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The frequency-response of pressure autoregulation is not well delineated; therefore, the optimal frequency of arterial blood pressure (ABP) modulation for measuring autoregulation is unknown. We hypothesized that cerebrovascular autoregulation is band-limited and delineated by a cutoff frequency for which ABP variations induce cerebrovascular reactivity. Neonatal swine ( n = 8) were anesthetized using constant minute ventilation while positive end-expiratory pressure (PEEP) was modulated between 6 and 0.75 cycles/min (min−1). The animals were hemorrhaged until ABP was below the lower limit of autoregulation (LLA), and PEEP modulations were repeated. Vascular reactivity was quantified at each frequency according to the phase lag between ABP and intracranial pressure (ICP) above and below the LLA. Phase differences between ABP and ICP were small for frequencies of >2 min−1, with no ability to differentiate cerebrovascular reactivity between ABPs above or below the LLA. For frequencies of −1, ABP and intracranial pressure (ICP) showed phase shift when measured above LLA and no phase shift when measured below LLA [above vs. below LLA at 1 min−1: 156° (139–174°) vs. 30° (22–50°); P < 0.001 by two-way ANOVA for both frequency and state of autoregulation]. Data taken above LLA fit a Butterworth high-pass filter model with a cutoff frequency at 1.8 min−1 (95% confidence interval: 1.5–2.2). Cerebrovascular reactivity occurs for sustained ABP changes lasting 30 s or longer. The ability to distinguish intact and impaired autoregulation was maximized by a 60-s wave (1 min−1), which was 100% sensitive and 100% specific in this model.

Objectives Neonates undergoing complex congenital heart surgery have a significant incidence of neurologic problems. Erythropoietin has antiapoptotic, antiexcitatory, and anti-inflammatory properties to prevent neuronal cell death in animal models, and improves neurodevelopmental outcomes in full-term neonates with hypoxic ischemic encephalopathy. We designed a prospective phase I/II trial of erythropoietin neuroprotection in neonatal cardiac surgery to assess safety and indicate efficacy. Methods Neonates undergoing surgery for D-transposition of the great vessels, hypoplastic left heart syndrome, or aortic arch reconstruction were randomized to 3 perioperative doses of erythropoietin or placebo. Neurodevelopmental testing using the Bayley Scales of Infant and Toddler Development III was performed at age 12 months. Results Fifty-nine patients received the study drug. Safety profile, including magnetic resonance imaging brain injury, clinical events, and death, was not different between groups. Three patients in each group died. Forty-two patients (22 in the erythropoietin group and 20 in the placebo group; 79% of survivors) returned for 12-month follow-up. In the group receiving erythropoietin, mean Cognitive Scale scores were 101.1 ± 13.6, Language Scale scores were 88.5 ± 12.8, and Motor Scale scores were 89.9 ± 12.3. In the group receiving placebo, Cognitive Scale scores were 106.3 ± 10.8 ( P = .19), Language Scores were 92.4 ± 12.4 ( P = .33), and Motor Scale scores were 92.6 ± 14.1 ( P = .51). Conclusions Safety profile for erythropoietin administration was not different than placebo. Neurodevelopmental outcomes were not different between groups; however, this pilot study was not powered to definitively address this outcome. Lessons learned suggest optimized study design features for a larger prospective trial to definitively address the utility of erythropoietin for neuroprotection in this population.

Options for long-term mechanical circulatory support to sustain pediatric heart failure patients requiring cardiac transplantation while they wait for donor hearts have been unsatisfactory. The conventional approach has been to use extracorporeal membrane oxygenation (ECMO), but its lack of feasibility for long-term use and the major complications associated with the technology have limited its use, especially in light of lengthy waiting lists for donor hearts. With the advent of the Berlin Heart EXCOR® Pediatric ventricular assist device (VAD), pediatric heart failure specialists have gained an important tool for helping this patient population survive until a donor heart can be identified. The EXCOR Pediatric VAD is designed to support pediatric patients of all age groups, from newborns to teenagers, and can be used successfully for many months. This paper describes the early experience with the EXCOR Pediatric VAD and the challenging journey undertaken to gain U.S. FDA approval, including successful completion of the first worldwide prospective clinical study of VADs in a pediatric population.

Background For neonates with atrioventricular septal defect and aortic arch obstruction including coarctation of the aorta, we sought to determine whether a difference in outcomes exists after a primary neonatal versus staged surgical repair (neonatal arch repair with delayed intracardiac repair). Methods This retrospective cohort study included consecutive neonates with atrioventricular septal defect and aortic arch obstruction who underwent cardiac surgery before 28 days of age at six centers from 1990 to 2009. Characteristics and outcomes between patients undergoing neonatal versus staged repair were compared. Results Of 66 study patients, 31 (47%) underwent primary neonatal repair and 35 (53%) underwent staged repair. At baseline echocardiogram, a greater percentage of neonatal repair patients had relative unbalanced ventricular size (56% versus 35%, p = 0.02). There were no other differences in demographic characteristics, cardiac anatomical or functional details, or surgical technique. Those undergoing neonatal repair tended to be more likely to have at least moderate left atrioventricular valve regurgitation early after repair (42% versus 19%, p = 0.05) and to have at least one major in-hospital complication (42% versus 20%, p = 0.06). After the initial cardiac operation, compared with the neonatal repair group, patients undergoing staged repair had greater survival (87% versus 57% at 6 years, log-rank p = 0.02) and freedom from the first unplanned cardiac reoperation (69% versus 45% at 6 years, log-rank p = 0.005). Conclusions For neonates with atrioventricular septal defect and aortic arch obstruction, when compared with neonatal repair, a staged approach was associated with improved survival and lower morbidity.

We describe a patient with anomalous left coronary artery with a short intramural course and a previously unrecognized myocardial bridge who presented with a recurrent episode of aborted sudden cardiac death. Intravascular ultrasound and fractional flow reserve showed significant compression at the left coronary artery ostium by the intercoronary pillar and at the myocardial bridge. Intravascular ultrasound and fractional flow reserve were normal after coronary translocation and unroofing of the myocardial bridge. All potential anatomic culprits should be addressed when operating on patients with anomalous coronaries.

The neonatal arterial switch operation has become the standard of care for transposition of the great arteries, including transposition with intact ventricular septum, transposition with ventricular septal defect with or without aortic arch hypoplasia, and double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly). While technically demanding, the operation is enormously gratifying and exciting for the congenital heart surgeon. In the current era, outcomes and expectations for the neonatal arterial switch operation are extremely high with many centers, including our own, reporting 30-day or hospital survivorship approaching 100%. Long-term results are also outstanding, although these patients do require lifelong follow-up and have the potential for need of remedial surgical intervention.

The last decade has witnessed significant advancement in the field of ventricular assist device (VAD) support. Although device options for pediatric patients were previously severely limited because of body size constraints, this frustrating situation has gradually been changing, owing to ongoing device miniaturization. Recognition of the superiority of VAD support compared with conventional extracorporeal membrane oxygenation support has spurred enthusiasm for VAD support in children. In this article, we discuss the current status of pediatric VAD support; where do we stand now and where will we be heading? Because this field is rapidly changing, it is anticipated that this article will provide a general overview of what is currently occurring in the field of pediatric VAD support.

Background The Infant Jarvik 2015 is an implantable axial-flow ventricular assist device (VAD) that has undergone the major evolutionary design modifications to improve hemocompatibility. This study was conducted in anticipation of data submission to the US Food and Drug Administration to obtain Investigational Device Exemption approval. Methods The VAD was implanted via a left thoracotomy in Barbado sheep (n = 10, 26 (19-34] kg). Anticoagulation was maintained with coumadin, with a target international normalized ratio of greater than the individual sheep's baseline values. The VAD was managed at the highest possible speed as clinically tolerable. Complete necropsy was performed at the end of the study. Results There were 2 early mortalities: tension pneumothorax (n = 1) and shower emboli of the fragmented myocardium (n = 1). The remaining 8 sheep (2 with 30-day and 6 with 60-day protocols) completed the anticipated study duration in excellent condition, with the 6 completing 60-day sheep showing appropriate weight gain during support. There were no signs of clinically significant hemolysis, with the final plasma-free hemoglobin of 2 (1-17) mg/dL. Necropsy showed old renal infarction in 7 sheep. Although thromboembolism can be the potential etiology, given the mild anticoagulation regimen, other sources of emboli were identified in 2 sheep (graft coating material and fragmented myocardium). Flow study demonstrated favorable increase in flow (up to 3.0 L/min) in proportion to change in pump speed. Conclusions This study has demonstrated that the Infant Jarvik 2015 VAD is capable of maintaining its functionality for an extended period of time with minimal hemolysis.

Premature infants are at risk of vascular neurological insults. Hypotension and hypertension are considered injurious, but neither condition is defined with consensus. Critical closing pressure (CrCP) is the arterial blood pressure (ABP) at which cerebral blood flow ceases. CrCP may serve to define subject-specific low or high ABP. Our objective was to quantify CrCP as a function of gestational age (GA). One hundred eighty-six premature infants with a GA range of 23-33 weeks, were monitored with umbilical artery catheters and transcranial Doppler insonation of middle cerebral artery flow velocity (FV) for 1-h sessions over the first week of life. CrCP was calculated using an impedance model derivation with Doppler-based estimations of cerebrovascular resistance and compliance. CrCP increased significantly with GA (r = 0.47; slope = 1.4 mmHg/week gestation), an association that persisted with multivariate analysis (p0.001). Higher diastolic ABP and higher GA were associated with increased CrCP (p0.001 for both). CrCP increases significantly at the end of the second and beginning of the third trimester. The low CrCP observed in premature infants may explain their ability to tolerate low ABP without global cerebral infarct or hemorrhage.

Premature infants are at an increased risk of intraventricular hemorrhage (IVH). The roles of hypotension and hyperemia are still debated. Critical closing pressure (CrCP) is the arterial blood pressure (ABP) at which cerebral blood flow (CBF) ceases. When diastolic ABP is equal to CrCP, CBF occurs only during systole. The difference between diastolic ABP and CrCP is the diastolic closing margin (DCM). We hypothesized that a low DCM was associated with IVH. One hundred eighty-six premature infants, with a gestational age (GA) range of 23-33 weeks, were monitored with umbilical artery catheters and transcranial Doppler insonation of middle cerebral artery flow velocity for 1-h sessions over the first week of life. CrCP was calculated linearly and using an impedance model. A multivariate generalized linear regression model was used to determine associations with severe IVH (grades 3-4). An elevated DCM by either method was associated with IVH (p 0.0001 for the linear method; p 0.001 for the impedance model). Lower 5-min Apgar scores, elevated mean CBF velocity, and lower mean ABP were also associated with IVH (p 0.0001). Elevated DCM, not low DCM, was associated with severe IVH in this cohort.

The goal of this study was to use decision analysis to evaluate the impact of varying uncertainties on the outcomes of patients with anomalous aortic origin of a coronary artery.Two separate decision analysis models were created: one for anomalous left coronary artery (ALCA) and one for anomalous right coronary artery (ARCA). Three strategies were compared: observation, exercise restriction, and surgery. Probabilities and health utilities were estimated on the basis of existing literature. Deterministic and probabilistic sensitivity analyses were performed.Surgery was the optimal management strategy for patients30 years of age with ALCA. As age increased, observation became an equivalent strategy and eventually surpassed surgery as the treatment of choice. The advantage on life expectancy for surgery over observation ranged from 2.6 ± 1.7 years for a 10-year-old patient to -0.03 ± 0.1 for a 65-year old patient. In patients with ARCA, observation was the optimal strategy for most patients with a life expectancy advantage over surgery of 0.1 ± 0.1 years to 0.2 ± 0.4 years, depending on age. Surgery was the preferred strategy only for patients25 years of age when the perceived risk of sudden cardiac death was high and the perioperative mortality was low. Exercise restriction was a suboptimal strategy for both ALCA and ARCA in all scenarios.The optimal management in anomalous aortic origin of a coronary artery depends on multiple factors, including individual patient characteristics. Decision analysis provides a tool to understand how these characteristics affect the outcomes with each management strategy and thus may aid in the decision making process for a particular patient.

Premature infants are at risk of vascular neurological insults. Hypotension and hypertension are considered injurious, but neither condition is defined with consensus. Critical closing pressure (CrCP) is the arterial blood pressure (ABP) at which cerebral blood flow ceases. CrCP may serve to define subject-specific low or high ABP. Our objective was to quantify CrCP as a function of gestational age (GA). One hundred eighty-six premature infants with a GA range of 23-33 weeks, were monitored with umbilical artery catheters and transcranial Doppler insonation of middle cerebral artery flow velocity (FV) for 1-h sessions over the first week of life. CrCP was calculated using an impedance model derivation with Doppler-based estimations of cerebrovascular resistance and compliance. CrCP increased significantly with GA (r = 0.47; slope = 1.4 mmHg/week gestation), an association that persisted with multivariate analysis (p < 0.001). Higher diastolic ABP and higher GA were associated with increased CrCP (p

Premature infants are at an increased risk of intraventricular hemorrhage (IVH). The roles of hypotension and hyperemia are still debated. Critical closing pressure (CrCP) is the arterial blood pressure (ABP) at which cerebral blood flow (CBF) ceases. When diastolic ABP is equal to CrCP, CBF occurs only during systole. The difference between diastolic ABP and CrCP is the diastolic closing margin (DCM). We hypothesized that a low DCM was associated with IVH. One hundred eighty-six premature infants, with a gestational age (GA) range of 23-33 weeks, were monitored with umbilical artery catheters and transcranial Doppler insonation of middle cerebral artery flow velocity for 1-h sessions over the first week of life. CrCP was calculated linearly and using an impedance model. A multivariate generalized linear regression model was used to determine associations with severe IVH (grades 3-4). An elevated DCM by either method was associated with IVH (p

The results of bridging patients with cardiac allograft failure to retransplantation (ReTx) with mechanical circulatory support (MCS) have not been well studied. The United Network of Organ Sharing (UNOS) database was used to analyze outcomes of patients successfully bridged with MCS to cardiac ReTx.Of 1,690 cardiac ReTx identified in the UNOS database from October 1987 to July 2011, 149 (8.8%) were bridged to ReTx with MCS.Patients bridged to ReTx with MCS had a poorer survival than patients not bridged (p0.0001). ReTx after ventricular assist device (VAD) support had better survival than ReTx after extracorporeal membrane oxygenation (ECMO; half-life, 3.9 years vs 61 days, p = 0.026). For patients bridged to ReTx, graft survival was 40% for ReTx within 1 year of primary Tx vs 64% (p = 0.003). When ReTx was performed 1 year after cardiac Tx, survival was similar in patients bridged with a VAD and those not bridged (mean, 7.5 vs 8.7 years; p = 0.8). Survival for patients bridged to ReTx with ECMO was consistently worse (p ≤ 0.05) in all analyses. The 1-year survival of ReTx after VAD performed in 2003 to 2011 (67%) was better than in the earlier era of 1987 to 2002 (37%, p = 0.005).Bridging patients to ReTx with ECMO at any time is not advisable. Bridging patients with MCS to ReTx within 1 year of primary cardiac Tx is not advisable. Survival after ReTx for patients bridged by VAD has improved considerably over time. Patients who survive the first year after cardiac Tx can be bridged by VAD to ReTx with an expectation that outcomes can be similar to ReTx patients who did not require MCS.

Background Expectations for outcomes after the neonatal arterial switch operation (ASO) continue to change. This cohort study describes neurodevelopmental outcomes at age 12 months after neonatal ASO, and analyzes both modifiable and nonmodifiable factors for association with adverse outcomes. Methods Patients who underwent an ASO (n = 30) were enrolled in a prospective outcome study, with comprehensive clinical data collection during the first 12 months of life. Brain magnetic resonance imaging was done preoperatively and 7 days postoperatively, and the Bayley Scales of Infant Development III was performed at age 12 months. Results Ten of 30 patients (33%) had preoperative magnetic resonance imaging injury; 13 of 30 patients (43%) had new postoperative magnetic resonance imaging injury. Twenty patients (67%) had Bayley Scales of Infant Development III: Cognitive Composite standard score mean was 104.8 ± 15.0, Language Composite standard score median was 90.0 (25th to 75th percentile, 83 to 94), and Motor Composite standard score mean was 92.3 ± 14.2. Best subsets multivariable analysis found associations between lower preoperative and intraoperative cerebral oxygen saturation, preoperative magnetic resonance imaging brain injury, total bypass time, and total midazolam dose and lower Bayley Scales of Infant Development III scores at age 12 months. Conclusions At 12 months after ASO, neurodevelopmental outcome means were within normal population ranges. The new associations reported in this study between potentially modifiable perioperative factors and outcomes require investigations in larger patient cohorts. Beyond survival, which was 100% in this cohort, factors influencing quality of life including neurodevelopmental outcomes should be routinely investigated in studies of ASO patients.

Rarely is closure of the atrial septal defect indicated in infants. Yet, an atrial septal defect in the presence of congenital mitral stenosis may cause significant congestive heart failure in infancy. We present the case of an infant with Shone's syndrome, left ventricular hypoplasia, and a large ostium secundum atrial septal defect who developed pulmonary overcirculation following repair of coarctation of the aorta. The infant underwent cardiac catheterization, hemodynamic assessment, and successful percutaneous closure of the atrial septal defect. The patient improved dramatically following the intervention. Follow-up echocardiograms have demonstrated growth of mitral valve annulus diameter and left ventricular dimensions. Atrial septal defect closure in this small infant was associated with immediate improved clinical status, and improved growth of the left heart structures. © 2012 Wiley Periodicals, Inc.

Children undergoing repair of aortic coarctation are at lifelong risk of developing recurrent aortic obstruction. Several surgical techniques have been described to address this challenging problem. Some centers have used extraanatomic aortic bypass techniques to avoid dissection on the previously operated field. We have favored a technique that relies on anatomic reconstruction. Although a repeat end-to

Missile embolus to the heart, although uncommon, is one of the most challenging scenarios in trauma. We describe a 36-year-old man who presented with a gunshot wound to the left chest and a chest x-ray revealing a foreign body in the mediastinum. A median sternotomy was performed and an injury to the left ventricle was identified. After intraoperative echocardiography and fluoroscopy confirmed a foreign body in the aortic root, cardiopulmonary bypass was implemented. A bullet was retrieved from the noncoronary sinus of the aortic valve. Injuries to the anterior leaflet of the mitral valve and left ventricle were repaired.

Takayasu arteritis (TA) is a large-vessel arteritis affecting the aorta and its major branches. It is a rare disease in children less than 10 years old, and its diagnosis is frequently delayed, likely because of TA's rarity and nonspecific symptoms early in the disease. Females are affected disproportionately, with a female to male ratio of 8.5 to 1. Recently, the European League against Rheumatism published an international consensus statement for making the diagnosis of childhood TA. Criteria include angiographic abnormalities of the aorta and/or its branches, pulse deficit or claudication, blood pressure discrepancy, bruits, hypertension, and elevated acute phase reactants. We described a 10-year-old female with severe TA of the ascending aorta and who presented with classic signs and symptoms of this rare disease.

Congenital cardiac valve disease is common, affecting ∼1% of the population, with substantial morbidity and mortality, but suboptimal treatment options. Characterization of the specific matrix and valve cell phenotypic abnormalities in these valves could lend insight into disease pathogenesis and potentially pave the way for novel therapies.Thirty-five human aortic and pulmonic valves were categorized based on gross and microscopic assessment into control valves (n=21); dysplastic valves, all except one also displaying hemodynamic changes (HEMO/DYSP, n=6); and hemodynamically altered valves (HEMO, n=8). Immunohistochemistry was performed on valve sections and flow cytometry on valvular interstitial cells.While both hemodynamically altered aortic and pulmonic valves demonstrated increased collagen turnover and cell activation, prolyl 4-hydroxylase and hyaluronan increased in hemodynamically altered aortic valves but decreased in hemodynamically altered pulmonic valves relative to control valves (P.001). HEMO/DYSP aortic valves demonstrated decreased collagen and elastic fiber synthesis and turnover compared to both hemodynamically altered aortic valves and control aortic valves (each P.006). Valvular interstitial cells from both hemodynamically altered and HEMO/DYSP pulmonic valves showed altered cell phenotype compared to control valves (each P.032), especially increased non-muscle myosin. Furthermore, valvular interstitial cells from hemodynamically altered pulmonic valves and HEMO/DYSP aortic and pulmonic valves each demonstrated greater size and complexity compared to control valves (each P.05).Dysplastic semilunar valves displayed alterations in collagen and elastic fiber turnover that were distinct from valves similarly exposed to altered hemodynamics as well as to control valves. These results demonstrate that dysplastic valves are not simply valves with gross changes or loss of leaflet layers, but contain complex matrix and cell phenotype changes that, with future study, could potentially be targets for novel nonsurgical treatments.

Background Coarctation of the aorta (CoA) is often associated with clinically significant hypoplasia of the aortic arch. Historically, patch aortoplasty or bypass procedures have been the preferred techniques when arch augmentation is required in children beyond infancy. While safe and effective, these approaches require prosthetic or biologic material without the potential for growth, or normal endothelial and physiologic elastic function. This retrospective study reviews the use of a novel technique, ascending sliding arch aortoplasty, that utilizes viable autologous tissue for repair of arch obstruction in children beyond infancy. Methods Between April 2002 and January 2007, 8 patients ranging in age from 18 months to 15 years underwent repair of CoA with arch hypoplasia using ascending sliding arch aortoplasty. All patients were approached through median sternotomy, utilizing cardiopulmonary bypass and selective antegrade cerebral perfusion. Results There was no mortality or major morbidity. One toddler had pneumonia, resulting in an increased length of stay. Median duration of hospitalization was 5.8 days, ranging from 3 to 10 days. No patient had evidence of residual obstruction or recurrent CoA at a mean follow-up interval of 36 months. Conclusions Ascending sliding arch aortoplasty for CoA with arch obstruction in children beyond infancy is a safe technique that can be accomplished without deep hypothermic circulatory arrest. There is no evidence of recurrence at midterm follow-up. Because the augmentation is accomplished with viable autologous aortic tissue, the potential for growth, preserved elasticity and endothelial function, and resistance to infection make this method attractive for use in the young.

The development of mechanical circulatory support devices for infants and small children with heart failure has significantly lagged behind the development of devices for adults. In the United States, there was really very little activity focused on the development and implementation of durable mechanical assist devices for small children until the early part of this millennium. At present, the Berlin Heart EXCOR Pediatric ventricular assist device (Berlin Heart Inc., The Woodlands, TX) is currently the only device available to provide long-term support to small children awaiting cardiac transplantation; this device is only available through either compassionate use solicitation or within the confines of an ongoing investigation device exemption trial. There are several other devices being developed at this time, but it will be years before they are tested in clinical trials. In this article, we will review the current status of mechanical circulatory support devices and therapy strategies for infants and small children presenting in acute heart failure.

Background Beginning in 2000 and accelerating in 2004, the Berlin Heart EXCOR (Berlin Heart Inc Woodlands, TX) became the first pediatric-specific ventricular assist device (VAD) applied throughout North America for children of all sizes. This retrospective study analyzed the initial Berlin Heart EXCOR pediatric experience as a bridge to transplantation. Methods Between June 2000 and May 2007, 97 EXCOR VADs were implanted in North America at 29 different institutions. The analysis is limited to 73 patients (75%) from 17 institutions, for which retrospective data were available. Results Median age and weight at VAD implant were 2.1years (range, 12 days–17.8 years) and 11 kg (range, 3–87.6 kg), respectively. The primary diagnoses were dilated cardiomyopathy in 42 (58%), congenital heart disease in 19 (26%), myocarditis in 7 (10%), and other cardiomyopathies in 5 (7%). Pre-implant clinical condition was critical cardiogenic shock in 38 (52%), progressive decline in 33 (45%), or other in 2 (3%). Extracorporeal membrane oxygenation was used as a bridge to EXCOR in 22 patients (30%). Device selection was left VAD (LVAD) in 42 (57%) and biventricular assist devices (BiVAD) in 31 (43%). The EXCOR bridged 51 patients (70%) to transplant and 5 (7%) to recovery. Mortality on the EXCOR was 23% ( n = 17) overall, including 35% (11 of 31) in BiVAD vs 14% (6 of 42) in LVAD patients ( p = 0.003). Multivariate analysis showed younger age and BiVAD support were significant risk factors for death while on the EXCOR. Conclusions This limited but large preliminary North American experience with the Berlin Heart EXCOR VAD as a bridge to cardiac transplantation for children of all ages and sizes points to the feasibility of this approach. The prospective investigational device evaluation trial presently underway will further characterize the safety and efficacy of the EXCOR as a bridge to pediatric cardiac transplantation.

Factor V and thrombin inhibitors may develop following exposure to bovine thrombin preparations. In patient populations where exposure to bovine thrombin is common, such as children undergoing cardiovascular surgery, the development of such inhibitors should be considered in the evaluation of prolonged prothrombin times. We present three cases of children developing factor V and thrombin inhibitors following repeated exposure during cardiac surgical procedures.

BACKGROUND A discrete subaortic membrane cannot only cause left ventricular outflow tract obstruction, but can grow onto the aortic valve leaflets. The late finding of this encroachment is aortic valve insufficiency or stenosis. Echocardiography is used to follow the progression of outflow tract obstruction, but its ability to show subaortic membrane encroachment onto the aortic valve is unclear. The purpose of this study is to determine the sensitivity and specificity of echocardiography for diagnosing whether a discrete subaortic membrane involves the aortic valve. METHODS A pre-operative determination of aortic valve involvement by a discrete subaortic membrane was obtained by review of the official pre-operative echocardiogram reading and a retrospective blinded review of the pre-operative echocardiogram by an independent echocardiographer. These findings were compared to the intra-operative findings. RESULTS A total of 48 consecutive patients underwent primary resection for isolated discrete subaortic membrane between October, 1995 and May, 2006. The pre-operative and blinded readings both predicted a statistically lower rate of aortic valve involvement - 35% in 11 of 31 patients and 31% in 10 of 31 patients, respectively - than found at surgery - 65% in 31 of 48 patients. The sensitivity and specificity of pre-operative echocardiography to diagnose aortic valve involvement is 35% and 76%. Overall survival was 100%. There were no strokes, re-operations for bleeding or wound infections, or need for a pacemaker. CONCLUSION Echocardiography is not sensitive in assessing whether a discrete subaortic membrane involves the aortic valve. Since the morbidity and mortality for discrete subaortic membrane resection is negligible, resection may be indicated at the time of diagnosis to minimise aortic valve impairment.

Intramyocardial (IM) coronary arteries are characterized by a segment of a coronary artery (CA) that travels into the myocardium instead of the normal epicardial course. It may occur in isolation or in association with anomalous aortic origin of a CA (AAOCA). They are uncommon lesions in children

Atrial arrhythmias are common in patients with complex congenital heart disease and associated with increased morbidity and mortality. Reactive antitachycardia pacing (ATP) can terminate atrial arrhythmias and reduce progression to persistent atrial fibrillation but utility is unreported in complex

166 Background: Esophagogastrectomy following neoadjuvant chemo-radiation therapy for the treatment of locally advanced esophageal cancer is associated with significant morbidity and mortality. We hypothesized that the avoidance of neck anastomoses unless absolutely necessary to achieve negative margins and standardization of the anastomotic technique among surgeons would significantly decrease perioperative morbidity and potential mortality and improve oncologic outcomes for these patients. Methods: A HIPAA-compliant, prospectively maintained data base was retrospectively analyzed on 115 patients who underwent esophagogastrectomy following chemo-RT from 2007-2017 performed via a laparotomy and right thoracotomy using a standardized anastomotic technique. These procedures were performed by 5 different attending surgeons at 2 different academic medical centers. Peri-operative morbidity was assessed by standard measures and post-operative survival was assessed using Kaplan-Meyer survival. Results: There was 1 anastomotic leak and 1 mortality at 30 and 90 days. The overall 5-year survival was 40%. Major morbidity, defined as re-operation for bleeding, anastomotic leak, pneumonia, reintubation, chyle leak, and initial mechanical ventilation > 48 hours was 26% (30/113). Conclusions: Low anastomotic complication rates are associated with decreased perioperative morbidity and prolonged oncologic survival when compared to historical controls.

Background Ventricular septal defect (VSD) is the most commonly recognized congenital heart defect. With the development of device closure for intracardiac defects, we sought to evaluate current expectations for surgical closure of isolated VSD. Methods Between January 1, 2000, and December 31, 2006, 215 patients underwent isolated VSD repair at a median age of 10 months (range, 20 days to 18 years) and a median weight of 7 kg (range, 2 to 66 kg). The following VSD types were found: 172 perimembranous (80%), 28 supracristal (13%), 6 inlet (3%), and 9 muscular (4%). One hundred eight patients (50%) had evidence of congestive heart failure or failure to thrive preoperatively. Thirty-one patients (14%) had aortic valve cusp prolapse, and 63 (29%) had genetic abnormalities. Results Incidence of significant postoperative complications was extremely low. No patient underwent reoperation for a residual VSD. None had complete heart block. One operative mortality (0.5%) and 2 late deaths (0.9%) occurred. Median postoperative hospital length of stay was 5 days (range, 2 to 187 days). In the immediate postoperative period, 6 patients (2.8%) required reoperation. No patients were discharged on antiarrhythmic agents, had complete heart block, or required permanent pacing. At mean follow-up of 2.1 ± 2.0 years, 99.5% (211 of 212) of patients were asymptomatic from a cardiac standpoint. None exhibited greater than mild new-onset tricuspid valve regurgitation. No aortic valve injuries occurred. Conclusions Surgical closure of isolated VSD is a safe, effective therapy. Risk of death, complete heart block, and reoperation is minimal. As new technologies for VSD closure evolve, results such as these should be considered when evaluating patients, choosing therapeutic options, and counseling families.

Objective The purpose of this study was to prospectively analyze the outcomes of patients with anomalous aortic origin of a coronary artery undergoing surgical intervention according to a standardized management algorithm. Methods All patients aged 2 to 18 years undergoing surgical intervention for anomalous aortic origin of a coronary artery between December 2012 and April 2017 were prospectively included. Patients underwent stress nuclear perfusion imaging, stress cardiac magnetic resonance imaging, and retrospectively electrocardiogram-gated computed tomography angiography preoperatively. Patients were cleared for exercise at 3 months postoperatively if asymptomatic and repeat stress nuclear perfusion imaging, stress cardiac magnetic resonance imaging, and computed tomography angiography showed normal results. Results A total of 44 patients, with a median age of 14 years (8-18 years), underwent surgical intervention: 9 (20%) for the anomalous left coronary artery and 35 (80%) for the anomalous right coronary artery. Surgical procedures included unroofing in 35 patients (80%), translocation in 7 patients (16%), ostioplasty in 1 patient (2%), and side-side-anastomosis in 1 patient (2%). One patient who presented with aborted sudden cardiac death from an anomalous left coronary and underwent unroofing presented 1 year later with a recurrent episode and was found to have an unrecognized myocardial bridge and persistent compression of the coronary requiring reintervention. At last follow-up, 40 patients (91%) are asymptomatic and 4 patients have nonspecific chest pain; 42 patients (95%) have returned to full activity, and 2 patients are awaiting clearance. Conclusions Surgical treatment for anomalous aortic origin of a coronary artery is safe and should aim to associate the coronary ostium with the correct sinus, away from the intercoronary pillar. After surgery, the majority of patients are cleared for exercise and remain asymptomatic. Longer follow-up is needed to assess the true efficacy of surgery in the prevention of sudden cardiac death.

Objective. Volume unloading of the functional single ventricle after a bidirectional Glenn anastomosis (BDG) prior to 1 year of age leads to improved global ventricular function as measured by the myocardial performance index (MPI), a Doppler-derived measurement of combined systolic and diastolic ventricular function. Systolic function remains unchanged after BDG according to previous studies; however, acute changes in global and diastolic function have not been previously investigated in this cohort. Our objective was to assess the short-term effects of the BDG on global ventricular function in patients with a functional single ventricle. Design. Echocardiograms to obtain MPI, isovolumic contraction time, and isovolumic relaxation time were performed at four time periods: in the operating room, in the operating room prior to BDG, shortly after separation from cardiopulmonary bypass, less than 24 hours postoperatively, and either prior to hospital discharge or at the first clinic follow-up visit. Results. Twenty-six patients were enrolled. There was significant ventricular dysfunction noted shortly after separation from cardiopulmonary bypass, median MPI 0.63 (0.39–0.81), that persisted in the short term postoperatively median MPI 0.50 (0.40–0.63). Isovolumic contraction time did not change, however, isovolumic relaxation time was significantly prolonged following BDG. Conclusion. In the postoperative patient after BDG, systolic function is preserved; however, there is evidence of diastolic and global ventricular dysfunction, at least in the short term.

Early or late systemic outlet obstruction in patients undergoing staged palliation for single ventricle variants may preclude a successful Fontan circulation. In assessing the patient presenting with real or impending obstruction, the surgeon is justified in pursuing an aggressive approach in protecting ventricular and semilunar valve function. Options include subaortic resection (transventricular, transatrial, or via a semilunar valve), modifications of the Damus-Kaye-Stansel connection, or a palliative arterial switch operation. In this review, the various options will be discussed and presented in the context of application to specific morphologic elements that make one more favorable than another in an individual patient.

Despite the excellent operative survival for tetralogy of Fallot (TOF) repair, well-documented long-term complications and reduced life expectancy remain challenges for these patients and their clinicians. In an attempt to change the natural history of repaired TOF, we at Texas Children's Hospital (Houston, TX) have developed a management strategy not focused on age, but rather focused on preserving the right ventricular (RV) infundibulum. The RV infundibulum sparing (RVIS) repair of TOF consists of a transatrial and transpulmonary approach to close the ventricular septal defect and resect RV infundibular muscle coupled with a mini (< 5 mm) transannular patch or no ventricular incision. This strategy is applied with the ambition of decreasing the well-documented, long-term complications of TOF repair with large right ventriculotomies such as RV dilation, arrhythmias, need for pulmonary valve replacement, and RV failure. The RVIS strategy is an attempt based on our current knowledge and experience to optimize the time of repair so that we can not only maximize the early operative results but the long-term effects of this approach as these children mature into adolescents and adults. We have uniformly applied the RVIS strategy since 1995, which includes over 320 isolated TOF patients. We are currently reviewing this cohort in hopes that it will strengthen our beliefs and known results as well as give us more insight into whether the RVIS strategy can change the natural history of repaired TOF.

Timely extubation is a well-accepted strategy in the postoperative intensive care unit management of Fontan patients to minimize the deleterious effects of positive-pressure ventilation. In October 2002, this strategy was extended to extubating selective Fontan patients in the operating room (EOR). This retrospective study examines how EOR has affected outcomes and practice in our Fontan population.Between October 2002 and June 2006, 112 patients underwent primary Fontan procedures; 38 (34%) were EOR and 74 (66%) were non-EOR. These two cohorts were not different (p0.05) in age, weight, surgery time, dominant ventricular morphology, hypoplastic left heart syndrome, prior bidirectional Glenn, concomitant procedures, atrioventricular valve regurgitation, and ventricular function. Analysis of variance was used to compare mean pulmonary artery pressure, mean arterial blood pressure, and mean common atrial pressure as a function of time.During the first 12 hours postoperatively, mean pulmonary artery pressure and mean common atrial pressure were significantly lower and mean arterial blood pressure was significantly higher in the EOR group than the non-EOR group (p0.05). No EOR patient required reintubation. Mean durations of inotropic agents (1.1 versus 2.4 days), chest tubes (5.8 versus 7.2 days), intensive care unit stay (3 versus 4.7 days), and hospital stay (8.6 versus 11.3 days) for EOR patients were shorter than for non-EOR patients (p0.05). Intensive care unit and hospital costs for EOR patients were 35% and 31% lower, respectively, than for non-EOR patients (p0.05). Kaplan-Meier survival for EOR patients (3 years, 100%) was not different (p = 0.3) than for non-EOR patients (1 and 3 years, 96%).After the Fontan procedure, selective EOR can be performed safely and improves postoperative hemodynamics, decreases hospital resource utilization, and reduces hospital recovery time.

ObjectiveTight glycemic control improves outcomes in critically ill adults. There are limited data regarding the effect of glycemic profiles in infants after cardiac operations. The aim of this study was to evaluate the association of hyperglycemia and hypoglycemia on adverse events in infants undergoing the arterial switch operation.MethodsFrom 2000 through 2005, 93 infants underwent the arterial switch operation (mean age, 2.5 ± 5.9 weeks; mean weight, 3.4 ± 0.8 kg). All serum glucose values during the first 24 postoperative hours were documented. The effect of time spent in specific glycemic bands on adverse events was determined.ResultsTwenty-three (25%; group 1) infants spent more than 50% of the time with glucose values between 80 and 110 mg/dL, and 13 (14%; group 2) spent more than 50% of the time with glucose values of greater than 200 mg/dL. A total of 71 adverse events was documented in 45 (48%) of 93 infants. Group 1 infants were more likely to have any adverse event (P = .001) and renal insufficiency (P < .001). Group 2 infants were not more likely to have adverse events. When controlling for preoperative and operative factors, being in group 1 was an independent predictor of postoperative adverse events (P = .004).ConclusionHyperglycemia does not appear to be detrimental in postoperative infants with congenital heart disease. Infants who spent the majority of the time with glucose values between 80 and 110 mg/dL were at increased risk for adverse events. The ideal glycemic profile in the postoperative cardiac infant has yet to be defined.

Severe mitral regurgitation predicts poor outcomes in adults with left ventricular dysfunction. Frequently, adult patients now undergo initial mitral valve surgery instead of heart transplant. Pediatric data are limited. This study evaluates the efficacy of mitral valve surgery for severe mitral regurgitation in children with dilated cardiomyopathy. This is a single-institution experience in seven children (range, 0.5-10.9 years) with severe mitral regurgitation and dilated cardiomyopathy who underwent mitral valve surgery between January 1988 and February 2005, with follow-up to January 2006. Children with dilated cardiomyopathy had a depressed fractional shortening preoperatively (24.4% +/- 6.1%) that remained depressed (22.9% +/- 7.6%) 1.3 +/- 1.2 years after surgery (p = 0.50). Left ventricular end-diastolic (6.5 +/- 1.5 to 4.8 +/- 1.8 z-scores, p0.01) and end-systolic (6.8 +/- 1.5 to 5.5 +/- 2.1 z-scores, p0.05) dimensions improved. Hospitalization frequency had a median decrease of 6.0 hospitalizations per year (p0.02). Three patients were transplanted 0.2, 2.4, and 3.5 years after surgery. There was no perioperative mortality. Mitral valve surgery in children with dilated cardiomyopathy was performed safely and improved symptoms, stabilizing ventricular dysfunction in most patients. Mitral valve surgery should be considered prior to heart transplant in children with dilated cardiomyopathy and severe mitral regurgitation.

Neonates with congenital cardiac disease are a special population. They are often critically ill, and need prolonged intravenous access. To date, no study has evaluated the efficacy and safety of peripherally inserted central venous catheters placed in this unique population. Our goal was to evaluate the use of such catheters in neonates with critical congenital cardiac disease, and to study features such as duration of use, reasons for removal of catheters, and complications. We inserted a total of 124 catheters in 115 neonates with critical congenital cardiac disease who were admitted to the Intensive Care Unit at Texas Children's Hospital from August 2002 to August 2004. The patients had a mean age of 10 days, and a mean weight of 3.1 kilograms. The peripherally inserted catheters were in place for a mean of 22.3 days. Therapy was completed in 76.6% patients at the time of removal of the catheter. The incidence of occlusion, dislodgement, and thrombus was 4.0%, 2.4%, and 1.6%, respectively. The infection rate was 3.6 per 1000 catheter-days, with a median onset on 37 days after placement. We conclude that central venous catheters, when inserted peripherally, provide reliable and safe access for prolonged intravenous therapy in neonates with critical congenital cardiac disease.