Cardiac and Pericardial Neoplasms in Children: Radiologic-Pathologic Correlation.

Primary cardiac and pericardial neoplasms are rare in the pediatric population and can include both benign and malignant lesions. Rhabdomyomas, teratomas, fibromas, and hemangiomas are the most common benign tumors. The most common primary cardiac malignancies are soft-tissue sarcomas, including undifferentiated sarcomas, rhabdomyosarcomas, and fibrosarcomas. However, metastatic lesions are more common than primary cardiac neoplasms. Children with primary cardiac and pericardial tumors may present with nonspecific cardiovascular symptoms, and their clinical presentation may mimic that of more common nonneoplastic cardiac disease. The diagnosis of cardiac tumors has recently been facilitated using noninvasive cardiac imaging. Echocardiography is generally the first-line modality for evaluation. Cardiac MRI and CT are used for tissue characterization and evaluation of tumor size, extension, and physiologic effect. The varied imaging appearances of primary cardiac neoplasms can be explained by their underlying abnormality. Treatment of these lesions varies from conservative management, with spontaneous regression of some lesions such as rhabdomyomas, to surgical resection, particularly in patients with associated heart failure. With adequate imaging techniques and knowledge of the pathologic basis of the neoplasm, it is often possible to differentiate benign from malignant tumors, which can greatly affect adequate and timely treatment. ©RSNA, 2023 Quiz questions for this article are available through the Online Learning Center.

Patients with Post-COVID-19 Vaccination Myocarditis Have More Favorable Strain in Cardiac Magnetic Resonance Than Those With Viral Myocarditis.

There have been reports of myocarditis following vaccination against COVID-19. We sought to describe cardiac magnetic resonance (CMR) findings among pediatric patients. Retrospective review at a large academic center of patients clinically diagnosed with post-vaccine myocarditis (PVM) undergoing CMR. Data collected included parametric mapping, ventricular function, and degree of late gadolinium enhancement (LGE). Post-processing strain analysis was performed using feature tracking. Strain values, T1/T2 values, and ventricular function were compared to age- and gender-matched controls with viral myocarditis using a Wilcoxon Signed Rank test. Among 12 patients with presumed PVM, 11 were male and 11 presented after the second vaccination dose, typically within 4 days. All presented with chest pain and elevated troponin. 10 met MRI criteria for acute myocarditis. All had LGE typically seen in the lateral and inferior walls; only five had prolonged T1 values. 10 met criteria for edema based on skeletal muscle to myocardium signal intensity ratio and only 5 had prolonged T2 mapping values. Patients with PVM had greater short-axis global circumferential and radial strain, right ventricle function, and cardiac output when compared to those with viral myocarditis. Patients with PVM have greater short-axis global circumferential and radial strains compared to those with viral myocarditis. LGE was universal in our cohort. Signal intensity ratios between skeletal muscle and myocardium may be more sensitive in identifying edema than T2 mapping. Overall, the impact on myocardial strain by CMR is less significant in PVM compared to more classic viral myocarditis.

Post-operative Chylothorax in Patients with Repaired Transposition of the Great Arteries.

Patients with dextro-transposition of the great arteries (d-TGA) require surgical repair as neonates. These patients are at risk for post-operative chylothorax. We sought to describe the presentation, imaging, and outcomes after intervention for patients with d-TGA with post-operative chylothorax. A retrospective chart review was performed in patients with repaired d-TGA who were referred from 1/1/2013 to 4/1/2020 for evaluation of chylothorax. Patient history, lymphatic imaging, and interventional data were collected. Impact of intervention on lymphatic drainage was evaluated with a student's t-test. Eight patients met inclusion criteria for this study. Five patients had a history of central venous thrombus leading to thoracic duct outlet occlusion. Five patients underwent intervention, two were managed conservatively, and one was not a candidate for intervention. Chylothorax resolved in six patients. There was a significant difference in output from 7 days prior to first intervention (114 mL/kg/day) compared to 28 days following final intervention (27 mL/kg/day, p = 0.034). There were no procedural complications. Chylothorax in patients with repaired transposition of the great arteries is often amenable to intervention. Early surveillance and management of central venous thrombosis may reduce the burden of lymphatic disease in these patients.

Total vascular resistance increases during volume-unloading in asymptomatic single ventricle patients.

OBJECTIVE: While the surgical stages of single ventricle (SV) palliation serve to separate pulmonary venous and systemic venous return, and to volume-unload the SV, staged palliation also results in transition from parallel to series circulation, increasing total vascular resistance. How this transition affects pressure loading of the SV is as yet unreported. METHODS: We performed a retrospective chart review of Stage I, II, and III cardiac catheterization (CC) and echocardiographic data from 2001-2017 in all SV pts, with focus on systemic, pulmonary, and total vascular resistance (SVR, PVR, TVR respectively). Longitudinal analyses were performed with log-transformed variables. Effects of SVR-lowering medications were analyzed using Wilcoxon rank-sum testing. RESULTS: There were 372 total patients who underwent CC at a Stage I (median age of 4.4 months, n=310), Stage II (median age 2.7 years, n = 244), and Stage III (median age 7.3 years, n = 113). Total volume loading decreases with progression to Stage III (P< 0.001). While PVR gradually increases from Stage II to Stage III, and SVR increases from Stage I to Stage III, TVR dramatically increases with progress towards series circulation. TVR was not affected by use of systemic vasodilator therapy. TVR, PVR, SVR, and CI did not correlate with indices of SV function at Stage III. CONCLUSIONS: TVR steadily increases with an increasing contribution from SVR over progressive stages. TVR was not affected by systemic vasodilator agents. TVR did not correlate with echo-based indices of SV function. Further studies are needed to see if modulating TVR can improve exercise tolerance and outcomes.

Risk Factors for Adverse Events in Children with Pulmonary Hypertension Undergoing Cardiac Catheterization.

Pulmonary hypertension (PH) can lead to progressive heart failure with high morbidity and mortality. Cardiac catheterization (CC) is the gold standard for diagnosis and response to vasodilatory medications. The invasive nature of CC and associated anesthesia predispose this patient population to adverse events including death. Catheterization records were queried from 1/1/2011 to 10/31/2016. Patients with PH, defined as pulmonary vascular resistance (PVR) greater than 3 WU m2, pulmonary artery pressure above 20 mmHg, and pulmonary wedge pressure less than or equal to 15 mmHg, who underwent hemodynamic CC were included in this retrospective study. Both patients with and without congenital heart disease were included. There were 198 CC in 191 patients. Adverse events (n = 28, 14.1%) included cardiac arrest, increased respiratory support requiring ICU care, PH crisis, bradycardia/hypotension requiring intervention, and arrhythmias. Odds of an adverse event increased by 22% for every 15-min increase in procedure times (OR 1.22, CI 1.01-1.39, p = 0.002) and were significantly increased for procedures longer than 80 min (OR 3.75, CI 1.56-9.00, p = 0.007) (Fig. 1). Patients with an adverse event had higher mean pulmonary artery pressures while breathing oxygen (43 [35-58] versus 34 [27-44] mmHg, p = 0.017) and oxygen with inhaled nitric oxide (37 [32-56] versus 32 [25-40] mmHg, p = 0.026). Females carried more risk than males (OR 3.88, CI 1.44-10.40, p = 0.007). Younger age, medication regimens, prematurity, and genetic disease did not carry an increased risk. Adverse events are common in pediatric patients with PH undergoing CC. The risk of adverse events correlates with greater procedure times and higher mean pulmonary artery pressure. Minimizing procedure time may improve patient outcomes.

Right ventricular myocardial performance index in pediatric patients with bronchopulmonary dysplasia-related pulmonary hypertension.

INTRODUCTION: This study aims to assess the utility of right ventricular myocardial performance index (RVMPI) as a potential echocardiographic tool to evaluate and serially follow patients with bronchopulmonary dysplasia (BPD) and pulmonary hypertension (PH). METHODS: We performed a retrospective chart review of all hemodynamic cardiac catheterizations performed January 2011 to December 2016 in patients born premature and with diagnosed BPD up to 4 years of age-excluding patients with significant congenital heart defects. Echocardiograms performed within 24 hours of the cardiac catheterization were reviewed, and a blinded RVMPI was calculated. The primary endpoint was correlation of invasive catheterization hemodynamics to noninvasive echocardiographic RVMPI measurement. RESULTS: A total of 49 individual patients met complete study criteria, and 10 of those patients underwent repeat cardiac catheterization. Median age at the time of assessment was 8 months (25%-75%, 4-18 months), and the cohort had a calculated RVMPI mean of 0.39 (±0.19), with 73% (43/59) having a RVMPI >0.28. A statistically significant correlation was found between the RVMPI and the baseline hemodynamics during catheterization with regard to the initial mean pulmonary arterial pressure (r = 0.58; P < 0.01) as well as the calculated pulmonary vascular resistance (r = 0.34; P = 0.01). CONCLUSIONS: This study demonstrates the utility of RVMPI for evaluation of PH in patients with prematurity and BPD. An increased RVMPI by noninvasive echocardiography was found to correlate with increased mean pulmonary arterial pressure and pulmonary vascular resistance measured during invasive cardiac catheterization. Large-scale validation of this study is being planned.

Critical care management of patients with lymphatic conduction disorders.

Lymphatic dysfunction in critical illness is complex. Primary complex lymphatic anomalies can lead to profound organ dysfunction, particularly respiratory failure and shock. Critical illness, the complications of critical illness, and the procedures and therapies used to treat critical illness, can lead to secondary lymphatic dysfunction. This is most often seen with congenital and acquired cardiovascular disease and respiratory disease. The critical care management of these patients requires an expert multidisciplinary team.

3-Dimensional Modeling Guided Transcatheter Repair of Dehisced Pulmonary Venous Baffle With Gore ASD Device.

A 38-year-old woman with sinus venosus atrial septal defect and partial anomalous return of the right upper pulmonary vein underwent a Warden procedure but experienced a large residual defect after patch dehiscence. Image-derived 3D modeling informed novel device closure with a Gore Cardioform atrial septal occluder. (Level of Difficulty: Advanced.).

Prolonged cardiopulmonary resuscitation and low flow state are not contraindications for extracorporeal support.

Outcomes of out-of-hospital cardiac arrest are poor irrespective of the patient age group and circumstances. Survival to discharge after out-of-hospital arrest in children is less than 10%. Use of extracorporeal cardiopulmonary resuscitation is increasing and has been shown to improve outcomes in some situations. However, the candidacy for such augmentation is based on patient selection, institutional practices, and availability of an extracorporeal membrane oxygenation center. Often, duration of resuscitation, low flow state, presenting pH, and circumstances of arrest dictate candidacy for extracorporeal membrane oxygenation. We present a case of extremely prolonged resuscitation for out-of-hospital arrest in a pediatric patient, and we describe the use of mechanical compression device and transition to extracorporeal membrane oxygenation. We present the case outcome as well as brief discussion about controversies in extracorporeal cardiopulmonary resuscitation. We hope the case provides an opportunity for further discussion regarding opportunities to improve selection, use of extracorporeal cardiopulmonary resuscitation, and impact outcomes.

Comparison of Two High-Dose Magnesium Infusion Regimens in the Treatment of Status Asthmaticus.

OBJECTIVES: To determine the feasibility and safety of a simplified high-dose magnesium sulfate infusion (sHDMI) for the treatment of status asthmaticus. METHODS: We retrospectively compared 2 different high-dose magnesium sulfate infusion regimens, as adjunctive treatment in status asthmatics, using data that were preciously collected. The initial high-dose, prolonged magnesium infusion (HDMI) regimen consisted of a loading dose of 75 mg/kg (weight ≤ 30 kg) or 50 mg/kg (weight > 30 kg) over a period of 30 to 45 minutes followed by a continuous infusion of 40 mg/kg/hr for an additional 4 hours. This was compared to the sHDMI regimen that consisted of 50 mg/kg/hr for 5 hours. No loading dose was given to the patients in the sHDMI arm. Obese patients were dosed by using ideal body weight. Physiologic parameters (i.e., heart rate, blood pressure, respiratory rate, oxygen saturation) and serum magnesium (SrMg) concentrations were monitored during administration of magnesium sulfate. RESULTS: Nineteen patients receiving the initial HDMI regimen were compared with 10 patients who received the sHDMI regimen. There was no significant difference in SrMg concentrations or physiologic parameters between the 2 dose regimens. CONCLUSIONS: The HDMI and sHDMI regimens both produced SrMg concentrations that are associated with bronchodilation. The safety profile was also similar for the 2 regimens. The unambiguity of sHDMI has the potential to reduce medication errors that are associated with calculation of the loading dose, product preparation, and ultimate administration.