Comprehensive management of type A acute aortic dissection in the paediatric population with ACTA 2 mutation: case reports and literature review of paediatric aortic dissection.

Acute aortic dissection in the paediatric population is rare but lethal. We present two paediatric cases of type A acute aortic dissection that required emergent procedures and were later found to have genetic mutations. High index of suspicion, early clinical diagnosis, prompt treatment, the advantageous collaboration between the paediatric team and aortic surgeons, and familial genetic testing are paramount to achieve a good outcome.

Influence of early extubation on post-operative outcomes after pediatric lung transplantation.

Lung transplantation has become an accepted therapeutic option for a select group of children with end-stage lung disease. We evaluated the impact of early extubation in a pediatric lung transplant population and its post-operative outcomes. Single-center retrospective study. PICU within a tertiary academic pediatric hospital. Patients <22 years after pulmonary transplant between January 2011 and December 2016. A total of 74 patients underwent lung transplantation. The primary pretransplantation diagnoses included cystic fibrosis (58%), pulmonary fibrosis (9%), and surfactant dysfunction disorders (10%). Of 60 patients, 36 (60%) were extubated within 24 hours and 24 patients after 24 hours (40%). A total of seven patients (11.6%) required reintubation within 24 hours. Median length of stay for the early extubation group was shorter at 3 days ([(IQR) 2.2-4.7]) compared to 5 days (IQR, 3-7) (P = .02) in the late extubation group. Median costs were lower for the early extubation group with 13,833 US dollars (IQR, 9980-22,822) vs 23 671 US dollars (IQR, 16 673-39 267) (P = .043). Fourteen patients were in the PICU prior to their transplantation; this did not affect their early extubation success. Neither did the fact of requiring invasive or non-invasive mechanical ventilation before transplantation. Early extubation appears to be safe in a pediatric population after lung transplantation and is associated with a shorter LOS and decreased hospital costs. It may prevent known complications associated with mechanical ventilation.

Waiting list outcomes in pediatric lung transplantation: Poor results for children listed in adult transplant programs.

BACKGROUND: Low case volume has been associated with lower survival after pediatric lung transplantation. Our aim was to analyze waitlist outcomes among pediatric lung transplant centers in the USA. METHODS: We studied a cohort of 1,139 pediatric candidates listed in the Organ Procurement and Transplantation Network for lung transplantation between 2002 and 2014. Of these candidates, 720 (63.2%) received a transplant. Candidates were divided into groups according to the clinical activity of the center of listing: high-volume pediatric (≥4 transplants per year); low-volume pediatric (<4 transplants per year); and adult (transplant volume predominantly in adults). We used multivariate Cox regression analysis to identify independent risk factors for waitlist mortality. We also determined the transplant rate-or likelihood of transplant after listing-over the study period. RESULTS: Fifty-eight percent of the children and adolescents were listed in adult centers where the resultant transplant rate was low-only 42% received a transplant compared with 93% in pediatric programs. Listing in an adult program was also the most significant risk factor for death on the waiting list (hazard ratio 15.6, 95% confidence interval 5.8 to 42.1). CONCLUSIONS: Most children (58%) are listed for lung transplantation in adult centers and have a reduced rate of transplantation and a greater chance of waitlist mortality.

Extubation in the operating room after Fontan's procedure: effect on practice and outcomes.

BACKGROUND: Timely extubation is a well-accepted strategy in the postoperative intensive care unit management of Fontan patients to minimize the deleterious effects of positive-pressure ventilation. In October 2002, this strategy was extended to extubating selective Fontan patients in the operating room (EOR). This retrospective study examines how EOR has affected outcomes and practice in our Fontan population. METHODS: Between October 2002 and June 2006, 112 patients underwent primary Fontan procedures; 38 (34%) were EOR and 74 (66%) were non-EOR. These two cohorts were not different (p < 0.05) in age, weight, surgery time, dominant ventricular morphology, hypoplastic left heart syndrome, prior bidirectional Glenn, concomitant procedures, atrioventricular valve regurgitation, and ventricular function. Analysis of variance was used to compare mean pulmonary artery pressure, mean arterial blood pressure, and mean common atrial pressure as a function of time. RESULTS: During the first 12 hours postoperatively, mean pulmonary artery pressure and mean common atrial pressure were significantly lower and mean arterial blood pressure was significantly higher in the EOR group than the non-EOR group (p < 0.05). No EOR patient required reintubation. Mean durations of inotropic agents (1.1 versus 2.4 days), chest tubes (5.8 versus 7.2 days), intensive care unit stay (3 versus 4.7 days), and hospital stay (8.6 versus 11.3 days) for EOR patients were shorter than for non-EOR patients (p < 0.05). Intensive care unit and hospital costs for EOR patients were 35% and 31% lower, respectively, than for non-EOR patients (p < 0.05). Kaplan-Meier survival for EOR patients (3 years, 100%) was not different (p = 0.3) than for non-EOR patients (1 and 3 years, 96%). CONCLUSIONS: After the Fontan procedure, selective EOR can be performed safely and improves postoperative hemodynamics, decreases hospital resource utilization, and reduces hospital recovery time.

Preliminary experience with the MicroMed DeBakey pediatric ventricular assist device.

Mechanical circulatory support for both acute and chronic heart failure is a widely applied therapeutic option in the adult population with a variety of devices clinically available. Technology in this field has advanced sufficiently such that long-term support or "destination therapy" has become a generally accepted reality. Similar progress has not occurred in the field of device support for heart failure in children. While the number of potential patients is significantly lower in the pediatric population, the clinical relevance and poignancy of individual need are nonetheless real. Until recently, children with heart failure have been largely disadvantaged in comparison to their adult counterparts. The DeBakey VAD Child (MicroMed Technology, Inc, Houston, TX) represents a hopeful initial step in the direction of reducing the technological gap between adults and children. While the clinical experience with this device is limited at present, preliminary results are encouraging. This report will provide an overview of the DeBakey VAD Child, including device specifications, indications for clinical use, surgical and postoperative considerations, and updated clinical experience.