Effect of preoperative extracorporeal membrane oxygenation therapy on postventricular assist device outcomes: an analysis of the STS Pedimacs database.

OBJECTIVES: Extracorporeal membrane oxygenation (ECMO) support prior to ventricular assist device (VAD) therapy is frequently used for stabilizing INTERMACS 1 and 2 paediatric patients. Data regarding outcomes with this strategy is limited. METHODS: Patient characteristics and outcomes post-VAD therapy with and without preop ECMO support were compared. Survival and risk factor analysis was performed for all patients and INTERMACS profile 1 subgroup. RESULTS: Of 541 INTERMACS 1 and 2 patients enrolled in Paediatric Interagency Registry for Mechanical Circulatory Support registry, 391 received primary VAD implantation and ECMO prior to VAD therapy was utilized in 150 patients. Younger age (P < 0.0001) and shock (P < 0.0001) were more common in group 2, with lower survival at 1, 6 and 12 months compared to group 1 (P < 0.0001). Freedom from infection (P = 0.03) was higher in group 1. Freedom from stroke (P = 0.7) was similar. Paracorporeal continuous flow devices led to poor survival in both groups 1 and 2 (P = 0.4). Implantable continuous flow (P < 0.0001) and paracorporeal pulsatile devices (P = 0.007) had better survival in primary VAD group. INTERMACS profile 1, lower weight, hepatic dysfunction, renal failure, paracorporeal continuous and percutaneous devices were associated with higher mortality. Preop ECMO therapy was not associated with higher mortality (P = 0.12). Survival of INTERMACS profile 1 patient was similar at 1, 6 and 12 months in both groups (P = 0.1). CONCLUSIONS: INTERMACS profile 1, lower weight, hepatic dysfunction, renal failure and use of paracorporeal continuous flow or percutaneous assist devices are associated with a higher postoperative mortality. Preop ECMO therapy however is not independently associated with higher postop mortality.

Periocardiophrenic Release to Prevent Conduit Compression After Neonatal Truncus Arteriosus Repair.

Sternal approximation postcardiac surgery in neonates can sometimes be challenging. Neonatal truncus arteriosus repair using a right ventricle-to-pulmonary artery homograft conduit is one such surgical procedure wherein there is a risk of developing conduit compression after sternal closure. We describe our technique of pericardiophrenic release at the time of delayed sternal closure to prevent hemodynamic compromise and conduit compression after sternal approximation in such cases.

Right Atrial Cannulation via Thoracotomy for Emergent Extracorporeal Membrane Oxygenation in Pediatric Patients with Prior Sternotomy.

Extracorporeal membrane oxygenation (ECMO) is the most common mechanical circulatory support strategy used to treat pediatric patients presenting with low cardiac output or cardiogenic shock. While transthoracic central cannulation is feasible and mostly utilized for early postoperative support, peripheral cannulation is preferred as a primary strategy in the late postoperative period. Redo-sternotomy and venous cannulation are difficult to achieve in patients with occluded peripheral veins or complex venous anatomy like Glenn circulation. In pediatric patients with multiple prior sternotomy and catheterization procedures, vascular access for cannulation is frequently limited. Peripheral cannulation for venoarterial ECMO (VA-ECMO) may be challenging or even impossible. In our case series, four pediatric patients with prior sternotomy underwent right atrial cannulation emergently in patients to secure venous drainage for ECMO support. Extracorporeal membrane oxygenation support could be established rapidly with adequate venous drainage in all cases. We conclude that right atrial cannulation via right thoracotomy can be a useful technique for venous cannulation in cases with prior sternotomy and is particularly useful in cases with Glenn circulation.

Mechanical Circulatory Support for the Failing Fontan: Conversion to Assisted Single Ventricle Circulation-Preliminary Observations.

BACKGROUND: Mechanical circulatory support (MCS) of a failing Fontan circulation remains challenging. We hypothesized that MCS can be provided by converting the Fontan circulation into a mechanically assisted single ventricle parallel circulation (MASVC). METHODS: A porcine model of functionally univentricular circulation was created under cardiopulmonary bypass (CPB) by performing an atrial septectomy, tricuspid valvectomy, and interrupting antegrade pulmonary blood flow. A centrifugal flow pump was placed with inflow from the common atrium. Eight millimeter Dacron grafts anastomosed to the ascending aorta and main pulmonary artery supplied systemic (Qs) and pulmonary (Qp) blood flow. Ultrasonic flow probes were used to measure Qs and Qp after weaning from CPB. The Qp/Qs ratio was regulated using an adjustable clamp. Hemodynamic and laboratory data were recorded. RESULTS: All four animals were successfully weaned from CPB onto the MASVC for a duration of two hours. Mechanically assisted single ventricle parallel circulation achieved satisfactory hemodynamics. As anticipated, the arterial oxygen saturation and partial pressure of oxygen in arterial blood were lower in the MASVC compared to baseline biventricular circulation. At the conclusion of the study, there was a trend towards a decrease in the mixed venous saturation with increasing oxygen extraction compared to the baseline. Serum lactate levels increased after weaning from CPB and did not return to baseline after two hours of support. CONCLUSION: Mechanically assisted single ventricle parallel circulation can be established in a single ventricle animal model. This strategy could potentially provide MCS of a single ventricle circulation. Studies with longer duration of support are required to assess adequacy of support and long-term sustainability.

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.

Choice of Peripheral Venoarterial Extra-Corporeal Membrane Oxygenation Cannulation Site in Patients Above 15 kilograms.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is a life-saving measure for pediatric patients with cardiopulmonary failure. The option of cannulating neck vessels versus those of the groin exists for patients over 15 kg; however, each carries the risk for complications. We present a single-center experience comparing the risks and benefits of these alternate peripheral ECMO cannulation sites. METHODS: All pediatric patients supported by venoarterial (VA) ECMO via peripheral cannulation from January 2006 to May 2013 were retrospectively reviewed, excluding those weighing less than 15 kg. We compared complications of neck versus groin cannulation sites, including neurologic dysfunction, limb ischemia, and in-hospital mortality. Univariate analysis and multivariable logistic regression were performed to identify factors for complications including mortality. RESULTS: Thirty-six patients (median age 10.8 years, interquartile range: 6.0-15.8) were cannulated for VA ECMO. Forty-four percent were cannulated via neck vessels and 47% were cannulated via groin. Nine percent were cannulated at both sites. Overall survival was 72% at decannulation and 67% at hospital discharge. No statistically significant survival difference between groin and neck cannulation subgroups was found. Rate of neurologic injury was higher in neck (25%) versus groin (12%) cannulation, but this was not statistically significant (p = 0.52). Extremity ischemia occurred in five patients having groin cannulation (29%, p = 0.04). CONCLUSIONS: Neck cannulation is a valuable alternative to groin vessels in patients above 15 kg. Risk of limb ischemia with groin cannulation is significant, and must be considered when choosing the cannulation site for ECMO support.

A contemporary, single-institutional experience of surgical versus expectant management of congenital heart disease in trisomy 13 and 18 patients.

The objective of this study was to examine a large institutional experience of patients with trisomy 13 and trisomy 18 in the setting of comorbid congenital heart disease and present the outcomes of surgical versus expectant management. It is a retrospective single-institution cohort study. Institutional review board approved this study. Thirteen consecutive trisomy 18 patients and three consecutive trisomy 13 patients (sixteen patients in total) with comorbid congenital heart disease who were evaluated by our institution's Division of Cardiovascular Surgery between January 2008 and December 2013 were included in the study. The primary outcome measures evaluated were operative mortality (for patients who received surgical management), overall mortality (for patients who received expectant management), and total length of survival during follow-up. Of the thirteen trisomy 18 patients, seven underwent surgical management and six received expectant management. With surgical management, operative mortality was 29 %, and 80 % of patients were alive after a median follow-up of 116 days. With expectant management, 50 % of patients died before hospital discharge. Of the three patients with trisomy 13, one patient underwent surgical management and two received expectant management. The patient who received surgical management with complete repair was alive at last follow-up over 2 years after surgery; both patients managed expectantly died before hospital discharge. Trisomy 13 and trisomy 18 patients with comorbid congenital heart disease can undergo successful cardiac surgical intervention. In this population, we advocate that nearly all patients with cardiovascular indications for operative congenital heart disease intervention should be offered complete surgical repair over palliative approaches for moderately complex congenital cardiac anomalies.

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.

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.

Twenty-four hour in-hospital congenital cardiac surgical coverage improves perioperative ECMO support outcomes.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) support is often required in the management of perioperative congenital heart surgery (CHS) patients. However, 24-hour in-hospital congenital cardiac surgical coverage (24-CCSC) is not available at all institutions. The purpose of this study is to evaluate the effect of 24-CCSC on perioperative ECMO outcomes in CHS patients. METHODS: An institutional review board approved, retrospective review of 128 perioperative CHS ECMO patients at a single, quaternary care children's hospital between January 2003 and December 2012 was performed. Primary endpoints evaluated were mortality in children supported with ECMO after undergoing cardiac surgery and ECMO-related morbidity after initiation of 24-CCSC with advanced congenital cardiac surgical fellows. Patients were divided into 2 groups based on whether 24-CCSC was absent (cohort 1: January 2003 to July 2007) or present (cohort 2: August 2007 to December 2012) at the time of ECMO management. RESULTS: The surgical procedures performed were similar in both cohorts based on STAT Mortality Categories (5 Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality Categories). The overall mortality rate in children supported with ECMO after undergoing cardiac surgery was 53%. This mortality was significantly reduced from 68% to 43% (p = 0.007) with 24-CCSC. Multivariate logistic regression analysis revealed that 24-CCSC (p = 0.009) and lower STAT Mortality Category (p = 0.042) were independent predictors of operative survival. Cardiac arrhythmias (36% to 16%; p = 0.012) and pulmonary complications (32% to 8%; p < 0.001) were significantly reduced with 24-CCSC. CONCLUSIONS: The presence of 24-CCSC significantly decreased the rate of mortality in children supported with ECMO after undergoing cardiac surgery, as well as cardiac arrhythmias and pulmonary complications for perioperative CHS patients receiving ECMO support. This study demonstrates that CHS programs would benefit from 24-CCSC in the care of this critically ill 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.