Multicenter Experience for Early and Mid-Term Outcome of MyVal Transcatheter Pulmonary Valve Implantation.

Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has recently been reported to be implanted in pulmonary position. Myval transcatheter valve were implanted in patients with stenosed dysfunctional conduits, severe regurgitation from transannular patch or dysfunctional surgical pulmonary valves (Bioprosthesis). Procedural details and post-TPVI follow-up were analysed. Myval TPVI was used in Fifty three patients with median age of 15 years (IQR 12-19.5 years). Almost sixty percent of the patients were male, with a median weight of 50 kg (31-63 kg). Prestenting was used in more than 80 percent of patients (n = 45 patients), while 6 patients had a prior surgical valve implantation. After Myval TPVI implantation, the peak instantaneous gradient across the RVOT decreased from a median of 23.5 mmHg (IQR 10-53 mmHg) pre-procedure to 10 mmHg (IQR 5-16 mmHg) post-procedure. The median fluoroscopy time for the procedure was 35 min (IQR 23.5-44 min). The large sizes-mainly the 29-mm and 32 mm Myval (Navigator, Meril Life Sciences Pvt Ltd, India), were the most used size in 40% (n = 22) of the cases each. The median contrast volume used during the procedure was 247 mL (IQR 120-300 mL). Patients were followed for a median period of 360 days (IQR 164-525 days). At the last clinic follow-up, there was no tricuspid valve regurgitation. Moderate neo-pulmonary valve regurgitation was reported in three cases. Early experience of TPVI with MyVal is encouraging with procedural success in all patients and acceptable mid-term outcomes.

Kawarabi: Administrative Structuring of a Multicenter Research Collaborative to Study Kawasaki Disease in the Arab Countries.

Kawasaki disease (KD), the leading cause of acquired heart disease in children in developed countries, merits conducting detailed studies in Arab countries. We introduce Kawarabi, as a multicenter research collaborative effort dedicated to improving diagnosis, care, and outcome of children and adults with KD in the Arab world. During the COVID-19 pandemic, there emerged a new multisystem inflammatory syndrome in children; a disease similar to KD. This highlighted the challenges that Arab physicians face in diagnosing and managing children with KD and KD-like illnesses. Kawarabi brings together experts in North America and Arab nations to study this family of diseases in a not-for-profit, voluntary scientific collaborative setting. Bylaws addressing the vision, objectives, structure, and governance of Kawarabi were established, and vetted by the 45 organizing members in 2021. An initial scientific publication showed evidence of a decreased level of awareness of the disease in the general population, as well as the lack of access to resources available for physicians caring for children with KD in Arab countries. Kawarabi has since held several educational webinars and an inaugural yearly meeting. The groundwork for future initiatives targeted at increasing awareness and understanding of the management and the long-term outcomes of children with KD in the region was established. Data on KD in the Arab world are lacking. Kawarabi is a multicenter research collaborative organization that has the unique resources, diversified ethnic makeup, and energy, to accomplish significant advances in our understanding and management of KD and its variants.

Pharmacogenomic prediction of anthracycline-induced cardiotoxicity in children.

PURPOSE: Anthracycline-induced cardiotoxicity (ACT) is a serious adverse drug reaction limiting anthracycline use and causing substantial morbidity and mortality. Our aim was to identify genetic variants associated with ACT in patients treated for childhood cancer. PATIENTS AND METHODS: We carried out a study of 2,977 single-nucleotide polymorphisms (SNPs) in 220 key drug biotransformation genes in a discovery cohort of 156 anthracycline-treated children from British Columbia, with replication in a second cohort of 188 children from across Canada and further replication of the top SNP in a third cohort of 96 patients from Amsterdam, the Netherlands. RESULTS: We identified a highly significant association of a synonymous coding variant rs7853758 (L461L) within the SLC28A3 gene with ACT (odds ratio, 0.35; P = 1.8 × 10(-5) for all cohorts combined). Additional associations (P < .01) with risk and protective variants in other genes including SLC28A1 and several adenosine triphosphate-binding cassette transporters (ABCB1, ABCB4, and ABCC1) were present. We further explored combining multiple variants into a single-prediction model together with clinical risk factors and classification of patients into three risk groups. In the high-risk group, 75% of patients were accurately predicted to develop ACT, with 36% developing this within the first year alone, whereas in the low-risk group, 96% of patients were accurately predicted not to develop ACT. CONCLUSION: We have identified multiple genetic variants in SLC28A3 and other genes associated with ACT. Combined with clinical risk factors, genetic risk profiling might be used to identify high-risk patients who can then be provided with safer treatment options.

Facilitated cardiac recovery in fulminant myocarditis: pediatric use of the Impella LP 5.0 pump.

We describe the successful use of the Impella LP 5.0 intracardiac microaxial pump (Abiomed, Danvers, MA) in a 13-year-old boy with fulminant biopsy-proven viral myocarditis. The patient, who previously was in refractory cardiogenic shock despite increasing inotropic and vasopressor support, immediately stabilized after Impella LP 5.0 implantation and was successfully bridged to a full recovery. Months later, he remains completely well, with no intracardiac or peripheral vascular sequelae of the procedure. In carefully selected pediatric patients the Impella may be a beneficial form of temporary mechanical circulatory support for fulminant cardiogenic shock.