Aortic valve replacement using On-X valve in a child postorthotopic heart transplantation.

A 20-month-old girl presented with severe dilated cardiomyopathy and decompensated congestive cardiac failure. Despite escalating inotropic and mechanical ventilation support, she required placement on extracorporeal membrane oxygenation and transfer to the transplant centre in Newcastle, England. She was placed on biventricular assist device and then Berlin Heart but failed to show any recovery of ventricular function. She underwent orthotopic heart transplantation at 2 years of age. She developed bacterial endocarditis with Enterococcus faecalis resulting in severe aortic valve regurgitation requiring aortic valve replacement with a 19 mm On-X valve (Airtivion) 11 days after her transplant. Given the size of the donor heart, it was possible to implant a 19-mm valve in this 12 kg child with minimal risk of patient prosthesis mismatch. She was anticoagulated with warfarin (On-X valve INR 2-3 for first 3 months; INR 1.5-2.0 thereafter). Although she suffered several other post-operative complications, including malabsorption, nasojejunal feeding, liver dysfunction, vertebral fractures, renal impairment and renal calcification, and need for repeat opening of her tracheostomy site following her initial decannulation, her aortic valve function has remained stable.

Nicolaides-Baraitser syndrome in a patient with hypertrophic cardiomyopathy and SMARCA2 gene deletion.

Nicolaides-Baraitser syndrome is a rare, neuro-developmental disorder caused by heterozygous pathogenic variants in the SMARCA2 gene, involved with chromatin regulation. Cardinal features include intellectual disability, short stature, microcephaly, triangular facies, sparse hair, brachydactyly, prominent interphalangeal joints and seizures. Genetic testing demonstrated a loss within SMARCA2 at 9p24.3 inclusive of basepairs 2094861_2141830 (hg19) in our patient. This case highlights a child with Nicolaides-Baraiter syndrome, a SMARCA2 gene deletion and a novel association of hypertrophic obstructive cardiomyopathy.

The Use of Virtual Reality Echocardiography in Medical Education.

Virtual reality (VR) is a relatively new technology that allows an individual to experience a virtual world. This new immersive video type may be of particular usefulness in procedure-based healthcare settings. We hypothesized that VR echocardiography was non-inferior to live demonstration. Our aim was to assess the usefulness of a VR echocardiographic approach in teaching echocardiography to pediatric trainees compared to live demonstration. This was a single center, cross-sectional observational design. We used a Garmin VIRB® 360 and a head-mount display to record live echocardiography exams in a pediatric population. An Oculus Go™ was used to view the 360° immersive/VR videos. Trainees responded to a written questionnaire afterwards. Fifteen trainees participated in the study, each of whom had previously seen echocardiography through live demonstration teaching. Eleven respondents had previous hands-on echocardiography experience. All 15 participants confirmed that VR echocardiography is a useful teaching tool with 87% (n = 13) rating it as good or very good on a 5-point Likert scale. When asked to compare VR to live demonstration, 67% (n = 10) rated VR echocardiography as the same or better than live demonstration. One of the participants reported a side effect, namely mild and self-resolving dizziness. VR echocardiography is a safe, inexpensive and practical way for trainees to learn echocardiography. The addition of VR echocardiography to the arsenal of teaching tools may enrich the learning experience for trainees.