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To investigate the influence of hypothermic cardiopulmonary bypass (HCPB) at 25°C and circulatory arrest at 18°C on the global and regional cerebral blood flow (CBF) during pulsatile perfusion, we performed the following studies in a neonatal piglet model. Using a pediatric physiologic pulsatile pump, we subjected six piglets to deep hypothermic circulatory arrest (DHCA) and six other piglets to HCPB. The DHCA group underwent hypothermia for 25 min, DHCA for 60min, cold reperfusion for 10 min, and rewarming for 40 min. The HCPB group underwent 15 min of cooling, followed by 60 min of HCPB, 10min of cold reperfusion, and 30 min of rewarming. The following variables remained constant in both groups: pump flow (150 ml/kg/min), pump rate (150 bpm), and stroke volume (1 ml/kg). During the 60-min aortic crossclamp period, the temperature was kept at 18°C for DHCA and at 25°C for HCPB. The global and regional CBF (ml/100g/min) was assessed with radiolabeled microspheres. The CBF was 48% lower during deep hypothermia at 18°C (before DHCA) than during hypothermia at 25°C (55.2± 14.3 ml/100 g/min vs 106.4±19.7 ml/100 g/min; p < 0.05). After rewarming, the global CBF was 45% lower in the DHCA group than in the HCPB group 48.3±18.1 ml/100 g/min vs (87±35.9 ml/100 g/min; p In conclusion, both HCPB and DHCA significantly decrease the regional and global CBF during CPB. Unlike HCPB, DHCA has a continued negative impact on the CBF after rewarming. However, 15 min after the end of CPB, there are no significant intergroup differences in the CBF.

Objectives: Patients undergoing cardiopulmonary bypass frequently manifest generalized systemic inflammation and occasionally manifest serious multiorgan failure. Inflammatory responses of bypass are triggered by contact of blood with artificial surfaces of the bypass circuits, surgical trauma, and ischemia-reperfusion injury. We studied the effects of specific inhibition of the alternative complement cascade by using an anti-factor D monoclonal antibody (166-32) in extracorporeal circulation of human whole blood used as a simulated model of cardiopulmonary bypass. Methods: Five healthy blood donors were used in the study. Monoclonal antibody 166-32 was added to freshly collected, heparinized human blood recirculated in a pediatric cardiopulmonary bypass circuit at a final concentration of 18 μg/mL. An irrelevant monoclonal antibody was used as a negative control with the same donor blood in a parallel bypass circuit on the same day. Blood samples were collected at different time points during recirculation for measurement of activation of complement, neutrophils, and platelets by immunofluorocytometric methods and enzyme-linked immunosorbent assays. Results: Monoclonal antibody 166-32 inhibited the alternative complement activation and the production of Bb, C3a, sC5b-9, and C5a. Upregulation of CD11b on neutrophils and CD62P on platelets was also significantly inhibited by monoclonal antibody 166-32. This is consistent with the inhibition of the release of neutrophil-specific myeloperoxidase and elastase and platelet thrombospondin. The production of proinflammatory cytokine interleukin 8 was also suppressed by the antibody. Conclusions: The alternative complement cascade is predominantly activated during extracorporeal circulation. Anti-factor D monoclonal antibody 166-32 is effective in inhibiting the activation of complement, neutrophils, and platelets. Inhibition of the alternative complement pathway by targeting factor D could be useful in reducing systemic inflammation in patients undergoing cardiopulmonary bypass. J Thorac Cardiovasc Surg 2001;122:113-22

Background . Over the past four decades, the surgical trend has been toward early, complete repair of tetralogy of Fallot (TOF). Many centers currently promote all neonates for total correction irrespective of anatomy and symptoms, with some surgeons advocating hypothermic circulatory arrest for repair in small infants. We believe this approach increases morbidity. Methods . Based on approximately 40 years' experience in 2,175 patients, we developed a management protocol focused on patient size, systemic arterial saturations, and anatomy. Symptomatic patients (hypercyanotic spells, ductal dependent pulmonary circulation) weighing less than 4 kg undergo palliative modified Blalock–Taussig shunt (BTS) followed by complete repair at 6 to 12 months. Asymptomatic patients, weighing less than 4 kg who have threatened pulmonary artery isolation, undergo BTS and repair at 6 to 12 months. All other patients undergo complete repair after 6 months. Results . From July 1, 1995, to December 1, 1999, 144 patients underwent operation for TOF (129 patients) or TOF with atrioventricular septal defect (TOF/AVSD, 15 patients). Ninety-four patients underwent one stage complete repair (88 TOF, 6 TOF/AVSD). Thirty-nine patients underwent repair after initial BTS (32 TOF, 7 TOF/AVSD). Ten patients are awaiting repair after BTS. The mean age and weight at complete repair were 18 months and 9 kg. There were no operative deaths. There have been 3 late deaths with complete follow-up (mortality 3 of 144 [2.1%]). Four of 133 patients (3%) have required reoperation after total correction. Conclusions . This management strategy optimizes outcomes by individualizing the operation to the patient. Advantages include avoidance of circulatory arrest, low morbidity and mortality, and low incidence of reoperation after complete repair.

OBJECTIVE The aim of this study was to determine the effect of prophylactic immune suppression on the incidence and severity of postpericardiotomy syndrome (PPS) in children after cardiac surgery with cardiopulmonary bypass (CPB). BACKGROUND Prophylactic suppression of the inflammatory response has an unknown effect on the incidence and severity of PPS in children undergoing surgery with CPB. METHODS This randomized double-blind placebo controlled trial included two study groups. Group A received pre-CPB intravenous methylprednisolone (1 mg/kg) plus four additional intravenous doses over 24 h, and Group B received intravenous saline placebo at identical intervals. Data included patient demographics, cardiac diagnosis/operation, CPB time, incidence and severity of PPS. Noncomplicated PPS—temperature >100.5°F, pericardial friction rub, patient irritability, small pericardial ± pleural effusion. Complicated PPS—noncomplicated PPS plus hospital readmission ± pericardiocentesis or thoracentesis. RESULTS We randomized 266 children: 20 exclusions (6 perioperative deaths, 14 reasons unrelated to treatment) leaving Group A (n = 126) and Group B (n = 120). There were no significant group differences in gender, cardiac diagnosis or CPB time. Group mean age differed (p = 0.05) and was treated as a covariate with no substantive outcome effect. In total, 39/246 children (16%) developed PPS (noncomplicated: n = 30, complicated: n = 9). There was no inter-group difference in overall PPS incidence (p = 0.73). However, Group A had a marginally significant increase in complicated PPS (p = 0.05). CONCLUSIONS Intravenous methylprednisolone at a standard anti-inflammatory dose administered pre-CPB and early post-CPB neither prevents nor attenuates PPS in children. Short-term pre-CPB and post-CPB methylprednisolone treatment may complicate PPS.

Mycotic pseudoaneurysm is a rare complication after orthotopic heart transplantation (OHT). This case describes a middle-aged OHT recipient who developed a mycotic pseudoaneurysm after an episode of acute cholecystitis. The region of involved aorta was effectively repaired using a patch of bovine pericardium and a brief period of hypothermic circulatory arrest. (J Card Surg 2010;25:749-751)

The purpose of this study was to quantify and compare pulsatile and nonpulsatile pressure and flow waveforms in terms of energy equivalent pressure (EEP) during cardiopulmonary bypass in a neonatal piglet model. EEP is the ratio of the area under the hemodynamic power curve and the flow curve. Piglets, mean weight of 3 kg, were used in physiologic pulsatile pump (n = 7), pulsatile roller pump (n = 6), and nonpulsatile roller pump (n = 7) groups. Data (waveforms of the femoral artery pressure, pump flow, and preaortic cannula extracorporeal circuit pressure) were collected during normothermic cardiopulmonary bypass at 35 degrees C (15 minutes on-pump), before deep hypothermic circulatory arrest (pre-DHCA) at 18 degrees C, and after cold reperfusion and rewarming (post-DHCA) at 36 degrees C. The pump flow rate was 150 ml/kg/min in all three groups. During pulsatile perfusion, the pump rate was 150 bpm in both pulsatile groups. Although there was no difference in mean pressures in all groups, EEP and the percentage increase of pressure (from mean pressure to EEP) of mean arterial pressure and preaortic cannula extracorporeal circuit pressure were higher with pulsatile perfusion compared with nonpulsatile perfusion (p < 0.001). In particular, the physiologic pulsatile pump group produced significantly higher hemodynamic energy compared with the other groups (p < 0.001). These results suggest that pulsatile and nonpulsatile flows can be quantified in terms of EEP for direct comparisons, and pulsatile flow generates higher energy, which may be beneficial for vital organ perfusion during cardiopulmonary bypass.

Organ injury (brain, kidney, and heart) has been reported in up to 30% of pediatric open heart surgery patients after conventional hypothermic non-pulsatile cardiopulmonary bypass (CPB) support with or without deep hypothermic circulatory arrest (DHCA). The effects of pulsatile (with a Food and Drug Administration approved modified roller pump) versus non-pulsatile perfusion on regional and global cerebral, renal, and myocardial blood flow were investigated during and after CPB with 60 minutes of DHCA in a neonatal piglet model.Piglets, mean weight 3 kg, were used in both pulsatile (n = 7) and non-pulsatile (n = 7) groups. After initiation of CPB, all animals were subjected to hypothermia for 25 minutes, reducing the rectal temperatures to 18 degrees C, 60 minutes of DHCA followed by 10 minutes of cold reperfusion and 40 minutes of rewarming with a pump flow of 150 mL/kg/min. During cooling and rewarming, alpha-stat acid-base management was used. Differently labeled radioactive microspheres were injected pre-CPB, on normothermic CPB, pre-DHCA, post-DHCA, and after CPB to measure the regional and global cerebral, renal, and myocardial blood flows.Global cerebral blood flow was significantly higher in the pulsatile group compared to the non-pulsatile group at normothermic CPB (100.4 +/- 6.3 mL/100 gm/min versus 70.2 +/- 8.1 mL/100 gm/min, p0.05) and pre-DHCA (77.2 +/- 5.2 mL/100 gm/min versus 56.1 +/- 6.7 mL/100 gm/min, p0.05). Blood flow in cerebellum, basal ganglia, brain stem, and right and left cerebral hemispheres had an identical pattern with the global cerebral blood flow. Renal blood flow appeared higher in the pulsatile group compared to the non-pulsatile group during CPB, but the results were statistically significant only at post-CPB (94.8 +/- 9 mL/100 gm/min versus 22.5 +/- 22 mL/100 gm/min, p0.05). Pulsatile flow better maintained the myocardial blood flow compared to the non-pulsatile flow after CPB (316.6 +/- 45.5 mL/100 gm/min versus 188.2 +/- 19.5 mL/100 gm/min, p0.05).Pulsatile perfusion provides superior vital organ blood flow compared to non-pulsatile perfusion in this model.

The mortality rate of infants who require renal replacement therapy after surgical repair of congenital heart disease has been reported to be 30%-79%. We report our experience with early initiation of continuous manual peritoneal dialysis (CPD) to treat fluid overload in 20 consecutive critically ill children who underwent CPD post cardiotomy. CPD catheters were inserted at the discretion of the cardiothoracic surgeon. CPD was started for evidence of total body fluid overload with inadequate urine output, and stopped when negative fluid balance was achieved and urine output improved. Median age was 10 days (range 3-186 days), mean time to start CPD post-operatively was 22 h (range 5-40 h), and mean duration of CPD was 50 h (range 13-92 h). CPD resulted in mean ultrafiltration of 93 ml/kg per day (range 43-233 ml/kg per day). Net negative fluid balance was 106 ml/kg per day (range 49-273 ml/kg per day). During CPD, the mean number of inotropes decreased from 2.2 to 1.6 (P

Several clinical and animal studies have demonstrated that pulsatile perfusion is more beneficial than nonpulsatile perfusion during short or long durations of extracorporeal circulation. Other investigators, however, have been unable to document these benefits. The issue remains controversial. Central to the debate is the issue of a precise definition of pulsatile flow. To help resolve the conflict, pulsatile flow may be quantified in terms of energy equivalent pressure. This formula contains both the arterial pressure and pump flow rate, which are the 2 most critical parameters for open heart surgery. This definition establishes common criteria for assessment of the effectiveness of extracorporeal support.

Blood trauma increases blood viscoelasticity by increasing red cell aggregation and plasma viscosity and by decreasing cell deformability. During extracorporeal circulation, the mode of perfusion (pulsatile or nonpulsatile) may have a significant impact on blood trauma. In this study, a hydraulically driven dual chamber pulsatile pump system was compared to a standard nonpulsatile roller pump in terms of changes in the blood viscosity and elasticity during cardiopulmonary bypass (CPB) and pre and post deep hypothermic circulatory arrest (DHCA). Piglets, with an average weight of 3 kg, were used in the pulsatile (n = 5) or nonpulsatile group (n = 5). All animals were subjected to 25 min of hypothermia, 60 min of DHCA, 10 min of cold reperfusion, and 40 min of rewarming with a pump flow of 150 ml/kg/min. A pump rate of 150 bpm, pump ejection time of 120 ms, and stroke volume of 1 ml/kg were used during pulsatile CPB. Arterial blood samples were taken pre-CPB (36 degrees C), during normothermic CPB (35 degrees C), during hypothermic CPB (25 degrees C), pre-DHCA (18 degrees C), post-DHCA (19 degrees C), post-rewarming (35 degrees C), and post-CPB (36 degrees C). Viscosity and elasticity were measured at 2 Hz and 22 degrees C and at strains of 0.2, 1, and 5 using the Vilastic-3 Viscoelasticity Analyzer. Results suggest that the dual chamber neonate-infant pulsatile pump system produces less blood trauma than the standard nonpulsatile roller pump as indicated by lower values of both viscosity and elasticity during CPB support.

Transesophageal echocardiography (TEE) and central venous catheter (CVC) placement are often used during congenital cardiac surgery. Complications of CVC placement include cardiac perforation, inadvertent arterial placement, and erroneous hemodynamic data from unrecognized malposition. In this study, we used a prospective, randomized, controlled design to evaluate the use of TEE to guide depth of insertion and confirm superior vena cava cannulation, and to improve the percentage of correctly placed CVCs and reduce complications of CVC placement. One hundred forty-five patients were studied. Eighty patients were randomized to have subclavian vein insertion, 64 to have internal jugular insertion, and 1 to have external jugular insertion of CVC. TEE-guided CVC placement resulted in 100% correct placement when assessed by preoperative TEE, versus 86% in the control group (72 of 72 vs 63 of 73; P 5 0.01). There was no difference in correct placement between the two groups when assessed by postoperative chest radiograph (81.9% TEE versus 75.3% control; P 5 not significant). One significant complication, a superior vena cava perforation, occurred in the control group. Time to placement was 9.6 min in the TEE group versus 8.0 min in the control group (P 5 0.015). Implications: Transesophageal echocardiography can be used to guide central venous catheter placement in congenital heart surgery. Central venous catheters that seem to be located high in the right atrium by chest radiograph in these patients are often actually in the superior vena cava and pose little risk of cardiac perforation. (Anesth Analg 1999;89:65‐70)

Cannulating a pediatric patient with an interrupted aortic arch presents many challenges. Two cannulas are necessary to allow for proper blood flow to the head and to the extremities. This case was made more difficult by the weight of the child and the small size of the ascending aorta. The available pediatric cannulas were too large. The perfusionist adapted two 14-gauge intravenous (i.v.) catheters as arterial cannulas which were incorporated into the 1/4-inch arterial line. The 14-gauge i.v. catheters worked successfully. While on cardiopulmonary bypass, the patient was both metabolically and hemodynamically stable. The patient survived the procedure and was eventually discharged from hospital.

6-mo-old infant was referred for closure of aventricular septal defect (VSD). Transthoracic echo-cardiography obtained for evaluation of a cardiacmurmur at 2 mo of age demonstrated a largeperimembranous VSD. Subsequent examination re-vealed partial occlusion of the defect by surround-ing aneurysmal tissue, billowing of this tissue intothe right atrium (RA), and the concern for a leftventricular (LV)-to-RA communication. Surgical in-tervention was undertaken in view of the clinicalevidence of a moderate intracardiac shunt.After induction of anesthesia, transesophageal echo-cardiography (TEE) was performed. Two-dimensionalimaging demonstrated a large, septated, cystic mass inthe posteroinferior aspect of the RA (Fig. 1; please seevideo clip available at www.anesthesia-analgesia.org).Color flow Doppler interrogation identified shuntingfrom the LV into the saccular structure, and from thisinto the right ventricle (RV) (Please see video clipavailableatwww.anesthesia-analgesia.org).Notricus-pid inflow obstruction was detected. A second area ofleft-to-right shunting was noted in the adjacent mem-branous ventricular septum.Surgical inspection demonstrated aneurysmal fi-brotic degeneration of the atrioventricular (AV) mem-branous septum protruding into the RA. Fenestrationsin the aneurysmal sac, in immediate proximity to thetricuspid valve (TV), appeared to allow for the egressof blood directly into the RV. Two distinct anatomiccommunications were identified in the membranousseptum: one between the LV and aneurysmal sacwithin the RA, and another between the ventricles.The intervention consisted of pericardial patch closureof the defects and repair of a TV leaflet incisionrequired for anatomic exposure. Because of the con-cern for creating AV block, the aneurysmal tissuecould not be resected in its entirety and a few fenes-trations remained on the septal aspect of the tricuspidannulus. Postoperative TEE demonstrated a smalleraneurysmal sac, mild tricuspid regurgitation, and noevidence of residual septal defect shunting. Agitatedsaline injection into a central venous catheter showeda small systolic stream from the RV into the aneurysm,consistent with residual fenestrations along the septalTV leaflet (Please see video clip available at www.anesthesia-analgesia.org).The membranous ventricular septum is divided bythe attachment of the septal leaflet of the TV into twocomponents: the AV portion, or region immediately

The use of mechanical circulatory support as a bridge to cardiac transplantation is now an accepted therapeutic option. Also, as the criteria for organ transplantation have broadened, multi-organ transplantation has become more feasible. We report the cases of 2 children with end-stage heart failure and renal failure who were successfully bridged to combined, single-donor heart and kidney transplantation with mechanical circulatory support.

Summary Congenital heart surgery is associated with a 2–25% reported incidence of neurological complication. Near-infrared spectroscopy (NIRS) can detect changes in regional cerebral saturation index (rSO2i) during cardiac surgery. If rSO2i decreases significantly, treatment algorithms are used to restore baseline values, potentially avoiding neurological complications. The efficacy of bilateral NIRS monitoring in pediatric congenital heart surgery has been debated. We report a case in which bilateral NIRS monitoring detected an abrupt decrease in rSO2i (right greater than left) after initiation of bypass without abnormalities detected by standard monitors. This resulted in prompt surgical intervention that restored rSO2i, potentially preventing neurological injury.

Anomalous left coronary artery from pulmonary artery (ALCAPA) is associated with high mortality if left uncorrected. We describe long-term outcomes after surgical repair and ventricular function changes on long-term follow-up.A retrospective review of patients who had ALCAPA repair from January 1996 to December 2011 was completed. Ventricular function was assessed by shortening fraction and ejection fraction, left ventricular end-diastolic dimension, and severity of mitral regurgitation. Speckle tracking echocardiography served as a marker of early myocardial dysfunction.In total, 34 patients underwent ALCAPA repair at median age of 5 months (range, 3 days to 39 years). Surgical interventions included coronary translocation in 31 patients (91%), Takeuchi repair in 2 (6%), and coronary ligation in 1 patient (3%). Concomitant mitral valve repair was performed in 5 patients (15%). No patient required mechanical circulatory support postoperatively. There was no early mortality. At median follow-up of 6 years (range, 1 month to 14 years), there were 5 reoperations: 2 heart transplants (6%), 1 mitral valve replacement (3%), 1 coronary artery bypass graft (3%), and 1 ventricular septal defect closure (3%); there was 1 death 2 years after surgery. Ejection fraction improved from 21% ± 6% to 60% ± 7% (p = 0.008), mean shortening fraction from 25% ± 14% to 38% ± 5% (p = 0.01), mean left ventricular end-diastolic dimension Z-score from 6.0 ± 3.8 to 0.9 ± 0.7 (p0.001), and number of patients with moderate-severe mitral regurgitation from 44% (15 of 34) to 5% (1 of 21). All patients had normal ejection fraction and shortening fraction at last follow-up. Speckle tracking echocardiography showed decreased global longitudinal (p = 0.01) and circumferential strain (p = 0.03) for 11 of 14 patients (79%).Excellent outcomes are achieved with repair of ALCAPA without mechanical circulatory support and with low reintervention rates. Normal ejection fraction and shortening fraction do not accurately convey myocardial dysfunction in these patients.

Background The optimal treatment for infants with aortic coarctation and hypoplastic aortic arch is controversial. The goal of this study was to report the short-term and mid-term outcomes of aortic arch advancement (AAA) in infants with hypoplastic aortic arch. Methods All infants who underwent AAA at our institution from 1995 to 2012 were included. AAA consisted of coarctectomy and end-to-side anastomosis of the descending aorta to the distal ascending aorta/proximal arch through a median sternotomy. The cohort was divided into four groups: (1) isolated AAA (n = 29, 11%), (2) AAA with closure of ventricular septal defect (n = 56, 20%), (3) AAA with other biventricular repairs (n = 115, 42%), and (4) AAA as part of single-ventricle palliation (n = 75, 27%). Results The cohort included 275 patients: 125 (45%) were female, and the median age was 14 days (interquartile range, 7–34 days). Genetic abnormalities were present in 48 patients (17%). Neurologic adverse events occurred in 3 patients (1%), all in group 4. Left bronchial compression was seen in 2 patients (0.7%); only one required intervention. Vocal cord dysfunction was noted in 36 of 95 patients (38%) on routine laryngoscopy. Only 1 patient had clinical residual dysfunction at the last follow-up visit. Perioperative mortality was 3% (n = 8). At a median follow-up time of 6 years, 8 patients (3%) had reinterventions at a median time of 5 months (3–17 months) after repair. Conclusions AAA is a safe, effective, and durable operation with low rates of adverse events and mid-term reintervention. The advantages include native tissue-to-tissue reconstruction and preserved potential for growth. As such, it is the ideal technique for the management of hypoplastic aortic arch in neonates and infants.

Background . Platypnea and Orthodeoxia have been described with congenital heart and severe lung diseases. Methods . We report 4 patients in whom platypnea and orthodeoxia developed after pneumonectomy. In these patients the mean oxygen saturation on room air was 65% (range, 45% to 79%) in the supine position. On O 2 therapy it improved to 94% (range, 80% to 99%). When the patients assumed the erect position and were receiving O 2 therapy the saturation dropped to a mean of 76% (range, 56% to 82%) and the patients complained of shortness of breath. Cardiac catheterization revealed a mean pulmonary capillary wedge pressure of 11.6 mm Hg (range, 7 to 18 mm Hg). All patients had normal right atrial pressure. A right-to-left interatrial shunt through a patent foramen ovale was documented by transesophageal echocardiography and dynamic ultrafast magnetic resonance imaging. The patients underwent surgical closure of the patent foramen ovale. Results . In the erect position, the room air O 2 saturation improved to a mean of 95% (range, 92% to 99%), and the shortness of breath disappeared. Conclusions . Postpneumonectomy patients complaining of shortness of breath should be assessed for platypnea and orthodeoxia. A right-to-left interatrial shunt through a patent foramen ovale can occur even in the absence of elevated right heart pressures, especially after right pneumonectomy, and is accentuated in the upright posture. Surgical correction of the patent foramen ovale can produce dramatic improvement. (Ann Thorac Surg 1997;63:198–201)

Background There is a paucity of data on the current outcomes of surgical intervention for recurrent aortic arch obstruction (RAAO) after initial aortic arch repair in children. The goal of this study is to report the long-term results in these patients. Methods All patients undergoing surgical intervention for RAAO at Texas Children's Hospital from 1995 to 2012 were included. The cohort was divided into four groups based on initial procedure: (1) simple coarctation repair, (2) Norwood procedure, (3) complex congenital heart disease, and (4) interrupted aortic arch. Results A total of 48 patients age 9 months (range, 22 days to 36 years) underwent 49 procedures for RAAO. All patients had an anatomic repair consisting of either patch aortoplasty (n = 27, 55%), aortic arch advancement (n = 8, 16%), sliding arch aortoplasty (n = 6, 12%), placement of an interposition graft (n = 2, 17%), reconstruction with donor allograft (n = 4, 8%), extended end-to-end anastomosis (n = 1, 2%), or redo Norwood-type reconstruction (n = 1, 2%). Most procedures (n = 46, 94%) were performed through a median sternotomy using cardiopulmonary bypass. At a median follow-up of 6.1 years (range, 9 days to 17 years), only 2 patients required surgical or catheter-based intervention for RAAO. Hypertension was present in 10% of patients at last follow-up. There were no neurologic or renal complications. There was 1 perioperative death after an aortic arch advancement in group 1. Four other patients have died during follow-up, none of the deaths related to RAAO. Conclusions Anatomic repair of RAAO is a safe procedure associated with low morbidity and mortality, and low long-term reintervention rates.

Improving mortality rates in children undergoing surgery for congenital heart disease has enabled a shift in focus to improving morbidity, particularly with respect to neurological complications. Various factors have been implicated in influencing neurological outcomes. We share our experience in formulating a customized cardiopulmonary bypass (CPB) protocol based on currently available evidence. Theoretical advantages of intraoperative neuromonitoring during CPB, specifically use of near-infrared spectroscopy, will be discussed in the context of methodologies to monitor cerebral perfusion during surgery.

The field of pediatric mechanical circulatory support with ventricular assist devices has lagged significantly behind that of adult patients. However, there is increasing attention on the emerging field of pediatric ventricular assist device support. In this review, part of the Pediatric Cardiac Intensive Care Society/Extracorporeal Life Support Organization Joint Consensus Statement on Mechanical Circulatory Support, we discuss several important aspects of pediatric ventricular assist device, focusing on biomechanics and selection of the most appropriate device.

Patients with right heart obstructive lesions develop residual or recurrent right ventricle outflow tract pathology as a result of native or implanted pulmonary valve (PV) dysfunction. Until recently, the standard of care has been surgical placement of a PV or valved right ventricle to pulmonary artery conduit. Catheter-based options are being increasingly applied in patients with PV dysfunction. The purpose of our study was to evaluate outcomes of surgical pulmonary valve/conduit replacement (PVR) at a large pediatric hospital to provide contemporary benchmark data for comparison with developing technologies.Retrospective review of patients undergoing PVR not associated with complex concomitant procedures from July 1995 to December 2010 was completed. Inclusion criteria were designed to generally match those applied to patients promoted for catheter-based valve replacement based on age and weight (age≥5 years and weight≥30 kg).There were 148 PVRs with all patients having undergone ≥1 previous interventions (tetralogy of Fallot [53%] and pulmonary atresia [17%]). Surgical indications were PV insufficiency (60%), PV stenosis (26%), and both (13%). Valves used included bioprosthetic (n=108; 73%) and homografts (n=40; 27%). Time-to-extubation, intensive care unit stay, and hospital length of stay were1 day (interquartile range, 0-1 day), 2 days (interquartile range, 1-2 days), and 5 days (interquartile range, 4-6 days), respectively, with no hospital deaths. Freedom from PV reintervention at 1, 3, and 5 years was 99%, 99%, and 94%, respectively. Multivariable analysis showed age13 years (P=.003), and smaller valve size (P=.025) were associated with increased risk of valve reintervention. Patient survival at follow-up (mean, 5.0±3.9 years) was 99%.Surgical PVR is safe with low in-hospital and midterm follow-up mortality and reoperation rates. These outcomes provide a useful benchmark for treatment strategy comparisons.

ObjectiveThere is limited information regarding the true incidence of and risk factors for chylothorax after pediatric cardiac surgery. The objective of this study was to determine, from a large multi-institution database, incidence, associated factors, and treatment strategy in patients undergoing pediatric cardiac surgery.MethodsAll patients younger than 18 years in the Pediatric Health Information System (PHIS) database who underwent congenital heart surgery or heart transplant from 2004 to 2011 were included. Procedure complexity was assessed by Risk Adjustment for Congenital Heart Surgery-1.ResultsIn all, 77,777 patients (55% male) of median age 6.7 months were included. Overall incidence of chylothorax was 2.8% (n = 2205), significantly associated with increased procedure complexity, younger age, genetic syndromes, vein thrombosis, and higher annual hospital volume. Patients with multiple congenital procedures had the highest incidence. Incidence increased with time, from 2% in 2004 to 3.7% in 2011 (P

To improve outcomes, including arrhythmia incidence, for patients with tetralogy of Fallot (TOF), the authors’ institution adopted an approach that minimizes or avoids transmural incision of the right ventricular outflow tract. When pulmonary blood flow is insufficient during the neonatal period, placement of an aortopulmonary artery shunt is preferred, followed by complete repair later in infancy. This study reviewed the perioperative and mid-term arrhythmia outcomes at the authors’ institution using this approach. Patients who underwent TOF repair from 1995 to 2008 were included in the study. Patient demographics and surgical history were collected. The primary end points of the study included documented perioperative arrhythmias and arrhythmias at the 10-year follow-up assessment. Of the 298 patients who underwent TOF repair, 50 (17 %) had undergone prior placement of a systemic-to-pulmonary artery shunt. The median age at repair was 9.7 months (interquartile range, 6.3–16.2 months). Clinically significant perioperative arrhythmias were found in 12 patients (4 %) including 6 junctional tachycardias, 4 atrial tachycardias, and 1 temporary complete heart block. No patients were receiving antiarrhythmic medications more than 24 months after surgery. Of the 298 patients, 86 (29 %) had a follow-up period of 10 years or longer (median, 12.2 years). No patients experienced new arrhythmias, received antiarrhythmic therapy, experienced post-discharge ventricular tachycardia, had atrioventricular block, or required a pacemaker or defibrillator. The right ventricular infundibulum sparing approach is associated with an extremely low incidence of perioperative and midterm arrhythmias. The perioperative and mid-term outcomes compare favorably with existing data from programs favoring neonatal repair. Long-term follow-up evaluation is essential to determine whether this strategy can effectively alter late pathophysiology and minimize late-term arrhythmias and associated mortality.

Background: There are limited studies analyzing pulsatile Glenns (PG) as a palliation strategy for complex single ventricle (SV) patients. This study sought to determine their outcomes at a single institution. Methods: All SV pts who underwent PG from 1995-2016 were included. Failure was defined as

Structural cardiac defects, such as Tetralogy of Fallot, often requires surgical placement of a patch to expand the right ventricular outflow tract (RVOT) in an area normally consisting of contractile myocardial tissue. Current cardiac patch materials are biologically inert and will not grow with a pediatric patient, often requiring reoperations. In this study, novel multi-layered scaffolds with a polycaprolactone core, a chitosan-based scaffold, and either gelatin or decellularized porcine heart matrix were implanted into a full thickness rat right ventricle defect for up to 8 weeks. The results show that engineered scaffolds were biodegradable and promoted tissue remodeling. Histological analysis of control fixed pericardium patches showed little to no cellular infiltration, while engineered scaffolds had significant muscular and vascular cell remodeling. Quantitative MRI revealed that left ventricular ejection fractions were stabilized in all patched hearts after 8 weeks, and the right ventricular ejection fraction in hearts with engineered patches was significantly greater than hearts with control pericardium patches. In addition, patches with heart matrix promoted a denser vascular network and a higher M2/M1 inflammatory macrophage ratio when compared to patches containing only gelatin. Collectively, these results show that these multi-layered patches are capable of full thickness defect repair and regeneration.

Anomalous aortic origin of coronary arteries (AAOCA) is the second most common cause of sudden cardiac death in young athletes and remains a challenging diagnosis with variable clinical and surgical approaches to management. We sought to review our 17-year institutional experience with functional

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore “hot topics” to highlight areas of emerging science for clinicians and scientists in moving toward a better understanding of the long-term management of patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac magnetic resonance is the gold standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta, and aortopulmonary collaterals, and on its ability to quantify biventricular size and function, pulmonary regurgitation (PR), and myocardial viability. Atrial re-entrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2%/yr. Risk stratification, even with electrophysiologic testing and cardiac magnetic resonance, remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite its high risks for complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia, and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, PR may be treated with transcatheter valve insertion. Ongoing surveillance of RV function is a crucial component of clinical assessment. Except for resynchronization with biventricular pacing, no medical therapies have been shown to be effective after RV dysfunction occurs. In patients with significant PR with RV dilation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging.

While survival of children with complex congenital heart defects has improved in recent years, roughly half suffer neurological deficits suspected to be related to cerebral ischemia. Here we report the first demonstration of optical diffuse correlation spectroscopy (DCS) for continuous and non-invasive monitoring of cerebral microvascular blood flow during complex human neonatal or cardiac surgery. Comparison between DCS and Doppler ultrasound flow measurements during deep hypothermia, circulatory arrest, and rewarming were in good agreement. Looking forward, DCS instrumentation, alone and with NIRS, could provide access to flow and metabolic biomarkers needed by clinicians to adjust neuroprotective therapy during surgery.

Two patients with functional single ventricle and a left superior vena cava, which drained to the coronary sinus, are presented. Each had undergone a Fontan modification that incorporated the coronary sinus into the Fontan pathway. Each developed severe right atrial and coronary sinus dilatation. Revision of a Fontan with an intraatrial lateral tunnel and an extracardiac conduit resulted in improved functional capacity of the patients.

The Blalock-Taussig shunt (BTS) was introduced 68 years ago before open repair of cyanotic congenital heart disease (CHD) was possible. The originally described technique has undergone many modifications but remains an integral component of the management of cyanotic CHD. We report our contemporary, single institution experience with the BTS.We performed a retrospective review of all patients treated with a BTS from June 1995 to December 2011.There were 730 BTS performed in 712 patients; 727 (99.6%) by interposition graft (modified). The BTS was predominantly right-sided (n = 657, 90%). Median age and weight at palliation were 8 days (range 0 days to 18.5 years) and 3.2 kg (1.5 to 51 kg). Median hospital length of stay was 16 days (range 0 to 347 days). There were 241 (33%) BTS performed as initial palliation for ultimate 2-ventricle (2V) circulation, 471 (65%) as part of staged palliation for patients with functionally univentricular lesions (1V), 6 (1%) as a part of 1.5-ventricle palliation, and 12 (1%) for Ebstein's anomaly. There were 473 (65%) BTS placed via sternotomy and the most common site of BTS was the right subclavian to right pulmonary artery (PA; n = 452, 62%). Hospital mortality was higher for BTS in 1V patients (1V 15% vs 2V 3%, p0.0001). Overall, 536 (73%) patients were bridged to complete repair or the second stage of 1V palliation after a median duration of 6.5 months (0 days to 15.3 years). Multivariable regression showed that sternotomy approach, use of cardiopulmonary bypass, innominate artery-PA shunt, and diagnosis of Ebstein's were risk factors for in-hospital mortality (p0.05).Although the BTS remains an important component of the surgical treatment of cyanotic congenital heart disease, patients with single ventricle circulation still face significant ongoing risk of mortality.

The pressure reactivity index (PRx) identifies optimal cerebral perfusion pressure after traumatic brain injury. We describe a method to improve PRx precision by induced variations in arterial blood pressure (ABP) using positive end-expiratory pressure (PEEP) modulation ( iPRx). Neonatal swine ( n = 10) were ventilated with static PEEP and then with PEEP oscillated between 5 and 10 cmH2O at a frequency of 1/min. PRx was recorded as a moving correlation coefficient between ABP and intracranial pressure (ICP) from spontaneous ABP activity (0.05-0.003 Hz) during static PEEP. iPRx was similarly recorded with PEEP oscillation-induced ABP waves. The lower limit of autoregulation (LLA) was delineated with continuous cortical laser Doppler flux monitoring. PEEP oscillation increased autoregulation-monitoring precision. The ratios of median absolute deviations to range of possible values for the PRx and iPRx were 9.5% (8.3–13.7%) and 6.2% (4.2–8.7%), respectively ( P = 0.006; median, interquartile range). The phase-angle difference between ABP and ICP above LLA was 161° (150°–166°) and below LLA, −31° (−42° to 12°, P < 0.0001). iPRx above LLA was −0.42 (−0.67 to −0.29) and below LLA, 0.32 (0.22–0.43, P = 0.0004). A positive iPRx was 97% specific and 91% sensitive for perfusion pressure below LLA. PEEP oscillation caused stable, low-frequency ABP oscillations that reduced noise in the PRx. Safe translation of these findings to clinical settings is expected to yield more accurate and rapid delineation of individualized optimal perfusion-pressure goals for patients.

Options for mechanical circulatory support as a bridge to heart transplantation in children with severe heart failure are limited.We conducted a prospective, single-group trial of a ventricular assist device designed specifically for children as a bridge to heart transplantation. Patients 16 years of age or younger were divided into two cohorts according to body-surface area (cohort 1,0.7 m(2); cohort 2, 0.7 to1.5 m(2)), with 24 patients in each group. Survival in the two cohorts receiving mechanical support (with data censored at the time of transplantation or weaning from the device owing to recovery) was compared with survival in two propensity-score-matched historical control groups (one for each cohort) undergoing extracorporeal membrane oxygenation (ECMO).For participants in cohort 1, the median survival time had not been reached at 174 days, whereas in the matched ECMO group, the median survival was 13 days (P0.001 by the log-rank test). For participants in cohort 2 and the matched ECMO group, the median survival was 144 days and 10 days, respectively (P0.001 by the log-rank test). Serious adverse events in cohort 1 and cohort 2 included major bleeding (in 42% and 50% of patients, respectively), infection (in 63% and 50%), and stroke (in 29% and 29%).Our trial showed that survival rates were significantly higher with the ventricular assist device than with ECMO. Serious adverse events, including infection, stroke, and bleeding, occurred in a majority of study participants. (Funded by Berlin Heart and the Food and Drug Administration Office of Orphan Product Development; ClinicalTrials.gov number, NCT00583661.).

ObjectivesSurgical treatment of aortic valve (AoV) disease in childhood involves complex decisions particularly in very small patients. There is no consensus regarding the optimum surgical option. The objective of this review was to analyze a contemporary experience of AoV surgery in a large children's hospital.MethodsA retrospective review of children (aged ≤18 years) undergoing AoV repair or replacement from June 1995 to December 2011 was carried out.ResultsA total of 285 AoV operations (97 repairs, 188 replacements) were performed on 241 patients. Hospital survival for repair was 98% and for replacements was 97%. At follow-up of repairs, there were 16 (17%) reoperations and 3 (3%) late deaths. Follow-up of AoV replacements demonstrated 31 (16%) reoperations (homograft 27, autograft 3, mechanical 1) and 8 (4%) late deaths (homograft 5, autograft 2, mechanical 1). Freedom from reintervention or death (FRD) was found to be lower in repairs for infants (P = .048) and truncal valves (P

Cardiovascular dysfunction is a primary independent predictor of age-related morbidity and mortality. Frailty is associated with activation of inflammatory pathways and fatigue that commonly presents and progresses with age. Interleukin 10 (IL-10), the cytokine synthesis inhibitory factor, is an anti-inflammatory cytokine produced by immune and non-immune cells. Homozygous deletion of IL-10 in mice yields a phenotype that is consistent with human frailty, including age-related increases in serum inflammatory mediators, muscular weakness, higher levels of IGF-1 at midlife, and early mortality. While emerging evidence suggests a role for IL-10 in vascular protection, a clear mechanism has not yet been elucidated. Methods In order to evaluate the role of IL-10 in maintenance of vascular function, force tension myography was utilized to access ex-vivo endothelium dependent vasorelaxation in vessels isolated from IL-10 knockout IL-10(tm/tm) and control mice. Pulse wave velocity ((PWV), index of stiffness) of vasculature was measured using ultrasound and blood pressure was measured using the tail cuff method. Echocardiography was used to elucidated structure and functional changes in the heart. Results Mean arterial pressures were significantly higher in IL-10(tm/tm) mice as compared to C57BL6/wild type (WT) controls. PWV was increased in IL-10(tm/tm) indicating stiffer vasculature. Endothelial intact aortic rings isolated from IL-10(tm/tm) mice demonstrated impaired vasodilation at low acetylcholine doses and vasoconstriction at higher doses whereas vasorelaxation responses were preserved in rings from WT mice. Cyclo-oxygenase (COX-2)/thromboxane A2 inhibitors improved endothelial dependent vasorelaxation and reversed vasoconstriction. Left ventricular end systolic diameter, left ventricular mass, isovolumic relaxation time, fractional shortening and ejection fraction were all significantly different in the aged IL-10(tm/tm) mice compared to WT mice. Conclusion Aged IL-10(tm/tm) mice have stiffer vessels and decreased vascular relaxation due to an increase in eicosanoids, specifically COX-2 activity and resultant thromboxane A2 receptor activation. Our results also suggest that aging IL-10(tm/tm) mice have an increased heart size and impaired cardiac function compared to age-matched WT mice. While further studies will be necessary to determine if this age-related phenotype develops as a result of inflammatory pathway activation or lack of IL-10, it is essential for maintaining the vascular compliance and endothelial function during the aging process. Given that a similar cardiovascular phenotype is present in frail, older adults, these findings further support the utility of the IL-10(tm/tm) mouse as a model of frailty.

Objectives Infective endocarditis is rare in children but potentially carries high mortality and morbidity. Few data exist regarding surgical therapy and the associated outcomes in children with infective endocarditis. The aim of the present study was to describe the characteristics and outcomes of children undergoing surgery for infective endocarditis. Methods A retrospective review of all patients aged 21 years or younger diagnosed with definitive infective endocarditis at a single center from 1996 to 2010 was performed. Results Of 76 identified patients with infective endocarditis (median age, 8.3 years; 73.9% boys), 46 patients (61%) required surgical intervention. Staphylococcus aureus was most commonly isolated (18 patients, 24%) followed by Streptococcus (17 patients, 22%). Common surgical indications included severe valvular insufficiency in 13 patients, septic embolization in 12, concomitant severe valvular insufficiency and ventricular dysfunction in 9, persistent vegetations in 9, and persistent bacteremia in 3. Although early surgery was performed within 7 days of diagnosis in 35 patients (76%), 25 (54%) underwent surgery within 3 days or less. The factors associated with surgery included the presence of ventricular dysfunction, left-sided vegetation, severe valvular insufficiency, septic embolization, and S aureus . Surgery within 3 days or less was associated with the presence of ventricular dysfunction and S aureus . Native valve repair was performed in 50% of patients with native-valve disease. Postoperatively, no septic embolization events occurred and recurrence was low (2%). The 1-, 5-, and 10-year survival was 98% ± 2%, 90% ± 8%, and 81% ± 11%, respectively. Conclusions Children with infective endocarditis can undergo successful early surgical therapy with a low risk of septic embolization, recurrence, and operative mortality.

This article presents 21 “Quality Measures for Congenital and Pediatric Cardiac Surgery” that were developed and approved by the Society of Thoracic Surgeons (STS) and endorsed by the Congenital Heart Surgeons’ Society (CHSS). These Quality Measures are organized according to Donabedian’s Triad of Structure, Process, and Outcome. It is hoped that these quality measures can aid in congenital and pediatric cardiac surgical quality assessment and quality improvement initiatives.

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.