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Patients with hypoplastic left heart syndrome who have been palliated with the Fontan procedure are at risk for adverse neurodevelopmental outcomes, lower quality of life, and reduced employability. We describe the methods (including quality assurance and quality control protocols) and challenges of a multi-center observational ancillary study, SVRIII (Single Ventricle Reconstruction Trial) Brain Connectome. Our original goal was to obtain advanced neuroimaging (Diffusion Tensor Imaging and Resting-BOLD) in 140 SVR III participants and 100 healthy controls for brain connectome analyses. Linear regression and mediation statistical methods will be used to analyze associations of brain connectome measures with neurocognitive measures and clinical risk factors. Initial recruitment challenges occurred that were related to difficulties with: (1) coordinating brain MRI for participants already undergoing extensive testing in the parent study, and (2) recruiting healthy control subjects. The COVID-19 pandemic negatively affected enrollment late in the study. Enrollment challenges were addressed by: (1) adding additional study sites, (2) increasing the frequency of meetings with site coordinators, and (3) developing additional healthy control recruitment strategies, including using research registries and advertising the study to community-based groups. Technical challenges that emerged early in the study were related to the acquisition, harmonization, and transfer of neuroimages. These hurdles were successfully overcome with protocol modifications and frequent site visits that involved human and synthetic phantoms.
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Background Surgical strategies to achieve biventricular (BiV) repair in children with borderline left ventricle (LV) continue to evolve. We report our innovative strategy of LV recruitment utilizing systemic to pulmonary artery shunt upsizing along with fenestrated atrial septation (FAS). Case The case is a 22mo old with hypoplastic left heart variant with type A aortic arch interruption and bilateral SVC. The LV, aortic and mitral valve were hypoplastic not meeting criteria for BiV repair. He underwent stage 1 palliation (Norwood with 4mm BTT shunt). Frequent COVID infections and over-circulation led to BiV dysfunction and cardiogenic shock requiring ECMO support for 4 days. At 5 months of age cardiac catheterization (CC) revealed good hemodynamic parameters for a stage 2 Glenn. An MRI also revealed growth of the left ventricle. Decision-making A decision was made to engage in a staged LV recruitment process to achieve BiV repair. We elected to avoid a volume offloading procedure in the form of a Glenn. To optimize continued volume loading on the LV, Stage 2 palliation consisted of upsizing to a 5mm BTT shunt with 4mm FAS. MRI at 22 months showed an LV volume of 60ml/m2 associated with CC hemodynamics showing LA pressure of 13mmHg, and LV end-diastolic pressure of 12mmHg. He underwent BiV repair with takedown of DKS, with primary anastomosis of the aorta and the pulmonary artery to their respective circulations. The postoperative echocardiogram illustrated a gradient of 5mmHg and 3mmHg through the mitral and aortic valve respectively. The pt was placed on a beta blocker and discharged on day 5 following BiV conversion. This strategy provides increased pulmonary blood flow with increased bloodflow across the mitral valve and inflow into the LV. In so doing may enhance the rate of LV growth. Furthermore, this strategy avoids the bidirectional Glenn (BDG), a volume offloading operation. Conclusion Shunt upsizing with FAS is well tolerated. It has the potential advantage for fewer operations to achieve BiV circulation due to rapid LV growth in comparison to other staged LV recruitment strategies involving the BDG.Copyright © 2023 American College of Cardiology Foundation
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Background Patients with hypoplastic left heart syndrome (HLHS) undergo a Fontan procedure as part of single ventricle surgical palliation. Post-Fontan, sluggish blood flow and an imbalance in coagulant factor proteins may predispose to thrombus formation. Other risk factors may include chylothorax as well as acute and chronic inflammation. Currently, there is no standardized surveillance strategy to detect thrombus in Fontan patients. Case A 34-month old male with HLHS underwent an extracardiac non-fenestrated Fontan complicated by chylothorax treated with 5 days of IV steroids and diuretics. He was on therapeutic aspirin. After progressive worsening of right pleural effusion, a chest tube was placed three weeks post-Fontan with continued chylous output. Stool alpha 1 antitrypsin was negative. Decision-making Given persistent chylothorax, a repeat echocardiogram was performed revealing a large mass in the Fontan circuit less than one month post-op. Cardiac CT showed occlusive thrombus filling the entirety of the Fontan conduit extending into hepatic veins and bilateral pulmonary arteries. He underwent extensive surgical thrombectomy and Fontan conduit revision. Hypercoagulable work-up revealed elevated factor 8 and von Willebrand factor activity which persisted more than one month post-op. Patient's history was also significant for COVID-19 infection 6 months prior. He was initially anticoagulated with bivalirudin with tirofiban initiated for antiplatelet therapy. He was ultimately transitioned to rivaroxaban, pentoxifylline and aspirin with chylothorax resolution over one month without thrombus recurrence. Conclusion Development of risk stratification tools to identify patients at higher risk for thrombi formation post-Fontan may facilitate patient selection for more aggressive anticoagulation. Consideration of elevated factor 8 as well as persistent or recurrent chylothorax may be beneficial, as increased thrombosis risk has been reported for both conditions in Fontan patients.Copyright © 2023 American College of Cardiology Foundation
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472 HOSPITAL-ACQUIRED ACHROMOBACTER DENITRIFICANS BACTEREMIA: CASE REPORT AND REVIEW OF LITERATURE 473 MULTISYSTEM INFLAMMATORY SYNDROME IN ADULTS (MIS-A) AFTER COVID-19 INFECTION 474 RISK FACTORS FOR EARLY-ONSET MDRO INFECTIONS IN CRITICALLY ILL PATIENTS 475 DESCENDING NECROTIZING MEDIASTINITIS FROM PERITONSILLAR ABSCESS PRESENTING AS A STEMI 476 OUTCOMES OF PATIENTS AGED 85 YEARS OR OLDER ADMITTED WITH COVID-19 477 BABESIOSIS AND RED BLOOD CELL EXCHANGE TRANSFUSION: CONSIDER IT EARLY 478 REFRACTORY TOXIC SHOCK SYNDROME: IV IMMUNOGLOBULIN TO THE RESCUE! 390 IMPACT OF VACCINATION ON COST AND COURSE OF HOSPITALIZATION ASSOCIATED WITH COVID-19 INFECTION Research Snapshot Theater: Infection III PUBLISHING NUMBER 391 THE PROGNOSTIC VALUE OF ICHIKADO SCORES AND CCI FOR HOSPITALIZED PATIENTS WITH COVID-19 PNEUMONIA 392 PROGNOSTIC MARKERS OF GERIATRIC PATIENTS IN THE ICU WITH RESPIRATORY FAILURE SECONDARY TO COVID-19 393 SEVERE SUPPURATIVE SIALADENITIS IN A COVID-19-POSITIVE PATIENT 394 A CASE OF EXTENSIVE NECROTIZING FASCIITIS DUE TO STRING TEST-NEGATIVE KLEBSIELLA PNEUMONIAE 395 IMPACT OF BMI ON MORTALITY, MECHANICAL VENTILATION, AND LENGTH OF STAY IN COVID-19 PATIENTS 396 IMPACT OF OBESITY ON CLINICAL OUTCOMES IN PATIENTS RECEIVING ACYCLOVIR FOR HSV ENCEPHALITIS 397 DEGREE OF THROMBOCYTOPENIA IN HOSPITALIZED COVID-19 PATIENTS WITH EBV AND CMV COINFECTION 398 ICU MANAGEMENT OF SEVERE BABESIOSIS IN AN IMMUNOCOMPROMISED ASPLENIC PATIENT Research Snapshot Theater: Infection IV PUBLISHING NUMBER 399 A CASE OF SEVERE BABESIOSIS REQUIRING EXCHANGE TRANSFUSION 400 BLOOD RNA BIOMARKERS DISTINGUISH IMMUNE RESPONSES TO COVID-19 VIREMIA VERSUS COMORBID INFECTIONS 401 HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: A HIDDEN SURPRISE UNDER THE GARB OF DENGUE FEVER?. [Extracted from the article]
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Background and Aim: COVID-19 pandemic caused by SARS-Cov-2 coronavirus affects all groups of patients. Although pediatric pop-ulation seems to be less affected with milder or asymptomatic course of SARS-CoV-2 infection, there are few groups of patients with potential high risk of severe or fatal course of coronavirus dis-ease. These include children with congenital heart defects. The aim of this study was to evaluate the course of SARS-Cov-2 infection in patients with univentricular heart after Fontan operation. Method(s): From September 2020 to May 2021 (before vccination started in pediatric population in Poland) we screen all 38 Fontan patients admitted to Cardiology Department, Polish Mother's Memorial Hospital Research Institute for SARS-Cov2 antibodies. Result(s): We found positive SARS-Cov-2 antibodies in 21 unvac-cinated Fontan patients (55% of all hospitalized Fontan patients), 15 boys (71%) and 6 girls in the age 3-22 years (mean 11 years). 14 patients (67%) had hypoplastic left heart syndrome. Course of SARS-CoV-2 infection: asymptomatic course in 11(52%) patients, fever in 7 (33%) patients, cough 4 (19%) patients, diar-rhoea in 2 patients, loss of smell and taste-1 patient. One, 18 years old patient suffered from Covid fog symptoms (impairment of sus-tained attention and memory problems), he hasn't notice any SARS-Cov-2 symptoms but the level of antiobodies was high. Only 3 patients were hospitalized in acute SARS Cov2 infection: 2 due beacause of need for intravenous rehydratation during severe diarrhoea, 1 because of JET (junctional ectopic tachycardia) during fever. There was no case of PIMS (pediatric inflammatory multi-system syndrome) in study group. Medications used in study group: aspirin in 19 (90 %), warfarin in 2, spironolactone in 18 (86%), sildenafil in 9 (43%), angiotensyn-converting enzyme inhibitors in 17 (81%), beta-blockers in 4 (19%) of patients. Conclusion(s): 1. In our study severe congenital heart defect such as univentricular heart was not a risk factor of severe course of SARS-Cov-2 infection. 2. Absence of PIMS in analized group of patients may be connected with changed immunologic response in Fontan patients and chronic use of ASA (acetylsalicylic acid). 3. The impact of SARS CoV 2 infection on patients with congenital heart defects needs further studies.
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INTRODUCTION: We report a case of MIS-C, confirmed on autopsy, in a toddler with hypoplastic left heart syndrome (HLHS). DESCRIPTION: The patient was a 15 month-old male with HLHS (aortic and mitral atresia) with a superior cavopulmonary anastomosis, and complete heart block with a pacemaker, admitted for respiratory distress. He had known exposure to multiple COVID+ family members, a positive SARS-COV-2 RT-PCR, fevers, elevated inflammatory markers, skin and mucosal changes, and multi-system organ dysfunction, evolving into severe, irreversible multiple organ failure. He met laboratory and clinical criteria for MIS-C. He was treated with high-dose Methylprednisolone, IVIG, Anakinra, and convalescent plasma, but continued to clinically deteriorate. Upon death, the family elected for autopsy. Significant autopsy findings included: acute ischemia of the papillary muscles;peripheral lung infarcts and hemophagocytosis in the bone marrow. The hemophagocytosis in the bone marrow has been noted on several of the small number of autopsies performed on children with fatal MIS-C. DISCUSSION: The patient met WHO and CDC criteria for MIS-C during his hospital stay. Both criteria require fever, elevated inflammatory markers without alternative cause, evidence of prior COVID-19 infection/exposure, and multi-organ involvement. While the pathophysiology of MIS-C remains unknown, the syndrome clinically and immunologically overlaps with other hyperferritinemic syndromes, such as hemophagocytic lymphohistiocytosis and macrophage activating syndrome, where immune-mediated multi-organ injury results from a dysregulated innate immune response-related cytokine storm. The finding of marrow hemophagocytosis on this patient's autopsy adds to the small body of literature on autopsies of children with fatal MIS-C, which also note hemophagocytosis in the marrow and/or spleen. The immune profile and pathology of patients with MIS-C suggests that Macrophages have a significant role in MIS-C. This case thus adds additional credence to this important role of Macrophages, which in turn can further direct studies into its management, to prevent similar deaths.
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We describe the case of a 2-month-old born with hypoplastic left heart syndrome who presented with fever and vomiting and was found to be infected with the novel corona virus (COVID-19). He underwent treatment with supplemental oxygen, heparin, and dexamethasone. After a 6-day hospitalisation, he recovered remarkably well without major adverse effects.