Prenatal Diagnosis of a Ductal-Dependent Branch Pulmonary Artery: Extra Vessels in the 3-Vessel and Trachea View.

Obtaining a three-vessel (3V) and three-vessel and trachea (3VT) view from the fetal upper mediastinum is now considered to be part of standard imaging protocol for routine obstetric cardiac screening examinations. We report two fetal cases of an anomalous pulmonary artery origin, utilizing the standard 3V and 3VT views. Further imaging led to a rare diagnosis of bilateral ductus arteriosus with discontinuous branch pulmonary arteries in the absence of any other congenital heart defect. We briefly discuss the imaging features, differential diagnoses, and management of this rare entity.

Relationship Between Obesity and Global Longitudinal Strain in the Pediatric Single Ventricle Fontan Population Across Ventricular Morphologies.

BACKGROUND: Obesity is associated with diminished myocardial function as measured by strain echocardiography in children and young adults with normal cardiac anatomy. Data are lacking about the effect of obesity on myocardial strain in patients with a single ventricle. In this study, the relationship between body mass index (BMI) and single ventricle myocardial strain in the Fontan population was assessed. METHODS AND RESULTS: Thirty-eight abnormal BMI Fontan cases (21 overweight and 17 obese) and 30 normal BMI Fontan controls matched based on single ventricular morphology, age, and sex were included in the study. Ventricular morphology was categorized as single right ventricle, single left ventricle, or biventricular. Single ventricle global longitudinal peak systolic strain (GLS) and other echocardiographic measurements were performed and compared between groups, with a P≤0.05 defined as significant. The abnormal BMI group demonstrated diminished GLS (-15.7±3.6% versus -17.2±3.2%, [P=0.03]) and elevated systolic blood pressure (P=0.04) compared with the normal BMI group. On subgroup analysis of those with single right ventricle morphology, the abnormal BMI group demonstrated diminished GLS compared with controls. There was no significant difference in GLS between the abnormal BMI and control groups in the single left ventricle and biventricular subgroups. Analyzed by ventricular morphology, no other variables were statistically different in the abnormal BMI group including systolic blood pressure. Inter-reader reproducibility for GLS and strain rate were excellent for both measures. CONCLUSIONS: Obesity has an adverse relationship with myocardial strain in the young Fontan population, with the most maladaptive response seen in the single right ventricle.

Echocardiographic findings in non-hospitalised children and adolescents following acute COVID-19.

BACKGROUND: Although COVID-19 is known to have cardiac effects in children, seen primarily in severe disease, more information is needed about the cardiac effects following COVID-19 in non-hospitalised children and adolescents during recovery. This study aims to compare echocardiographic markers of cardiac size and function of children following acute COVID-19 with those of healthy controls. METHODS: This single-centre retrospective case-control study compared 71 cases seen in cardiology clinic following acute COVID-19 with 33 healthy controls. Apical left ventricle, apical right ventricle, and parasternal short axis at the level of the papillary muscles were analysed to measure ventricular size and systolic function. Strain was analysed on vendor-independent software. Statistical analysis was performed using t-test, chi-square, Wilcoxon rank sum, and regression modelling as appropriate (p < 0.05 significant). RESULTS: Compared to controls, COVID-19 cases had slightly higher left ventricular volumes and lower left ventricular ejection fraction and right ventricular fractional area change that remained within normal range. There were no differences in right or left ventricular longitudinal strain between the two groups. Neither initial severity nor persistence of symptoms after diagnosis predicted these differences. CONCLUSIONS: Echocardiographic findings in children and adolescents 6 weeks to 3 months following acute COVID-19 not requiring hospitalisation were overall reassuring. Compared to healthy controls, the COVID-19 group demonstrated mildly larger left ventricular size and lower conventional measures of biventricular systolic function that remained within the normal range, with no differences in biventricular longitudinal strain. Future studies focusing on longitudinal echocardiographic assessment of patients following acute COVID-19 are needed to better understand these subtle differences in ventricular size and function.

Utility of Fetal Echocardiography with Acute Maternal Hyperoxygenation Testing in Assessment of Complex Congenital Heart Defects.

Fetal echocardiography is an excellent tool for accurately assessing the anatomy and physiology of most congenital heart defects (CHDs). Knowledge gathered from a thorough initial fetal echocardiogram and serial assessment assists with appropriate perinatal care planning, resulting in improved postnatal outcomes. However, fetal echocardiography alone provides limited information about the status of the pulmonary vasculature, which can be abnormal in certain complex CHDs with obstructed pulmonary venous flow (hypoplastic left heart syndrome with restrictive atrial septum) or excessive pulmonary artery flow (d-transposition of the great arteries, usually with a restrictive ductus arteriosus). Fetuses with these CHDs are at high risk of developing severe hemodynamic instability with the immediate transition from prenatal to postnatal circulatory physiology at the time of birth. Adjunctive use of acute maternal hyperoxygenation (MH) testing in such cases can help determine pulmonary vascular reactivity in prenatal life and better predict the likelihood of postnatal compromise and the need for emergent intervention. This comprehensive review discusses the findings of studies describing acute MH testing in a diverse spectrum of CHDs and congenital diagnoses with pulmonary hypoplasia. We review historical perspectives, safety profile, commonly used clinical protocols, limitations, and future directions of acute MH testing. We also provide practical tips on setting up MH testing in a fetal echocardiography laboratory.

Comprehensive evaluation of left ventricular deformation using speckle tracking echocardiography in normal children: comparison of three-dimensional and two-dimensional approaches.

BACKGROUND: Three-dimensional (3D) speckle tracking echocardiography (STE) can overcome some of the inherent limitations of two-dimensional (2D) STE; however, clinical experience is lacking. We aimed to assess and compare the feasibility, agreement, and reproducibility of left ventricular (LV) global longitudinal (GLS), and regional strain by 3D vs 2D STE in normal children. METHODS: Healthy pediatric subjects (n = 105, age mean = 11.2 ± 5.5 years) were prospectively enrolled. Three-dimensional and 2D LV GLS, as well as regional strain in 16 myocardial segments were quantified. Bland Altman analysis, intra- class correlation coefficients (ICC), percent error and linear regression were used for agreement and correlation between the two techniques. Analysis and acquisition times were compared. Inter- and intra-observer reproducibility was assessed in 20 studies. RESULTS: There was good to excellent agreement for 2D and 3D global longitudinal strain (ICC =0.82) and modest agreement for regional strain (ICC range 0.43-0.71). Both methods had high feasibility (88.6% for 2D vs 85.7% for 3D, p = 0.21), although 3D STE required significantly shorter acquisition and analysis time than 2D STE (acquisition time 1 ± 1.2 mins vs 2.4 ± 1 mins; p = 0.03, analysis time = 3.3 ± 1 mins vs 8.2 ± 2.5 mins; p = 0.001, respectively). Inter and intra-observer reproducibility was excellent for GLS by the two techniques (ICC = 0.78-0.93) but moderate to poor for regional strain (ICC = 0.21-0.64). CONCLUSION: Three-dimensional global LV strain is as feasible and reproducible as 2D strain, with good agreement yet significantly more efficient acquisition and analysis. Regional strain is less concordant and 2D and 3D values should not be used interchangeably. 3D LV GLS may represent a viable alternative in evaluation of LV deformation in pediatric subjects.

The Apical View Sign for Easier Identification of Left Juxtaposition of the Right Atrial Appendage.

Left juxtaposition of the right atrial appendage (RAA) is a rare congenital cardiac malformation, where both atrial appendages are located leftward of the great arteries. Despite well-described echocardiographic markers, this diagnosis remains challenging to establish, especially for the novice imager. We describe the apical view sign in three patients with juxtaposed RAA. We noted that the normal left atrial appendage was visualized from the apical four-chamber or equivalent coronal view at the level of the atrioventricular valves.

Transseptal puncture using surgical electrocautery in children and adults with and without complex congenital heart disease.

BACKGROUND: Atrial transseptal puncture (TSP) for cardiac catheterization procedures remain challenging in children and adults with complex congenital heart disease (CHD). OBJECTIVES: We sought to evaluate our experience using radiofrequency (RF) current via surgical electrocautery needle for TSP to facilitate diagnostic and interventional procedures. METHODS: Retrospective chart review of all patients (pts) who underwent TSP using RF energy (10-25 W) via surgical electrocautery from three centers from January 2011 to January 2017 were evaluated. Echocardiograms were reviewed to define the atrial septum as normal and complex (thin aneurysmal, thick/fibrotic, synthetic patch material, and extra cardiac conduit). RESULTS: A total of 54 pts underwent 55 successful TSP. Median age was 12.5 years (1 day-54 years) and weight was 52.7 kg (2-162). Indications for TSP included; EP study and ablation procedures in structurally normal hearts (n = 24) and in complex atrial septum/CHD and structural heart disease pts (n = 30): Electrophysiology study and ablation in 4, diagnostic catheterization in 9, and interventional procedures in 17 pts were performed. Atrial TSP was successful in 54/55 (98%). Atrial perforation with tiny-small pericardial effusion not requiring intervention was noted in 2 pts. TSP was unsuccessful in one critically ill neonate with unobstructed TAPVR and restricted atrial septum who experienced cardiac arrest requiring CPR, ECMO, and emergent surgery. CONCLUSIONS: RF current delivery using surgical electrocautery for TSP is a feasible and an effective option in patients with complex CHD for diagnostic, interventional, and electrophysiology procedures.

Echocardiographic Diagnosis of Left Main Coronary Artery Atresia.

This case describes an infantile presentation of left main coronary artery atresia (LMCAA), which involves complete absence of the left coronary ostium and left main coronary artery. The echocardiographic features of LMCAA that assist in making this diagnosis are detailed. Important imaging features that distinguish LMCAA from anomalous left coronary artery from the pulmonary artery are highlighted.

Is Interarterial Course a Requisite for Ischemia in Anomalous Origin of Coronary Arteries?

Anomalous origin of the left main coronary artery from the noncoronary sinus (LCANS) is a rare variant of anomalous aortic origin of a coronary artery that is not characterized by an interarterial course. Despite the absence of an interarterial course, there are rare reports of LCANS presenting with sudden death, life-threatening arrhythmia, exercise-induced myocardial ischemia, premature ventricular contractions, and syncope. We report a patient with LCANS presenting with acute myocardial infarction who recovered completely with emergent surgical unroofing of the coronary artery.

Moving towards universal prenatal detection of critical congenital heart disease in southern Nevada: a community-wide program.

This study compares the current, prenatal detection rate for critical congenital heart disease in Southern Nevada with the previously reported rate, after developing and expanding a comprehensive, community-wide fetal cardiology program. For the current-period analysis, we inquired our database and electronic health records for patients born in Clark County, Nevada, with critical congenital heart disease between May 2012 and April 2014, and we compared the results with the previous period between May 2003 and April 2006. The major components of the community-wide program include fetal congenital heart disease screening via general obstetric ultrasound studies performed in obstetrician's offices, radiology imaging centers, or maternal-fetal medicine specialty practices; subsequent referral for comprehensive fetal echocardiography performed in maternal-fetal medicine offices under the on-site supervision by fetal cardiologists; and recurring community educational programs teaching the 5-axial plane, fetal echocardiographic screening protocol to general obstetric sonographers and instructing perinatal sonographers in advanced imaging topics. For the current period, the prenatal detection rate for critical congenital heart disease in Southern Nevada was 71 versus 36% for the previous period (p < 0.001). The temporal improvement in prenatal detection of critical congenital heart disease may be related to our expanded decentralized, community-wide fetal cardiology program, and our experiences may be applicable to other metropolitan areas.

Comparison between proximal thoracic vascular measurements obtained by contrast-enhanced magnetic resonance angiography and by transthoracic echocardiography in infants and children with congenital heart disease.

Accurate assessment of the proximal thoracic vasculature in infants and children with congenital heart disease (CHD) is vital for deciding the appropriate surgical or interventional procedure and predicting outcomes. This information usually is obtained by transthoracic echocardiography (TTE). Contrast-enhanced magnetic resonance angiography (CE-MRA) frequently is used to obtain diagnostic data when the image quality by TTE is limited. Calculation of z-scores for measurements obtained by CE-MRA in this population currently is not possible due to the lack of normative data. A reasonable agreement between vessel dimensions by CE-MRA and TTE will allow the use of TTE-based z-scores on measurements from CE-MRA. This study examines the accuracy and agreement of proximal thoracic vascular measurements obtained by CE-MRA versus TTE. Infants and children younger than 3 years with CHD who had a CE-MRA between August 2006 and May 2011 were retrospectively identified. Main and branch pulmonary arteries, ascending aorta, distal transverse arch, and aortic isthmus were measured from CE-MRA and TTE in analogous imaging planes and locations by two investigators blinded to each other. The study enrolled 35 subjects with CHD. The median age was 129 days (range, 0-1077 days), and the median weight was 5.8 kg (range, 2.16-17 kg). The median interval between the two imaging methods was 9 days (range, 0-60 days). Data analysis was performed with 129 of the 210 possible paired measurements. The remaining 81 paired measurements could not be performed due to inaccurate visualization of vessel borders or an unavailable imaging plane from TTE, CE-MRA, or both. The range of vessel sizes measured from 2.8 to 23.4 mm. There was excellent correlation between CE-MRA and TTE (r = 0.94, p < 0.001). The mean difference between the measurements was -0.1 ± 1.2 mm, and the limits of agreement were -2.5 to 2.3 mm. Proximal thoracic vascular measurements obtained by CE-MRA and TTE in infants and children with CHD have a strong correlation. The agreement between these two imaging methods is adequate. Until normative data for vessel size measurements obtained from CE-MRA are available for this population, TTE-based z-scores can be applied to the measurements obtained by CE-MRA.

Evaluation of cardiac manifestations in pediatric liver transplant candidates.

Knowledge concerning the involvement of the cardiovascular system in children awaiting liver transplant is limited. Therefore, no guidelines have been established on evaluating this group of patients for cardiac disease. This review examines the diverse cardiovascular manifestations of liver disease in children. We also discuss the available testing and its applicability in screening for cardiac disease in this vulnerable population.

Transcatheter repair of Fontan baffle stenosis and fenestration in two patients using Cook Zenith aortic endografts.

Patients with Fontan baffles for single ventricle may have cyanosis from right-to-left shunt through leaks in the baffle or due to intentionally created fenestrations. Typically this right-to-left shunt may be addressed with catheter-based occlusion devices. However, in narrowing of the Fontan baffle, placement of occluders within the Fontan baffle may additionally narrow the pathway and is therefore undesirable. We describe 2 patients with the combination of Fontan baffle stenosis and patent fenestration treated with a Zenith abdominal aortic aneurysm endograft (Cook Medical). The covered stent graft both occluded the right-to-left shunt and eliminated the baffle stenosis. Both patients have had symptomatic improvement.

Peak circulatory power as an indicator of clinical status in children after Fontan procedure.

Peak circulatory power (CircP), a product of peak exercise oxygen uptake (VO(2)) and peak mean or systolic arterial blood pressure, has proved to be a strong predictor of poor outcome in adults with congenital heart disease. This study sought to compare CircP with other cardiopulmonary exercise (CPX) test variables and to assess whether CircP is superior in categorizing patients into well-functioning vs. poorly functioning at-risk groups in the pediatric population after a Fontan procedure. The CPX test reports of 50 patients were retrospectively reviewed after the Fontan procedure. The patients were divided into two groups. The well-functioning group included patients in New York Heart Association (NYHA) classes 1 and 2 (n = 36). The poorly functioning at-risk group included patients in NYHA classes 3 and 4 and those with significant indicators or outcomes of a poor prognosis (n = 14). The patients in the well-functioning group had significantly higher CircP values based on mean blood pressure (MBP) (P < 0.001), higher CircP values based on systolic blood pressure (SBP) (P < 0.001), and higher peak VO(2) (P = 0.004) than those in the poorly functioning at-risk group. At a cutoff value less than 2100.4 mmHg/mlO(2)/kg/min, CircP MBP had a sensitivity of 85% in categorizing children to the poorly functioning at-risk group. CircP correlated well with the clinical status of our patients. CircP and peak VO(2) did not differ significantly in ability to identify poorly functioning patients. Further prospective analysis is needed to assess whether CircP can serve as a prognostic marker for the pediatric population after Fontan procedure.