In adults with cardiomyopathy, tissue Doppler imaging (TDI) has been shown to correlate with left ventricular filling pressures (LVFPs) and has been advocated as a surrogate to catheterization. However, this has not been validated in children. This is a retrospective review of children ≤18 years old with dilated, hypertrophic, restrictive, and left ventricular noncompaction cardiomyopathy who underwent cardiac catheterization within 3 months of an echocardiogram. Spearman's correlation coefficients were calculated to assess a correlation between LVFP and mitral inflow E/A ratio, lateral mitral E/E', and septal E/E'. Thirty-eight patients were included in the study; median age was 8.6 years old. The median LVFP was 19 mm Hg, median mean pulmonary artery pressure was 25 mm Hg, and median pulmonary vascular resistance index (PVRi) was 2.4 Wu. There was no significant correlation between LVFP or PVRi with lateral mitral E/E' or septal E/E'. There was a positive correlation between LVFP and mitral inflow E/A ratio (rs = 0.59, P < .01). In a subgroup analysis of patients with hypertrophic or restrictive cardiomyopathy, there was a negative correlation (rs = 0.56, P = .02) between the mean pulmonary artery pressure and septal A'. TDI measures of diastolic function are not reliable surrogates for LVFP, mean pulmonary artery pressures, and PVRi at catheterization in children.

We reviewed our single institutional experience with pediatric ventricular assist device (VAD) support over the last 2 decades, with an aim to improve our current management and gain an insight into the future direction. A retrospective review was conducted on all patients that had undergone VAD support between 1996 and 2017. Outcomes were analyzed based on the type of VADs, whether temporary or durable devices. Primary end points were positive outcomes, including bridge-to-transplantation, bridge-to-recovery, alive on device, and bridge-to-bridge to another VAD, or negative outcomes, including death during VAD support or in-hospital death after bridge-to-recovery. The Pediatric Interagency Registry for Mechanical Circulatory Support definition was used to classify adverse events. Overall, 201 VADs were implanted in 159 patients, with 82 (41%) and 119 (59%) being temporary and durable support, respectively. There has been a trend toward an increasing annual implant volume both with temporary and durable VADs. Positive outcomes were achieved in 80% (66 of 82) of those with temporary support, with bridge-to-recovery (53% [35 of 66]) and bridge-to-bridge to another VAD (38% [25 of 66]) being the predominant outcomes. Of those on durable support, 84% (100 of 119) achieved positive outcomes, with bridge-to-transplant (66% [78 of 119]) being the leading destination. The most notable change during the study period was the introduction of implantable continuous-flow VADs, resulting in outpatient management becoming a routine practice. No patients were discharged on VAD support before 2004, but 85% were discharged on VADs with discharge capability after 2013. The present study has demonstrated the evolutional changes of pediatric VAD support and their effect on clinical outcomes over the last 2 decades.

The Jarvik 2015 Ventricular Assist Device (VAD) (Jarvik Inc, New York, NY) is the first and currently only continuous-flow VAD specifically designed for small children, and it is being evaluated in the so-called Pump for Kids, Infants, and Neonates (PumpKIN) trial. Due to the strict inclusion criteria of the trial, there have been a group of patients who failed to meet the criteria and therefore received the Jarvik 2015 VAD under the designation of “compassionate use.” This is the same phenomenon seen previously during the Berlin Heart EXCOR trial. While we await the results of the PumpKIN trial, which will report the device performance in a strictly selected population, the compassionate use cases represent actual “real world” experiences. We describe herein our experience of two compassionate use cases. In particular, this report has a special emphasis on the power consumption and hemolysis and inflammatory lab profile of the Jarvik 2015 VAD as hemocompatibility was the primary focus of the developmental and the preclinical phases.

Arrhythmogenic cardiomyopathy (AC) is a disease that involves electromechanical uncoupling of cardiomyocytes. This leads to characteristic histologic changes that ultimately lead to the arrhythmogenic clinical features of the disease. Initially thought to affect the right ventricle predominantly, more recent data show that it can affect both the ventricles or the left ventricle alone. Throughout the recent era, diagnostic modalities and criteria for AC have continued to evolve and our understanding of its clinical features in different age groups as well as the genotype to the phenotype correlations have improved. In this review, we set out to detail the epidemiology, etiologies, presentations, evaluation, and management of AC across the age continuum.