Explore the vast range of titles available.

Use of correct nomenclature is important in all aspects of medicine. Many of the controversies that have bedeviled paediatric cardiology have devolved from the inappropriate use of words to describe the lesions to be found when the heart is congenitally abnormal. A continuing area of disagreement is the situation currently described by many as representing “heterotaxy”. When used literally, this word means any departure from the normal. Thus, all congenitally malformed hearts represent examples of heterotaxy. By convention, nonetheless, the term is used to describe the arrangement in which the bodily organs, including parts of the heart, are not in their usual or in their mirror-imaged patterns. The arrangements, therefore, represent the presence of the organs on the right and left sides of the body being mirror imaged, in other words isomeric; however, not all the organs are uniformly isomeric. In this review, we show that, when assessed on the basis of the morphology of the atrial appendages, specifically the extent of the pectinate muscles relative to the atrioventricular junctions, isomerism is an unequivocal finding within the heart. Only the atrial appendages, however, are truly isomeric. The potential problem of disharmony between the various systems of organs is resolved simply by accounting specifically for each of the systems. On these bases, we suggest that the isomeric arrangements can now readily be diagnosed in the clinical setting, and differentiated into their right and left isomeric variants. We propose that such distinctions will provide the key for establishing the genetic cues responsible for the formation of the isomeric as opposed to the lateralised arrangements.

The aim of the study is to describe the spectrum of indications for pediatric ECG-synchronized CT angiography (CTA), the main determinants of radiation exposure, and trends in radiation dose over time at a single, tertiary referral center. The study was IRB approved and HIPAA compliant with informed consent waived. Between 2005 and 2013, 324 pediatric patients underwent ECG-synchronized CTA to evaluate known or suspected cardiovascular abnormalities (109 female, median age 8.1 years). The effective dose (ED) was calculated using age-specific correction factors. Univariate and multivariate regression analyses were performed to identify predictors of radiation dose. The most common primary indications for the CTA examinations included known or suspected coronary pathologies (n = 166), complex congenital heart disease (n = 73), and aortic pathologies (n = 41). Median radiation exposure decreased from 12 mSv for patients examined in the years 2005–2007 to 1.2 mSv for patients examined in the years 2011–2013 (p < 0.001). Patients scanned using a tube potential of 80 kV (n = 259) had a significantly lower median radiation dose (1.4 mSv) compared to patients who were scanned at 100 kV (n = 46, median 6.3 mSv) or 120 kV (n = 19, median 19 mSv, p < 0.001). Tube voltage, followed by tube current and the method of ECG-synchronization were the strongest independent predictors of radiation dose. Growing experience with dose-saving techniques and CTA protocols tailored to the pediatric population have led to a tenfold reduction in radiation dose over recent years and now allow routinely performing ECG-synchronized CTA in children with a radiation dose on the order of 1 mSv.

The validity and reproducibility of echocardiographic methods used to quantify mitral regurgitation (MR) in children with congenital heart disease are unknown. We evaluated the usefulness of methods used to quantify MR in children enrolled in a multicenter trial of enalapril 6 months after surgical repair of an atrioventricular septal defect (AVSD). MR severity in this trial was assessed using body surface area (BSA)-adjusted vena contracta lateral (i-VCWlat) and anterior-posterior (i-VCWap) dimensions and cross-sectional area (i-VCA), regurgitant volume/BSA, regurgitant fraction, and qualitative MR grade. For each method, association with left ventricular end-diastolic volume (LVEDVz) and end-diastolic dimension (LVEDDz) z-scores and interobserver agreement were assessed. In 149 children (median age 1 year), i-VCWlat, i-VCWap, and i-VCA were best associated with LVEDVz (r2 = 0.54, r2 = 0.24, and r2 = 0.46, respectively; p < 0.001 for all) and showed the highest interobserver agreement (intraclass correlation coefficient = 0.62, 0.73, and 0.68, respectively). Qualitative MR grade was also associated with LVEDVz (r2 = 0.31, p < 0.001) and showed modest interobserver agreement (kappa 0.56). Regurgitant volume/BSA and regurgitant fraction were associated with LVEDVz (r2 = 0.45 and r2 = 0.45, p < 0.001 for both) but showed poor interobserver agreement [ICC = 0.28 (n = 91) and ICC = 0.17 (n = 76), respectively], and their values were negative in 75% of subjects. In conclusion, echocardiographic assessment of MR severity after AVSD remains challenging. Among the quantitative methods used in this trial, i-VCW and i-VCA performed the best but offered little advantage compared with qualitative MR grade. The utility of regurgitant volume and fraction was severely limited by poor interobserver agreement and frequently negative values.

Single ventricle hearts are congenital cardiovascular defects in which the heart has only one functional pumping chamber. The treatment for these conditions typically requires a three-staged operative process where Stage 1 is typically achieved by a shunt between the systemic and pulmonary arteries, and Stage 2 by connecting the superior venous return to the pulmonary circulation. Surgically, the Stage 2 circulation can be achieved through a procedure called the Hemi-Fontan, which reconstructs the right atrium and pulmonary artery to allow for an enlarged confluence with the superior vena cava. Based on pre-operative data obtained from two patients prior to Stage 2 surgery, we developed two patient-specific multi-scale computational models, each including the 3D geometrical model of the surgical junction constructed from magnetic resonance imaging, and a closed-loop systemic lumped-parameter network derived from clinical measurements. “Virtual” Hemi-Fontan surgery was performed on the 3D model with guidance from clinical surgeons, and a corresponding multi-scale simulation predicts the patient's post-operative hemodynamic and physiologic conditions. For each patient, a post-operative active scenario with an increase in the heart rate (HR) and a decrease in the pulmonary and systemic vascular resistance (PVR and SVR) was also performed. Results between the baseline and this “active” state were compared to evaluate the hemodynamic and physiologic implications of changing conditions. Simulation results revealed a characteristic swirling vortex in the Hemi-Fontan in both patients, with flow hugging the wall along the SVC to Hemi-Fontan confluence. One patient model had higher levels of swirling, recirculation, and flow stagnation. However, in both models, the power loss within the surgical junction was less than 13% of the total power loss in the pulmonary circulation, and less than 2% of the total ventricular power. This implies little impact of the surgical junction geometry on the SVC pressure, cardiac output, and other systemic parameters. In contrast, varying HR, PVR, and SVR led to significant changes in theses clinically relevant global parameters. Adopting a work-flow of customized virtual planning of the Hemi-Fontan procedure with patient-specific data, this study demonstrates the ability of multi-scale modeling to reproduce patient specific flow conditions under differing physiological states. Results demonstrate that the same operation performed in two different patients can lead to different hemodynamic characteristics, and that modeling can be used to uncover physiologic changes associated with different clinical conditions.

The efficacy of a specialized pediatric cardiac rapid response team is unknown. We hypothesized that a specialized cardiac rapid response team would facilitate team-wide communication between the cardiac stepdown unit and cardiac intensive care unit (ICU) teams and improve patient care. A specialized pediatric cardiac rapid response team was implemented in June 2015. All pediatric cardiac rapid response team activations and outcomes from implementation through December 2018 were reviewed. Cardiac arrests and unplanned transfers to the cardiac ICU were indexed to 1000 patient-days to account for inpatient volume trends and evaluated over time. There were 202 cardiac rapid response team activations in 108 unique patients during the study period. After implementation of the pediatric cardiac rapid response team, unplanned transfers from the cardiac stepdown unit to the cardiac ICU decreased from 16.8 to 7.1 transfers per 1000 patient days (p = 0.012). The stepdown unit cardiac arrest rate decreased from 1.2 to 0.0 arrests per 1000 patient-days (p = 0.015). There was one death on the cardiac stepdown unit in the 5 years since the implementation of the cardiac rapid response team, compared to four deaths in the previous 5 years. A reduction in unplanned cardiac ICU transfers, cardiac arrests, and mortality on the cardiac stepdown unit has been observed since the implementation of a specialized pediatric cardiac rapid response team. A specialized cardiac rapid response team may improve communication and empower the interdisciplinary care team to escalate care for patients experiencing clinical decline.

Computed tomographic angiography (CTA) and cardiac catheterization are useful adjuncts to echocardiography for delineating cardiovascular anatomy in pediatric patients. These studies require ionizing radiation, and it is paramount to understand the amount of radiation pediatric patients receive when these tests are performed. Modern dosimetry methods facilitate the conversion of radiation doses of varying units into an effective radiation dose. To compare the effective radiation dose between nongated CTA of the chest and diagnostic cardiac catheterization in pediatric patients. This is a retrospective cohort study of patients of patients who underwent either nongated CTA of the chest or diagnostic cardiac catheterization between July 2009 and April 2010. Fifty patients were included in each group as consecutive samples at a single tertiary care center. An effective radiation dose (mSv) was formulated using conversion factors for each group. The median effective dose (ED) for the CTA group was 0.74 mSv compared with 10.8 mSv for the catheterization group (p < 0.0001). The median ED for children <1 year of age in the CTA group was 0.76 mSv compared with 13.4 mSv for the catheterization group (p < 0.0001). Nongated CTA of the chest exposes children to 15 times less radiation than diagnostic cardiac catheterization. Unless hemodynamic data are necessary, CTA of the chest should be considered in lieu of diagnostic cardiac catheterization in patients with known or presumed cardiac disease who need additional imaging beyond echocardiography

ObjectivesTo evaluate a self-navigated free-breathing three-dimensional (SNFB3D) radial whole-heart MRA technique for assessment of main coronary arteries (CAs) and side branches in patients with congenital heart disease (CHD).MethodsSNFB3D-MRA datasets of 109 patients (20.1±11.8 years) were included. Three readers assessed the depiction of CA segments, diagnostic confidence in determining CA dominance, overall image quality and the ability to freeze cardiac and respiratory motion. Vessel sharpness was quantitatively measured.ResultsThe percentages of cases with excellent CA depiction were as follows (mean score): left main, 92.6 % (1.92); left anterior descending (LAD), 88.3 % (1.88); right (RCA), 87.8 % (1.85); left circumflex, 82.8 % (1.82); posterior descending, 50.2 % (1.50) and first diagonal, 39.8 % (1.39). High diagnostic confidence for the assessment of CA dominance was achieved in 56.2 % of MRA examinations (mean score, 1.56). Cardiac motion freezing (mean score, 2.18; Pearson’s r=0.73, P<0.029) affected image quality more than respiratory motion freezing (mean score, 2.20; r=0.58, P<0.029). Mean quantitative vessel sharpness of the internal thoracic artery, RCA and LAD were 53.1, 52.5 and 48.7 %, respectively.ConclusionsMost SNFB3D-MRA examinations allow for excellent depiction of the main CAs in young CHD patients; visualisation of side branches remains limited.Key Points• Self-navigated free-breathing three-dimensional magnetic resonance angiography (SNFB3D-MRA) sufficiently visualises coronary arteries (CAs).• Depiction of main CAs in patients with congenital heart disease is excellent.• Visualisation of CA side branches using SNFB3D-MRA is limited.• SNFB3D-MRA image quality is especially correlated to cardiac motion freezing ability.

Objectives To evaluate image quality (IQ) of low-radiation-dose paediatric cardiovascular CT angiography (CTA), comparing iterative reconstruction in image space (IRIS) and sinogram-affirmed iterative reconstruction (SAFIRE) with filtered back-projection (FBP) and estimate the potential for further dose reductions. Methods Forty neonates and children underwent low radiation CTA with or without ECG synchronisation. Data were reconstructed with FBP, IRIS and SAFIRE. For ECG-synchronised studies, half-dose image acquisitions were simulated. Signal noise was measured and IQ graded. Effective dose (ED) was estimated. Results Mean absolute and relative image noise with IRIS and full-dose SAFIRE was lower than with FBP ( P  < 0.001), while SNR and CNR were higher ( P  < 0.001). Image noise was also lower and SNR and CNR higher in half-dose SAFIRE studies compared with full-and half-dose FBP studies ( P  < 0.001). IQ scores were higher for IRIS, full-dose SAFIRE and half-dose SAFIRE than for full-dose FBP and higher for half-dose SAFIRE than for half-dose FBP ( P  < 0.05). Median weight-specific ED was 0.3 mSv without and 1.36 mSv with ECG synchronisation. The estimated ED of half-dose SAFIRE studies was 0.68 mSv. Conclusions IR improves image noise, SNR, CNR and subjective IQ compared with FBP in low-radiation-dose paediatric CTA and allows further dose reductions without compromising diagnostic IQ. Key Points • Iterative reconstruction techniques significantly improve non-invasive cardiovascular CT in children . • Using half traditional radiation dose image quality is higher with iterative reconstruction . • Iterative reconstruction techniques may allow further radiation reductions in paediatric cardiovascular CT .

Many, if not most, of the controversies regarding the description of the congenitally malformed heart have been resolved over the turn of the 20th century. A group of lesions that remains contentious is the situation in which both arterial trunks, in their greater part, are supported by the morphologically right ventricle. It was considered, for many years, that presence of bilateral infundibulums, or conuses, was a necessity for such a diagnosis. It has now been appreciated that this suggestion founders on many counts. In the first instance, such bilateral infundibulums are to be found in patients with other ventriculo-arterial connections, including the otherwise normal heart. In the second instance, it is clear that such an approach abrogates the important principle now known as the morphological method. This states that entities should be defined in terms of their intrinsic morphology and not on the basis of other variable features. It is now also clear that, when assessed simply on the basis of the ventricular origin of the arterial trunks, a significant number of patients fulfil the criteria for so-called “200%” origin of the trunks from the right ventricle when there is fibrous continuity between the leaflets of the atrioventricular and arterial valves. In this review, we show how attention to the morphology of the channel between the ventricles now provides the key to accurately diagnose the ventriculo-arterial connection in patients with suspected double-outlet right ventricle. This is because, when both arterial trunks arise exclusively or predominantly from the morphologically right ventricle, the outlet septum, of necessity, is itself a right ventricular structure. The channel between the ventricles, therefore, is roofed by the inner heart curvature, whether that structure is fibrous or muscular. Our observations then confirm that it is the attachment of the outlet septum, which itself can be muscular or fibrous, which determines the commitment of the interventricular communication to the subarterial outlets. The interventricular communication itself, when directly committed to the ventricular outlets, opens between the limbs of the septomarginal trabeculation or septal band. The defect is subaortic when the outlet septum is attached to the cranial limb of the trabeculation, subpulmonary when attached to the caudal limb, and doubly committed when attached to the inner heart curvature in the roof of the defect. Non-committed defects are no longer positioned within the limbs of the septomarginal trabeculation. Although readily demonstrable by a skilled echocardiographer, we show how these anatomical features are more easily demonstrated with added accuracy when using CT data sets.

We utilized the multicenter Pediatric Acute Care Cardiology Collaborative (PAC3) 2017 and 2019 surveys to describe practice variation in therapy availability and changes over a 2-year period. A high acuity therapies (ATs) score was derived (1 point per positive response) from 44 survey questions and scores were compared to center surgical volume. Of 31 centers that completed the 2017 survey, 26 also completed the 2019 survey. Scores ranged from 11 to 34 in 2017 and 11 to 35 in 2019. AT scores in 2019 were not statistically different from 2017 scores (29/44, IQR 27–32.5 vs. 29.5/44, IQR 27–31, p = 0.9). In 2019, more centers reported initiation of continuous positive airway pressure (CPAP) and Bi-level positive airway pressure (BiPAP) in Acute Care Cardiology Unit (ACCU) (19/26 vs. 4/26, p < 0.001) and permitting continuous CPAP/BiPAP (22/26 vs. 14/26, p = 0.034) compared to 2017. Scores in both survey years were significantly higher in the highest surgical volume group compared to the lowest, 33 ± 1.5 versus 25 ± 8.5, p = 0.046 and 32 ± 1.7 versus 23 ± 5.5, p = 0.009, respectively. Variation in therapy within the ACCUs participating in PAC3 presents an opportunity for shared learning across the collaborative. Experience with PAC3 was associated with increasing available respiratory therapies from 2017 to 2019. Whether AT scores impact the quality and outcomes of pediatric acute cardiac care will be the subject of further investigation using a comprehensive registry launched in early 2019.