Coarctation of the Aorta and Anomalous Origin of the Left Coronary from the Opposite Sinus of Valsalva in a 6-Year-Old Male.

Coarctation of the aorta is a form of congenital heart disease requiring surgical intervention. If is often associated with other forms of congenital heart disease. Anomalous origin of the left coronary artery (ALCA) from the opposite sinus of Valsalva is implicated in sudden cardiac death. We report the first case in the literature of coarctation of the aorta associated with ALCA from the opposite aortic sinus and describe surgical correction of these two lesions.

Incidence of Multisystem Inflammatory Syndrome in Children Among US Persons Infected With SARS-CoV-2.

Importance: Multisystem inflammatory syndrome in children (MIS-C) is associated with recent or current SARS-CoV-2 infection. Information on MIS-C incidence is limited. Objective: To estimate population-based MIS-C incidence per 1 000 000 person-months and to estimate MIS-C incidence per 1 000 000 SARS-CoV-2 infections in persons younger than 21 years. Design, Setting, and Participants: This cohort study used enhanced surveillance data to identify persons with MIS-C during April to June 2020, in 7 jurisdictions reporting to both the Centers for Disease Control and Prevention national surveillance and to Overcoming COVID-19, a multicenter MIS-C study. Denominators for population-based estimates were derived from census estimates; denominators for incidence per 1 000 000 SARS-CoV-2 infections were estimated by applying published age- and month-specific multipliers accounting for underdetection of reported COVID-19 case counts. Jurisdictions included Connecticut, Georgia, Massachusetts, Michigan, New Jersey, New York (excluding New York City), and Pennsylvania. Data analyses were conducted from August to December 2020. Exposures: Race/ethnicity, sex, and age group (ie, ≤5, 6-10, 11-15, and 16-20 years). Main Outcomes and Measures: Overall and stratum-specific adjusted estimated MIS-C incidence per 1 000 000 person-months and per 1 000 000 SARS-CoV-2 infections. Results: In the 7 jurisdictions examined, 248 persons with MIS-C were reported (median [interquartile range] age, 8 [4-13] years; 133 [53.6%] male; 96 persons [38.7%] were Hispanic or Latino; 75 persons [30.2%] were Black). The incidence of MIS-C per 1 000 000 person-months was 5.1 (95% CI, 4.5-5.8) persons. Compared with White persons, incidence per 1 000 000 person-months was higher among Black persons (adjusted incidence rate ratio [aIRR], 9.26 [95% CI, 6.15-13.93]), Hispanic or Latino persons (aIRR, 8.92 [95% CI, 6.00-13.26]), and Asian or Pacific Islander (aIRR, 2.94 [95% CI, 1.49-5.82]) persons. MIS-C incidence per 1 000 000 SARS-CoV-2 infections was 316 (95% CI, 278-357) persons and was higher among Black (aIRR, 5.62 [95% CI, 3.68-8.60]), Hispanic or Latino (aIRR, 4.26 [95% CI, 2.85-6.38]), and Asian or Pacific Islander persons (aIRR, 2.88 [95% CI, 1.42-5.83]) compared with White persons. For both analyses, incidence was highest among children aged 5 years or younger (4.9 [95% CI, 3.7-6.6] children per 1 000 000 person-months) and children aged 6 to 10 years (6.3 [95% CI, 4.8-8.3] children per 1 000 000 person-months). Conclusions and Relevance: In this cohort study, MIS-C was a rare complication associated with SARS-CoV-2 infection. Estimates for population-based incidence and incidence among persons with infection were higher among Black, Hispanic or Latino, and Asian or Pacific Islander persons. Further study is needed to understand variability by race/ethnicity and age group.

Multisystem Inflammatory Syndrome in U.S. Children and Adolescents.

BACKGROUND: Understanding the epidemiology and clinical course of multisystem inflammatory syndrome in children (MIS-C) and its temporal association with coronavirus disease 2019 (Covid-19) is important, given the clinical and public health implications of the syndrome. METHODS: We conducted targeted surveillance for MIS-C from March 15 to May 20, 2020, in pediatric health centers across the United States. The case definition included six criteria: serious illness leading to hospitalization, an age of less than 21 years, fever that lasted for at least 24 hours, laboratory evidence of inflammation, multisystem organ involvement, and evidence of infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) based on reverse-transcriptase polymerase chain reaction (RT-PCR), antibody testing, or exposure to persons with Covid-19 in the past month. Clinicians abstracted the data onto standardized forms. RESULTS: We report on 186 patients with MIS-C in 26 states. The median age was 8.3 years, 115 patients (62%) were male, 135 (73%) had previously been healthy, 131 (70%) were positive for SARS-CoV-2 by RT-PCR or antibody testing, and 164 (88%) were hospitalized after April 16, 2020. Organ-system involvement included the gastrointestinal system in 171 patients (92%), cardiovascular in 149 (80%), hematologic in 142 (76%), mucocutaneous in 137 (74%), and respiratory in 131 (70%). The median duration of hospitalization was 7 days (interquartile range, 4 to 10); 148 patients (80%) received intensive care, 37 (20%) received mechanical ventilation, 90 (48%) received vasoactive support, and 4 (2%) died. Coronary-artery aneurysms (z scores ≥2.5) were documented in 15 patients (8%), and Kawasaki's disease-like features were documented in 74 (40%). Most patients (171 [92%]) had elevations in at least four biomarkers indicating inflammation. The use of immunomodulating therapies was common: intravenous immune globulin was used in 144 (77%), glucocorticoids in 91 (49%), and interleukin-6 or 1RA inhibitors in 38 (20%). CONCLUSIONS: Multisystem inflammatory syndrome in children associated with SARS-CoV-2 led to serious and life-threatening illness in previously healthy children and adolescents. (Funded by the Centers for Disease Control and Prevention.).

Imaging of congenital coronary artery anomalies.

Imaging of the coronary arteries is an important part of the evaluation of children with congenital heart disease and isolated congenital coronary artery anomalies. Echocardiography remains the main imaging modality and is complemented by MRI and CT angiography in the older or difficult-to-image child. We review echocardiography, MRI, and CT angiography for coronary artery imaging, with emphasis on techniques. The clinical implications of isolated congenital coronary artery anomalies are also addressed, along with a discussion about the current consensus on optimal management of these anomalies.

Outcomes of mitral regurgitation associated with large ventricular septal defect and a normal mitral valve apparatus: does intact atrial septum have an impact?

The hemodynamics of isolated large ventricular septal defect with an intact atrial septum results in greater mitral valve (MV) annular dilation and an increased incidence of mitral regurgitation (MR). MR will resolve in most patients after surgical VSD closure alone without MV repair.

Repeatability of cardiac-MRI-measured right ventricular size and function in congenital heart disease.

BACKGROUND: The measurement error for right ventricular (RV) size and function assessed by cardiac MRI (CMRI) in congenital heart disease has not been fully characterized. As CMRI parameters are being increasingly utilized to make clinical decisions, defining error in the clinical setting is critical. OBJECTIVE: This investigation examines the repeatability of CMRI for RV size and function. MATERIALS AND METHODS: Forty consecutive people with congenital heart disease involving the RV were retrospectively identified. Contouring of RV volumes was performed by two expert CMRI clinicians. The coefficient of variability and repeatability coefficients were calculated. Repeatability coefficients were multiplied by the mean value for each group studied to define a threshold beyond which measurement error was unlikely to be responsible. RESULTS: The variability for indexed RV end-diastolic volume = 3.2% and 3.3% for intra- and interobserver comparisons, respectively. The repeatability coefficients were 13.2% and 14.9% for intra- and interobserver comparisons, which yielded threshold values of 15.1 ml/m^2 and 20.2 ml/m^2, respectively. For RV ejection fraction (EF), the repeatability coefficients for intra- and interobserver comparisons were 5.0% and 6.0%, which resulted in threshold values of 2.6 EF% and 3.0 EF%. CONCLUSION: The threshold values generated can be used during serial assessment of RV size and function.

Normal values for left ventricular volume in infants and young children by the echocardiographic subxiphoid five-sixth area by length (bullet) method.

BACKGROUND: Left ventricular (LV) end-diastolic volume (LVEDV) can be estimated by the formula (5/6) x area x length, or the "bullet" method. The aim of this study was to determine the range of normal LVEDV values in infants and young children (aged 0-3 years) by the subxiphoid bullet method. METHODS: Echocardiograms from 100 normal subjects aged ≤ 3 years were retrospectively analyzed. Subjects with systemic disease, abnormal body size, cardiovascular disease, or nondiagnostic subxiphoid images were excluded. Measurements of LV short-axis cross-sectional diastolic area at the midventricular level and LV length were made offline from subxiphoid images. LVEDV was indexed to body surface area (BSA) to the powers of 1.0 and 1.38. Relationships between indexing methods, age, and gender were explored. RESULTS: The median age was 0.98 years (range, 0-2.9 years), the median weight was 9.5 kg (range, 3.1-16.0 kg), and the median BSA was 0.45 m(2) (range, 0.21-0.66 m(2)). The mean LVEDV/BSA(1.38) was 70.4 ± 9.1 mL/m(2.6), with an excellent correlation between LVEDV and BSA(1.38) (r = 0.96, P < .01). There was no residual relationship between LVEDV/BSA(1.38) and BSA (r = 0.06, P = NS) and no significant relationship between LVEDV/BSA(1.38) and age (r = 0.10, P = NS) or LVEDV/BSA(1.38) and gender. CONCLUSIONS: The normal range for LVEDV by the subxiphoid echocardiographic bullet method is reported for newborns, infants, and young children. LVEDV should be indexed to BSA(1.38), which is consistent with the known relationship between LV size and body size. In children aged ≤ 3 years, these data can be used to calculate Z scores for LVEDV by the subxiphoid bullet technique independent of age or gender.

Incessant pericardial effusion in a 9 year old male responding to infliximab.

Recurrent idiopathic pericardial effusion can be a challenging medical problem. Multiple medical interventions may yield minimal improvement. We describe a patient with an incessant pericardial effusion that responded to infliximab. The use of infliximab should be considered in the management of recurrent pericardial effusions resistant to other treatment modalities.

Enteroviral sepsis and ischemic cardiomyopathy in a neonate: case report and review of literature.

Neonatal entero-viral sepsis is a rare but fulminant infection with multisystem involvement, often presenting with hepatitis, meningo-encephalitis, disseminated intravascular coagulation (DIC), and myocarditis. Neonatal myocarditis often proves fatal. We report here a case of neonatal enteroviral myocarditis with multisystem organ failure and ischemic cardiomyopathy that was managed medically.

Stent dilatation of a right ventricle to pulmonary artery conduit in a postoperative patient with hypoplastic left heart syndrome.

A 10-day-old child with hypoplastic left heart syndrome (HLHS) underwent first-stage palliation for HLHS, Norwood procedure with a Sano modification, i.e., placement of a right ventricular to pulmonary artery (RV-PA) conduit. The patient developed progressively worsening systemic oxygen desaturation in the immediate postoperative period. Stenosis of the proximal RV-PA conduit was diagnosed by echocardiography. In the catheterization laboratory stent placement in the conduit was performed. This resulted in increased systemic oxygen saturation. The patient was eventually discharged from the hospital with adequate oxygen saturations.

N-terminal B-type natriuretic peptide levels in pediatric patients with congestive heart failure undergoing cardiac surgery.

OBJECTIVES: The objectives of this study were to measure circulating N-terminal B-type natriuretic peptide levels in pediatric patients undergoing surgical repair of congenital heart lesions with left ventricular volume overload and to determine whether presurgical and immediate postoperative N-terminal B-type natriuretic peptide levels could predict patient outcomes after surgical intervention. METHODS: Thirty-eight children aged 1 to 36 months undergoing surgical repair of cardiac lesions with left ventricular volume overload were studied. Plasma N-terminal B-type natriuretic peptide levels were measured preoperatively and at 2, 12, 24, 48, and 72 hours after surgical intervention and were assessed for their predictive value of postoperative outcomes. Plasma N-terminal B-type natriuretic peptide levels were also measured in 34 similarly aged healthy children. RESULTS: Patient preoperative N-terminal B-type natriuretic peptide levels were significantly higher than those of healthy control subjects (3085 +/- 4046 vs 105 +/- 78 pg/mL). Preoperative N-terminal B-type natriuretic peptide levels correlated with the complexity of surgical repair, as measured by cardiopulmonary bypass time (r = 0.529, P < .001), and with postoperative measures, including fractional inhaled oxygen requirements registered at 12 hours (r = 0.443, P = .005) and duration of mechanical ventilation (r = 0.445, P = .005). Plasma N-terminal B-type natriuretic peptide levels increased 5-fold within 12 hours after cardiopulmonary bypass (14,685 +/- 14,317 pg/mL). Multivariable regression analysis showed that the preoperative N-terminal B-type natriuretic peptide level was a significant predictor of duration of intensive care unit stay (P = .02) and that the peak postoperative N-terminal B-type natriuretic peptide level was a significant predictor of the intensity of overall medical management, as assessed by using the therapeutic intervention scoring system (P = .01). CONCLUSION: Plasma N-terminal B-type natriuretic peptide levels measured preoperatively and postoperatively can be a prognostic indicator in the management of the pediatric patient after surgical intervention for congenital heart repair.

Congenital infantile hepatic hemangioendothelioma type II treated with orthotopic liver transplantation.

The authors describe a 10-week-old girl with infantile hepatic hemangioendothelioma who initially presented with difficulty feeding, hepatomegaly, and multiple hemangiomas of the skin. Six weeks of steroid therapy and 2 weeks of chemotherapy failed to produce clinical improvement. The patient underwent split liver transplantation. A definitive diagnosis of hemangioendothelioma type II was made. Imaging studies cannot differentiate between hemangioendothelioma and angiosarcoma. Treatment modalities for this condition remain unclear. The patient continues to do well.

Echocardiographic analysis of ventricular geometry and function during repair of congenital septal defects.

BACKGROUND: This study investigated changes in left ventricular (LV) geometry and systolic function after corrective surgery for atrial (ASD) and ventricular septal defects (VSD). METHODS: Transesophageal LV short-axis echocardiograms were recorded before and after operative repair of ASD (n = 11) and VSD (n = 7). Preload was measured using LV end-diastolic area indexed for body surface area. Measurements of septal-freewall (D1) and anterior-posterior (D2) endocardial diameters were used to assess LV symmetry from D1/D2. Systolic indices included stroke area, area ejection fraction, and fractional shortening. RESULTS: Preload, stroke area, area ejection fraction, and fractional shortening of D1 increased after ASD repair but decreased after VSD repair (p < 0.05). End-diastolic symmetry increased after ASD closure and decreased after VSD closure (p < 0.05). Increases in stroke area and ejection fraction after ASD correction primarily reflected increased shortening of D1. A positive correlation was found overall between percent change in end-diastolic area (EDA) and percent change in area ejection fraction (r(2) = 0.80, p < 0.0001, n = 18). CONCLUSIONS: Preload was the primary determinant of changes in LV function in this series of ASD and VSD repairs. Intraoperative changes in position of the interventricular septum affected systolic and diastolic LV symmetry and septal free wall shortening. Additional studies are needed to define changes in afterload and contractility as well as diastolic compliance and systolic mechanics.

Sodium-hydrogen exchange inhibition preserves ventricular function after ventricular fibrillation in the intact swine heart.

BACKGROUND: We tested the hypothesis that sodium-hydrogen exchange inhibition attenuates ventricular dysfunction after ischemia-reperfusion injury in the intact porcine heart. METHODS: Twelve pigs (weight, 30-45 kg) were evenly divided into 2 groups. Baseline ventricular function studies were based on echocardiography, conductance, aortic flow, and left ventricular pressure. Animals were given vehicle (control) or benzamide-N-(aminoiminomethl)-4-(4-[2-furanylcarbonyl]-1-piperazinyl)-3-(methylsulfonyl)methanesulfonate (BIIB 513; 3 mg/kg administered intravenously). Ten minutes later, hearts were subjected to 75 seconds of ventricular fibrillation. After reperfusion for 40 minutes, function studies were repeated. Hearts were arrested and excised. Postmortem data included passive pressure-volume curves and myocardial water content. RESULTS: Preload recruitable stroke work was significantly decreased from baseline after ischemia and reperfusion in the control group (27.7 +/- 2.5 vs 48.0 +/- 5.6 mm Hg [+/- SEM], P =.001) but not in the BIIB 513 group (43.0 +/- 5.8 vs 45.5 +/- 4.1 mm Hg, P = not significant). In vivo diastolic and postmortem passive left ventricular compliance were reduced after ischemia and reperfusion for control animals but remained unchanged for animals receiving BIIB 513. Time required to recover baseline blood pressure after ventricular fibrillation was significantly longer for control animals (159 +/- 15 vs 88 +/- 14 seconds [+/- SEM], P =.008). Myocardial water content (78.97% +/- 0.94% vs 77.86% +/- 0.46% [+/- SEM]) and normalized left ventricular mass (137.24 +/- 6.17 vs 128.41 +/- 1.96 g [+/- SEM]) were insignificantly increased in control animals. CONCLUSIONS: Sodium-hydrogen exchange inhibition attenuates ventricular dysfunction after 75 seconds of ventricular fibrillation and 40 minutes of reperfusion. This family of agents might prove useful in patients with severe left ventricular dysfunction undergoing ventricular fibrillation for implantable cardioverter defibrillator testing.

Left ventricular end-diastolic volume from ejection fraction and stroke volume in pigs during IVC occlusion.

BACKGROUND: Real-time measurement of left ventricular end-diastolic volume (LVEDV), combined with left ventricular end-diastolic pressure (LVEDP), would allow continuous measurement of intraoperative diastolic function. In pursuit of this goal, we examined stroke volume divided by ejection fraction for calculation of LVEDV(sv/ef). METHODS: Five anesthetized pigs underwent median sternotomy and pericardiotomy. A transit-time ultrasonic flow probe on the ascending aorta provided cardiac output. A micromanometer provided LV end-diastolic pressure. End-diastolic and end-systolic areas were measured from LV short-axis cross sections to obtain ejection fraction. LVEDV(sv/ef) was calculated during IVC occlusion. Steady-state LVEDV(echo) was determined using a three-plane echocardiography model. LVEDV(echo) was used to validate steady-state LVEDA in each experiment. RESULTS: Correlation coefficients for linear and pressure-volume relation analyses ranged from 0.46 to 0.99. The two methods for measuring LVEDV generated compliance curves with an overall reliability coefficient of 0.84. CONCLUSIONS: The LVEDV(sv/ef) method may facilitate real-time determination of LV compliance.