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Background: Takotsubo cardiomyopathy (TCM) has some distinctive features like greater proportion of reverse-TCM and central nervous system disease as a prevalent triggering cause. We expose the case of a child with cardiogenic shock presenting an atypical echocardiographic TCM pattern on an echocardiography, after an acute neurologic trigger. We also include a systematic literature review of previously described cases of atypical-TCM in children. Case Report: A previously healthy 9 year-old boy with status epilepticus presented abrupt cardiogenic shock. The EKG showed signs of myocardial ischemia, cardiac biomarkers NT-proBNP (2756 pg/mL ) and Troponin I (1707 pg/mL ) , and the echocardiography exposed a dilated LV with severely reduced systolic function (LVEF 28%) along with hypokinetic mid-basal segments (circumferential ballooning), and preserved hypercontractile apical segments, with the normal origin of both coronary arterial systems. A presumptive diagnosis of “reverse”, “inverse” or atypical Takotsubo cardiomyopathy was built based on the echocardiographic findings, apart from the ACS-like EKG findings, the raised cardiac biomarkers, and the neurological trigger of the hyper catecholaminergic state. Despite cardiovascular improvement with supportive treatment, the patient eventually expired on day 2 after PICU admission due to neurological complications. As shown in our systematic review, only 19 similar cases have been reported to date. Conclusion: With the report of this unusual case, we aim to point out the fundamental role of bedside echocardiography as a diagnostic test for critically ill children presenting with ACS-like in the context of neurosurgical emergencies, where bedside echocardiography itself can accurately establish a presumptive diagnosis of TCM.

Background: Schimke immuno-osseous dysplasia (SIOD) is a very rare autosomal recessive genetic disease caused by mutations in the SMARCAL1 gene. It is characterized by spondyloepiphyseal dysplasia, T-cell immunodeficiency, hypercromic nevi, hypercholestero-le-mia, and steroid-resistant nephrotic syndrome with progressive renal failure to end-stage kidney disease. Case presentation: We report two cases of SIOD in sisters, diagnosed after the debut of nephrotic syndrome. Both had a personal history of short stature, acetabular hip dysplasia, and hypercho-lesterolemia. The first case, a 6-year-old girl, presented peripheral refractory edema, severe arte-rial hypertension, and progressive decrease of the glomerular filtration rate. Steroid-resistance of nephrotic syndrome was confirmed, treated with tacrolimus without response. Renal function worsened over the following 4 months, so haemodialysis was started. Her sister, a 5-year-old girl, had the steroid-resistant nephrotic syndrome and normal blood pressure and renal function under enalapril treatment. In view of the suspicion of SIOD, genetic studies were carried out, revealing the same mutation in homozygosis. Conclusions: SIOD has a variable expression with multi-systemic involvement with a short life expectancy. Early diagnosis is important, which can encourage the early start of treatment and anticipation of complications that may be life-threatening.

Background We aimed to explore and to compare the association between the NT-proBNP and high-sensitivity troponin I (hs-cTnI) at early stages of acute bronchiolitis with echocardiographic alterations, clinical severity and outcomes. Methods A single centre, prospective observational study including previously healthy infants aged 1–12 months with bronchiolitis admitted to a tertiary hospital from April 2019 to March 2020. All patients underwent clinical, laboratory and echocardiographic evaluation at the same time point within 12 h of hospital admission. NT-proBNP > 1121 pg/ml and hs-cTnI > 26 ng/L were considered elevated. The primary outcome measure was the association of raised cardiac biomarkers with the need for PICU admission. Results We enrolled 40 infants with median levels of NT-proBNP of 1176 (520–3030) pg/ml and hs-cTnI of 11.5 (5–21) ng/L at the time of hospital admission. Raised levels of NT-proBNP and hs-cTnI in 50% and 20% of cases, respectively. Of them, 15 (37%) required PICU admission during the hospitalization. Increased NT-proBNP was associated with PICU admission (adjusted OR 9.5 (CI95% 1.4–64); p = 0.020), prolonged hospitalization (β = 2.7; p = 0.012) and duration of oxygen administration (β = 2.7; p = 0.004) in the multivariate analysis. There were no differences in hs-cTnI levels regarding PICU admission (p = 0.866). Increased hs-cTnI levels were only associated with oxygen administration duration (Spearman rho = 0.38; p = 0.017), but this association disappeared in the multivariate analysis. Only NT-proBNP was associated with echocardiographic parameters of myocardial dysfunction (p Conclusion Early elevated NT-proBNP but not hs-cTnI could be used as a biomarker for myocardial strain and disease severity in bronchiolitis.

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We aimed to delineate cardiopulmonary interactions in acute bronchiolitis and to evaluate the capacity of a combined cardiopulmonary ultrasonography to predict the need for respiratory support. This was a prospective observational single-center study that includes infants < 12 month of age admitted to a hospital due to acute bronchiolitis. All the included patients under-went clinical, laboratory and cardiopulmonary ultrasonographic evaluation at the same time point within 24 hours of hospital admission. The existence of significant correlation between car-diac and respiratory parameters was the primary outcome. The association of different cardio-pulmonary variables with the need of respiratory support higher than O2, the length of stay hos-pitalization, the PICU stay, and the duration of respiratory support were a secondary outcome. We enrolled 112 infants (median age 1 (0.5-3) months; 62% males) hospitalized with acute bron-chiolitis. Increased values of the pulmonary variables (BROSJOD score, pCO2 and LUS) showed moderate correlations with NT-proBNP and all echocardiographic parameters indicative of pulmonary hypertension and myocardial dysfunction. Up to 36 (32%) infants required respira-tory support during the hospitalization. This group presented with higher lung ultrasound score (p

Introduction: The real risk of Sudden Cardiac Death (SCD) in Asymptomatic Wolff-Parkinson-White (AWPW) syndrome is still unclear, and the literature is controversial about the best management strategy. Usually, SCD has been reported as the first event in asymptomatic or undiagnosed infants with AWPWs. So adequate risk stratification is warranted to prevent the occurrence of life-threatening arrhythmias in these patients. However, none of the available diagnostic tests is satisfactory to predict SCD. Case Presentation: We report two cases of AWPW syndrome in infants that experienced SCD as their first clinical manifestation. Conclusion: AWPW syndrome in infants is not rare. It is also a challenging condition that implies a very weak but real risk of SCD, which is very difficult to ascertain with current diagnostic methods. In this article, we review the literature about these cases and discuss the adequate management of these patients.

Aim: To explore NT-proBNP as biomarker for pulmonary hypertension (PH) in infants with respiratory syncytial virus infection (RSVI). Patients & methods: We prospectively enrolled 93 healthy infants with RSVI aged 1–12 months. NT-proBNP determination and echocardiography were performed at admission. Results: PH was found in 22% of patients and associated with a severe course of the disease. NT-proBNP >1635 pg/ml resulted an independent predictor for PH (odds ratio: 16.46 [95% CI: 4.10–66; p

X-linked hypophosphatemia (XLH) is a rare genetic disorder with X-linked dominant inheritance. Mutations in the PHEX gene increase fibroblast growth factor 23 (FGF23) concentrations, causing loss of phosphorus at the proximal tubule. Most pediatric patients debut in the first two years with short stature and bowed legs. Conventional treatment consists of oral supplements with phosphorus and calcitriol. Since 2018, burosumab has been approved as a novel therapeutic option for XLH, with promising results. The purpose of this study was to share our experience with two cases of XLH treated with burosumab. These patients presented with a broad phenotypical differences. One had the most severe radiological phenotype and developed left ventricular hypertrophy (LVH) and left ventricular dysfunction with preserved ejection fraction. Treatment with burosumab was well-tolerated and was followed by radiological stability and a striking improvement in both blood biochemistry and quality of life. The LVH was stable and left ventricular function normalized in the patient with cardiac involvement. In recent years many studies have been carried out to explain the role of FGF23 in cardiovascular damage, but the exact pathophysiological mechanisms are as yet unclear. The most intensively studied populations are patients with XLH or chronic kidney disease, as both are associated with high levels of FGF23. To date, cardiovascular involvement in XLH has been described in patients treated with conventional treatment, so it would be of interest to investigate if early use of burosumab at the time of diagnosis of XLH would prevent the occurrence of cardiovascular manifestations.

We present the rare case of lipomatous atrial septal hypertrophy associated with adrenocorticotropin hormone therapy in an infant with West syndrome, highlighting their relatively benign nature and good prognosis in children, and the relevance of the differential diagnosis with more dangerous cardiac masses in order to avoid aggressive diagnostic and therapeutic interventions.

Dystrophin-deficient cardiomyopathy (DDC) is currently the leading cause of death in patients with dystrophinopathies. Targeting myocardial fibrosis (MF) has become a major therapeutic goal in order to prevent the occurrence of DDC. We aimed to review and summarize the current evidence about the role of the renin–angiotensin–aldosterone system (RAAS) in the development and perpetuation of MF in DCC. We conducted a comprehensive search of peer-reviewed English literature on PubMed about this subject. We found increasing preclinical evidence from studies in animal models during the last 20 years pointing out a central role of RAAS in the development of MF in DDC. Local tissue RAAS acts directly mainly through its main fibrotic component angiotensin II (ANG2) and its transducer receptor (AT1R) and downstream TGF-b pathway. Additionally, it modulates the actions of most of the remaining pro-fibrotic factors involved in DDC. Despite limited clinical evidence, RAAS blockade constitutes the most studied, available and promising therapeutic strategy against MF and DDC. Conclusion: Based on the evidence reviewed, it would be recommendable to start RAAS blockade therapy through angiotensin converter enzyme inhibitors (ACEI) or AT1R blockers (ARBs) alone or in combination with mineralocorticoid receptor antagonists (MRa) at the youngest age after the diagnosis of dystrophinopathies, in order to delay the occurrence or slow the progression of MF, even before the detection of any cardiovascular alteration.

ArticleTitle: Left ventricular myocardial dysfunction secondary to adverse ventricular-ventricular interactions in previously healthy infants with Respiratory Syncytial Virus Bronchiolitis…Moises Rodriguez-Gonzalez 1,4*, Alvaro Antonio Perez-Reviriego1,4, Ana Castellano-Martinez2,4, Simon Lubian-Lopez3,4 and Isabel Benavente-Fernandez3,41 Pediatric Cardiology Division, Puerta del Mar University Hospital, Cadiz, Spain;doctormoisesrodriguez@gmail.com;alvaro.apr@hotmail.com2 Pediatric Nephrology Division, Puerta del Mar University Hospital, Cadiz, Spain;anacastellanomart@gmail.com3 Neonatology Division, Puerta del Mar University Hospital, Cadiz, Spain;isabenavente@gmail.com;slubian@yahoo.es4 Biomedical Research and Innovation Institute of Cadiz (INiBICA), Research Unit, Puerta del Mar University Hospital* Correspondence: doctromoisesrodriguez@gmail.com.Received: date; Accepted: date; Published: dateAbstract: Aim: To investigate if the presence of left ventricular myocardial dysfunction (LVMD) assessed by Tei index (LVTX) may have a direct impact on the outcomes in Respiratory Syncytial Virus bronchiolitis (RSVB), and if NT-proBNP will increase the accuracy of traditional clinical and laboratory markers in predicting the severity of the disease. Methods: A single-centre, prospective, cohort study including healthy infants aged 1-12 month-old admitted due to RSVB between October 1, 2016 and April 1, 2017. All patients underwent clinical, laboratory and echocardiographic evaluation within 24 hours of admission. PICU admission was defined as severe disease. Results: We enrolled 50 cases of RSVB (median age of 2 (1-6.5) months; 40% female) and 50 age-matched controls. We observed higher values of LVTX in infants with RSVB than in controls (0.42 vs 0.36; p=0.008). A total of 9 (18%) cases presented LVMD (LVTX>0.5), with higher incidence of PICU admission (89% vs 5%; p 45 mmHg coexisted in the same patient. Plasma NT-proBNP levels at admission were determined using a commercially available electrochemiluminescent immunoassay kit (ElecSys 2010, Roche Diagnostics). The primary outcome was PICU admission during the hospitalization. PICU admission criteria for RSVB at our institution rely on the presence of: apnea, extreme bradycardia, need of respiratory support greater than high-flow nasal cannula oxygen therapy, or inotropic support.

Objective: To evaluate if hypoalbuminemia on admission predict disease severity in children with acute bronchiolitis (AB). Working hypothesis: Hypoalbuminemia is associated with worse outcome in infants with AB. Study design: Single-centre prospective cohort study. Patient-subject selection: Infants aged

We present our recent experience with a 6-month-old infant with a personal history of short bowel syndrome that presented with fever, cyanosis, and cardiogenic shock secondary to severe pulmonary hypertension and right ventricular failure without pulmonary thromboembolism. He did not present signs of toxin-mediated disease or Kawasaki disease. He was finally diagnosed with SARS-CoV-2 infection. If this presentation is confirmed in future research, the severe cardiovascular impairment in children with COVID-19 could be also attributable to the primary pulmonary infection, not only to a multisystem inflammatory syndrome but also in children without heart disease.

This study aims to systematically review the incidence of myocardial strain detected by echocardiography in previously healthy infants with acute bronchiolitis and its role as a predictor for adverse outcomes in this setting. Methods: Pubmed/Medline, Excerpta Medica Data Base (EMBASE), and Cochrane Library were searched in April 2020 to identify original observational prospective studies that systematically performed echocardiography for the screening of myocardial strain in healthy infants with acute bronchiolitis. Pooled estimates were generated using random-effects models. Heterogeneity within studies was assessed using Cochran’s Q and I2 statistics. Funnel plots and Egger´s regression method were constructed to evaluate publication bias. Sensitivity analyses were also conducted to evaluate potential sources of heterogeneity. Results: After a detailed screening of 305 articles, a total of 10 studies with 395 participants (mean of 40 participants per study) was included. Five of them were classified as high-quality studies. Up to 28% of cases presented adverse outcomes. The echocardiographic screening for myocardial strain was performed within the first 24 h of admission in 92% cases. Tissue Doppler imaging and Speckle-Tracking echocardiography were performed only in 20% of cases. The presence of pulmonary hypertension was evaluated with methods different from the tricuspid regurgitation jet in 64% of cases. Seven studies found some grade of myocardial strain with a pooled incidence of 21% (CI 95%, 11–31%), in the form of pulmonary hypertension (pooled incidence of 20% (CI 95%, 11–30%)), and myocardial dysfunction (pooled incidence of 5% (CI 95%, 1–9%)). The presence of these echocardiographic alterations was associated with adverse outcomes (pooled relative risk = 16; CI 95%, 8.2–31.5). After a subgroup analysis based on the echocardiographic techniques used, no significant heterogeneity across the studies was observed. There was no evidence of publication bias when assessed by Egger´s test. Cardiac biomarkers to assess myocardial strain were used in five studies. Only N-terminal-pro-brain natriuretic peptide accurately predicted the presence of myocardial strain by echocardiography. Conclusions: Myocardial strain is not infrequent in previously healthy infants with acute bronchiolitis, and it could be present at the early stages of the disease with prognostic implications. There is a need for sufficiently powered prospective studies with a similar methodology, preferably employing advanced imaging techniques, to conclusively address the usefulness of the assessment of myocardial strain in this setting.

Background: The aim of the study was to document cardiovascular clinical findings, cardiac imaging and laboratory markers in children presenting with the novel multisystemic inflammatory syndrome associated with COVID-19. Methods: A real-time internet based survey was sent via the member mailing database for Association for European Paediatric and Congenital Cardiologists (AEPC) working groups for Cardiac Imaging and Cardiovascular Intensive Care member. Inclusion criteria was children 0-18 years admitted to hospital between March 1 and June 6, 2020 with diagnosis of an inflammatory syndrome and acute cardiovascular complications. Findings: A total of 286 children from 55 centres from 17 European countries were included. The median age was 8·4 years (IQR 3·8-12·4 years) and 67% were males. Most common cardiovascular complications were shock (40%), cardiac arrhythmias (35%), pericardial effusion (28%) and coronary artery dilatation (24%). Reduced left ventricular ejection fraction was present in 52% of patients and 93% had raised cardiac troponin (cTnT). The biochemical markers of inflammation were raised in majority of patients on admission: elevated CRP (99%), ferritin (79%), procalcitonin (96%), NT-proBNP (93%), IL-6 level (88%) and D-dimers (90%). There was a statistically significant correlation between degree of elevation in cardiac and biochemical parameters and need of intensive care support (p

Resumen: Introducción: La Sociedad Española de Cardiología Pediátrica y Cardiopatías Congénitas recomienda realizar ecocardiografía transtorácica (ETT) en todo menor de 2 años con soplo. En 2014 la Asociación Americana de Pediatría publicó los primeros criterios de uso apropiado de ETT pediátrica ambulatoria (CUA) como guía para promover un uso costo-eficiente de la misma. Nuestro objetivo fue analizar los CUA y otros factores clínicos como predictores de cardiopatía congénita (CC) en menores de 2 años con soplo para desarrollar una estrategia de derivación eficiente y segura. Pacientes y método: Estudio de casos y controles en menores de 2 años derivados por pediatría de atención primaria a cardiología pediátrica por soplo durante 4 años. Mediante análisis multivariante se determinó un modelo predictivo de CC. Resultados: Se incluyeron 688 pacientes con 129 casos (19%) de CC. La edad menor a 3 meses (odds ratio ajustada [ORa] 3,8[1,5-8,4]; p=0,030) y cumplimiento de CUA (ORa 16,3[9,4-28,3]; p

In approximately 5% of patients with idiopathic recurrent pericarditis, the disease usually follows a chronic relapsing course, and children can develop dependence and side effects of prolonged high-dose corticosteroid regimens. In this setting anakinra, a recombinant human interleukin-1 competitive receptor antagonist that blocks the biologic effects of interleukin-1, thereby reducing systemic inflammatory responses, appears to be one of the most promising strategies. We report an adolescent with steroid-dependent idiopathic recurrent pericarditis that was successfully treated with anakinra, highlighting that this therapeutic option seems to be an effective, rapidly acting, steroid-sparing, and relatively safe agent for the treatment of this entity in children.

Hypertrophic obstructive cardiomyopathy (HOCM) is the most common known cause of sudden death in children beyond infancy and in young athletes. Cases reported indicate that steroid-induced HOCM is usually a benign disorder. The normalization of cardiac morphological changes and clinical signs observed after the discontinuation of steroid therapy indicates that the effects on cardiac muscle are dose-dependent and reversible. However, the management of patients with symptomatic-HOCM presenting in infancy represents a major challenge because left ventricular outflow tract obstruction is a major risk factor associated with increased mortality in pediatric patients. We report a critically ill infant with steroid-induced HOCM resistant to beta-blockers who was successfully treated with disopyramide without relevant adverse events. Adult guidelines and pediatric experts suggest pharmacological therapy with beta-blockers or verapamil as the first- and second-line approach. However, these drugs are not always an option, especially in critical patients, hence, alternative therapeutic options are required. For these cases, disopyramide could be an alternative drug in spite of the little evidence on its safety and efficacy in pediatric patients. Our experience supports this cause, and the need for prospective studies on its use in the management of hypertrophic cardiomyopathy in children. .

Background Kawasaki disease (KD) is a multisystem vasculitic disease. Coronary artery aneurysms (CAAs) are the most important and life-threatening complication of KD. Various neurologic complications have been described to occur in 1–30% of patients with KD, but peripheral facial nerve palsy (FNP) is rare (0.9%). Case Report We describe a 5-month-old male infant who presented to us with unilateral left infranuclear FNP in the convalescent phase (day 18 of illness) of incomplete KD. The initial diagnosis was not made during the first 10 days of illness (therapeutic window for immunoglobulin treatment) as he was suspected to have hand-mouth-foot disease. We believe that both the delay in diagnosis and treatment of an atypical presentation of KD, combined with the more severe vasculitis and inflammatory burden reported in these cases, contributed to the development of CAA in our patient. Why Should An Emergency Physician Be Aware of This? This case highlights the importance of considering KD diagnosis in children with prolonged unexplained fever, even with incomplete diagnostic features, as well as the need to be aware of unusual manifestations, such as FNP. Atypical cases like this may be at increased risk of CAA because of delayed diagnosis and a higher inflammatory burden; therefore, a more aggressive treatment approach may be necessary.

Coronary vasospasm can result from silent myocardial ischaemia to sudden death. There are many precipitant factors including different pharmacological agents. Kounis syndrome is defined by acute coronary syndromes associated with anaphylactic or anaphylactoid reactions. We report, to the best of our knowledge, the first paediatric case of Kounis syndrome due to intravenous atropine.

In Respiratory Syncytial Virus infection, the early identification of infants at risk for severe disease in order to potentially decrease morbidity could be considered a major goal. Current guidelines recommend only clinical observation for this purpose in infants without known comorbidities. However, recent evidence shows that the presence of pulmonary hypertension in this population is a relevant risk factor for the development of a severe illness, even in healthy infants. The determination of plasmatic NT-proBNP levels could help to identify those cases that benefit of echocardiographic screening to detect pulmonary hypertension in this population during hospitalization.

Kounis syndrome consists of the simultaneous occurrence of anaphylaxis and acute coronary syndrome. It is a rare entity that may be underdiagnosed in paediatrics. The clinical presentation is variable, atypical and usually unexpected, and it carries possible serious complications such as ventricular arrhythmias, myocardial infarction and sudden death. Therefore, an early diagnosis and treatment for myocardial revascularization and the anaphylactic reaction are crucial. We report the case of an 11-year-old male who, after contact with latex, presented an anaphylactic reaction associated with coronary vasospasm, with rapid and complete recovery after administration of intramuscular adrenaline. The cardiological study ruled out coronary pathology as the cause of the event. The allergy study revealed a latex-fruit (kiwi and pineapple) cross-reactivity syndrome. The patient was diagnosed with type I Kounis syndrome triggered by latex, recommending the avoidance of possible triggers.

Kawasaki disease (KD) is a systemic childhood vasculitis with peculiar tropism for the heart. Coronary artery aneurysms are the primary cause of morbidity and mortality in these patients. The timely administration of gammaglobulin decreases the risk for development of coronary artery aneurysms, highlighting the importance of early KD recognition. However, the most significant dilemma in the management of KD is the diagnosis itself. In this article, we review the recent literature focusing on the diagnostic utility of N-terminal probrain natriuretic peptide as a biomarker for diagnosis of KD. The main conclusion is that N-terminal probrain natriuretic peptide is an useful biomarker for KD diagnostic that represents a valuable addition to the current diagnostic workup of patients with suspected KD, increasing the diagnostic accuracy.

Pulmonary sequestration is an unusual cause of heart failure in infants. We report a preterm newborn with signs of congestive heart failure supposed secondary to a ductus arteriosus that was finally diagnosed as a coexistent extralobar pulmonary sequestration. In this case, Doppler echocardiography was essential for diagnosis, revealing an abnormal systemic arterial supply to the sequestered lung and abnormal venous drainage.

Listeriosis is a sporadic infectious disease, which affects high-risk populations, such as the elderly, pregnant women, newborns, and immunocompromised patients. During pregnancy, listeriosis usually presents like a mild non-specific infection, but it may be responsible for fetal loss, preterm labor, early onset neonatal sepsis, and neonatal death. We report the case of a late stillbirth secondary to maternal chorioamnionitis. Listeria monocytogenes was isolated from the amniotic fluid and the fetal pleural fluid. The fetal autopsy revealed a disseminated inflammatory response with multi-organ involvement. This case illustrates the importance of the prevention and the diagnosis of listeriosis during gestation and may help us to understand the physiopathology of fetal loss due to listeriosis.

Asymptomatic VPE refers to the presence of this abnormal ECG pattern in the absence of any symptoms. The natural history in these patients is usually benign, and most children (60%) with VPE are usually asymptomatic. However, Sudden Cardiac Death (SCD) has been reported to be the initial symptom in many patients too. The increased risk of SCD is thought to be due to the rapid conduction of atrial arrhythmias to the ventricle, via the AP, which degenerates into Ventricular Fibrillation (VF). The best method to identify high-risk patients with asymptomatic VPE for SCD is the characterization of the electrophysiological properties of the AP through an Electrophysiological Study (EPS). Also, catheter ablation of the AP with radiofrequency as definitive treatment to avoid SCD can be performed by the same procedure with high rates of success. However, the uncertainty over the absolute risk of SCD, the poor positive predictive value of an invasive EPS, and complications associated with catheter ablation have made the management of asymptomatic VPE challenging, even more in those children younger than 8-year-old, where there are no clear recommendations. This review provides an overview of the different methods to make the risk stratification for SCD in asymptomatic children with, as well as our viewpoint on the adequate approach to those young children not included in current guidelines.