Continuous Prostanoid Initiation in Severe Pulmonary Hypertension in the Pediatric Cardiac Intensive Care Unit.

OBJECTIVE: Limited data exists regarding prostanoid (PGI2) use in critically ill patients with pulmonary hypertension. (PH) in the pediatric cardiac intensive care unit (CICU) setting. MATERIALS AND METHODS: Single center, retrospective study of patients with diagnosis of PH who received continuous PGI2 and were admitted to CICU from January/2015 to April/2022. Data collected included patient demographics and clinical characteristics including diagnosis, etiology of PH, vasoactive and ventilatory support, length of stay, and survival. Type, initial, maximum, and final dose of PGI2 as well as hemodynamic data was obtained. Data reported as mean ± standard deviation. Significance taken p value < 0.05. RESULTS: 24 patients received PGI2 therapy at a mean age of 3.1 years, range (0-16.6 years). PGI2 was in the form of IV epoprostenol in 12 patients, IV treprostinil in 6, and SQ treprostinil in 6 patients. Mean initial dose was 2.79 ng/kg/min, max dose 18.75 ng/kg/min, and mean duration of therapy was 38.5 days. At PGI2 initiation, 21 (87.5%) were on vasoactive infusions, 19 (79.2%) mechanically ventilated (MV), and 6 (25%) were on extracorporeal membrane oxygenation (ECMO). The in-hospital mortality rate was 37.5% (n = 9). Patients MV and on ECMO support had higher risk of death (p = 0.04, and < 0.01, respectively). CONCLUSION: PGI2 therapy was tolerated in approximately 50% of patients with the most common side effect being hypotension leading to discontinuation in 1/3rd of patients. Ongoing evaluation of the benefits of PGI2 for patients in the CICU setting will help better identify patient selection, type, and dosing of PGI2.

Dilated cardiomyopathy associated with a mutation in the dispatched RND transporter family member 1 gene.

Dilated cardiomyopathy is the most common presentation of cardiomyopathy in children with 20-35% of patients having an identified genetic component. There are more than 30 genes implicated in the pathogenesis of dilated cardiomyopathy. We present the first report of a female infant with dilated cardiomyopathy with a genetic variant in the dispatched RND transporter family member 1 gene.

Vitamin D and morbidity in children with Multisystem inflammatory syndrome related to Covid-19.

Background: Multisystem inflammatory syndrome (MIS-C) is a clinical presentation reported in children related to Coronavirus-19 infection who present with a toxic shock like syndrome. Vitamin D deficiency has been postulated to play a role with severity of coronavirus infection in adult patients and other viral respiratory infections. Objective: This study aims to investigate if severe vitamin D deficiency was associated with increased disease severity and cardiac involvement in MIS-C. Methods: This is a retrospective and single center study. We included hospitalized patients less than 18 years of age with diagnosis of MIS-C between March and July 2020. Severe vitamin D deficiency was defined as 25-OH vitamin D level < 10 ng/ml within 48 h of admission. The composite outcome severe disease included patients requiring inotropes, mechanical ventilation, and extracorporeal membrane oxygenation. Results: Of the 31 patients with MIS-C, 45% were male and 58% were African American. The median age was 8 (1-13) years. Ten patients had severe vitamin D deficiency with a mean level of 7.2 ng/ml. Ninety percent of patients with severe vitamin D deficiency had severe disease (P < 0.001). Patients with severe vitamin D deficiency had an increased risk of cardiac involvement (P < 0.001). Conclusions: We describe a potential association between severe vitamin D deficiency and severe disease in children presenting with MIS-C. Severe vitamin D deficiency predisposes patients for cardiovascular involvement and may play a critical role in the host immune response to COVID-19 infection. Future prospective studies at the basic science and clinical level should be pursued to better delineate this association.

Inferior Vena Cava Collapsibility Index to Assess Central Venous Pressure in Perioperative Period Following Cardiac Surgery in Children.

The inferior vena cava collapsibility index (IVCCI) is an ultrasound method used to assess central venous pressure (CVP). Our objective was to evaluate the correlation between IVCCI and CVP in children during the early period following surgery for congenital heart disease (CHD). Prospective study performed in a single tertiary care center. Patients ≤ 18 years old, who underwent cardiopulmonary bypass surgery for CHD, were enrolled. Ultrasound images of the inferior vena cava (IVC) were obtained at two time points; the first was within 2 h of arrival to the CICU and the second was 12-18 h from the first measurement. CVP measured by catheter placed during surgery was recorded within minutes of performing ultrasound. Maximum and minimum IVC diameters were measured by 2D images and M mode method. Seventy patients (47.1% males), with median age 7 months (IQR 4-47 months) and weight 6.9 kg (IQR 4.8-13.5 kg), were evaluated. The 2D IVCCI had inverse correlation with CVP in patients breathing spontaneously; r = - 0.76 (p < 0.01) and r = - 0.73 (p < 0.01), during the first and second measurements, respectively. The 2D IVCCI ≤ 0.24 had sensitivity, specificity, and negative predictive value of 94%, 79%, and 88.9% , respectively, to detect CVP ≥ 10 mmHg. No correlation was found between IVCCI and CVP during positive pressure ventilation. There is a significant inverse correlation between 2D IVCCI and CVP in spontaneously breathing children after surgery for CHD. Use of 2D IVCCI for monitoring CVP could reduce the frequency and duration of CVP catheters and their inherent complications.

Vasoactive-ventilation-renal score: a reliable prognostic index for perioperative outcomes following congenital heart surgery in adults.

INTRODUCTION: The number of adults requiring surgeries for CHD is increasing. We sought to evaluate the utility of the vasoactive-ventilation-renal (VVR) score as a predictor of prolonged length of stay in adults following CHD surgery. METHODS: This is a retrospective review of 158 adult patients who underwent CHD surgery involving cardiopulmonary bypass. VVR score was calculated upon arrival to ICU and every 6 hours for the first 48 hours post-operatively. Our primary outcome was prolonged length of stay defined as hospital length of stay greater than 75th percentile for the cohort (≥8 days). RESULTS: The study cohort had a median age of 25.6 years (18-60 years), and 83 (52.5%) were male. The groups with and without prolonged length of stay were comparable in age, gender, race, and surgical severity score. VVR score was significantly higher at all time points in the group with prolonged length of stay. The first post-operative day peak VVR score ≥13 had a sensitivity of 81% and specificity of 75% for predicting prolonged length of stay (p = 0.0001). On regression analysis, peak VVR score during the first day was independently associated with prolonged length of stay. CONCLUSIONS: Peak VVR score during the first post-operative day was a strong predictor of prolonged length of stay in adults following CHD surgery.

Does obesity affect the short-term outcomes after cardiothoracic surgery in adolescents with congenital heart disease?

BACKGROUND: Obesity is a modifiable, independent risk factor for mortality and morbidity after cardiovascular surgery in adults. Our objective was to evaluate the impact of obesity on short-term outcomes in adolescents undergoing surgery for congenital heart disease (CHD). METHODS: This retrospective chart review included patients 10-18 years of age who underwent CHD surgery. Our exclusion criteria were patients with a known genetic syndrome, heart transplantation, and patients with incomplete medical records. The clinical data collected included baseline demographics and multiple perioperative variables. Charting the body mass index in the Centers for Disease Control and Prevention growth curves, the entire cohort was divided into three categories: obese (>95th percentile), overweight (85th-95th percentile), and normal weight (<85th percentile). The composite outcome included survival, arrhythmias, surgical wound infection, acute neurologic injury, and acute kidney injury. RESULTS: The study cohort (n = 149) had a mean standard deviation (SD), body mass index (BMI) of 22.6 ± 6.5 g/m2, and 65% were male. There were 27 obese (18.1%), 24 overweight (16.1%), and 98 normal weight (65.8%) patients. Twenty-seven (18%) patients had composite adverse outcomes. Overweight and obese patients had significantly higher adverse outcomes compared with normal weight patients (odds ratio (OR): 2.9; confidence interval (CI): 1-8.5, p = 0.04 and OR: 3; CI: 1-8.5, p = 0.03, respectively). In multivariate analysis, obesity was an independent predictor of adverse outcome in our cohort (p = 0.04). CONCLUSIONS: Obesity is associated with short-term adverse outcome and increased health resource utilisation in adolescents following surgery for CHD. Further studies should evaluate if intervention in the preoperative period can improve outcomes in this population.

Acute kidney injury following first-stage palliation in hypoplastic left heart syndrome: hybrid versus Norwood palliation.

OBJECTIVE: The aim of this study was to evaluate the prevalence of acute kidney injury after first-stage surgical palliation in patients with a single ventricle and to explore associated risk factors and outcomes. Design and patients This single-centre retrospective study included neonates who underwent either Norwood or Hybrid procedure from 2008 to 2015 for a single ventricle. Postoperative acute kidney injury was defined using the paediatric risk, injury, failure, loss, end-stage renal disease (pRIFLE), criteria within 72 hours of the procedure. Main results Our cohort (n=48) underwent surgical palliation at a mean (SD) age of 12 (11) days. Postoperative acute kidney injury was diagnosed in 14 (29%) patients. The prevalence of acute kidney injury in the Hybrid group was 16% and 53% in the Norwood group. Infants who developed acute kidney injury underwent surgery at younger ages [6 (5-10) versus 10 (8-16) days, p=0.016], and had a higher peak lactate level in the initial 24 hours [5.9 (4.2-9.1) versus 3.4 (2.4-6.7), p=0.007]. Norwood procedure was significantly associated with acute kidney injury [odds ratio 11.7 (95% confidence interval 1.3-101.9), p=0.03]. ICU stay [38 (21-84) versus 16 (6-45) days, p=0.038] and time to extubation [204 (120-606) versus 72 (26-234) hours, p=0.014] were longer in those with acute kidney injury. The two patients who developed early postoperative renal failure as per pRIFLE died before discharge from associated comorbidities. CONCLUSIONS: Acute kidney injury occurs in a third of the patients with single ventricle after surgical palliation but is mostly transient. Norwood, compared with Hybrid procedure, is a risk factor for postoperative acute kidney injury, which, in turn, is associated with longer ICU stay and time to extubation.

Hematuria in an Adult with Congenital Heart Disease.

BACKGROUND: Adults with congenital heart disease (CHD) in the United States now outnumber children with CHD, due in part to the improvement in surgical and medical management. This growing population may present postoperatively to the emergency department (ED) with nonspecific complaints from unforseen complications secondary to cardiac intervention. CASE REPORT: We describe a 39-year-old male who presented to the ED with hematuria and dysuria after he underwent percutaneous device ventricular septal defect (VSD) closure 10 days before. Upon initial evaluation, laboratory results confirmed a urinary tract infection and hematuria. Given persistent red discoloration of urine and easy fatigability, further investigation and re-evaluation found him to be anemic secondary to intravascular hemolysis. Cardiac catheterization showed residual shunting through the VSD device margins causing the hemolysis. Although this is a rare complication of VSD device closure, the patient's initial presentation of hematuria and dysuria presented a unique diagnostic challenge. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Patients with underlying CHD require emergency physicians to consider a multidisciplinary approach to properly diagnose and facilitate treatment.

Vasoactive Inotropic Score (VIS) as Biomarker of Short-Term Outcomes in Adolescents after Cardiothoracic Surgery.

Our aim was to evaluate the Vasoactive Inotropic Score (VIS) as a prognostic marker in adolescents following surgery for congenital heart disease. This single-center retrospective chart review included patients 10-18 years of age, who underwent cardiac surgery from 2009 to 2014. Hourly VIS was calculated for the initial 48 postoperative hours using standard formulae and incorporating doses of six pressors. The composite adverse outcome was defined as any one of death, resuscitation or mechanical support, arrhythmia, infection requiring antibacterial therapy, acute kidney injury or neurologic injury. Surgeries were risk-stratified by the type of surgical repair using the validated STAT score. Statistical analysis (SPSS 19.0) included Mann-Whitney U test, Chi-square test, ROC curves, and binary regression analysis. Our cohort (n = 149) had a mean (SD) age of 13.9 (2.4) years and included 97 (65.1 %) males. Maximal VIS at 24 and 48 h following surgery was significantly higher in subjects (n = 27) who suffered an adverse outcome. Subjects with adverse outcome had longer bypass and cross-clamp times, durations of stay in the hospital, and a higher rate of acute kidney injury, compared to those (n = 122) without postoperative adverse outcomes. The area under the ROC for maximum VIS at 24-48 h after surgery was 0.76, with sensitivity, specificity, and positive and negative predictive values with 95 % CI of 67 (48-82) %, 74 (70-77) %, and 36 (26-44) % and 91 (86-95) %, respectively, at a cutoff >4.75. On binary logistic regression, maximum VIS on second postoperative day remained significantly associated with adverse outcome (OR 1.35; 95 % CI> 1.12-1.64, p = 0.002). Maximal VIS at 24 and 48 h correlated significantly with length of stay and time to extubation. Maximal VIS on the second postoperative day predicts adverse outcome in adolescents following cardiac surgery. This simple yet robust prognostic indicator may aid in risk stratification and targeted interventions in this population.

Echocardiographic Indicators Associated with Adverse Clinical Course and Cardiac Sequelae in Multisystem Inflammatory Syndrome in Children with Coronavirus Disease 2019.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 causes significant cardiovascular involvement, which can be a determinant of clinical course and outcome. The aim of this study was to investigate whether echocardiographic measures of ventricular function were independently associated with adverse clinical course and cardiac sequelae in patients with MIS-C. METHODS: In a longitudinal observational study of 54 patients with MIS-C (mean age, 6.8 ± 4.4 years; 46% male; 56% African American), measures of ventricular function and morphometry at initial presentation, predischarge, and at a median of 3- and 10-week follow-up were retrospectively analyzed and were compared with those in 108 age- and gender-matched normal control subjects. The magnitude of strain is expressed as an absolute value. Risk stratification for adverse clinical course and outcomes were analyzed among the tertiles of clinical and echocardiographic data using analysis of variance and univariate and multivariate regression. RESULTS: Median left ventricular apical four-chamber peak longitudinal strain (LVA4LS) and left ventricular global longitudinal strain (LVGLS) at initial presentation were significantly decreased in patients with MIS-C compared with the normal cohort (16.2% and 15.1% vs 22.3% and 22.0%, respectively, P < .01). Patients in the lowest LVA4LS tertile (<13%) had significantly higher C-reactive protein and high-sensitivity troponin, need for intensive care, and need for mechanical life support as well as longer hospital length of stay compared with those in the highest tertile (>18.5%; P < .01). Initial LVA4LS and LVGLS were normal in 13 of 54 and 10 of 39 patients, respectively. There was no mortality. In multivariate regression, only LVA4LS was associated with both the need for intensive care and length of stay. At median 10-week follow-up to date, seven of 36 patients (19%) and six of 25 patients (24%) had abnormal LVA4LS and LVGLS, respectively. Initial LVA4LS < 16.2% indicated abnormal LVA4LS at follow-up with 100% sensitivity. CONCLUSION: Impaired LVGLS and LVA4LS at initial presentation independently indicate a higher risk for adverse acute clinical course and persistent subclinical left ventricular dysfunction at 10-week follow-up, suggesting that they could be applied to identify higher risk children with MIS-C.

Three Cases of Pediatric Multisystem Inflammatory Syndrome Associated with COVID-19 Due to SARS-CoV-2.

BACKGROUND Coronavirus disease 2019 (COVID-19) infection commonly presents as fever, cough, and shortness of breath in adults. Children are thought to have milder respiratory symptoms and to recover more quickly. We describe a new presentation of COVID-19 infection in children consisting of multisystem inflammation with decreased left ventricular function and evidence of lung disease. CASE REPORT Three children presented with fever, conjunctivitis, dry and cracked lips, rash, and/or cervical lymphadenopathy for at least 5 days. Two of these children required mechanical ventilation, and 1 of the 2 needed extracorporeal membrane oxygenation (ECMO) to support cardiorespiratory function. All of these children had moderate to severe hyponatremia and lymphopenia, which is usually seen in COVID-19. They were treated with intravenous immunoglobulin and high-dose aspirin. All of the children recovered. CONCLUSIONS Early recognition of children with multisystem inflammation is important because they are at increased risk for deterioration. Treatment with intravenous immunoglobulin and aspirin was used because this regimen has been shown to be beneficial in vasculitis of Kawasaki disease. The development of shock due to cardiac involvement may require ECMO.

Noonan syndrome associated with anomalous left coronary artery from the pulmonary artery in a patient with the rare RAF1 mutation: A case report and review of literature.

We present the case of a 7-week-old male infant diagnosed with anomalous left coronary artery from the pulmonary artery (ALCAPA) who underwent repair by left coronary artery reimplantation, followed by an eventful postoperative period including need for venous arterial extracorporeal membrane oxygenation and mitral valve replacement due to mitral calcification and severe insufficiency. He also required heart transplant due to severe rapidly progressive biventricular hypertrophy. The pathology examination of the explanted heart showed massive cardiomegaly. Subsequently, the infant's cardiomyopathy panel was positive for RAF1 mutation, consistent with diagnosis of a rare form of Noonan syndrome. To our knowledge, this autosomal dominant condition in association with ALCAPA has not been previously reported in the literature.

Heart Disease in Children.

The pediatric cardiology field has developed rapidly over the past few decades. More children than ever born with congenital heart disease (CHD) are growing into adulthood. Primary care providers play a key role in diagnosis, management, and referral of children with CHD because many common cardiac complaints (eg, feeding intolerance, cyanosis, chest pain, palpitations, and syncope) are first addressed in the primary care setting. The spectrum of heart disease in children ranges from common complaints to complex single-ventricle physiology, acute myocarditis, and heart transplantation. This article reviews the pathophysiology and management of the most frequent cardiac conditions encountered in primary care.

Parental perception of functional status and impact on the family of children with congenital heart surgery.

AIMS: To assess the functional status and the family impact of children with congenital heart defects (CHD), using the parental Functional Status II (FS-II) and the Impact on Family (IOF) questionnaires. METHODS: In this prospective observational study, parents of children who underwent surgery for CHD during the first year of life completed the FS-II and the IOF questionnaires. Genetic syndromes and prematurity <32weeks were exclusion criteria. The FS-II generates a total score and age specific general health (GH), activity (A) and, responsiveness (R) subscales. The IOF generates a total scale and financial and sibling subscales. RESULTS: Our cohort (n=100), comprised 54% males; the median (IQR) age was 32 (10-56) months. Eighteen children had age-specific scores in the 1-2 SD below mean range (n=17) or more than 2 SD below the mean (n=1) for "ill children". There were significant negative correlations between the total FS-II and total IOF (r=-0.35, p<0.001) and financial IOF (r=-0.35, p<0.001). RACHS-1 category of CHD 4-6 was associated with higher likelihood of lower functional status. CONCLUSIONS: More complex CHD was associated with lower functional status, which correlated with a greater impact on the families. Parental questionnaires may be useful instruments for developmental surveillance in this population.