Pediatric Cardiac Tumors: A 45-year, Single Institution Review

Background: Cardiac tumors in children are rare. Of the cases reported in the literature, nearly all are benign and managed conservatively. Methods: This is a retrospective, observational study of pediatric patients <18 years who presented for surgical evaluation of a cardiac tumor, between 1969 and 2014 at a tertiary care children’s hospital. Presentation, pathology, management, and outcomes were evaluated. Results: Over the last 45 years, 64 patients were evaluated for surgical resection of a cardiac tumor. Rhabdomyoma was the most common neoplasm (58%), and 17% of the tumors had malignant pathologies. While 42% of benign cardiac neoplasms required surgical intervention for significant hemodynamic concerns, 73% of malignant neoplasms underwent radical excision, if possible, followed by adjuvant chemotherapy. Despite a 37% mortality in patients with malignant pathology, an aggressive surgical approach can yield long-term survival in some patients. There were no deaths among patients with benign tumors and 17% had postoperative complications mostly related to mitral regurgitation. Conclusion: Cardiac tumors in children are rare but can be managed aggressively with good outcomes. Benign tumors have an excellent survival with most complications related to tumor location. Malignant tumors have a high mortality rate, but surgery and adjuvant chemotherapy allow for prolonged survival in selected patients

Multiblock High Order Large Eddy Simulation of Powered Fontan Hemodynamics: Towards Computational Surgery

Children born with only one functional ventricle must typically undergo a series of three surgeries to obtain the so-called Fontan circulation in which the blood coming from the body passively flows from the Vena Cavae (VCs) to the Pulmonary Arteries (PAs) through the Total Cavopulmonary Connection (TCPC). The circulation is inherently inefficient due to the lack of a subpulmonary ventricle. Survivors face the risk of circulatory sequelae and eventual failure for the duration of their lives. Current efforts are focused on improving the outcomes of Fontan palliation, either passively by optimizing the TCPC, or actively by using mechanical support. We are working on a chronic implant that would be placed at the junction of the TCPC, and would provide the necessary pressure augmentation to re-establish a circulation that recapitulates a normal two-ventricle circulation. This implant is based on the Von Karman viscous pump and consists of a vaned impeller that rotates inside the TCPC. To evaluate the performance of such a device, and to study the flow features induced by the presence of the pump, Computational Fluid Dynamics (CFD) is used. CFD has become an important tool to understand hemodynamics owing to the possibility of simulating quickly a large number of designs and flow conditions without any harm for patients. The transitional and unsteady nature of the flow can make accurate simulations challenging. We developed and in-house high order Large Eddy Simulation (LES) solver coupled to a recent Immersed Boundary Method (IBM) to handle complex geometries. Multiblock capability is added to the solver to allow for efficient simulations of complex patient specific geometries. Blood simulations are performed in a complex patient specific TCPC geometry. In this study, simulations without mechanical assist are performed, as well as after virtual implantation of the temporary and chronic implants being developed. Instantaneous flow structures, hepatic factor distribution, and statistical data are presented for all three cases

Does the degree of preoperative mitral regurgitation predict survival or the need for mitral valve repair or replacement in patients with anomalous origin of the left coronary artery from the pulmonary artery?

BackgroundAnomalous origin of the left coronary artery from the pulmonary artery causes severe myocardial ischemia, global left ventricular dysfunction, and annular dilatation producing varying degrees of mitral regurgitation. Mitral regurgitation secondary to the left ventricular or papillary muscle dysfunction in infants will usually improve in the absence of ongoing ischemia. The aim of this study is to determine the influence of the degree of preoperative mitral regurgitation on the early and late outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery who underwent coronary reimplantation.MethodsTwenty-five patients underwent coronary reimplantation and 1 early patient had ligation during a 30-year period (median age, 4 months; range, 1 month to 16 years), with a median follow-up of 7 years (range, 4 months to 25 years). Before repair, 7 infants (27%) presented in extremis requiring ventilatory and inotropic support, and 17 patients (65%) presented with heart failure. Mitral regurgitation was present in all patients: trivial in 6 patients, mild in 12 patients, moderate in 5 patients, and severe in 3 patients. No patient underwent mitral valve repair or replacement at the time of anomalous origin of the left coronary artery from the pulmonary artery repair.ResultsHospital survival was 92%. Three patients underwent mitral valve repair or replacement at the mean time of 3.5 years (all with severe preoperative mitral regurgitation). The degree of mitral regurgitation gradually improved in all remaining patients with preoperative mild and moderate mitral regurgitation. Echocardiographic studies demonstrated improvement in left ventricular function in all children. None of the patients showed any evidence of supravalvar pulmonary stenosis as a result of their pulmonary artery reconstruction.ConclusionLong-term clinical outcome and left ventricular function are good despite severe left ventricular dysfunction at presentation. Mitral valve repair or replacement is generally not necessary at the time of anomalous origin of the left coronary artery from the pulmonary artery repair. Significant residual mitral regurgitation is present in some patients and can usually be managed surgically at a later date depending on its degree of severity

Anterior pericardial tracheoplasty for long-segment tracheal stenosis: Long-term outcomes

ObjectiveAlthough several techniques have been described for the treatment of tracheal stenosis, including slide tracheoplasty, tracheal autograft, rib grafting, and use of a pericardial patch, the optimal repair remains controversial because of a lack of long-term follow-up data. The purpose of this study is to examine the long-term results of anterior pericardial tracheoplasty.MethodsTo assess the long-term outcomes of patients who underwent repair of tracheal stenosis with anterior pericardial tracheoplasty, we reviewed the case histories of 26 consecutive patients (1984–present). All but 5 had long-segment tracheal stenosis with more than 10 complete tracheal rings. Twenty-one had significant cardiac disease, and 10 had their cardiac lesions repaired at the time of their tracheoplasty. The median age was 6 months (range, 2 days–25 years). All patients underwent anterior pericardial tracheoplasty through a median sternotomy during normothermic cardiopulmonary bypass. We have previously described our tracheoplasty technique. An average of 14 tracheal rings (range, 5–22) was divided anteriorly, and a patch of fresh autologous pericardium was used to enlarge the trachea to 1.5 times the predicted diameter for age and weight.ResultsThere were 3 hospital deaths (at 1, 2, and 7 months, respectively) and 2 late deaths (at 2 and 13 years postoperatively, respectively). No deaths were related to airway obstruction. Two survivors required tracheostomy postoperatively, one after formation of granulation tissue and stenosis and the other after failure to wean from mechanical ventilation. All survivors remain asymptomatic, with minimal to no evidence of airway obstruction. Median follow-up is 11 years (range, 3 months–22 years).ConclusionAnterior pericardial tracheoplasty for tracheal stenosis provides excellent results in the majority of patients at long-term follow-up

Vasoactive-ventilation-renal score reliably predicts hospital length of stay after surgery for congenital heart disease

Objectives We aimed to further validate the vasoactive-ventilation-renal score as a predictor of outcome in patients recovering from surgery for congenital heart disease. We also sought to determine the optimal time point within the early recovery period at which the vasoactive-ventilation-renal score should be measured. Methods We prospectively reviewed consecutive patients recovering from cardiac surgery within our intensive care unit between January 2015 and June 2015. The vasoactive-ventilation-renal score was calculated at 6, 12, 24, and 48 hours postoperatively as follows: vasoactive-ventilation-renal score = ventilation index + vasoactive-inotrope score + Δ creatinine [change in serum creatinine from baseline*10]. Primary outcome of interest was prolonged hospital length of stay, defined as length of stay in the upper 25%. Receiver operating characteristic curves were generated, and areas under the curve with 95% confidence intervals were calculated for all time points. Multivariable logistic regression modeling also was performed. Results We reviewed 164 patients with a median age of 9.25 months (interquartile range, 2.6-58 months). Median length of stay was 8 days (interquartile range, 5-17.5 days). The area under the curve value for the vasoactive-ventilation-renal score as a predictor of prolonged length of stay (>17.5 days) was greatest at 12 hours postoperatively (area under the curve = 0.93; 95% confidence interval, 0.89-0.97). On multivariable regression analysis, after adjustment for potential confounders, the 12-hour vasoactive-ventilation-renal score remained a strong predictor of prolonged hospital length of stay (odds ratio, 1.15; 95% confidence interval, 1.10-1.20). Conclusions In a heterogeneous population of patients undergoing surgery for congenital heart disease, the novel vasoactive-ventilation-renal score calculated in the early postoperative recovery period can be a strong predictor of prolonged hospital length of stay

Cavopulmonary assist: Long-term reversal of the Fontan paradox

Objective Fontan circulatory inefficiency can be addressed by replacing the missing subpulmonary power source to reverse the Fontan paradox. An implantable cavopulmonary assist device is described that will simultaneously reduce systemic venous pressure and increase pulmonary arterial pressure, improving preload and cardiac output, in a univentricular Fontan circulation on a long-term basis. Methods A rotary blood pump that was based on the von Karman viscous pump was designed for implantation into the total cavopulmonary connection (TCPC). It will impart modest pressure energy to augment Fontan flow without risk of obstruction. In the event of rotational failure, it is designed to default to a passive flow diverter. Pressure-flow performance was characterized in vitro in a Fontan mock circulatory loop with blood analog. Results The pump performed through the fully specified operating range, augmenting flow in all 4 directions of the TCPC. Pressure rise of 6 to 8 mm Hg was readily achieved, ranging to 14 mm Hg at highest speed (5600 rpm). Performance was consistent across a wide range of cardiac outputs. In stalled condition (0 rpm), there was no discernible pressure loss across the TCPC. Conclusions A blood pump technology is described that can reverse the Fontan paradox and may permit a surgical strategy of long-term biventricular maintenance of a univentricular Fontan circulation. The technology is intended for Fontan failure in which right-sided circulatory inefficiencies predominate and ventricular systolic function is preserved. It may also apply before clinical Fontan failure as health maintenance to preempt the progression of Fontan disease

Tracheostomy Following Surgery for Congenital Heart Disease: 14-year Institutional Experience

Background: Tracheostomy following congenital heart disease (CHD) surgery is a rare event and associated with significant mortality. Hospital survival has been reported from 20% to 40%. Late mortality for these patients is not well characterized. Methods: We performed a retrospective observational study of patients who had a tracheostomy following CHD surgery (excluding isolated patent ductus arteriosus ligation) between January 2000 and December 2013. Patients were categorized into single-ventricle or biventricular physiology groups. Demographics, genetic syndromes, pulmonary disease, and comorbidities were collected. Outcomes including hospital survival, long-term survival, and weaning from positive pressure ventilation are reported. Bivariate and time-to-event models were used. Results: Over a 14-year period, 61 children (0.9% incidence) had a tracheostomy placed following CHD surgery. There were 12 single-ventricle patients and 49 biventricular patients. Prematurity, genetic syndromes, lung/airway disease, and other comorbidities were common in both CHD groups. Gastrostomy tubes were used more frequently in biventricular physiology patients (91.8%) versus single-ventricle patients (66.7%, P = .04). Survival to hospital discharge was 50% in the single-ventricle group compared to 86% in biventricular patients (P = .01). Long-term survival continued to be poor in the single-ventricle group comparatively (three years, 27.8% vs 64.8%, P = .01). Gastrostomy tube placement was independently associated with survival in both groups (P = .002). Conclusion: Tracheostomy is performed following many types of surgery for CHD and is commonly associated with other comorbidities. Both hospital and long-term survival are substantially lower in children with single-ventricle physiology as compared to patients with biventricular physiology

A Randomized, Controlled Trial of Catheter-Related Infectious Event Rates Using Antibiotic-Impregnated Catheters Versus Conventional Catheters in Pediatric Cardiovascular Surgery Patients

We conducted a randomized, controlled clinical trial to determine whether a difference in catheter-associated blood stream infection (CABSI) incidence existed between children who underwent cardiac surgery and had a central venous catheter impregnated with minocycline and rifampin versus those who had a conventional, nonimpregnated catheter after cardiac surgery. Due to a lower number of infections than expected, the study was terminated early. Among 288 evaluable patients, the rates of CABSI and line-related complications were similar between the 2 groups

Outcomes in patients with interrupted aortic arch and associated anomalies: a 20-year experience

Objective: The surgical results for the repair of interrupted aortic arch (IAA) have evolved in recent years. We report our results for staged repair of this complex congenital malformation. Methods: Sixty-five patients (mean age, 16.9 ± 41.7 days) were diagnosed with IAA and referred for surgical therapy. The surgical management strategy at our institution between 1982 and 2005 has been one-stage complete repair (n = 13) or staged repair (n = 52) in selected patients. Non-complex patients (group I, n = 51) had a ventricular septal defect (87%), aortopulmonary window (8%), and left ventricular outflow tract obstruction (27%). Group II (n = 14) were patients with Taussig–Bing double outlet right ventricle (n = 6) or truncus arteriosus (n = 8). Method of staged repair of IAA was to transect and turn down the left carotid artery and anastomosis it to the descending aorta (n = 41) or graft interposition (n = 2) combined with a pulmonary artery (PA) banding followed in a few months by delayed ventricular septal defect (VSD) closure and PA de-banding. Results: There were 5 early and 10 late deaths. The actuarial survival including early mortality was 92% at 1 year, 81% at 5 years, and 76% at 10 and 15 years. There was an 81% 15-year survival for children in group I compared with a 54% for children in group II (p ≪ 0.001). Risk factors for increased mortality by univariate analysis were as follows: (1) primary aortic anastomosis (p = 0.03), (2) presence of complex anomalies (p = 0.05), and (3) initial IAA repair performed before 1994 (p = 0.05). Actuarial freedom from any type of aortic reoperation or intervention was 86% at 1 year, 69% at 5 years, and 60% at 10 and 15 years. Univariate and multivariate analyses identified no tested variables as risk factors for reoperation. The majority (86%) was in New York Heart Association (NYHA) class I, and 14% remained in NYHA class II. During the postoperative course there were no neurologic deficits, seizures, and growth disturbances in any patient. Conclusion: Staged repair of IAA using a left carotid artery turn down can be safely applied in IAA patients with and without other intracardiac anomalies with good results. Use of the left carotid artery for arch reconstruction did not result in any detectable neurological events or growth disturbances later in life. Associated anomalies played an important role in outcomes. The long-term probability for reoperation and/or reintervention remains high regardless of operative technique

Cavopulmonary assist for the failing Fontan circulation: impact of ventricular function on mechanical support strategy

Mechanical circulatory support--either ventricular assist device (VAD, left-sided systemic support) or cavopulmonary assist device (CPAD, right-sided support)--has been suggested as treatment for Fontan failure. The selection of left- versus right-sided support for failing Fontan has not been previously defined. Computer simulation and mock circulation models of pediatric Fontan patients (15-25 kg) with diastolic, systolic, and combined systolic and diastolic dysfunction were developed. The global circulatory response to assisted Fontan flow using VAD (HeartWare HVAD, Miami Lakes, FL) support, CPAD (Viscous Impeller Pump, Indianapolis, IN) support, and combined VAD and CPAD support was evaluated. Cavopulmonary assist improves failing Fontan circulation during diastolic dysfunction but preserved systolic function. In the presence of systolic dysfunction and elevated ventricular end-diastolic pressure (VEDP), VAD support augments cardiac output and diminishes VEDP, while increased preload with cavopulmonary assist may worsen circulatory status. Fontan circulation can be stabilized to biventricular values with modest cavopulmonary assist during diastolic dysfunction. Systemic VAD support may be preferable to maintain systemic output during systolic dysfunction. Both systemic and cavopulmonary support may provide best outcome during combined systolic and diastolic dysfunction. These findings may be useful to guide clinical cavopulmonary assist strategies in failing Fontan circulations