Syed Murfad Peer

Surgical cardiac denervation therapy for treatment of congenital ion channelopathies in pediatric patients: a contemporary, single institutional experience.

Background: Congenital ion channel disorders, including congenital long QT syndrome (LQTS), cause significant morbidity in pediatric patients. When medication therapy does not control symptoms or arrhythmias, more invasive treatment strategies may be necessary. This study examines our institution’s clinical experience with surgical cardiac denervation therapy for management of these arrhythmogenic disorders in children. Methods: An institutional review board–approved retrospective review identified ten pediatric patients with congenital ion channelopathies who underwent surgical cardiac denervation therapy at a single institution between May 2011 and April 2014. Eight patients had a diagnosis of congenital LQTS, two patients were diagnosed with catecholaminergic polymorphic ventricular tachycardia (CPVT). All patients underwent sympathectomy and partial stellate ganglionectomy via video-assisted thoracoscopic surgery (VATS). Results: Six of the ten patients had documented ventricular arrhythmias preoperatively, and 70% of the patients had preoperative syncope. The corrected QT interval decreased in 75% of patients with LQTS following sympathectomy. Postoperative arrhythmogenic symptoms were absent in 88% of congenital LQTS patients, but both patients with CPVT continued to have symptoms throughout the duration of follow-up. All patients were alive after a median follow-up period of 10 months. Conclusions: Surgical cardiac denervation therapy via VATS is a useful treatment strategy for congenital LQTS patients who fail medical management, and its potential benefit in the management of CPVT is unclear. A prospective comparison of the efficacy of surgical cardiac denervation therapy and implantable cardioverter-defibrillator use in congenital ion channelopathies is timely and crucial.

Tricuspid atresia with aortopulmonary window: challenges in achieving a balanced circulation.

Tricuspid atresia and aortopulmonary window are rare congenital cardiac anomalies. The occurrence of both these anomalies in the same patient is extremely rare, with only 1 case reported in the literature. We report the surgical management of one such patient and discuss the management issues with respect to Stage 1 single ventricle palliation.

Outcomes of Tracheostomy Following Congenital Heart Surgery: A Contemporary Experience

INTRODUCTION Following congenital heart surgery, pediatric patients may experience persistent respiratory failure that requires tracheostomy placement. Currently, definitive knowledge of the optimal timing for tracheostomy placement in this patient population is lacking. METHODS An 8-year retrospective review of 17 pediatric patients who underwent congenital heart surgery and subsequently required tracheostomy placement was performed. Patients were evaluated with regard to the timing of tracheostomy and mortality. RESULTS The overall study mortality was 24%. The median duration of intubation prior to tracheostomy was 60 days (interquartile range: 19-90 days); there was no difference in the average time between intubation and tracheostomy for survivors compared with nonsurvivors (51 vs. 73 days, P = .37). No difference was observed in the overall duration of positive pressure ventilation when tracheostomy was performed within 30 days of intubation compared with greater than 30 days following intubation (481 vs. 451 days, P = .88). Overall, 18% of patients were successfully weaned from the ventilator after a median duration of positive pressure ventilation of 212 days. CONCLUSION The timing of tracheostomy placement may be an important factor in clinical outcomes for pediatric patients with persistent dependence on mechanical ventilatory support following congenital heart surgery. A larger, multi-institution study may help further elucidate our observed clinical findings in this patient population.

Intermediate-Term Results of Extracorporeal Membrane Oxygenation Support Following Congenital Heart Surgery

Background: Although there are considerable data regarding in-hospital results of congenital heart surgery (CHS) patients requiring postoperative extracorporeal membrane oxygenation (ECMO) support, there is limited information on intermediate-term outcomes. Methods: A single-institution retrospective review of 25 consecutive postoperative CHS patients who required ECMO and survived to hospital discharge between January 2003 and June 2008. Survival was estimated by the Kaplan-Meier method. Results: At a median follow-up of 3.3 years (interquartile range: 1.2-5.9 years), there was one death which occurred at six months postsurgery. Kaplan-Meier-estimated survival at three years was 95% (95% confidence interval: 90%-100%). Indications for ECMO included extracorporeal cardiopulmonary resuscitation (48%), systemic hypoxia (4%), postoperative low-cardiac output syndrome (28%), and intraoperative failure to wean off cardiopulmonary bypass (20%). Following ECMO support, 65% of patients had unplanned cardiac reinterventions (three requiring operative interventions, six requiring percutaneous interventions, and four requiring both), and 47% of patients required unplanned hospitalizations. In all, 29% of patients developed neurological deficits and 12% of patients developed chronic respiratory failure. No patients developed renal failure. Overall, systemic ventricular (SV) function normalized in 83% of patients, whereas 17% of patients had persistent mild-to-moderate SV dysfunction. Conclusions: Intermediate-term patient survival of ECMO following CHS is encouraging. However, neurological impairment and unplanned cardiac reinterventions remain significant concerns. Further delineation of risk factors to improve patient outcomes is warranted.

Ethical Dilemma Offering Short-Term Extracorporeal Membrane Oxygenation Support for Terminally Ill Children Who Are Not Candidates for Long-Term Mechanical Circulatory Support Or Heart Transplantation

The use of extracorporeal membrane oxygenation (ECMO) in terminally ill pediatric patients who are not candidates for long-term mechanical circulatory support or heart transplantation requires careful deliberation. We present the case of a 16-year-old female with a relapse of acute lymphoid leukemia and acute-on-chronic cardiomyopathy who received short-term ECMO therapy. In addition, we highlight several ethical considerations that were crucial to this patient’s family-centered care and demonstrate that this therapy can be accomplished in a manner that respects patient autonomy and family wishes.

Patency of common carotid artery and internal jugular vein after a simple vessel sparing cannulation for extracorporeal membrane oxygenation support.

BACKGROUND Common carotid artery and internal jugular vein are commonly cannulated for establishment of peripheral venoarterial extracorporeal membrane oxygenation (VA ECMO) support. We present our results of a vessel sparing cannulation technique for neck vessels, which helps maintain vessel patency after decannulation. METHODS All patients who underwent ECMO, between January 2004 and January 2013 at a single center, were retrospectively reviewed. Follow up data for the patency of common carotid artery (CCA) and internal jugular vein (IJV) after decannulation were recorded. RESULTS Twenty-four consecutive patients who were successfully decannulated after VA ECMO support who underwent vessel sparing cannulation were retrospectively reviewed. Follow up data were unavailable in 4 and 1 patient did not survive. Amongst the remaining 19 patients the median duration of ECMO support in the remaining was 7 (IQR; 4-10) days. Follow up studies documenting vessel patency were available for IJV in 18 patients and CCA in 14 patients. At a median follow up of 137days (IQR; 35-7240) 15 (78%) patients had patent IJVs and 14 (100%) patients had patent CCAs. CONCLUSION The simple vessel sparing technique is effective in allowing restoration of the patency of the neck vessels after ECMO decannulation. LEVEL OF EVIDENCE Case series with no comparison group (Level IV).

Rapid Response Extracorporeal Membrane Oxygenation Deployment—Surgical Technique:

The availability of extracorporeal membrane oxygenation (ECMO) is the standard of care at most high-acuity congenital heart surgery programs. Extracorporeal membrane oxygenation is also an integral part of managing children with in-hospital cardiac arrest refractory to conventional cardiopulmonary resuscitation (extracorporeal cardiopulmonary resuscitation). In this article, we aim to provide a practical guide to initiate rapid ECMO in a nonoperating room setting.

eReply. Re: Balancing the pulmonary circulation in tricuspid atresia with an aortopulmonary window

We thank Dr Sainathan for his eComment on our article [1, 2]. The author suggests an alternative strategy of placing bilateral branch pulmonary artery bands while leaving the aortopulmonary (AP) window intact as was used by Hosein et al. [3] for the management of patients with hypoplastic left heart syndrome (HLHS). While there may be different ways of managing rare complex anatomies such as tricuspid atresia and aortopulmonary window, in addition to the surgical strategy, we once again would like to highlight the interesting physiology in this patient, which we believe is related to ventricular volume unloading following AP window ligation leading to restriction at the level of the ventricular septal defect. We have successfully used bilateral branch pulmonary artery bands as a bridge to decision in high-risk patients with HLHS [4]. While this approach is to be recommended in certain selected patients, it is not a procedure free of problems, as Hosein et al. themselves in their report have accepted as ‘fundamentally flawed’ [2]. Conflict of interest: none declared