BackgroundOur study evaluated progression of and identified potential factors contributing to outcomes of ROME III defined-functional gastrointestinal disorders (FGIDs) in children treated symptomatically in a biopsychosocial model of care with a long-term follow-up.MethodsWe performed a retrospective review of pediatric patients who were diagnosed with ROME III defined-FGIDs including functional abdominal pain, functional dyspepsia, irritable bowel syndrome and abdominal migraine. Patients were managed symptomatically in a biopsychosocial model of care from the time of initial diagnosis. Demographics, management, progression and response to treatment assessed as complete, partial, and no improvement were reviewed.ResultsTwo hundred fifty-eight patients were included with mean age of 10.6 years, female 55.4%, mean number of encounters 3.3 visits, and mean follow-up was 18.7 months (range 2 - 59, SD 15.8). Diagnoses were functional abdominal pain 45%, irritable bowel syndrome 20.9%, multiple 13.2%, functional dyspepsia 12.8%, and abdominal migraine 8.1%. Investigations were performed in most patients: laboratory studies in 93.4% (non-contributory abnormal 23.6%), imaging studies in 45.3% (non-contributory abnormal 5%) and endoscopies in 43.0% (non-contributory abnormal 1.2%). Treatment included medication in 93.7%, and surgery in 1.9% (normal pathology). There were new functional gastrointestinal diagnosis in 11.6%, evolution of FGIDs, from one to another in 12.0%, and recurrence found in 35.7% of patients. There were 60.1% patients in the complete improvement group (CIG) and 39.1% in the partial/no improvement group (PIG/NIG). No statistical difference was found between CIG and PIG/NIG regarding demographics or evaluation. PIG/NIG had more encounters (mean 3.63 vs. 3.11; P = 0.03), had non-contributory lab abnormalities (34.4% vs. 20.0%; P = 0.01), needed more endoscopies (52.4% vs. 36.8%; P = 0.02), required more treatment changes (mean 1.41 vs. 0.81; P < 0.01) and developed new functional gastrointestinal diagnoses (19.4% vs. 6.5%; P < 0.01) with long-term follow-up.ConclusionsPatients with ROME III defined-FGIDs who experience partial or no improvement with treatment develop new FGID diagnosis, need more number of follow-up visits, require more number of endoscopies, need more treatment changes, and have more non-contributory laboratory abnormalities, compared to those who experience complete improvement. Symptomatic treatment offered in a biopsychosocial model of care is possibly beneficial in managing children with FGIDs.

Introduction: Right ventricular pacing is associated with pacemaker induced cardiomyopathy and lesser degrees of pacing-induced LV dysfunction (PIVD) manifested by a reduction in left ventricular ejection fraction (LVEF). Our objective was to determine whether apical 4 chamber strain (A4C) by echocardiography can identify patients at risk of PIVD before LVEF declines.Methods and Results: A retrospective chart review of patients (0-21 years) who had a pacemaker with a ventricular lead placed between 2011 and 2017 was performed.Patients were divided into group A (LVEF <55% and/or >10% decline in LVEF within 12 months of pacemaker placement) and group B. Data have collected before and 1 and 12 months postpacemaker implantation. There were 30 patients in the group A and 60 in group B. At 1 and 12 months postpacemaker implantation, the LVEF was significantly lower while the A4C and QRS duration on electrocardiogram were significantly higher in the group A. While the LVEF and A4C became markedly abnormal in group A as early as 1 month, the A4C did not seem to demonstrate such marked abnormalities in group B. However, a sub-analysis of patients in the group A with preserved LVEF at 1 month demonstrated significant worsening in their A4C at that time.Conclusion: Myocardial deformation imaging may be a clinically useful tool for the prediction of a decline in LV systolic function following pacemaker implantation.Abnormalities in A4C seem to appear before LVEF decline and as soon as 1-month postpacemaker implantation. K E Y W O R D S cardiomyopathy, deformation, dysfunction, electrocardiogram, pacemaker, pediatric

An infant with coarctation of the aorta and Williams syndrome was noted to have petechiae in cardiology clinic prior to planned surgical intervention. Workup revealed acquired von Willebrand syndrome secondary to the high shear force generated by the aortic coarctation. He was treated with intra- and postoperative Humate P; there were no postoperative bleeding complications. His acquired von Willebrand syndrome resolved postoperatively.

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Introduction: A subset of patients who develop post-surgical heart block have recovery of atrioventricular-node function. Factors predicting recovery are not understood. We sought to investigate our center’s incidence of post-surgical heart block and examine factors associated with recovery of atrioventricular-node function. Methods: Patients 0 – 21 years who underwent cardiac surgery between January 2010 – December 2019 at a free-standing children’s hospital and experienced postoperative heart block were included. Demographics, clinical and operative variables were analyzed. Results: Of 6333 surgical hospitalizations, 128 (2%) patients were included. Of the 128 patients included, 90 (70%) had return of atrioventricular-node function, and 38 (30%) had permanent pacemaker placement. Of the 38 patients who underwent pacemaker placement, 6 (15.8%) had recovery of atrioventricular-node function noted on long-term follow-up. Median time to from onset of heart block to late atrioventricular-node recovery was 13 days (IQR 5 – 117). Patients with single-ventricle physiology ( p = 0.04), greater weight ( p = 0.03) and shorter cardiopulmonary bypass time ( p =0.015) were more likely to have recovery. The use of postoperative steroids was similar between all groups ( p = 0.445). Infectious or wound complications were similar between pacemaker groups ( p = 1). Conclusions: Two percent of patients who underwent cardiac surgery developed postoperative heart block, and 0.6% underwent pacemaker placement. Early recovery of atrioventricular-node was associated with greater weight at time of surgery, single-ventricle physiology and shorter cardiopulmonary bypass time. Late recovery of atrioventricular-node conduction following pacemaker placement occurred in 15.8% of patients.

Background: Multisystem inflammatory syndrome in children is a rare, post-infectious complication of SARS-CoV-2 infection in children. We aimed to assess the long-term sequelae, particularly cardiac, in a large, diverse population. Methods: We performed a retrospective cohort study of all children (aged 0–20 years, n = 304) admitted to a tertiary care centre with a diagnosis of multisystem inflammatory syndrome in children from March 1, 2020 to August 31, 2021 and had at least one follow-up visit through December 31, 2021. Data were collected at hospitalisation, 2 weeks, 6 weeks, 3 months, and 1 year after diagnosis, where applicable. Cardiovascular outcomes included left ventricular ejection fraction, presence or absence of pericardial effusion, coronary artery abnormalities, and abnormal electrocardiogram findings. Results: Population was median age 9 years (IQR 5–12), 62.2% male, 61.8% African American (AA), and 15.8% Hispanic. Hospitalisation findings included abnormal echocardiogram 57.2%, mean worst recorded left ventricular ejection fraction 52.4% ± 12.4%, non-trivial pericardial effusion 13.4%, coronary artery abnormalities 10.6%, and abnormal ECG 19.6%. During follow-up, abnormal echocardiogram significantly decreased to 6.0% at 2 weeks and 4.7% at 6 weeks. Mean left ventricular ejection fraction significantly increased to 65.4% ± 5.6% at 2 weeks and stabilised. Pericardial effusion significantly decreased to 3.2% at 2 weeks and stabilised. Coronary artery abnormalities significantly decreased to 2.0% and abnormal electrocardiograms significantly decreased to 6.4% at 2 weeks and stabilised. Conclusion: Children with multisystem inflammatory syndrome in children have significant echocardiographic abnormalities during the acute presentation, but these findings typically improve within weeks. However, a small subset of patients may have persistent coronary abnormalities.

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