World Journal for Pediatric and Congenital Heart Surgery

Data collection and dissemination have been a part of the US transplant experience since its earliest days. As part of this process, the United Network for Organ Sharing (UNOS) has provided open access to its data. In addition, multiinstitutional groups such as the Pediatric Heart Transplant Study (PHTS) have collected data of particular interest to pediatric and congenital transplants. This wealth of data enables quality improvement along several pathways including individual program assessment and improvement and development of both structure and process measures for ongoing improvement. Extensive literature exists utilizing these data, but must be read critically, recognizing the limitations presented by missing variables (whether uncollected or collected but left blank), reproducibility, and small sample sizes among pediatric patients. However, despite these limitations, opportunity continues to exist to apply these data sets to ongoing questions of quality and optimize organ allocation and long-term survival among pediatric patients with heart failure.

Background: Perioperative bleeding is common in pediatric cardiac surgery patients. Traditional laboratory tests do not adequately characterize coagulation derangements in patients with bleeding. We sought to establish preoperative thromboelastography parameters in children prior to cardiopulmonary bypass, to compare thromboelastography assessment with standard coagulation parameters postoperatively, and to assess thromboelastography in children with significant hemorrhage. Methods: Sixty patients requiring cardiopulmonary bypass were enrolled in a prospective observational study of perioperative thromboelastography. Thromboelastography measures were obtained preoperatively, intraoperatively after protamine administration, upon admit to the intensive care unit, and when patients were treated for bleeding. Thromboelastography measures were not used for clinical care. Postoperative thromboelastography measurements were compared with the standard coagulation parameters. Intraoperative thromboelastography, postoperative thromboelastography, and clinical outcomes were compared among patients who did and did not have significant postoperative bleeding. Results: Preoperative thromboelastography parameters were similar to other published normal values for pediatric patients. Transfusion recommendations based on thromboelastography measurements were significantly different from those based on the standard coagulation testing. Thromboelastography measures after initial protamine administration were significantly different in patients with postoperative bleeding. This difference was not present upon arrival to the intensive care unit. Patients with significant bleeding tended to cease bleeding when clinical interventions were in agreement with recommendations based on thromboelastography. Conclusions: Pediatric patients with significant postoperative bleeding after surgery are more likely to have abnormal thromboelastography early after cessation of cardiopulmonary bypass. Thromboelastography illustrates derangements in the coagulation system and may aid in the treatment of postoperative bleeding.

Background: We previously demonstrated that a dose of glucocorticoids (GCs) administered prior to cardiopulmonary bypass (CPB) is effective at suppressing the inflammatory response to CPB and leads to an improved postoperative course. We evaluated whether an additional dose of GC administered eight hours prior to CPB would lead to further clinical benefit. Methods: We conducted a prospective study in which patients were randomized to receive placebo or GC eight hours prior to CPB, in addition to a dose of GC administered following induction of anesthesia. We measured serum inflammatory mediator levels and postoperative clinical parameters. Results: Thirty-one patients were included in the study. Eighteen patients received two doses of GC and 13 patients received a single does of GC. Complement C3a levels were significantly lower at 24 hours following surgery in those patients who received two doses of GC (3136 ± 1650 vs 1779 ± 1616 ng/mL, P = .04). There was no significant difference in tumor necrosis factor (TNF)-α or interleukin (IL)-6 levels at any time between groups. There was no significant difference in core body temperature or renal function (based on serum creatinine levels) between groups. There was no significant difference between groups in duration of mechanical ventilation (2.4 ± 1.5 vs 3.6 ± 3.7 days, two vs one dose, respectively, P = .33) or length of stay in the intensive care unit ([ICU]; 3.4 ± 1.4 vs 4.9 ± 3.6 days, 2 vs 1 dose, respectively, P = .15). Conclusion: While those patients who received two doses of GC prior to surgery had significantly less complement activation postoperatively, clinical outcomes did not differ between groups. We conclude that the practice of administering an additional dose of GC prior to CPB is not supported. However, a large randomized study is needed to conclusively discount the potential benefit of this strategy.

Objective: To report our experience with perventricular closure of muscular (apical) ventricular septal defects (VSDs) in small infants, with echocardiographic guidance only, in a nonhybrid suite. Methods: Eight infants with nine large muscular (apical) VSDs underwent perventricular device closure in a nonhybrid operating room, with transesophageal and epicardial echocardiography guidance, at a mean age and weight of 3.07 (0.3-7.28) months and 3.7 (2.5-6.2) kg, respectively. Five patients had multiple VSDs. Four had associated cardiac defects. Results: Nine Amplatzer muscular VSD devices with a mean size of 10 (4-14) mm were deployed. Seven patients were discharged from the intensive care unit with a mean length of stay of 8.6 days. Four patients had minimal postprocedural residual shunt; no one had a residual shunt at six-month follow-up. Mid-term results are excellent. Conclusion: Perventricular closure is feasible under echocardiographic guidance only in small patients, even without hybrid suite. This may be a good approach for very symptomatic low-weight infants with apical VSD and may also be useful, in any center, at any time, and in any operating room, to treat an associated apical VSD, even unexpected.

Few studies have characterized the surgical outcomes following epicardial pacemaker placement in very low-birth weight infants with congenital complete heart block. This study was undertaken to review the surgical experience with this patient population based on data from a large multi-institutional registry. Methods: The Pediatric Cardiac Care Consortium (PCCC) multi-institutional database was retrospectively reviewed to identify premature, low-birth weight neonates that underwent surgical placement of an epicardial pacing system for heart block. We reviewed 179 patients with birth weights less than 1.5 kg that underwent a major operative procedure. Of these, 10 patients underwent surgical placement of an epicardial pacing system for heart block. Patients had heart block in otherwise structurally normal hearts (n = 6) or heart block associated with complex structural congenital cardiac anomalies (n = 4). Results: There were no deaths directly related to the surgical placement of the epicardial pacing system. There were no immediate complications with either lead or generator placement. One generator pocket was revised three months following placement. Survival to discharge was 60%. The four deaths occurred at a mean of 11 days (range 1-45 days) following the procedure. Conclusions: Neonates born with prematurity and congenital heart block represent a challenging subset of patients with significant mortality. Generator pocket breakdown and infection have been considered barriers to optimal short- and long-term outcomes. Among cases in the PCCC, there were no deaths or major complications that could be attributed to permanent epicardial pacemaker placement. These data suggest that an aggressive surgical strategy may be justified.

Objectives: Incisions in the left ventricle have previously been associated with increased mortality and morbidity, particularly in infants. In order to determine whether this assumption is still true in the current era, we reviewed our recent experience with apical left ventriculotomy in neonates and infants. Methods: The records of five consecutive patients requiring a left ventriculotomy between 2007 and 2010 were reviewed. Weight and age ranged from 2.6 to 16 kilograms and 5 days to 2 years. The diagnoses were three multiple ventricular septal defects, one rhabdomyoma, and one apical aneurysm. The primary end point was left ventricular ejection fraction, with other end points being intensive care unit length of stay, time to extubation, inotrope requirement, arrhythmias, and mitral valve function. Results: There were no early or late deaths. Although lower than their preoperative values, early postoperative ejection fractions were greater than 50% in all patients. Two patients required no inotropes, and 3 required only minimal support. Hospital length of stay was 9 ± 7 days for multiple ventricular septal defect patients, with intensive care unit stays of 2 to 5 days. There were no postoperative arrhythmias requiring pharmacological therapy, and one patient had a significant reduction in mitral insufficiency postoperatively. Conclusions: Based on our experience, we believe that an apical left ventriculotomy does not significantly impair left ventricular function even in small infants, and is not associated with significant morbidity, based on short-term follow-up. Although the long-term effects are still unknown, early results suggest that a left ventriculotomy may safely be used when alternative approaches are inadequate for complex cardiac defects.

Background: Sternal wound infections (SWIs) are a costly complication for children after cardiac surgery, increasing morbidity, mortality, and financial cost. There are no pediatric guidelines to reduce the incidence of SWI in this vulnerable population. Methods: A quality improvement, multidisciplinary team was formed, and a protocol to prevent SWI was developed. A prospective review of patients who underwent pediatric cardiac surgery was conducted over a two-year period to follow adherence to the protocol and incidence of SWI. The Centers for Disease Control definitions for surgical site infections were used to determine the depth and presence of infection. Results: Three hundred and eight children <18 years of age had sternotomies during the study period. There was a reduction in all SWI between the first and second years of the study (odds ratio [OR] = 0.35; confidence interval [CI] 95% 0.12-1.01; P = .059). Delayed sternal closure (DSC) was associated with increased risk of SWI (OR = 5.4; CI 95% 2.13-14.9; P ≤ .001). Institution of a protocol in patients with DSC was associated with decreased infections during the second year (first year: n = 7 (14%), second year: n = 2 (4%), P = .14). Conclusions: Institution of a protocol was associated with a decreased number of infections in children. A multicenter study of a bundled protocol approach to SWI prevention is needed. Children with DSC had a significantly higher risk of developing a wound infection. Initiating strategies to reduce SWI with a focus on children with DSC may result in improved overall infection rates.

Children in the cardiac intensive care unit (CICU) with congenital or acquired heart disease are at risk for hematologic complications, both hemorrhage and thrombosis. The overall incidence of hematologic complications in the CICU is unknown, but risk factors and target groups have been identified where the essential physiologic balance between bleeding and clotting has been disrupted. Although the best management of life-threatening bleeding and clotting is prevention, the cardiac intensivist is often faced with managing life-threatening hematologic events involving patients from within the unit or those who present from outside. Part I of this review deals with the propensity of children with congenital and acquired heart disease to complications of both bleeding and clotting, and includes discussions of perioperative bleeding, thromboses in single-ventricle patients, clotting of Blalock-Taussig shunts and thrombotic complications of mechanical valves. Part II deals with the subject of stroke in children with heart disease. Part III reviews monitoring the effectiveness of anticoagulation and thrombolysis in the CICU. Currently available diagnostics modalities, medications and management strategies are reviewed and future directions discussed.

While surgical repair of tetralogy of Fallot (TOF) is generally associated with good early outcomes, late complications affect long-term survival and may require reoperation. Pulmonary regurgitation (PR) and tricuspid regurgitation (TR) may increase the risk of arrhythmias, reduced cardiac function, and sudden death. Tricuspid valve function can be compromised secondarily in the setting of PR or directly as a result of injury or alteration of the valve related to the original TOF repair. This article reviews the etiologic mechanisms, pathophysiological implications, and surgical interventions for TR. Effective management following TOF repair requires consideration of TR to optimize late outcomes.

In this report, we describe the surgical management of a patient with tetralogy of Fallot and pulmonary atresia, in whom a fistula from the anterior interventricular coronary artery was the predominant source of arterial supply to both the lungs.

We describe a rare association of aortopulmonary window and double aortic arch in a 1.7-kg newborn who presented with severe respiratory distress. A staged surgical approach was used because of the size of the patient and significant comorbidity. This approach resulted in excellent outcome.

A seven-month-old girl with partial anomalous pulmonary venous connection and atrial septal defect underwent cardiac magnetic resonance for further evaluation. Anatomical images and flow quantification confirmed the diagnosis and demonstrated a commitment of the superior vena cava to the left atrium, resulting in a right-to-left shunt and pulmonary/systemic blood flow ratio of 0.85. The findings were confirmed during surgery.

Congenital tracheal stenosis is frequently associated with heart malformations. Simultaneous correction of both anomalies has been advocated by several authors. We describe our experience with a premature neonate with congenital tracheal stenosis and tetralogy of Fallot. The anomalies were corrected during the same surgical procedure with the aid of extracorporeal circulation. The implications of the operative and postoperative courses, concerning both the cardiac anomaly and the tracheal anomaly are discussed.

The association of atrioventricular canal defect and cor triatriatum sinister is very rare and only a few anecdotal reports of successful surgical repair and outcome have been reported. We report a case of Rastelli type-B atrioventricular canal defect with cor triatriatum which was successfully repaired.

Tetralogy of Fallot (TOF) is a cyanotic congenital heart defect typically diagnosed in infancy and treated with early surgical correction. We report a patient with TOF diagnosed at age 78. Successful surgical repair was performed at age 83, the oldest reported age of surgical correction of this condition. Despite a complicated surgical and postoperative course, the patient is doing well almost four years later.

We report a two-year-old patient with isomerism of the right atrial appendages, a functionally univentricular heart, and associated totally anomalous pulmonary venous connection. The unusual finding was an intrapulmonary course of the vertical vein. We discuss the anatomical findings, management, and outcome.

We describe spontaneous rupture of a congenital left ventricular (LV) aneurysm with subsequent tamponade and cardiac arrest in a 4-year-old male with staphylococcal septicemia. Emergency resuscitation, thoracotomy, and oversewing were successfully undertaken in the pediatric intensive care unit. There was complete cardiovascular recovery without adverse neurodevelopmental sequelae.  This article details the difficulties in determining the etiology of ventricular aneurysms but highlights the importance of attempting to do so, particularly in distinguishing between congenital and acquired forms. Congenital aneurysms are usually a stable pathology; mycotic aneurysms are not and should be managed emergently, as survival after rupture is rare.

Esophageal perforation is a rare, but life threatening, entity in children. The most common iatrogenic causes include nasogastric tube insertion, stricture dilation, or endotracheal intubation. Recently, transesophageal echocardiography (TEE) has been increasingly used in pediatric cardiac surgery to assess cardiac function and structural abnormalities. The safety of TEE in children is still controversial and complications such as airway obstruction, hemodynamic compromise, and esophageal injury have been reported. We recently experienced a case of esophageal perforation caused by TEE probe insertion during neonatal cardiac surgery; two weeks of conservative management resulted in complete resolution of the injury.

We describe a case of revascularization for anomalous left coronary artery from the pulmonary artery (ALCAPA) with severe left ventricular (LV) dysfunction and severe mitral regurgitation (MR). Extracorporeal membrane oxygenation (ECMO), later successfully converted to an indigenous left ventricular assist device (LVAD) functionally resulted in the spontaneous resolution of MR and satisfactory recovery of LV function.