Chronic disease management may be improved with the integration of mobile technologies capable of real-world physiologic monitoring. We performed a prospective, single-centre, pilot study to assess the feasibility of a consumer-based activity monitor use in outpatient management of adolescent cardiomyopathy patients. During the 3-month study period, the activity monitor was worn consistently and provided detailed physiologic and activity data with no adverse events related to the device use. Survey data revealed that the majority of subjects approve of these technologies becoming integrated into routine clinical care. Ongoing study is in need to define optimal disease management for this complex patient population in the age of consumer-driven healthcare.

The care of children with hypoplastic left heart syndrome is constantly evolving. Prenatal diagnosis of hypoplastic left heart syndrome will aid in counselling of parents, and selected fetuses may be candidates for in utero intervention. Following birth, palliation can be undertaken through staged operations: Norwood (or hybrid) in the 1st week of life, superior cavopulmonary connection at 4–6 months of life, and finally total cavopulmonary connection (Fontan) at 2–4 years of age. Children with hypoplastic left heart syndrome are at risk of circulatory failure their entire life, and selected patients may undergo heart transplantation. In this review article, we summarise recent advances in the critical care management of patients with hypoplastic left heart syndrome as were discussed in a focused session at the 12th International Conference of the Paediatric Cardiac Intensive Care Society held on 9 December, 2016, in Miami Beach, Florida.

Hypertrophic cardiomyopathy has a range of clinical severity in children. Treatment options are limited, mainly on account of small patient size. Disopyramide is a sodium channel blocker with negative inotropic properties that effectively reduces left ventricular outflow tract gradients in adults with hypertrophic cardiomyopathy, but its efficacy in children is uncertain. A retrospective chart review of patients ⩽21 years of age with hypertrophic cardiomyopathy at our institution and treated with disopyramide was performed. Left ventricular outflow tract Doppler gradients before and after disopyramide initiation were compared as the primary outcome measure. Nine patients received disopyramide, with a median age of 5.6 years (range 6 days–12.9 years). The median left ventricular outflow tract Doppler gradient before initiation of disopyramide was 81 mmHg (range 30–132 mmHg); eight patients had post-initiation echocardiograms, in which the median lowest recorded Doppler gradient was 43 mmHg (range 15–100 mmHg), for a median % reduction of 58.2% (p=0.002). With median follow-up of 2.5 years, eight of nine patients were still alive, although disopyramide had been discontinued in six of the nine patients. Reasons for discontinuation included septal myomectomy (four patients), heart transplantation (one patient), and side effects (one patient). Disopyramide was effective for the relief of left ventricular outflow tract obstruction in children with hypertrophic cardiomyopathy, although longer-term data suggest that its efficacy is not sustained. In general, it was well tolerated. Further study in larger patient populations is warranted.

Heart failure in children is a complex disease process, which can occur secondary to a variety of aetiologies, including CHD, cardiomyopathy, or acquired conditions as well. Although the overall incidence of disease is low, the associated morbidity and mortality are high. Mortality may have decreased slightly over the last decade, and this is likely due to our ability to shepherd patients through longer periods of significant morbidity, with lasting effects. Costs of heart failure are significant – on the order of $1 billion annually as hospital charges for inpatient admissions alone. The value, or benefit to patient life and quality of life at this cost, is not well delineated. Further research is needed to optimise not only outcomes for these patients but also the high costs associated with them.

Acute heart failure is a common and serious complication of congenital and acquired heart disease, and it is associated with significant morbidity, mortality, and costs. When a patient is admitted to the hospital with acute heart failure, there are several important goals for the hospital admission, including maintaining adequate perfusion, establishing the underlying aetiology for the heart failure, patient and family education, and discharge from the hospital in a stable condition. The pathway to home discharge is variable and may include inotropic therapy, mechanical circulatory support, and/or heart transplantation. This review will cover the epidemiology, presentation, and management of acute heart failure in children.

In the United States alone, ∼14,000 children are hospitalised annually with acute heart failure. The science and art of caring for these patients continues to evolve. The International Pediatric Heart Failure Summit of Johns Hopkins All Children’s Heart Institute was held on February 4 and 5, 2015. The 2015 International Pediatric Heart Failure Summit of Johns Hopkins All Children’s Heart Institute was funded through the Andrews/Daicoff Cardiovascular Program Endowment, a philanthropic collaboration between All Children’s Hospital and the Morsani College of Medicine at the University of South Florida (USF). Sponsored by All Children’s Hospital Andrews/Daicoff Cardiovascular Program, the International Pediatric Heart Failure Summit assembled leaders in clinical and scientific disciplines related to paediatric heart failure and created a multi-disciplinary “think-tank”. The purpose of this manuscript is to summarise the lessons from the 2015 International Pediatric Heart Failure Summit of Johns Hopkins All Children’s Heart Institute, to describe the “state of the art” of the treatment of paediatric cardiac failure, and to discuss future directions for research in the domain of paediatric cardiac failure.

We describe a case of influenza A myocarditis and transient left ventricular wall thickening in a 14-year-old girl presenting with acute heart failure. Admission echocardiogram revealed significant left ventricular hypertrophy with depressed left ventricular systolic function. The aetiology of the ventricular thickening was demonstrated to be myocardial oedema using cardiac magnetic resonance imaging. The natural course of this unusual clinical presentation of acute myocarditis and the importance of cardiac magnetic resonance imaging in this challenging clinical setting are discussed.

Although the Fontan operation may provide a durable circulation for some patients with functionally univentricular hearts for several decades, circulatory failure becomes more common over time. Medical and/or surgical interventions can improve the circulation for some period of time; however, many patients will experience end-stage circulatory failure. Heart transplantation may be considered in these patients. This review will cover the indications and evaluation for heart transplantation, management of patients while waiting for heart transplantation and the current outcomes of transplantation in this population.

Background: Viral genome in cardiac allograft has been associated with early graft loss in children who have undergone cardiac transplant from unknown mechanisms. Methods: This study is a retrospective review of children who have undergone cardiac transplant at a single institution from 1/2004 to 5/2008. Patients underwent cardiac catheterisations with endomyocardial biopsies to evaluate for rejection – graded on Texas Heart Institute scale – and the presence of virus by polymerase chain reaction. Patients with virus identified during the first year post transplant were compared at 1 year post transplant with virus-free patients. Results: The cohort comprised 59 patients, and the median age at transplant was 5.1 years. Viral genomes were isolated from 18 (31%) patients. The PCR + group had increased inflammation on endomyocardial biopsies, with a median score of 4 (ISHLT IR) versus 1 (ISHLT 1R) in the PCR – group (p = 0.014). The PCR + group had a similar cardiac index (median 3.7 ml/min/m2), pulmonary capillary wedge pressure (median 10 mmHg), and pulmonary vascular resistance index (median 1.7 U m2) comparatively. PCR + patients were more likely to have experienced an episode of rejection (p = 0.004). Conclusions: Children who developed viral endomyocardial infections after a cardiac transplant have increased allograft inflammation compared with virus-free patients. However, the haemodynamic profile is similar between the groups. The ongoing subclinical inflammation may contribute to the early graft loss associated with these patients.

The patient with a functionally univentricular heart is at increased risk for ventricular dysfunction for a variety of reasons. At birth, the pulmonary and systemic circulations are in parallel, leading to pulmonary overcirculation and a volume-loaded functional ventricle. Significant atrioventricular valvar regurgitation, abnormal ventriculoarterial coupling, diastolic dysfunction, and altered ventricular geometry can also contribute to long-term ventricular dysfunction. These collected circumstances place the patient at increased risk for perioperative morbidity and mortality. We will discuss in this review the pathophysiology that leads to ventricular dysfunction at each stage of surgical palliation, as well as the strategies for perioperative management. In addition, we will highlight novel strategies for management of ventricular dysfunction.