Anthony Trela

Laparoscopic Nissen Fundoplication During Gastrostomy Tube Placement and Noninvasive Ventilation May Improve Survival in Type I and Severe Type II Spinal Muscular Atrophy

Progressive respiratory muscle weakness with bulbar involvement is the main cause of morbidity and mortality in type I and severe type II spinal muscular atrophy. Noninvasive positive pressure ventilation techniques coupled with laparoscopic gastrointestinal procedures may allow for improved morbidity and mortality. The authors present a series of 7 spinal muscular atrophy patients (6 type I and 1 severe type II) who successfully underwent laparoscopic gastrostomy tube insertion coupled with Nissen fundoplication and early postoperative extubation using noninvasive positive pressure ventilation techniques. The authors measured the length of survival and the frequencies of pneumonia and hospitalization before and after surgery as outcomes of these new surgical and medical interventions. All 7 patients had respiratory symptoms (unmanageable oropharyngeal secretions, cough, pneumonia), difficulty feeding, and weight loss. Six patients had documented reflux via diagnostic testing preoperatively. Five patients were on noninvasive positive pressure ventilation and other supportive respiratory therapies prior to surgery. All 7 patients survived the procedures. By August 2006, 5 patients with type I and 1 with severe type II spinal muscular atrophy were alive and medically stable at home 1.5 months to 41 months post-op. One patient with type I expired approximately 5 months post-op due to obstructive apnea. This case series demonstrates that laparoscopic gastrostomy tube placement coupled with Nissen fundoplication and noninvasive positive pressure ventilation can be successfully used as a treatment option to allow for early postoperative extubation and to optimize quality of life in type I and severe type II spinal muscular atrophy patients.

Ventricular pacing in single ventricles—A bad combination

BACKGROUND Chronic ventricular pacing (VP) is associated with systolic dysfunction in a subset of pediatric patients with heart block and structurally normal hearts. The effect of chronic VP in congenital heart disease is less well understood, specifically in the single-ventricle (SV) population. OBJECTIVE To determine the longitudinal effect of VP in SV patients. METHODS SV patients with heart block and dual-chamber pacemakers requiring >50% VP were compared with nonpaced (controls) SV patients matched for age, sex, and SV morphology. Patients were excluded if a prepacing echocardiogram was not available. Echocardiogram and clinical parameters were compared at baseline (prepacing) and at last follow-up in the paced group, and in controls when they were at ages similar to those of their paced-group matches. RESULTS Twenty-two paced and 53 control patients from 2 institutions were followed for similar durations (6.6±5 years vs 7.6±7.6 years; P = .59). There was no difference between groups regarding baseline ventricular function or the presence of moderate-to-severe atrioventricular valvar regurgitation (AVVR). Paced patients were more likely to develop moderate-to-severe systolic dysfunction (68% vs 15%; P < .01) and AVVR (55% vs 8%; P < .001) and require heart failure medications (65% vs 21%; P < .001). Chronic VP was also associated with a higher risk of transplantation or death (odds ratio, 4.9; 95% confidence interval, 1.05-22.7; P = .04). CONCLUSIONS SV patients requiring chronic VP are at higher risk of developing moderate-to-severe ventricular dysfunction and AVVR with an increased risk of death or transplantation compared with controls. New strategies to either limit VP or improve synchronization in this vulnerable population is imperative.

Is There a Difference in Tachycardia Cycle Length during SVT in Children with AVRT and AVNRT

There are limited adult data suggesting the tachycardia cycle length (TCL) of atrioventricular reentry tachycardia (AVRT) is shorter than atrioventricular nodal reentry tachycardia (AVNRT), though little data exist in children. We sought to determine if there is a difference in TCL between AVRT and AVNRT in children.

50 is the new 70: Short ventriculoatrial times are common in children with atrioventricular reciprocating tachycardia

BACKGROUND One of the basic electrophysiological principles of atrioventricular reciprocating tachycardia (AVRT) is that ventriculoatrial (VA) times during tachycardia are >70 ms. We hypothesized, however, that children may commonly have VA times <70 ms in AVRT. OBJECTIVE This study sought to determine the incidence and characteristics associated with short-VA AVRT in children. METHODS A retrospective single-center review of children with AVRT from 2000 to 2014 was performed. All patients ≤18 years of age with AVRT at electrophysiology study were included. Patients with persistent junctional reciprocating tachycardia, atrioventricular nodal reentry tachycardia, and tachycardia not unequivocally proven to be AVRT were excluded. VA time was defined as the time between earliest ventricular activation and earliest atrial activation in any lead and was confirmed by 2 electrophysiologists. Patients with VA times <70 ms (SHORT-VA) and those with standard VA times ≥70 ms (STD-VA) were compared. Logistic regression analysis identified characteristics of SHORT-VA patients. RESULTS A total of 495 patients with AVRT were included (mean age 11.7 ± 4.1 years). There were 265 patients (54%) with concealed accessory pathways (APs) and 230 (46%) with Wolff-Parkinson-White syndrome. AP location was left-sided in 301 patients (61%) and right-sided in 194 (39%). The mean VA time in AVRT was 100 ± 33 ms. A total of 63 patients (13%) had VA times <70 ms (SHORT-VA). The shortest VA time during AVRT was 50 ms. There was no difference in age, AV nodal block cycle, or body surface area between SHORT-VA and STD-VA patients, but SHORT-VA patients had lower weight (43 ± 17 vs 51 ± 23 kg, P = .02), lower AV nodal effective refractory period (AVNERP; 269 ± 50 vs 245 ± 52 ms, P < .01), and more left-sided APs (50 [79%] vs 251 [58%]; P < .01]. On multivariate logistic regression, factors associated with SHORT-VA included left-sided AP (odds ratio [OR] 5.79, confidence interval [95% CI] 2.21-15.1, P < .01), shorter AVNERP (OR 0.99, CI 0.98-0.99, P < .01), and lower weight (OR 0.97, CI 0.95-0.99, P < .01). CONCLUSIONS Children with AVRT can frequently have VA times <70 ms, with 50 ms being the shortest VA time. This finding debunks the classic electrophysiology principle that VA times in AVRT must be >70 ms. SHORT-VA AVRT was more common in children with left-sided APs.

Antibiotic Prophylaxis Practices in Pediatric Cardiac Implantable Electronic Device Procedures: A Survey of the Pediatric And Congenital Electrophysiology Society (PACES)

Cardiac implantable electronic device (CIED) infections are associated with significant morbidity in the pediatric device population, with a tenfold higher risk of infection in children compared to adults. The 2010 American Heart Association (AHA) guidelines recommend a single dose of systemic antibiotic (ABX) prophylaxis prior to CIED implantation and no post-operative (OP) ABX. However, there is limited data regarding adherence to this recommendation among the pediatric community. To assess current clinical practices for CIED ABX prophylaxis in pediatrics; whether the AHA guidelines are being followed; and if not, the reasons for non-adherence. An anonymous web-based survey was sent to physician members of the Pediatric And Congenital Electrophysiology Society regarding ABX prophylaxis for new CIED implants and reoperations. 75 (25%) members responded. Only 7% of respondents follow the 2010 AHA guidelines. While all respondents give pre-OP IV ABX, 64% routinely treat patients with 24-h post-OP IV ABX with additional oral or IV therapy. 69% of respondents are cognizant of the guidelines but 88% of those cognizant do not follow the guidelines for a variety of reasons including lack of data and different substrate (pediatric patients). 79% stated that pediatric-specific data would be required for them to change their practice and follow the published guidelines. The majority of pediatric EP physicians who responded to this survey do not follow the current AHA guidelines on ABX prophylaxis and administer post-OP ABX. Most pediatric EP physicians believe that the increased risk of infection in children merits additional ABX.

IS THERE A DIFFERENCE IN TACHYCARDIA CYCLE LENGTH DURING SUPRAVENTRICULAR TACHYCARDIA IN CHILDREN WITH ATRIO-VENTRICULAR RECIPROCATING TACHYCARDIA AND ATRIO-VENTRICULAR NODAL RE-ENTRY TACHYCARDIA?

Atrio-ventricular reciprocating tachycardia (AVRT) and Atrio-ventricular nodal re-entry tachycardia (AVNRT) are the two most common forms of Supraventricular Tachycardia (SVT) in children. There is limited adult data suggesting the tachycardia cycle length (TCL) of AVRT is shorter than AVNRT, though