Use of the Occlutech pmVSD Device for Hybrid Closure of a Large Mid-Muscular Ventricular Septal Defect in a Small Infant Born With Hypoplastic Aortic Arch.

We describe the perventricular device closure of a large mid-muscular ventricular septal defect (VSD) in a 2.9 kg infant born with hypoplastic aortic arch and VSD using an Occlutech perimembranous occluder. In this case, the anatomy required a short low-profile device and hence a perimembranous occluder was used. To our knowledge, this is the first described use of this device for hybrid closure of a muscular VSD and the application of this technique in a patient <3 kg.

Is there a role for endovascular stent implantation in the management of postoperative right ventricular outflow tract obstruction in the era of transcatheter valve implantation?

BACKGROUND: The optimal management pathway for the dysfunctional right ventricular outflow tract (RVOT) is uncertain. We evaluated the long-term outcomes and clinical impact of stent implantation for obstructed RVOTs in an era of rapidly progressing transcatheter pulmonary valve technology. METHODS: Retrospective review of 151 children with a biventricular repair who underwent stenting of obstructed RVOT between 1991 and 2017. RESULTS: RVOT stenting resulted in significant changes in peak right ventricle (RV)-to-pulmonary artery (PA) gradient (39.4 ± 17.1-14.9 ± 8.3; p < 0.001) and RV-to-aortic pressure ratio (0.78 ± 0.22-0.49 ± 0.13; p < 0.001). Subsequent percutaneous reinterventions in 51 children to palliate recurrent stenosis were similarly effective. Ninety-nine (66%) children reached the primary outcome of subsequent pulmonary valve replacement (PVR). Freedom from PVR from the time of stent implantation was 91%, 51%, and 23% at 1, 5, and 10 years, respectively. Small balloon diameters for stent deployment were associated with shorter freedom from PVR. When additional children without stent palliation (with RV-to-PA conduits) were added to the stent cohort (total 506 children), the multistate analysis showed the longest freedom from PVR in those with stent palliation and subsequent catheter reintervention. Pulmonary regurgitation was well-tolerated clinically. Indexed RV dimensions and function estimated by echocardiography remained stable at last follow up or before primary outcome. CONCLUSION: Prolongation of conduit longevity with stent implant remains an important strategy to allow for somatic growth to optimize the risk-benefit profile for subsequent surgical or transcatheter pulmonary valve replacement performed at an older age.

Adrenergic receptor genotypes influence postoperative outcomes in infants in the Single-Ventricle Reconstruction Trial.

OBJECTIVES: Adrenergic receptor (ADR) genotypes have been associated with adverse outcomes in heart failure. Our objective was to evaluate the association of ADR genotypes with post-Norwood outcomes in infants with hypoplastic left heart syndrome (HLHS). METHODS: Infants with HLHS participating in the Pediatric Heart Network Single-Ventricle Reconstruction Trial underwent genotyping for 4 single-nucleotide polymorphisms in 3 ADR genes: ADRB1_231A/G, ADRB1_1165G/C, ADRB2_5318C/G, and ADRA2A_2790C/T. The association of genotype with freedom from serious adverse events (SAEs) (death, transplant, extracorporeal membrane oxygenation, cardiopulmonary resuscitation, acute shunt failure, unplanned reoperations, or necrotizing enterocolitis) during 14 months' follow-up was assessed with Cox regression and the association with post-Norwood complications was assessed with Poisson regression. Models were adjusted for clinical and surgical factors. RESULTS: The study included 351 eligible patients (62% male; 83% white). The mean age at Norwood procedure was 5.6 ± 3.6 days. A total of 152 patients had SAEs during 14-month follow-up including 84 deaths and 10 transplants. ADRA2A_2790CC genotype had lower SAE-free survival compared with CT/TT genotypes during follow-up (Log rank test, P = .02), and this association was independent of clinical and surgical risk factors (adjusted Cox regression, hazard ratio 1.54 [95% confidence interval 1.04, 2.30] P = .033). Post-Norwood complication rate did not differ by genotype. CONCLUSIONS: Infants with HLHS harboring ADR genotypes that are associated with greater catecholamine release or sensitivity had lower event-free survival after staged palliation. Excess catecholamine activation may adversely affect cardiovascular adaptation after the Norwood procedure. Future studies should explore whether targeting adrenergic activation in those harboring risk genotypes can improve outcomes. (ClinicalTrials.gov number NCT00115934).

Longitudinal assessment of myocardial function in childhood chronic kidney disease, during dialysis, and following kidney transplantation.

BACKGROUND: Childhood chronic kidney disease (CKD) and dialysis are associated with increased long-term cardiovascular risk. We examined subclinical alterations in myocardial mechanics longitudinally in children with CKD, during dialysis, and following renal transplantation. METHODS: Forty-eight children with CKD (stage III or higher) who received kidney transplants from 2008 to 2014 were included in a retrospective study and compared to 192 age- and sex-matched healthy children. Measurements of cardiac systolic and diastolic function were performed, and global longitudinal strain (GLS) and circumferential strain (GCS) were measured by speckle-tracking echocardiography at CKD, during dialysis, and 1 year following kidney transplantation. Mixed-effects modeling examined changes in GLS and GCS over different disease stages. RESULTS: Children with CKD had a mean age of 10 ± 5 years and 67% were male. Eighteen children received preemptive transplantation. Children with CKD had increased left ventricular mass, lower GLS, and impaired diastolic function (lower E/A ratio and E' velocities) than healthy children. Changes in left ventricular diastolic parameters persisted during dialysis and after renal transplantation. Dialysis was associated with reduced GLS compared to CKD (ß = 1.6, 95% confidence interval 0.2-3.0); however, this was not significant after adjustment for systolic blood pressure and CKD duration. Post-transplantation GLS levels were similar to those at CKD assessment. GCS was unchanged during dialysis but significantly improved following transplantation. CONCLUSIONS: There are differences in diastolic parameters in childhood CKD that persist during dialysis and after transplantation. Systolic parameters are preserved, with significant improvement in systolic myocardial deformation following transplantation. The impact of persistent diastolic changes on long-term outcomes requires further investigation.