Faculty Bibliography

With growing number of pediatric cases of COVID-19, a unique hyper-inflammatory syndrome, linked to SARS-CoV-2 infection, has emerged in children referred to as multisystem inflammatory syndrome in children (MIS-C). This Kawasaki Disease (KD)-like illness has been described across the world. This syndrome shares features of KD, toxic shock syndrome, and macrophage activation syndrome and is associated with significantly elevated inflammatory markers. Everyday there are new data emerging improving the care of these patients. The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a collaborative network designed to improve the outcomes of pediatric patients with end-stage heart failure and involves centers from across North America. The committee gathered information concerning COVID-19 anticoagulation practices at various centers and harmonized the data to formulate a set of recommendations.

Right heart failure (RHF) is a vexing problem in children after left ventricular assist device (LVAD) implantation that can negatively impact transplant candidacy and survival. Anticipation, prevention, early identification and appropriate medical and device management of RHF are important to successful LVAD outcomes. However, there is limited pediatric evidence to guide practice. This pediatric-focused review summarizes the relevant literature and describes the harmonized approach to RHF from the Advanced Cardiac Therapies Improving Outcomes Network (ACTION). This review seeks to improve RHF outcomes through the sharing of best practices and experience across the pediatric VAD community.

Femoral artery thrombosis (FAT) is an adverse event post-catheterization. We decided to study the incidence and risk factors of FAT before and after initiation of a pulse loss monitoring program. All cardiac catheterization cases performed using femoral arterial vascular access were retrospectively reviewed. The study period was divided into two eras: before (era 1), and after the initiation of the protocol (era 2). The primary outcome was FAT requiring therapy. Univariable and multivariable logistic regression models were used to identify factors associated with FAT. Receiver operating characteristic curve were generated to predict FAT. Our cohort consisted of 2,388 cases including 467 (19.6%) infants weighing < 10 kg. The overall incidence of FAT was 1.7% (n = 41). The incidence of FAT was 3.5 times higher in era 2 (p < 0.001). Multivariable model showed that era, weight, sheath exchange, and coarctation intervention were significantly associated with FAT. Infants younger than 7.7 months and less than 6.7 kg were found to be at highest risk for FAT. Our study shows that the incidence of FAT may be underestimated without a monitoring program. Small infants and coarctation interventions requiring larger sheaths or sheath exchanges are at highest risk for FAT.

Cardiac paraganglioma (PGL) is a rare catecholamine-secreting tumor forming 1% to 3% of cardiac tumors. Although most PGL occur sporadically, evidence exists that 40% of them may be related to familial cancer predisposition syndromes. We present a unique case of a 14-year-old female who presented with persistent hypertension and was found to have a cardiac PGL. During surgical resection, even though the main right coronary was preserved, the sinoatrial (SA) nodal artery was surrounded by the tumor and required resection with the tumor. The patient subsequently developed SA node dysfunction and is currently being evaluated for placement of a permanent pacemaker.

We report the case of a premature newborn diagnosed with coarctation of the aorta after spontaneous closure of ductus arteriosus who was successfully managed with prostaglandin E₁ infusion until surgical repair could be performed. This case, together with a review of the literature, suggests an important role for prostaglandin in the management of coarctation even in the absence of a patent ductus arteriosus. The putative mechanism for the utility of prostaglandin infusion is that it may relieve the obstruction in neonates with severe coarctation by not only opening of the ductus but, in select cases, relaxing the ductal tissue encircling the aortic isthmus region. We also found a possible dose dependence of the efficacy of the prostaglandin infusion when the ductus is closed.

Purulent pericarditis is a rare infectious disease with significant mortality, even in the modern antibiotic era. The presenting signs can often be subtle and patients can deteriorate rapidly with cardiac tamponade. We report a previously healthy 16-month-old female who developed purulent pericarditis associated with paronychia and sepsis caused by methicillin-sensitive Staphylococcus aureus. In addition to antibiotic treatment, she required emergent pericardiocentesis for cardiac tamponade, followed by two surgical interventions including full median sternotomy incision and partial pericardiectomy. At 4-month follow-up, she did well with no evidence of constrictive pericarditis on echocardiogram.

Pompe disease is a type-II glycogen storage disease, and clinical manifestations include hypertrophic cardiomyopathy and generalised muscular hypotonia. Enzyme replacement therapy has proven to be effective in reversing the ventricular hypertrophy. The outcomes are variable depending on time to diagnosis and severity of the cardiac disease. We describe two contrasting cases of patients with infantile-onset Pompe disease. The first child was diagnosed late and had severe cardiac hypertrophy with respiratory decompensation and ventilator dependence and eventual death. The second case was diagnosed at birth with early initiation of therapy resulting in a good outcome. Our cases highlight the importance of early initiation of enzyme replacement therapy to improve clinical outcomes.

Over the past few decades, the diagnosis and management of children with various malignancies have improved tremendously. As a result, there are an increasing number of children who are long-term cancer survivors. With improved survival, however, has come an increased risk of treatment-related cardiovascular complications that can appear decades after treatment. These problems are serious enough that all caregivers of childhood cancer survivors, including oncologists, cardiologists, and other health care personnel, must pay close attention to the short- and long-term effects of chemotherapy and radiotherapy on these children. This review discusses the effects of treatment-related cardiovascular complications from anthracyclines and radiotherapy and the methods for preventing, screening, and treating these complications.

Premature ventricular contractions (PVCs) in pediatric patients without structural heart disease and normal left ventricular systolic function rarely require therapy, though it is unknown whether these patients have subclinical cardiac dysfunction. Speckle tracking echocardiography is an additional means of evaluating cardiac function in asymptomatic pediatric PVC patients with normal standard measures of left ventricular (LV) function. Asymptomatic pediatric patients (< 21 years) without congenital heart disease, LV ejection fraction (LVEF) ≥ 55% and PVC burden ≥ 5% on 24-h Holter monitor were included. Demographic information, exercise stress test results, standard echocardiographic measures of LV systolic function and PVC morphology by 12-lead ECG were collected. Peak global systolic longitudinal strain (GLS) from apical four-chamber view was analyzed offline. 29 patients were identified (mean age 11.7 ± 5.8 years, 49.2 ± 25.3 kg, 59% male). Mean PVC burden was 12.0 ± 7.0% (range 5-37.5%). 14/29 (48%) had exercise stress testing with evidence of PVCs; 9/14 (64%) had PVC suppression at a mean heart rate (HR) of 160 ± 23 bpm and 5/14 (36%) did not suppress at a mean maximum HR of 188 ± 9 bpm. All patients had normal strain values by speckle tracking echocardiography (mean LV GLS - 22.5 ± 2.0%, LV global circumferential strain - 25.3 ± 3.9 and RV GLS - 24.1 ± 3.0%). There was no correlation between PVC burden and cardiac function parameters. Asymptomatic pediatric patients without structural heart disease, preserved LVEF/shortening fraction and PVC burden ≥ 5% demonstrated normal cardiac function including strain patterns indicating no evidence of subclinical cardiac dysfunction. Larger scale studies and longitudinal evaluation of left ventricular function using speckle tracking echocardiography is warranted in this population.