Resilience and response of the congenital cardiac network in Italy during the COVID-19 pandemic

: The worldwide response to the current COVID-19 pandemic has been focused on how to prevent the disease and to protect the high-risk patient from a potentially lethal infection. Several consensus and guidelines articles have been published dealing with the cardiac patient with systemic hypertension, heart transplant or heart failure. Very little is known about the patients, both in the pediatric as well as in the adult age, with congenital heart disease. The peculiar physiology of the heart with a native, repaired or palliated congenital heart defect deserves a specialized care. Hereby we describe the early recommendations issued by the Italian Society of Pediatric Cardiology and Congenital Heart Disease and how the network of the congenital cardiac institutions in Italy reacted to the threat of potential wide spread of the infection among this fragile kind of patient

Efficacy of sildenafil and high-dose anakinra in an MIS-C patient with pulmonary vasculitis: A case report

Multisystem inflammatory syndrome in children (MIS-C) is a newly identified clinical entity still not very well known in terms of epidemiology, pathogenesis, and long-term outcome. Pulmonary involvement with acute respiratory failure is an unusual life-threatening complication of MIS-C, often a reason for admission to the pediatric intensive care unit (PICU) and the use of mechanical ventilation. We present a case of a 7-year-old male patient, previously healthy, hospitalized for MIS-C, treated with intravenous immunoglobulins (IVIG), high dose methylprednisolone, and anakinra. After 2 days of the aforementioned therapy, the patient presented with hypoxia (SatO2: 85% in ambient air room) and breathing difficulties. A chest computed tomography (CT) scan showed the presence of multiple bilateral basal parenchymal thickening and small basal pleural effusion and an arterial blood gas analysis revealed severe hypoxia (PaO2/FiO2 ratio, 170 mmHg). Because of a worsening of respiratory distress, the patient was transferred to the PICU, where invasive mechanical ventilation and a continuous infusion of anakinra (12 mg/kg/day) were started. An echocardiogram was performed, which showed an increase in pulmonary pressure (40 mmHg) with normal heart ejection fraction (55%), and the hypothesis of pulmonary vasculitis involving the pulmonary arterioles was made. Therefore, therapy with sildenafil (0.15 mg/kg/day) was promptly set up, with an immediate improvement of the clinical picture of respiratory failure, reduction of pulmonary pressure (23 mmHg), and subsequent extubation at 36 h with a regular clinical course until discharge. As far as we know, our case represents the first report of pulmonary vasculitis in an MIS-C patient. The use of sildenafil and high-dose continuous anakinra may represent a rescue therapy in cases of MIS-C with pulmonary vasculitis or with difficulty in extubation, allowing a short-term hospitalization in intensive care and improving the long-term outcome in these patients

Transcatheter Interventions for Neonates with Congenital Heart Disease: A Review

Newborns with congenital heart disease often require interventions linked to high morbidity and mortality rates. In the last few decades, many transcatheter interventions have become the first-line treatments for some critical conditions in the neonatal period. A catheter-based approach provides several advantages in terms of procedural time, length of hospitalization, repeatability and neurodevelopmental issues (usually related to cardiopulmonary bypass). The main transcatheter procedures will be reviewed, as they are now valid alternatives to conventional surgical management

A large basal left ventricular pseudoaneurysm following pediatric cardiac surgery

Left ventricular pseudoaneurysm (LV-PSA) is a rare complication in children, usually developing after cardiac surgery, percutaneous procedures, infections, or trauma. Herein, we report a case of large basal submitral LV-PSA in a 36-day-old baby, detected 26 days after cardiac operation for hypoplastic arch, aortic coarctation, and small ventricular septal defect. No complications occurred in the first postoperative course, and early postoperative echocardiograms were normal. Despite large dimension of pseudoaneurysm, the baby presented with only mild tachypnea. The baby was successfully operated. Pseudoaneurysm, besides rare, could have an extremely broad and insidious clinical presentation and had to be considered in post-cardiac surgery follow-up echocardiogram at any time lapse

Off-Label Use of Cardiovascular Drugs in the Home Therapy of Children With Congenital or Acquired Heart Disease

Most medications are not labeled for use in the pediatric population because they have not been formally studied in children. Data on off-label use of cardiovascular (CV) drugs in the home therapy of children with CV disease are scanty. The study included 325 pediatric patients with CV disease and on >= 1 CV medication who underwent >= 1 visit during 2019 at the Pediatric Cardiology outpatient clinic of Giovanni XXIII Pediatric Hospital in Bari, Italy. A total of 287 patients (88.3%) received >= 1 off-label medication, whereas 113 patients (34.7%) received >= 2 off-label medications, and 22 patients (6.7%) >= 3 off-label medications. In CV medications (n = 27) 85% were used off-label in all cases, and 92.5% were used off-label in >= 50% of patients. Adverse events occurred in 8 patients, leading to drug discontinuation in 2 of them. In all 8 cases, medications were used off-label. In multivariate analysis, congenital heart disease patients with single-ventricle physiology (odds ratio 8.4, 95% confidence interval 2.25 to 54.4) and those with heart failure (odds ratio 2.0, 95% confidence interval 1.1 to 3.6) were at higher risk for receiving >= 2 off-label drugs. The off-label use of CV drugs in the home therapy of children with congenital or acquired heart disease is common and adverse events may occur. Patients with congenital heart disease with single-ventricle physiology and those with heart failure have a higher probability to receive >= 2 off-label medications. This study highlights the need for larger safety and efficacy trials in this specific cohort of pediatric patients. (C) 2021 Elsevier Inc. All rights reserved

[Treatment of heart failure in adult congenital heart disease]

The progressive shifting in adult patients with congenital heart disease (ACHD) epidemiology with aging and superimposed acquired cardiac pathology on top of complex congenital heart defects is leading to an increase of hospitalizations for heart failure (HF), which nowadays represents one of the main causes of death in this patient population. Although there is a theoretical evidence to support the use of conventional drugs indicated for the general population with HF, randomized controlled trials do often exclude ACHD patients. Anatomical and physiological heterogeneity makes it difficult to define the role of cardiac resynchronization, and indications are less established. Timing of assessment for heart transplantation is challenging and referral often occurs too late. The present review wants to offer a summary of current therapeutic strategies and discuss future perspectives for ACHD-related HF treatment

Endomyocardial biopsy safety and clinical yield in pediatric myocarditis: An Italian perspective

ObjectivesThe objective of this investigation is to evaluate the safety, the impact of endomyocardial biopsy (EMB) results in myocarditis management and the incidence of different etiologies of myocarditis in a pediatric population. BackgroundAlthough EMB is an established diagnostic tool to evaluate suspected myocarditis, there is lack of clear diagnostic and management guidelines for myocarditis in pediatric patients, particularly in infants. MethodsWe performed a retrospective database review and subsequent outcomes analysis from five Italian pediatric cardiology centers to identify patients aged 0-18 years who underwent EMB for suspected myocarditis or inflammatory cardiomyopathy (ICMP) between 2009 and 2011. ResultsEMB was performed in 41 children, of which 16 were male. The population ranged between 16 days of age to 17 years (mean age at EMB=5.24.9 years). The overall incidence of EMB-related complications was 15.5% (31.2% in infants, and 6.8% in children>1 year of age; P=0.079) while the incidence of EMB-driven treatment changes was 29.2%. Histological examination together with PCR on heart biopsy specimens allowed an etiological diagnosis in 26/41 patients (63%). Among the 15 patients (36.5%) with diagnosis of dilated cardiomyopathy (DCM) 11 had idiopathic DCM. Finally, we found an overall incidence of death/cardiac transplantation of 24%. ConclusionsIn a pediatric population with suspected myocarditis/ICMP, EMB was useful in confirming the diagnosis only in 41% of cases but showed an overall diagnostic power of 63%. As complications of EBM are not negligible, particularly in infants, the risk/benefit ratio should be taken into account in each patien

Incidence and Prevalence of Multisystem Inflammatory Syndrome in Children (MIS-C) in Southern Italy

Multisystem inflammatory syndrome in children (MIS-C) is a pediatric hyperinflammatory syndrome related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection whose epidemiology is not very well known at present. The objective of the study was to better understand the incidence of MIS-C in the Apulia region in southern Italy. Our primary goal was to estimate the incidence of newly identified cases of MIS-C in children aged 0–18 years, during a period of six months, encompassing the second pandemic wave. We also analyzed the characteristics of our cohort in terms of clinical features, treatment, and outcomes. The cumulative incidence of MIS-C was 3.27 per 100,000 residents between 0 and 18 years of age. In our cohort, gastrointestinal, mucocutaneous, and cardiac involvement were the most common clinical features. With our step-up approach to therapy, no patients required intensive care unit (ICU) admission and no cardiac sequelae after 6 months of onset were found in echocardiograms. Conclusion: Our epidemiological study of MIS-C in southern Italy showed unexpectedly overlapping figures with other US studies