Background Residual lesions may be important in both in-hospital and post-discharge outcomes following congenital heart surgery. We sought to understand their impact (measured by residual lesion score, RLS) on Norwood outcomes. Methods We reviewed outcomes post-Norwood procedure (2015-2017) in the Pediatric Heart Network’s RLS study, including transplant-free survival, reinterventions, and days alive and out of hospital 12-months post-Norwood among survivors to Norwood discharge. Residual lesions were assessed by echocardiography and clinical characteristics at discharge. Global rank scores of outcomes of interest weighted on severity, and win/loss ratios were calculated. RLS defined Class-1 (no residua), Class-2 (minor residua), Class-3 (major residua). Results Among 249 neonates, 239 could be assigned an RLS Class. Of 206 survivors to hospital discharge, RLS was not associated with post-discharge re-interventions. At 12 months, 53 (22%) died, including 3/4 after transplant. Of 192 with follow-up to assess days alive and out of hospital through 12 months, RLS Class-3 had 49 fewer days. Transplant-free survival 12 months post-Norwood was lower for Class-3 vs. Class-1 (HR:3.08, 95% CI: 1.51, 6.28, P=0.002). Global rank score identified a higher ratio of wins for RLS Classes 1&2 vs. Class-3. Conclusions Major residual lesions post-Norwood are associated with poorer transplant-free survival and fewer days alive and out of hospital at 12 months but are not associated with increased rates of reintervention post-Norwood discharge. Global rank scores confirmed poorer outcomes in Class-3. Given these findings, patients with RLS Class-3 warrant close follow-up.

Objectives: The objective of our study was to semiautomatically generate echocardiogram indices in pediatric sepsis using novel algorithms and determine which indices were associated with mortality. We hypothesized that strain and diastolic indices would be most associated with mortality. Design: Retrospective cohort study of children with sepsis from 2017 to 2022. Survivors and nonsurvivors were compared for echocardiogram indices. Multivariate Cox proportional hazard models were constructed for our primary outcome of in-hospital mortality. Linear regression was performed for secondary outcomes, which included multiple composite 28-day outcomes. Results: Of the 54 patients in the study, 9 (17%) died. Multiple echocardiogram indices of both right (RV) and left ventricles (LV) were associated with in-hospital mortality [RV GLS adjusted hazard ratio (aHR): 1.16 (1.03-1.29), P = 0.011; RV global longitudinal early diastolic strain rate (GLSre) aHR: 0.24 (0.07 to 0.75), P = 0.014; LV GLSre aHR: 0.33 (0.11-0.97), P = 0.044]. Impairment in GLS was associated with fewer ventilator-free days [RV GLS beta-coefficient: -0.47 (-0.84 to -0.10), P = 0.013; LV GLS beta-coefficient -0.62 (-1.07 to -0.17), P = 0.008], organ-support free days [RV GLS beta-coefficient: -0.49 (-0.87 to -0.11), P = 0.013; LV GLS beta-coefficient: -0.64 (-1.10 to -0.17), P = 0.008], and days free from ICU [RV GLS beta-coefficient: -0.42 (-0.79 to -0.05), P = 0.026; LV GLS beta-coefficient: -0.58 (-1.03 to -0.13), P = 0.012]. Systolic indices were not associated with mortality in this cohort. Conclusion: Our study demonstrates the feasibility of obtaining echocardiogram indices in a semiautomatic method using our algorithms. We showed that abnormal strain is associated with worse outcomes in a cohort of children with sepsis.

Introduction: Sepsis induced myocardial dysfunction is common in pediatric sepsis. We sought to utilize novel, specialized algorithms of echocardiogram data that allow for automatic acquisition of echocardiographic indices from all four chambers without advanced interpretation by cardiologists. We hypothesized that these algorithms could predict mortality and morbidity in septic children. Methods: A retrospective cohort study of 59 children admitted to the ICU with sepsis and echocardiogram data. Three user inputted points outlining each heart chamber were applied to existing echocardiograms. Novel algorithms then computed the relevant echocardiographic indices. We examined right and left sided indices for systolic function [ejection fraction (EF), stroke volume (SV) systolic wave (S’)], diastolic function [early diastolic annular velocity (E’), late diastolic annular velocity (A’)], chamber sizes, and strain indices. Our primary outcome was mortality, secondary outcomes included ventilator free days (VFD) at 28 days, and length of stay (LOS). Linear regression models were constructed. Results: Six (10.2%) of 59 patients died. Baseline characteristics were similar between survivors and nonsurvivors. Survivors had higher right ventricular (RV) SV [11.2 mL (4.7, 52.3) versus 3.04 mL (2.1, 4.2), p=0.010], RV (S’) [6.2 cm/s (5.0, 7.6) versus 4.5 cm/s (2.9, 6.2), p=0.046] and RV end-diastolic volume (EDV) [29.6 mL (12.3, 70.9) versus 9.1 mL (5.8, 17.6), p=0.016]. Multiple right chamber indices were associated with more VFD at 28 days [RV S’: β 2.7 (1.3 to 4.1), p=< 0.000, RV E’: β 2.3 (0.9 to 3.6), p=0.002, right atrial (RA) SV: β 0.3 (0.1 to 0.5), p=0.010, RA EF: β 0.2 (0.0 to 0.4) p=0.047, and RA EDV: β 0.4 (0.1 to 0.8) p=0.017]. Multiple diastolic indices were associated with differences in both ICU LOS in hours [left ventricular (LV) A’: β -129.5 (-208.4 to -50.6), p=0.020; RV S’: β -96.1 (-168.7 to -23.4), p=0.01] and hospital LOS in hours [LV A’: β -144.9 (-266.6 to -23.4), p=0.02; RV S’: β -145.1 (-260.7 to -29.6), p=0.015]. Conclusions: In this cohort of children with sepsis, echocardiographic indices as measured by our point of care, novel algorithms can be utilized to risk stratify these high-risk patients. Right sided chambers appear to be more predictive of both mortality and morbidity.

Background:There is little known about the spectrum of cardiac injury in acute COVID-19 infection in children. Methods:A single-centre, retrospective chart analysis was performed. The protocol was deemed IRB exempt. All patients under the age of 21 years admitted from 20 March, 2020 to 22 June, 2021 for acute symptomatic COVID-19 infection or clinical suspicion of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 were included. Past medical history, lab findings, echocardiogram and electrocardiogram/telemetry findings, and clinical outcomes were reviewed. Results:Sixty-six patients with MIS-C and 178 with acute COVID-19 were reviewed. Patients with MIS-C had more cardiac testing than those with acute COVID-19. Inflammatory markers were more likely elevated, and function was more likely abnormal on echocardiogram in those with MIS-C with testing performed. Among patients with MIS-C, 17% had evidence of coronary dilation versus 0% in the acute COVID-19 group. One (0.6%) patient with acute COVID-19 had clinically significant electrocardiogram or telemetry findings, and this was in the setting of prior arrhythmias and CHD. Four (6%) patients with MIS-C had clinically significant findings on electrocardiogram or telemetry. Among patients with acute COVID-19, extracorporeal membrane oxygenation support was required in 0.6% of patients with acute COVID-19, and there was a 2.8% mortality. There were no deaths in the setting of MIS-C. Conclusions:Patients with acute COVID-19 and clinical suspicion of cardiac injury had a lower incidence of abnormal laboratory findings, ventricular dysfunction, or significant arrhythmia than those with MIS-C.

Introduction: Obesity is a pro-inflammatory state and may influence presentation and outcomes of both Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19. Methods: From January 2020 to January 2022, n=2566 contemporaneous KD, MIS-C and acute COVID-19 pediatric patients from 39 sites in 8 countries were enrolled into the International KD Registry. The study population was confined to 1077 MIS-C patients meeting CDC criteria with confirmed or probable COVID-19 infection, and 657 KD patients meeting AHA guideline criteria without COVID-19 infection who had body mass index (BMI) recorded at presentation. BMI was converted to Z scores using WHO formulas for age and sex, and associated factors were sought using general linear regression modeling. Results: Mean zBMI was higher for MIS-C (+0.58) vs KD patients (-0.06; p<0.001), and MIS-C patients were more likely to be overweight (BMI 85 th -<95 th %ile; 18% vs 11%), obese (95 th -<99 th %ile; 14% vs 6%) or morbidly obese ( > 99 th %ile; 8% vs 2%; p<0.001). MIS-C patients were significantly more likely to present with shock, and had higher peak troponin I and NTproBNP, with no association or interaction with adiposity. Higher adiposity was significantly associated with higher peak white cell count for MIS-C but not KD. Higher peak CRP for MIS-C and higher peak ESR for KD were not associated with adiposity. Higher peak creatinine was associated higher adiposity, more so for MIS-C. Both higher adiposity and MIS-C were significantly associated with lower LV ejection fraction, although there was no significant interaction (Figure A). Higher maximum coronary artery Z score was associated with KD but not with adiposity, with no significant interaction (Figure B). Conclusions: MIS-C patients have higher levels of adiposity than KD patients. Higher adiposity is associated with greater renal dysfunction in the setting of MIS-C, and greater cardiac dysfunction in both MIS-C and KD.

Purpose The Blalock-Taussig (BT) shunt is a surgical procedure that establishes a permanent source of pulmonary or systemic blood flow in children with severely obstructed or hypoplastic vessels. Post-operatively, one of the major challenges for the practitioner is to maintain a balanced pulmonary to systemic blood flow ratio. For those patients with persistently low oxygen saturations or concern for pulmonary hypertension, some practitioners empirically trial selective phosphodiesterase inhibitors in an attempt to increase pulmonary blood flow. The purpose of this retrospective study was to evaluate the patient characteristics, indication, side …

Despite prenatal diagnosis, prenatal intervention, and immediate postnatal intervention, patients with hypoplastic left heart syndrome and intact or highly restrictive atrial septum have the highest risk for mortality. Charts for all infants diagnosed with hypoplastic left heart syndrome from 2009 to 2017 were retrospectively reviewed and compared, including pulmonary vein Doppler patterns on fetal echocardiogram and evidence of pulmonary lymphangiectasia on fetal MRI. Of the 81 newborns with hypoplastic left heart syndrome, we defined two groups. Group 1 patients had an adequate atrial septal communication (n = 69), while Group 2 met criteria for intact/restrictive septum (n = 12). No patient in Group 1 had a type C pulmonary vein Doppler pattern, while no patient in Group 2 had a type A pulmonary vein Doppler pattern. The two patients with pulmonary lymphangiectasia had type C pulmonary vein Doppler pattern and an intact atrial septum and did not survive. Survival to discharge for Group 1 was 83% compared to 58% for Group 2 (p = 0.116). Survival to stage 2 palliation was 71% for Group 1 compared to 50% for Group 2 (p = 0.186). Only 4 of the initial 12 patients from Group 2 are alive, which is an overall survival of 33%. Our experience supports previous evidence that fetal echocardiography can identify those patients with the greatest likelihood for postnatal intervention as well as those at highest risk for mortality. Fetal MRI is a novel imaging modality that may help providers separate patients at highest risk for mortality, regardless of pulmonary vein Doppler pattern.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but serious, sequala of acute pulmonary embolism. Symptoms can be subtle and non-specific and the prognosis is poor if severe pulmonary hypertension (PH) and right ventricular dysfunction are present. While PH-targeted therapies are often used, there is only one FDA approved therapy, and only for disease that is deemed inoperable. The greatest chance for potential cure and long-term survival is surgical pulmonary endarterectomy. We report a 6-year-old male with a history of asthma and two unprovoked deep venous thromboses who presented with syncope. Chest x-ray showed cardiomegaly and an echocardiogram showed severe PH with severely decreased right ventricular (RV) function. Ventilation-perfusion scan showed mismatched perfusion defects involving the right lower lobe, and CT of the chest showed right lower lobe subsegmental pulmonary thrombus, suspicious for chronic thromboembolism. Given his clinical presentation and the severity of his right ventricular dysfunction, he was started on ambrisentan, sildenafil and subcutaneous treprostinil. Bilateral pulmonary endarterectomy was performed with resection of level 2 to 3 disease and he was successfully weaned off all PH therapy. Four months post-op, he is clinically asymptomatic and his echo shows normal RV function without PH. In conclusion, CTEPH is a rare but likely underdiagnosed disease process in pediatrics. Clinicians should have a high index of suspicion for at risk patients with unexplained dyspnea. Even if right ventricular dysfunction and severe PH are present, surgical pulmonary endarterectomy can be performed successfully in young children.

Objective To compare the incidence of post-PDA ligation syndrome after surgical vs. percutaneous closure of PDAs in very low birth weight (VLBW) infants. Study design Cohort study comparing 59 infants who underwent surgical ligation with 25 infants who underwent percutaneous closure. Comparisons between groups were made using two-sample t-tests and a Fisher's exact test. Comparisons of pre vs. post variables within each group were made using paired t-tests. Results Patients who underwent surgical ligation had a higher need for initiation of post-procedure hemodynamic support (12/59 vs. 0/24, p = 0.016), a higher post-procedure peak FiO(2) (0.64 vs. 0.43, p = 0.004), and a larger absolute change in peak FiO(2) (0.23 vs. 0.09, p = 0.008). Conclusion VLBW infants who had percutaneous closure of their PDA did not experience post ligation syndrome and had less escalation of respiratory support compared with infants who underwent surgical ligation.

Patent ductus arteriosus is the most common cardiovascular abnormality in premature infants. With newly available percutaneous devices, centres are reporting high rates of success and favourable safety profiles with percutaneous closure of haemodynamically significant ductus arteriosi in infants under 1000 g. We report the case of a 5-week-old, previous 25-week gestation, 1200-g infant who underwent successful percutaneous closure of a ductus arteriosus with a Medtronic Microvascular Plug but who developed late-term coarctation from the device. This case should prompt practitioners to consider the need and timing of follow-up echocardiograms in this population and sheds light on a newly reported long-term complication of device closure in premature infants.

Severe PPHTN is a contraindication to liver transplantation and predicts an abysmal 5-year outcome. It is defined as a resting mPAP >45 mm Hg with a mean pulmonary artery wedge pressure of 3 wood units in the setting of portal hypertension. There have been limited reports of successful treatment of PPHTN leading to successful liver transplantation in adults, and one reported use of monotherapy as a bridge to successful liver transplant in pediatrics. To our knowledge, we describe the first use of combination therapy as a successful bridge to liver transplantation in a pediatric patient with severe PPHTN. This report adds to the paucity of data in pediatrics on the use of pulmonary vasodilator therapy in patients with severe PPHTN as a bridge to successful liver transplantation. Early diagnosis in order to mitigate or avoid the development of irreversible pulmonary vasculopathy that would preclude candidacy for liver transplantation is crucial, but our report demonstrates that combination therapy can be administered safely, quickly, and may allow for successful liver transplantation in patients with severe PPHTN.

AbstractTakotsubo cardiomyopathy is characterised by akinesis and ballooning of the left ventricular apex during contraction of the otherwise normal base of the heart. We describe the case of a 7-month-old previously healthy female who presented with an unwitnessed cardiac arrest. Workup raised suspicion for non-accidental trauma. Despite progression to brain death, the severely decreased ventricular function and apical akinesis of the left ventricle improved within 40 hours of admission. This report will familiarise paediatricians with this rare cardiomyopathy and emphasise the importance of considering non-accidental trauma as an inciting event for patients with unwitnessed cardiac arrest found to have decreased ventricular function.

For most patients, adenoviruses cause few acute health concerns and are often self-limiting. Patients who are immunocompromised or immunosuppressed, however, are at risk for disseminated adenovirus and suffer high morbidity and mortality, without well-defined treatment options. We report the case of a 9-month-old boy who was successfully treated for disseminated adenovirus infection with intravenous immunoglobulin and cidofovir 3 months post heart transplant, tailored to serum adenoviral load and clinical response. We emphasise the importance of early identification, monitoring, and a potentially novel treatment in the paediatric cardiac transplant population with disseminated adenovirus infection.