The Early “Unnatural” History Following Surgical Repair of Ventricular Septal Defects

Introduction Surgical outcomes for simple ventricular septaldefects (VSD) have been excellent in the past three decades. Forthis project, the timing of resolution of left-sided dilation and mitralregurgitation (MR) following VSD repair was assessed. Methods Echocardiographic data surrounding surgery of 42 consecutivechildren who underwent surgical patch repair of a VSD werereviewed. The echocardiograms were reviewed up to a mean of 12months post-operatively (range 9 - 14 months). Quantitative dataindexed to body surface area including left atrial (LA) volume, mitralvalve annulus diameter, and left ventricular end-diastolic dimension(LVEDD) was analyzed. Results The majority of our pre-surgical cohort had only trace(44%) or no MR (31%), with a small proportion having mild (16%)or moderate MR (9%). No patients had moderate or greater MR followingrepair at follow-up. The median mitral valve annular Z-scorewas 1.8 (SD 1.6; range: -1.2 to 4.1) pre-operatively, improving to a 0.6(range: -1.7 to 2.4; p < 0.001) at follow-up. LA dilation was present in70% of patients, with a median LA volume Z-score of 1.1 (range: -2.6to 15.5), decreasing to 13% median Z-score -1.2 (range: -3.5 to 2.9; p< 0.001) at follow-up. LV dilation was present in 81% of pre-operativepatients with a median LVEDD Z-score of 3.0 (range: -2.0 to 7.9).There was significant improvement in qualitative assessment of LVenlargement (25%) with a median LVEDD Z-score of 0.5 (range:-2.1 to 2.9; p < 0.001) at follow-up. Discharge echocardiogram wasperformed at a mean of 5.7 days (range: 3 - 12 days) following surgery. Conclusions Normalization of LA, mitral valve annulus, and LV sizeoccurred within the first three months in the majority of patients, withsignificant changes occurring within the first post-operative weekfollowing surgical repair for VSD

Use of echocardiographic subxiphoid five-sixth area length (bullet) method in evaluation of adequacy of borderline left ventricle in hypoplastic left heart complex

Indexed left ventricular end-diastolic volume (ILVEDV) is commonly used in evaluating "borderline left ventricle (LV)" in hypoplastic left heart complex (HLHC) to determine if the LV can sustain adequate systemic cardiac output. Commonly used quantification methods include biplane Simpson or the traditional five-sixth area length "bullet" methods, which have been shown to underestimate true LV volumes, when septal position is mildly abnormal. Subxiphoid five-sixth area length method is proposed as a more accurate estimate of true LV volume in the evaluation of borderline LV

Novel use of an ultrafiltration device as an alternative method for fluid removal in critically ill pediatric patients with cardiac disease: a case series

Fluid overload (FO) is a common complication for pediatric patients in the intensive care unit. When conventional therapy fails, hemodialysis or peritoneal dialysis is classically used for fluid removal. Unfortunately, these therapies are often associated with cardiovascular or respiratory instability. Ultrafiltration, using devices such as the AquadexTM system (Baxter Healthcare, Deerfield, IL, USA), is an effective tool for fluid removal in adult patients with congestive heart failure. As compared to hemodialysis, ultrafiltration can be performed using smaller catheters, and the extracorporeal volume and minimal blood flow rates are lower. In addition, there is no associated abdominal distension as is seen in peritoneal dialysis. Consequently, ultrafiltration may be better tolerated in critically ill pediatric patients. We present three cases of challenging pediatric patients with FO in the setting of congenital heart disease in whom ultrafiltration using the AquadexTM system was successfully utilized for fluid removal while cardiorespiratory stability was maintained

Intimal spindle cell sarcoma masquerading as adult-onset symptomatic pulmonic stenosis: a case report and review of the literature

Abstract Background Pulmonary artery intimal spindle cell sarcomas are rare and carry with them a poor prognosis and high rate of recurrence. In extremely rare cases, this tumor can infiltrate the pulmonic valve and manifest as adult-onset pulmonic stenosis. Case presentation We report an unusual case of a patient with symptomatic, adult-onset severe pulmonic stenosis who was referred for possible balloon valvuloplasty but was subsequently found to have pulmonary artery intimal sarcoma infiltrating the pulmonary valve leading to progressive exertional dyspnea. Conclusion The presence of adult-onset pulmonic stenosis should prompt the clinician to investigate further as most cases of pulmonic stenosis are congenital in nature and present early in life. Careful diagnostic evaluation in concert with multimodal imaging should take place to arrive at the correct and challenging diagnosis of sarcoma-induced adult-onset severe pulmonic stenosis. Given the poor prognosis and rapid progression of disease, early diagnosis is crucial

Procalcitonin as a biomarker of bacterial infection in pediatric patients after congenital heart surgery

Background: Bacterial infection (BI) after congenital heart surgery (CHS) is associated with increased morbidity and is difficult to differentiate from systemic inflammatory response syndrome caused by cardiopulmonary bypass (CPB). Procalcitonin (PCT) has emerged as a reliable biomarker of BI in various populations. Aim: To determine the optimal PCT threshold to identify BI among children suspected of having infection following CPB. Setting and Design: Single-center retrospective observational study. Setting and Design: Single-center retrospective observational study. Statistical Analysis: The Wilcoxon rank-sum test was used for nonparametric variables. The diagnostic performance of PCT was evaluated using a receiver operating characteristic (ROC) curve. Results: Ninety-eight patients were included. The median age was 2 months (25th and 75th interquartile of 0.1-7.5 months). Eleven patients were included in the BI group. The median PCT for the BI group (3.42 ng/mL, 25th and 75th interquartile of 2.34-5.67) was significantly higher than the median PCT for the noninfected group (0.8 ng/mL, 25th and 75th interquartile 0.38-3.39), P = 0.028. The PCT level that yielded the best compromise between the sensitivity (81.8%) and specificity (66.7%) was 2 ng/mL with an area under the ROC curve of 0.742. Conclusion: A PCT less than 2 ng/mL makes BI unlikely in children suspected of infection after CHS

Strain in children with MIS-C and acute COVID-19

Context : Cardiac injury has been described in both acute COVID-19 and the multisystem inflammatory syndrome in children (MIS-C). Echocardiographic strain has been shown to be a sensitive measure of systolic function. Aims : We sought to describe strain findings in both the groups on initial presentation and follow-up. Settings and Design : A retrospective study analyzing echocardiograms of all patients presenting with acute COVID-19 infection and MIS-C at our institution between March 2020 and December 2020 was performed. Subjects and Methods : TOMTEC software was used for strain analysis in both the study groups (COVID-19 and MIS-C) and age-matched healthy controls. Strain was correlated with LV ejection fraction (EF) and serum troponin levels. Results : Forty-five patients (34 – MIS-C and 11 – COVID-19) met the inclusion criteria. There was a statistically significant decrease in LV longitudinal strain (P < 0.001), LV circumferential strain (P < 0.001), and left atrial strain (P = 0.014) in the MIS-C group when compared to the control group. There was a statistically significant decrease in LV longitudinal strain (P = 0.028) in the acute COVID-19 group. All patients with abnormal left ventricular EF (LVEF) had abnormal strain. However, 14 (41%) patients in the MIS-C group and 3 (27%) in the acute COVID-19 group had preserved LVEF but abnormal strain. There was a significant correlation with LV longitudinal strain (P = 0.005) and LVEF (P = 0.002) and troponin in patients with MIS-C. Abnormal strain persisted in one-third of patients in the MIS-C and acute COVID-19 groups on outpatient follow-up. Conclusions : Patients with MIS-C and acute COVID-19 can develop myocardial dysfunction as seen by abnormal strain. LV longitudinal strain correlates with cardiac injury as measured by serum troponin in patients with MIS-C. Strain may provide an additional tool in detecting subtle myocardial dysfunction. It can be routinely employed at diagnosis and at follow-up evaluation of these patients