significantly with rehabilitation, and he tolerated heart transplantation without further complications. MCS is a feasible option as a bridge to recovery or heart transplant eligibility for patients with hemodynamic collapse requiring chemotherapy but it does necessitate close titration of the anti-coagulation regimen to coincide with changes in the inflammatory state.

Comparison of area-length method by echocardiography versus full-volume quantification by cardiac magnetic resonance imaging for the assessment of left atrial volumes in children, adolescents, and young adults.

Left atrial (LA) size is a known predictor of adverse cardiovascular events. Echocardiography is the modality of choice for the evaluation of atrial size; however, cardiac magnetic resonance imaging (cMRI) remains the "gold standard." We sought to calculate atrial volumes using the area-length method by both echocardiography and cMRI and compare them with area-volume quantification by cMRI. Thiry-eight patients (mean age 20 ± 12 years, 71% male) who underwent cMRI and echocardiography between September 2010 and June 2012 were retrospectively identified. The time interval between the two studies was ≤ 6 months. LA volumes by echocardiogram were estimated using the area-length method: LA volume = (0.85 × area(4ch) × area(2ch))/(shortest atrial length). The atrial length and area were measured in standard apical two-chamber and four-chamber planes. Measured values were indexed to body surface area (BSA). CMRI measurements were obtained from prospectively gated steady-state free precession cine stack images obtained in a standard four-chamber plane. LA volumes were calculated using Simpson's method: LA volume = LA area × (slice thickness + gap) per slice. Slice thickness ranged from 5 to 7 mm with contiguous slices of 5 to 7 mm. The values were indexed to BSA. Statistics were summarized using measures of central tendency. LA volumes by echocardiography were significantly less than those by full-volume cMRI quantification. The mean LA volume by echocardiography and full-volume cMRI were 35 ± 14.5 and 42.4 ± 17.2, respectively (p = 0.05). The mean difference between LA volumes obtained by the two methods was 7.4 ± 10.6. LA volume measured by cMRI using the area-length method closely approximated full-volume assessment by cMRI with mean values of 42.9 ± 17.4 versus 42.4 ± 17.2, respectively (p = 0.91). There were no significant differences in the patient characteristics between the two study modalities. LA volumes as measured by echocardiography using the area-length method consistently underestimated the true volume when compared with cMRI. LA volumes measured using the area-length method by cMRI is an alternative technique for accurately quantifying chamber size and can be useful in decreasing scan time or when full-volume data sets are incomplete.

Predictors of exaggerated exercise-induced systolic blood pressures in young patients after coarctation repair.

BACKGROUND: In normotensive subjects, an exaggerated blood pressure response to exercise is associated with the development of resting hypertension. We sought to determine the prevalence of elevated blood pressures during exercise in post-operative coarctation patients with normal resting blood pressure, and investigate associations with exercise-induced hypertension in this population. METHODS: A total of 38 patients were enrolled after end-to-end anastomosis repair and resting normotension. All patients underwent anthropometric and blood pressure measurements, echocardiographic evaluation of function, arterial stiffness assessment by pulse wave velocity, and a graded exercise test. An abnormal response was defined as a maximum systolic blood pressure greater than the 95th percentile of published normal values. Correlation analyses and stepwise regression analyses were performed. RESULTS: The mean age was 12.7 years, including 79% male patients. The mean resting systolic blood pressure was 111.3 millimetres of mercury and the mean exercise systolic blood pressure was 178.1 millimetres of mercury. The prevalence of a systolic blood pressure greater than the 95th percentile was 16.7%. In multivariate analysis, the exercise systolic blood pressure index was associated with body mass index, age, aortic valve annulus, shortening fraction, and pulse wave velocity (R2 equal to 0.79, p equal to 0.0009). Estimates of ventricular filling and indexed left ventricular mass were elevated. CONCLUSIONS: There is a risk of elevated systolic blood pressure during exercise in normotensive patients after coarctation repair. Resting blood pressures are useful but not sufficient. Echocardiography demonstrated abnormalities suggestive of a chronic cardiac burden despite resting normotension. Regular imaging may be necessary to improve long-term outcomes. New paradigms for the continued follow-up of these patients are necessary.