Search

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.

Background Newborns with single-ventricle anatomy, transposition of the great arteries, and systemic outflow obstruction are challenging patients most often managed with a Norwood or Damus-Kaye-Stansel (DKS) procedure. The palliative arterial switch operation (pASO) offers the theoretical advantage of avoiding a systemic-to-pulmonary artery shunt physiology and posterior entrapment of the left pulmonary artery while aligning the single left ventricle with the posterior semilunar valve. Limited outcome data exist for the pASO. The purpose of this study was to examine the clinical course of patients after this operation. Methods We conducted a retrospective review of all neonates undergoing a pASO at our institution from July 1995 to June 2011. Results Fourteen patients underwent pASO at a median age of 7 days (2–16 days). Primary diagnoses included double-inlet left ventricle (6 patients [43%]), tricuspid atresia (TA) (4 patients [29%]), and other (4 patients [29%]). Concomitant procedures at initial operation included aortic arch reconstruction (13 patients [93%]), pulmonary artery banding (6 patients [43%]), and placement of a systemic-to-pulmonary artery shunt (1 patient [7%]). Median intensive care unit stay and hospital length of stay were 11 days (4–60 days) and 21 days (9–84), respectively. There were no deaths. All patients had advanced to a bidirectional cavopulmonary shunt (BCPS). Four patients required intervention between the pASO and BCPS procedures: systemic-to-pulmonary artery shunt (3 patients [21%]) and pulmonary artery banding revision (1 patient [7%]). Eleven patients had Fontan completion; the remaining 3 patients are candidates for this procedure. At the time of the Fontan operation, 7 (64%) patients required pulmonary artery augmentation. All patients were alive at last follow-up (median, 5 years [3 months–14 years]). At last echocardiographic follow-up (median, 5 years [3 months–10 years]), all patients had normal ventricular function, trivial to mild neoaortic insufficiency, and no left ventricular outflow obstruction. Conclusions The pASO should be considered in neonates with single ventricle, transposition, and systemic outflow obstruction because it affords a favorable anatomic arrangement for long-term palliation, with excellent survival and preserved ventricular function.

Congenital heart disease remains the most common birth defect affecting almost 1% of the population and if one considers congenitally bicuspid aortic valves, the number of affected individuals rises to as much as 4% by many estimates. The spectrum of congenital heart disease is broad. Patients may have very complex structural anomalies including an absence of a ventricle (single ventricle), lack of connection to a great artery (aortic or pulmonary atresia), anomalies of venous connections (systemic or pulmonary venous), valvar abnormalities (semilunar and/or atrioventricular), abnormal connections (transposition or malalignment), and vascular hypoplasia (aortic coarctation, hypoplasia, or interruption). Individuals affected by variations of these problems may present with acute circulatory compromise immediately at birth, later in infancy or on more rare occasions, later in life. The physiology at presentation relates to the structural morphology and may include cyanosis, low cardiac output (shock), arterial or venous obstruction, excessive pulmonary blood flow, and congestive heart failure. It has long been recognized that infants presenting with symptomatic congenital heart disease have an overall very poor prognosis if left untreated. On the less structurally complex end of the spectrum, patients may present with defects between cardiac chambers in isolation or in combination with other problems. Defects of the atrial or ventricular septa are most commonly observed. Again, there is very broad range of symptomatology and physiology relating to the type, size, location, and number of septal defects. For larger defects, with significant intracardiac shunting of blood, the patient’s shortand long-term prognosis may be significantly affected if the lesion is left untreated. Surgical, and more recently, catheter-based treatments for serious congenital cardiac defects represent a true success story of modern medicine. The earliest palliative surgeries, performed in the 1930s (ligation of patent ductus arteriosus and coarctation repair) and 1940s (creation of a systemicto-pulmonary artery shunt) ushered in the era of physiologic treatment for congenital heart disease. In subsequent decades, enormous progress was made in addressing more and more complex conditions including aggressive reconstructions. In the 1950s, cardiopulmonary bypass to support the systemic circulation offered the prospect of intracardiac correction (tetralogy of Fallot, atrioventricular canal, and atrial/ventricular septal defects). In the 1960s and 1970s, durable palliations were developed for single ventricle (Glenn and Fontan procedures). The 1980s saw increasingly aggressive operations for serious defects in newborns and small babies (transposition of the great arteries, truncus arteriosus, aortic interruption, and ultimately, very complex lesions including hypoplastic left heart syndrome). In the 1990s and the first decade of the new millennium, treatments have been further refined to improve operative survival, reduce morbidity, and consider long-term outlook including neurodevelopmental opportunities. Recent striking advances have included successful surgical correction of complex lesions in very small, preterm neonates, the development and implementation of catheter-based treatments for many conditions, and in utero interventions for fetuses with critical cardiac conditions. This article is a commentary on the following article: 10.1007/s12265011-9289-8.

Background The Society of Thoracic Surgeons (STS) Workforce on Congenital Heart Surgery undertook a second subspecialty Practice and Manpower Survey (2005, 2010) to obtain contemporary data. Methods Preliminary research suggested a potential target group of 273 congenital heart surgeons, including 258 at 125 US centers and 15 at 8 Canadian centers. The web-based survey was sent to these surgeons, plus all individuals listing pediatric cardiac surgery on CTSNet. Results Two hundred forty-six responders included 213 active congenital heart surgeons, 16 retired congenital heart surgeons, and 17 surgeons in training. Retirement age was 63.5 ± 7.5 years. Two hundred thirteen active congenital heart surgeon responders represent 78% of the original estimate. Their responses generated the following data: The mean age was 49.2 ± 8.5 (range, 35 to 75 years). American medical school graduates included 159 of 201 respondents (79%). Years of postgraduate training was 9.7 ± 1.7. One hundred ninety-seven (92%) respondents were certified in thoracic surgery by the American Board of Thoracic Surgery (ABTS). Twenty-eight of 200 (14%) received their congenital heart surgery training outside the United States or Canada. One hundred forty-three of 190 respondents (75%) perform exclusively congenital heart operations; 31 (16%) perform exclusively pediatric heart operations. Of 186 respondents, 54 (29%) perform fewer than 100 major congenital heart operations per year, 78 (42%) perform 100 to 199 procedures, and 54 (29%) perform 200 procedures or more. Active congenital heart surgeons have been in their current positions for 9.3 ± 8.6 years. Eight respondents are in their first year of practice. For 203 respondents, mean anticipated years to retirement is 16.1 ± 7.6. Twenty-eight anticipate retirement within 5 years; 31 in 6 to 10 years. Conclusions These data should help facilitate rational plans to meet workforce needs for an expanding patient population.

Fetal ultrasound provides the capacity for early detection of a variety of congenital heart diseases. We report a case of aneurysmal dilatation of a patent ductus arteriosus detected in utero and subsequently confirmed by transthoracic echocardiography in the neonatal period. Prompt recognition of the ductus aneurysm with resultant surgical ligation may have averted potentially serious complications from this condition.

Background Currently, there are no Food and Drug Administration–approved devices available that can provide long-term mechanical circulatory support to smaller children with severe heart failure as a bridge to heart transplant (HT). In recent years, the Berlin Heart EXCOR Pediatric ventricular assist device (VAD) has emerged as a potential treatment option. Systematic data on the safety and efficacy of the EXCOR are limited. Methods The Investigational Device Exemption (IDE) clinical study is designed to evaluate the safety and probable benefit of the EXCOR to support regulatory review of the device under the Humanitarian Device Exemption regulation. The study design and rationale are reviewed in light of the well-described challenges inherent in small population studies. Results The Berlin Heart EXCOR IDE clinical study is a prospective, multicenter, single-arm, clinical cohort study. Children aged 0 to 16 years with severe heart failure (Interagency Registry for Mechanically Assisted Circulatory Support profile 1 or 2) due to 2-ventricle heart disease and actively listed for HT comprise the primary study cohort. The control population is a propensity-matched retrospective cohort of children supported with extracorporeal membrane oxygenation, the only bridge device available to smaller children before the EXCOR. The primary efficacy end point is survival to heart transplantation or recovery. The primary safety end point is the incidence of serious adverse events as defined by pediatric Interagency Registry for Mechanically Assisted Circulatory Support criteria. The study will enroll a total of 48 subjects in 2 cohorts based on body surface area (cohort 1 2 , cohort 2 0.7-1.5 m 2 ) and is powered to show safety superiority to a prespecified performance goal of 0.25 serious adverse events per day of support. Children ineligible for the primary cohort will still have access to the device in a third compassionate-use cohort where adverse event data will be collected for additional safety characterization of the device. Conclusion The Berlin Heart IDE clinical study will be the first bridge-to-HT VAD study designed exclusively for children. It is anticipated that the study will provide important information on the safety and efficacy of the Berlin Heart EXCOR Pediatric in children while providing valuable lessons into the design and conduct of future VAD studies in children.

Neurological injury still occurs in association with pediatric cardiac surgery, despite major progress in reducing mortality. Forty to 50% of 5 year olds who underwent complex open cardiac surgery as neonates and young infants have impaired neurodevelopmental outcomes. In order to apply neuroprotective strategies, it is necessary to develop markers for acute neurological injury, and surrogates that predict later abnormal neurodevelopmental outcomes. Near infrared spectroscopy holds promise as a marker of acute brain injury, with several pediatric cardiac surgery studies indicating a relationship between prolonged low cerebral oxygen saturation and clinical or MRI change. EEG seizures have not been found to have a relationship with acute or longer term neurological injury in recent studies. Brain MRI indicates that approximately 30% of neonates have MRI injury before cardiac surgery, and 40% have new injury postoperatively, predominately white matter injury. Serum or urine biomarkers, such as S100β protein, neuron–specific enolase, or creatine kinase brain band, lack specificity for nervous system injury. Newer biomarkers, such as glial fibrillary acidic protein, ubiquitin C-terminal hydrolase 1, and phosphorylated axonal neurofilament heavy chain, have potential for much greater specificity. Neuroprotective strategies include cardiopulmonary bypass techniques which avoid circulatory arrest and low flows. Certain anesthetic regimens such as halogenated anesthetic agents, ketamine, and dexmedetomidine may be neuroprotective in pediatric cardiac surgery. Therapeutic hypothermia, and remote ischemic preconditioning are two simple techniques that may prove to have neuroprotective properties. Erythropoietin pretreatment, and treatment with neurotrophic factors such as brain-derived neurotrophic factor, glial cell line derived neurotrophic growth factor, vascular endothelial growth factor, and nerve growth factor also have potential to prevent and treat neuronal loss in the perioperative period. Finally, neural stem cells derived from the patient’s own umbilical cord blood could potentially be utilized for neuroprotection in neonates with a prenatal diagnosis of congenital heart disease.

Objective With improving operative mortality for staged palliation of hypoplastic left heart syndrome, interstage death accounts for an increasing proportion of hypoplastic left heart syndrome mortality. We investigated risk factors for death or cardiac transplantation during the interstage period between bidirectional Glenn and Fontan procedures in children with hypoplastic left heart syndrome. Methods Patients with hypoplastic left heart syndrome who underwent bidirectional Glenn between August 1995 and June 2007 were screened. Standard risk patients, defined by having been discharged after both Norwood and bidirectional Glenn, were included for analysis. Patient demographic, echocardiographic, cardiac catheterization, and operative data were reviewed. Interstage attrition was defined as death or cardiac transplantation more than 30 days after bidirectional Glenn and before the Fontan procedure. Statistical analysis was carried out using the Student t test, Pearson chi-square correlation, and Cox proportional hazard modeling for multivariable analysis. Results Ninety-two patients with hypoplastic left heart syndrome were alive at 30 days after bidirectional Glenn. Of these patients, 8 died and 3 underwent cardiac transplantation at a median of 391 days (range, 59–1175 days) after bidirectional Glenn, yielding an interstage attrition rate of 12%. Removing the 7 patients who are still awaiting Fontan (but all of whom are at least 3.5 years after bidirectional Glenn) adjusts the attrition rate to 13%. Interstage attrition did not correlate with hemodynamic data obtained at cardiac catheterization, aortic arch obstruction, or right ventricular dysfunction. Multivariable analysis demonstrated that the presence of moderate or severe tricuspid valve regurgitation (hazard ratio, 6.02; 95% confidence interval, 1.56–23.24; P z score (hazard ratio, 0.38; 95% confidence interval, 0.16–0.88; P = .02) were independent preoperative risk factors for interstage attrition. Conclusions Interstage attrition between bidirectional Glenn and Fontan procedures occurred in 12% of our study population. Moderate or greater tricuspid valve regurgitation and low weight z score at the time of bidirectional Glenn are important risk factors for interstage attrition between the bidirectional Glenn and Fontan procedures in children with hypoplastic left heart syndrome.

Objective. Discontinuous pulmonary arteries are believed to portend poor outcomes for a single ventricle palliation leading to Fontan's operation. This is a single institutional review of patients with single ventricle and discontinuous pulmonary arteries who underwent pulmonary artery centralization as part of staged surgical palliation. Design. The study is a retrospective case series. Patients. From November 1997 to December 2005, 12 centralization procedures were performed on 12 single ventricle patients with discontinuous pulmonary arteries. The diagnoses at surgery were as follows: heterotaxy 67%, pulmonary atresia 75%, a single morphologic right ventricle 58%, a single morphologic left ventricle 33%, and functional single ventricle with atrial situs inversus 8%. Outcome Measures. The outcome was assessed by hospital survival, actuarial survival, and New York Heart Association (NYHA) classification at follow-up. Results. The overall actuarial survival following centralization is 100% (95% confidence interval = 0.698 to 1). Seventy-five percent of the patients have undergone a Fontan procedure. Median McGoon ratio pre-Fontan = 1.65 (range: 1–2.1). Median follow-up after Fontan = 4.4 years (range: 1.2–9 years). Overall actuarial survival following Fontan is 100% (95% confidence interval = 0.428–0.911). Following the Fontan, there have been no thromboembolic complications, protein-losing enteropathy, nor Fontan takedowns. One hundred percent of the Fontan patients are NYHA class I. Conclusions. This experience indicates that a resuscitative strategy for discontinuous pulmonary arteries can result in good outcomes after the Fontan procedure. The presence of discontinuous pulmonary arteries in patients with single ventricle physiology should not preclude a management strategy with the goal of Fontan candidacy.

Right aortic arch with associated arch hypoplasia, coarctation, and an aberrant isolated left subclavian artery is a very uncommon variant of vascular rings, which itself accounts for fewer than 1% of all congenital cardiac defects. Several cases have been reported, and most commonly, extraanatomic grafts have been used for repair. We present a unique technique that involves the resection of the circumflex arch, aortic arch advancement, and end-to-side anastomosis to repair this rare aortic arch malformation.

Recently, there has been a surge of interest by clinicians, industry, and the government in the development and use of ventricular assist devices (VAD) in children. Despite this rapidly expanding interest, the incidence of VAD use in children across the United States is unknown. The Healthcare Cost and Utilization Project (H-CUP) Kids' Inpatients Database (KID) was analyzed to characterize the current utilization of VADs in children nationwide.The most recent HCUP-KID (2006) was analyzed (n = 7.5 million). This database is a nationwide sampling of hospital discharges of patients less than 20 years old weighted to provide national estimates.In 2006, 187 children had a VAD implanted in the United States. Mean age was 13 ± 7 years. Forty patients (21%) were bridged to VAD by extracorporeal membrane oxygenation. Forty-nine patients (26%) were bridged to heart transplant. Fifty-six patients (30%) died in hospital. Eighty-six patients (46%) were discharged or transferred. Length of stay was 29 days (range, 0 to 285). Total cost was $174,743 (range, $4,230 to $1,911,588). Sixty-seven hospitals placed VADs; 66% of VADs (124) were implanted at large teaching hospitals (more than 500 beds), and 46% (85) were at high-volume hospitals (more than 5 VADs per year). High-volume, large teaching hospitals (10) had better survival (89% versus 61%; p0.001) and lower hospital cost ($236,000 ± $184,000 versus $300,000 ± $355,000; p = 0.002) compared with all other hospitals. On multivariate analysis, acute renal failure and extracorporeal membrane oxygenation were risk factors for mortality, whereas transplant and being at a high-volume large teaching hospital were protective factors from death.Preliminary data suggest that the growing use of VADs in children may be better served in regard to resource utilization and outcomes if centralized to high-volume large teaching hospitals.

Background Mechanical support using a left ventricular assist device (LVAD) can lead to functional recovery of the myocardium in patients with end-stage heart failure (HF). Molecular remodeling, cytoskeletal disruption, and apoptosis activation are associated with abnormal gene expression in the failing ventricular myocardium of HF subjects and can normalize in response to medium- and long-term mechanical unloading in adults. However, there is little knowledge of the changes in gene expression after short-term mechanical support in children with HF. Methods We evaluated left ventricular biopsies from 4 children with HF. The children had implantation of a continuous- or a pulsatile-flow LVAD for 8 to 16 days before undergoing heart transplantation. At the time of LVAD insertion and removal, we performed quantitative real-time polymerase chain reaction (QPCR) to study the expression of 326 genes encoding for structural, transcriptional, and signaling pathways proteins, and immunoblot analysis on dystrophin and apoptotic factors. Results Short-term LVAD therapy significantly decreased brain natriuretic peptide (BNP) levels from pre-LVAD (3,584.5 ± 378.3 pg/ml [95% CI]) to post-LVAD (447.5 ± 52.7 pg/ml [95% CI]) in 2 patients in whom comparative BNP measurements were available. In addition, short-term LVAD therapy reduced HF and apoptosis markers, whereas it upregulated structural proteins, including dystrophin, as well as pro-hypertrophic and pro-inotropic markers. Furthermore, LVAD therapy normalized expression of genes involved in calcium homeostasis, cell growth, and differentiation. Conclusions Our pilot study suggests that even short-term LVAD therapy in children with severe HF can reverse molecular remodeling. This favorable effect should be taken into consideration in eligible children with significant ventricular dysfunction.

Objective: To assess the effects of transesophageal echocardiography (TEE) on hemodynamic variables during cardiac surgery in small infants. Design: A prospective clinical study. Setting: A medical college-affiliated tertiary care children's hospital. Participants: Twenty-three infants weighing 2 to 5 kg undergoing cardiac surgery. Interventions: Baseline heart rate, arterial pressure, and central venous pressure were recorded. A pediatric TEE probe was inserted, and the hemodynamic variables were again recorded. Postoperatively the hemodynamic measurements were measured again before and after probe removal, with the addition of left atrial pressure and pulmonary artery pressure when available. Hemodynamic parameters were carefully observed during all phases of the TEE examinations for any changes attributable to probe manipulation. Measurements and Main Results: No statistically significant changes occurred in this group of patients during TEE. No clinically significant changes in any individual patient occurred during the measurement or during manipulation of the TEE probe for the complete examination. Conclusion: Although hemodynamic compromise can occur in small infants, this study suggests that it is infrequent. Fear of hemodynamic compromise should not prevent use of intraoperative TEE in small infants when otherwise indicated.

UNLABELLED Transesophageal echocardiography (TEE) is frequently used during congenital cardiac surgery. Complications are infrequent, but interference with ventilation has been reported, especially in small infants. Ventilation variables were measured prospectively in 22 infants, 2-5 kg, undergoing heart surgery with TEE. Measurements were made preoperatively before and after TEE probe insertion and postoperatively before and after TEE probe removal. The variables measured included arterial blood gases, expired tidal volume, peak inspiratory pressure, positive end-expiratory pressure, minute ventilation, airway resistance, dynamic compliance, and peak inspiratory and expiratory flow rates. No significant change in any ventilatory variable at either time period was noted in the infants. IMPLICATIONS Ventilatory compromise is infrequent in small infants undergoing transesophageal echocardiography (TEE) examination. Careful ventilatory monitoring rapidly detects changes in ventilation during TEE examination. Small infants who benefit from TEE during heart surgery should not be excluded from receiving a TEE examination because of concern of ventilatory compromise.

The natural history of standard (large, transmural right ventriculotomy) repair of tetralogy of Fallot (TOF) is associated with a concerning incidence of right ventricular (RV) failure and reoperation. We believe preserving the infundibulum using a TOF repair method with a mini-(5 mm) or no ventricular incision optimizes RV function and confers long-term benefit. Over the past 13 years, we have uniformly applied this RV infundibulum sparing (RVIS) strategy.Using a retrospective cohort study design, 304 TOF patients who underwent the RVIS strategy (July 1995-June 2008) were reviewed. Median weight and age at repair: 8 kg (3-62 kg) and 9 months (2 days-23 years). Seventeen percent (51) of patients required a systemic-to-pulmonary artery shunt.Ninety-nine percent of patients had a mini- 73% (222) or no 26% (79) ventricular incision. Postoperative morbidity included arrhythmias 3% (10), postoperative bleeding 2% (7), temporary renal failure 1% (3), and neurologic injury1% (2). Thirty-day survival was 99.7%. Overall 1 and 7-year Kaplan-Meier survivals were 97% and 96%. In nonsyndromic children, only 1 patient has died in the RVIS strategy. A total of 3.2% (10) of patients had reoperations. Twenty-one percent (65/304) of patients have been followed for7 years (median: 8.5 years). None of them have severe dilation,mild RV outflow obstruction, an arrhythmia, or a pacemaker/AICD. Ninety-five percent of these patients have normal RV function; 3 (4.6%) had mild dysfunction. This cohort has excellent exercise tolerance (MaxVO2 (mean): 41 ± 12 mL/kg/min).The RVIS strategy has allowed morbidity, mortality, and reoperation rates to be minimized. Midterm results suggest that RVIS does appear to preserve RV function. Longer term follow-up will be essential in establishing if the RVIS strategy can change the natural history of repaired TOF.

Objective. Pulmonary autograft aortic root replacement (Ross' operation) is now associated with low operative risk. Recent series suggest that patients with primary aortic insufficiency have diminished autograft durability and that patients with large discrepancies between pulmonary and aortic valve sizes have a low but consistent rate of mortality. Therefore, Ross' operation in these patients has been avoided when possible at Texas Children's Hospital. Our objective was to report outcomes of Ross' operation when selectively employed in pediatric patients with aortic valve disease. Methods. Between July 1996 and February 2006, 55 patients (mean age 6.8 ± 5.5 years) underwent Ross' procedure. Forty-seven patients (85%) had a primary diagnosis of aortic stenosis, three (5%) patients had congenital aortic insufficiency, and five (9%) patients had endocarditis. Forty-two (76%) patients had undergone prior aortic valve intervention (23 [55%] percutaneous balloon aortic valvotomies, 12 [29%] surgical aortic valvotomies, 12 [29%] aortic valve replacements, 2 [5%] aortic valve repairs). Fourteen (25%) patients had ≥2 prior aortic valve interventions. Thirty-two patients (58%) had bicuspid aortic valves. Follow-up was 100% at a mean of 3 ± 2.5 years. Results. Hospital and 5-year survival were 100% and 98%, respectively. Morbidity included one reoperation (2%) for bleeding. Median length of hospital stay was 6 days (3 days–3 months). Six (11%) patients needed a right ventricular to pulmonary artery conduit exchange at a median time of 2.3 years. Freedom from moderate or severe neoaortic insufficiency at 6 years is 97%. Autograft reoperation rate secondary to aortic insufficiency or root dilation was 0%. Conclusions. By selectively employing Ross' procedure, outcomes of the Ross procedure in the pediatric population are associated with minimal autograft failure and mortality at mid-term follow-up.

A 4-year-old girl with a history of a moderate-sized secundum atrial septal defect and mild mitral valve prolapse underwent transcatheter closure with a 22-mm Amplatzer septal occluder (ASO) (AGA Medical Corporation, Golden Valley, Minn) device. Approximately 12 months later, she presented to the emergency department in septic shock with a 5-day history of fevers up to 105°F and was transferred to the pediatric intensive care unit. Her blood and urine cultures grew methicillin-sensitive Staphylococcus aureus within 24 hours. Multiple septic emboli were found on a brain magnetic resonance imaging scan. A transthoracic echocardiogram revealed vegetations on the mitral and tricuspid valves (Figures 1 and 2⇓ and Movies I and II), with a perforation in the mitral valve and severe mitral insufficiency. The patient was taken to the cardiovascular operating room, and severe mitral and tricuspid valve endocarditis was …

Antegrade cerebral perfusion (ACP) is a cardiopulmonary bypass technique that uses special cannulation procedures to perfuse only the brain during neonatal and infant aortic arch reconstruction. It is used in lieu of deep hypothermic circulatory arrest (DHCA), and thus has the theoretical advantage of protecting the brain from hypoxic ischemic injury. Despite this, recent comparative studies have shown no difference in neurodevelopmental outcomes with ACP versus DHCA for neonatal arch repair. This article presents animal and human data demonstrating that ACP flows less than 30 mL/kg/min are inadequate for many patients, and may be the explanation for lack of outcome difference versus DHCA. A technique for ACP, its physiologic basis, and a neuromonitoring strategy are presented, and then the results of an outcome study are reviewed, showing that with ACP technique at higher flows of 50 to 80 mL/kg/min guided by neuromonitoring, periventricular leukomalacia is eliminated on postoperative brain magnetic resonance imaging after neonatal cardiac surgery.

Objective. No ideal option exists for restoring pulmonary valve competence late after repair of the congenitally abnormal right ventricular outflow tract (RVOT). This has driven a continued search for new alternatives. Texas Children’s Hospital has recently used the Carpentier-Edwards Perimount RSR Pericardial Aortic Prosthesis (Edwards Lifesciences, Irvine, Calif, USA) for this indication and reports the initial experience. Design. Retrospective chart review. Setting. Academically affiliated tertiary-care pediatric hospital. Patients. Twenty-six patients who underwent pulmonary valve replacement with the Perimount® valve late after RVOT reconstruction between June 2002 and November 2005. Interventions. No prospective interventions. Outcomes Measures. Hospital morbidity and mortality. Valve function assessed by follow-up visits and echocardiograms. Results. Mean age and weight of the patients were 20.3 ± 9.8 years (range 7.0–45.1 years) and 56.2 ± 18.1 kg (range 35.8–109 kg). Twenty-two patients (85%) had severe pulmonary insufficiency (PI), 23 (89%) had symptomatic right heart failure, and 14 (54%) had moderate to severe right ventricular dysfunction. Average prosthetic valve size was 23 mm (range 19–27 mm). Twenty-one (88%) patients were extubated within 24 hours. There was no hospital mortality. Median length of stay for all patients from day of surgery was 6 days (range 3–56 days). Median length of last echocardiography follow-up was 12.4 months (range 0.1–37.6 months). At that time, 16 of the 26 (62%) patients had improved right ventricular function, no patient demonstrated significant RVOT obstruction, and 24 patients (92%) have no PI or mild PI. Freedom from death, reintervention, or reoperation on the pulmonary valve is 100% at 2.5 years. Conclusion. Initial results with the Perimount® bovine pericardial tissue prosthesis for pulmonary valve replacement are encouraging. Further follow-up is required to define long-term function and durability.

Patients suffering from acute cardiac graft rejection can die because of hemodynamic collapse while being treated with vigorous immunosuppressive therapies. There is little pediatric data on the use of mechanical circulatory support (MCS) in patients with acute cardiac graft rejection accompanied by hemodynamic instability. This report reviews our experience using MCS in patients with severe acute allograft rejection and cardiogenic shock. Between July 1995 and December 2006, 7 of 117 heart transplant recipients (6%) had MCS placed in 8 cases of acute graft rejection with hemodynamic instability. Devices used were BioMedicus (five), Thoratec (two), and extracorporeal membrane oxygenation machine (one). Mean age was 12 +/- 6.6 years. Median duration of support was 7.5 days (range, 3-28 days). Medical therapy applied included pulse steroids (eight), antithymocyte globulin (five), intravenous immunoglobulins (five), and plasmapheresis (five). Eighty-eight percent (seven of eight cases) weaned from MCS. Five patients weaned to recovery and two were bridged to retransplant. Five of the seven patients weaned (71%) were discharged home, all with normal left ventricular function. Median follow-up was 3.0 years (4.5 months to 3.5 years). One-year survival is 50% and 3 year survival is 38%. Mechanical circulatory support can be applied in patients with acute cardiac graft rejection causing hemodynamic instability with acceptable weaning and discharge rates. Unfortunately, late survival for this cohort remains poor.

We present a case of intrapericardial teratoma diagnosed by ultrasound at 26 weeks of gestation presenting as a large tumour mass and rapid development of hydrops fetalis. The fetus died in utero one day before scheduled open fetal surgery.

A retrospective study was undertaken of 157 patients who underwent a Glenn anastomosis between January 1996 and May 2001. Of these, 33 had heterotaxy syndrome: 20 males and 13 females, with a mean age of 1.26 ± 2.8 years. Twenty-five had right atrial isomerism and 5 had left isomerism. A common atrioventricular valve was found in 24 patients, 18 had bilateral superior venae cavae, and 18 had anomalous pulmonary venous return. Repair was carried out in 8 patients with anomalous pulmonary venous return, and pulmonary artery augmentation was performed in 11. Compared to the 124 patients who had a Glenn operation for single ventricle without heterotaxy, there were significantly longer durations of mechanical ventilation, intensive care unit stay, and inotropic support, as well as higher mortality in the heterotaxy group. Heterotaxy syndrome with single ventricle still has a high rate of morbidity and mortality. Patients with severe atrioventricular valve regurgitation are at risk of early death. Complete Fontan circulation may not be possible in all patients, and Glenn anastomosis may be their final palliation.

Limited data exist regarding the perioperative morbidity profile of adults who have cardiac operations at pediatric facilities.A retrospective review (January 2000 to December 2004) of patients (aged 18 years or older) who underwent cardiac surgery at our pediatric institution was performed.There were 149 cardiac operations performed in 135 patients. There were 2 early deaths. There were 70 preoperative noncardiac morbidities in 49 patients (36%) and 140 preoperative cardiac morbidities in 78 patients (58%). Preoperative arrhythmia (n = 76) and moderate or greater ventricular systolic dysfunction (n = 24) were most common. There were 51 postoperative adverse noncardiac events in 32 patients (24%). Renal insufficiency (0.5 mg/dL baseline change; n = 8) was most common. There were 53 postoperative adverse cardiac events in 44 patients (33%). Ventricular tachycardia (n = 13) was most common. Risk factors for postoperative adverse noncardiac events included preoperative histories of New York Heart Association (NYHA) class III or greater (p0.001), seizure (p = 0.04), and psychiatric disorder (p = 0.002). Risk factors for postoperative adverse cardiac events included older patient age (p = 0.001), preoperative functional single ventricle (p = 0.006), NHYA class III or greater (p = 0.003), atrial fibrillation/flutter (p0.001), and ventricular tachycardia or fibrillation (p = 0.04).Postoperative adverse events occur frequently when adults undergo cardiac operations at children's hospitals. Older patient age, preoperative arrhythmias, and preoperative NHYA class are predictors of postoperative adverse cardiac events.

Background Surgical repair of interrupted aortic arch (IAA) remains challenging and is associated with significant mortality and incidence of late arch obstruction, as recently reported by the Congenital Heart Surgeons' Society (CHSS). In particular, the CHSS reported that any technique other than direct anastomosis with patch augmentation is a risk factor for arch reintervention. The experience at Texas Children's Hospital with IAA repair using an aortic arch advancement technique without a patch was examined. Methods Between July 1995 and December 2005, 60 patients underwent IAA repair using aortic arch advancement without a patch. Selective cerebral perfusion was used in 25 patients (42%). Cox proportional hazards models were used to analyze 20 variables to determine risk factors for death, arch reintervention, and left ventricular outflow tract (LVOT) reintervention. Results Median age was 8 days (range, 2 to 271 days) and weight was 3.0 kg (range, 1.7 to 6.1 kg). IAA types were A in 18 (30%) and B in 42 (70%). Associated anomalies were multiple congenital anomalies in 30 (50%) patients, DiGeorge syndrome in 21 (35%), LVOT obstruction in 26 (43%), a single ventricle in 11 (18%), and truncus arteriosus in 6 (10%). Mean follow-up was 3.0 ± 2.6 years. Five-year freedom from aortic arch reintervention was 100%. Survival at 30 days, 1 year, and 5 years was 93%, 78%, and 76%, respectively. Since July 2000, two of 32 patients have died for an overall survival of 94%. Risk factors for death are older age, multiple congenital anomalies, DiGeorge syndrome, and bicuspid aortic valve. Selective cerebral perfusion was an independent protective variable for survival. Survival for an IAA patient with a ventricular septal defect and no complicating cardiac anomalies was 100%. Conclusions Aortic arch advancement without a patch can be applied to IAA patients, with the expectation of a minimal need for arch reintervention. This technique affords an excellent survival, to which selective cerebral perfusion may be a contributing factor.

A 1,700-g premature baby who presented with interrupted aortic arch type B, hypoplastic aortic annulus, severe subaortic stenosis, and large ventricular septal defect underwent successful surgical repair. A Ross-Konno operation was used to address the left ventricular outflow tract obstruction, hypoplastic aortic annulus, and ventricular septal defect. The interruption was repaired with a direct anastomosis.

For patients with transposition of the great arteries and a systemic right ventricle, complex late arterial-switch operations (double switch, switch conversion, Senning-Rastelli) after the newborn period have been described recently to restore the morphologic left ventricle to the systemic circulation. The purpose of this study was to describe the anesthetic management and perioperative outcome of this group of patients and to compare them with a control group of patients who had primary arterial-switch operations in the neonatal period.Retrospective database and medical record review with 3:1 control:case ratio.Tertiary care academic children's hospital.Patients undergoing complex late-arterial switch operations after the newborn period.None.Thirteen patients were identified in the complex late-switch group and 43 in neonatal arterial-switch group. There were no perioperative deaths, no new gross neurologic deficits, and all patients were discharged home in both groups. Anesthetic and bypass times were significantly longer in the late-switch group (745 v 558 minutes, p0.001, and 382 v 243 minutes, p0.001, respectively). Transfusion requirements were similar between the groups. The incidence of arrhythmia (92% v 9%, p0.001), use of pacing systems (69% v 9%, p0.001), cardioversion (15% v 0%, p = 0.05), and pharmacologic treatment of arrhythmias (69% v 0%, p0.01) intraoperatively were significantly higher in the complex late-switch group.Patients presenting for complex late corrective operations for transposition of the great arteries require long and complex anesthetics. Despite these challenges, perioperative outcomes are excellent.

We report a case of an infant with complex congenital heart disease who was placed on captopril for afterload reduction following cardiac surgery and subsequently developed pulmonary infiltrates with eosinophilia. The patient was readmitted with symptoms of rhinorrhea, poor feeding, and decreased activity level. She was found to have diffuse pulmonary infiltrates on chest radiograph and a marked peripheral eosinophilia without leukocytosis. After discontinuing captopril and starting systemic steroids, her symptomatology rapidly improved, and her eosinophilia and radiographic abnormalities both resolved.

Background: Near-infrared spectroscopy (NIRS) is a noninvasive optical monitor of regional cerebral oxygen saturation (rSO 2 ). The aim of this study was to validate the use of NIRS by cerebral oximetry in estimating invasively measured mixed venous oxygen saturation (SvO 2 ) in pediatric postoperative cardiac surgery patients. Methods: Twenty patients were enrolled following cardiac surgery with intraoperative placement of a pulmonary artery (PA) or superior vena cava (SVC) catheter. Five patients underwent complete biventricular repair - complete atrioventricular canal (n = 3) and other (n = 2). Fifteen patients with functional single ventricle underwent palliative procedures - bidirectional Glenn (n = 11) and Fontan (n = 4). Cerebral rSO 2 was monitored via NIRS (INVOS 5100) during cardiac surgery and 6 h postoperatively. SvO 2 was measured from blood samples obtained via an indwelling PA or SVC catheter and simultaneously correlated with rSO 2 by NIRS at five time periods: in the operating room after weaning from cardiopulmonary bypass, after sternal closure, and in the CICU at 2, 4, and 6 h after admission. Results: Each patient had five measurements (total = 100 comparisons). SvO 2 obtained via an indwelling PA or SVC catheter for all patients correlated with rSO 2 obtained via NIRS: Pearson's correlation coefficient of 0.67 (P < 0.0001) and linear regression of r2 = 0.45 (P < 0.0001). Separate linear regression of the complete biventricular repairs demonstrated an r = 0.71, r2 = 0.50 (P < 0.0001). BlandAltman analysis showed a bias of +3.3% with a precision of 16.6% for rSO 2 as a predictor of SvO 2 for all patients. Cerebral rSO 2 was a more accurate predictor of SvO 2 in the biventricular repair patients (bias -0.3, precision 11.8%), compared with the bidirectional Glenn and Fontan patients. Conclusions: Regional cerebral oximetry via NIRS correlates with SvO 2 obtained via invasive monitoring. However, the wide limits of agreement suggest that it may not be possible to predict absolute values of SVO 2 for any given patient based solely on the noninvasive measurement of rSO 2 . Near-infrared spectroscopy, using the INVOS 5100 cerebral oximeter, could potentially be used to indicate trends in SVO 2 , but more studies needs to be performed under varying clinical conditions.

OBJECTIVE. We present 10 patients with double aortic arch with atresia of the distal left arch segment, a form of incomplete double aortic arch, and describe the distinct MRI and CT findings for this potentially symptomatic vascular ring.CONCLUSION. Knowledge of the distinctive imaging appearance of this congenital arch anomaly can direct the radiologist to the correct preoperative diagnosis.

Some neonates with coarctation of the aorta (COA) present with cardiogenic shock and secondary end-organ injury. The management of this subgroup imposes unique challenges. We review our perioperative strategy and outcomes for neonates with COA who presented with cardiogenic shock.Neonates (younger than 30 days) with isolated COA or COA with aortic arch hypoplasia were identified. Retrospective review was performed to identify and characterize patients who presented with cardiogenic shock, defined as impaired left ventricular (LV) or right ventricular (RV) systolic function, or both, respiratory failure requiring tracheal intubation, and metabolic acidosis.Thirteen neonates presented in cardiogenic shock and underwent surgical repair. No patients required catheter or surgical reintervention for recoarctation. There were no deaths at a mean follow-up of 54 months. Group I neonates (isolated COA, n = 7) underwent end-to-end anastomosis through left thoracotomy. The mean age and pH at presentation were 9 (+/-1.1) days and 7.07 (+/-0.21), respectively. The mean preoperative and postoperative LV myocardial performance indices (MPI) were 0.81 (+/-0.22) and 0.37 (+/-0.16), respectively (p = 0.002). Group II neonates (COA with arch hypoplasia +/- ventricular septal defect, n = 6) underwent aortic arch advancement and ventricular septal defect closure through median sternotomy. The mean time from diagnosis to surgery in group II was 5.5 (+/-1.9) days versus 2.4 (+/-1.5) days in group 1 (p = 0.01). The mean age and pH at presentation were 11.8 (+/-9.3) days and 7.02 (+/-0.21), respectively. The mean preoperative and postoperative LV MPI were 0.46 (+/-0.13) and 0.35 (+/-0.11), respectively (p = 0.02). The total hospital length of stay in group II patients was 18 (+/-6.23) days versus 11.3 (+/-5. 7) days in group I (p = 0.04).Timely intervention with a strategy individualized to the patient anatomy can be performed with excellent outcomes in neonates with COA and cardiogenic shock. Neonates with isolated COA had worse preoperative LV MPI, which reflects more significant global left ventricular systolic dysfunction in this subgroup. The elapsed time from diagnosis to surgery was decreased in neonates with isolated COA.

Deep hypothermic circulatory arrest (DHCA) is commonly used for neonatal cardiac surgery. However, prolonged exposure to DHCA is associated with neurologic morbidity. The Norwood operation and aortic arch advancement are procedures that typically require DHCA during surgical correction. Regional low flow perfusion (RLFP) can be used to limit or exclude the use of circulatory arrest. This technique involves cannulation of the innominate or subclavian artery using a Gore-Tex graft, allowing isolated cerebral perfusion. Data was collected in 34 patients undergoing either neonatal aortic arch reconstruction or the Norwood procedure using RLFP. All patients had two arterial pressure monitors using either the umbilical or femoral artery catheters and radial or brachial catheters. Adequacy of perfusion was determined using cerebral saturation, blood flow velocity, mean arterial pressures, and arterial blood gas results. Cerebral saturation and blood flow velocity were monitored using the near-infrared spectroscopy (NIRS) (INVOS 5100, Somanetics Corp, Troy, MI) and a transcranial Doppler pulse-wave ultrasound (TCD) (EME Companion, Nicolet Biomedical, Madison, WI), respectively throughout the entire bypass period. Blood gases were monitored using a point of care blood gas analyzer (Gem Premier, Mallinckrodt Sensor System, Inc., Ann Arbor, MI). Data collected revealed total bypass times for repair between 69-348 min, with a mean of 180 min. Regional low flow perfusion times lasted between 6-158 min, with an average of 50 min., and DHCA times ranged from 0-66 min, with a mean of 19 min. The perfusion techniques used allowed patient clinical data to remain consistent throughout the cardiopulmonary bypass period, regardless of lower flows (Figure 1) The 30-day postoperative mortality rate was 2.9 %, with no evidence of neurologic injury during follow up. In conclusion, regional low flow cerebral perfusion might benefit patients by limiting the use of circulatory arrest during cardiac surgery. Further study is necessary to evaluate patient outcomes, comparing regional cerebral perfusion and circulatory arrest techniques.

Low flow hypothermic cardiopulmonary bypass, deep hypothermic circulatory arrest, and regional low-flow cerebral perfusion are special techniques used to facilitate complex intracardiac and aortic surgery in neonates and infants. Each carries a risk of cerebral hypoxia and neurologic morbidity. Neurologic monitoring in the form of near-infrared spectroscopy for cerebral oxygenation, transcranial Doppler ultrasound, and the bispectral index electroencephalogram can monitor the brain during these techniques to determine the minimum acceptable bypass flow rates or maximum acceptable duration of deep hypothermic circulatory arrest. The use of this monitoring has the potential to improve long-term neurologic and developmental outcome.

Physiologic cerebral monitoring has become an important part of our cardiovascular surgical unit. We recently encountered an unusual variant of aortic atresia that was first suggested by physiologic cerebral monitoring and required modification of our operative technique. We describe and discuss the anatomy, its translation into cerebral monitor findings, and how we modified our operative technique.

The spectrum of sternal wound infections after cardiac surgery ranges from superficial infections to a deep sternal infection known as mediastinitis. Mediastinitis is a rare but clinically relevant source of postoperative morbidity and mortality in adult and pediatric patients after cardiac surgery.We retrospectively identified all patients diagnosed with mediastinitis after cardiac surgery from January 1987 to December 2002 (17 patients/7,616 surgeries = 0.2%). Demographic data, cardiac diagnosis, cardiac surgery, hospital length of stay, associated medical diagnosis, and surgical treatment for mediastinitis were collected.Fifteen pediatric patients (age18 years) were diagnosed with mediastinitis (mean age at diagnosis 37.5 months, range 21 days to 17 years. The median postoperative day of diagnosis was 14 days (6 to 50 days). The most common organism was Staphylococcus species (n = 9). Six patients had an associated bacteremia. The median hospital length of stay for all patients was 42.5 days (range 16 to 163 days). The hospital mortality was 1 of 15 (6%). Each patient was treated with intravenous antibiotics; sternal debridement; and rectus abdominus flap reconstruction (n = 7), pectoralis muscle flap reconstruction (n = 3), omentum reconstruction (n = 1), or primary sternal closure (n = 4). Three patients have undergone redo-sternotomy with orthotopic heart transplantation, bidirectional cavopulmonary anastomosis, and replacement of a right ventricle to pulmonary artery homograft.Timely diagnosis, aggressive sternal debridement, and liberal use of rotational muscle flaps can potentially minimize the morbidity and mortality in pediatric postoperative cardiac patients. Subsequent redo-sternotomy has not been problematic.

Background A growing number of adults with functional single ventricles are presenting as candidates for first-time and redo-Fontan operations. This study describes the clinical presentation and early operative results of adults who have undergone Fontan modifications. Methods Between July 1995 and April 2003, 23 patients (>18 years old) had Fontan operations. We retrospectively reviewed their perioperative courses. Results Twenty-three Fontan operations (first-time [n = 8] and redo [n = 15]) were performed with no early or late deaths. No patient has required reoperation. One patient has been listed for orthotopic heart transplantation. The overall mean age is 23 years (18 to 41 years); mean follow-up, 30 months; median postoperative hospital stay, 8 days (4 to 34 days); and median duration of chest tube drainage, 4 days (2 to 12 days). The postoperative New York Heart Association (NYHA) functional class was improved in 22 of 23 patients. Eight first-time Fontan operations (7 of 8 nonfenestrated) were performed; lateral tunnel (n = 7) and extracardiac conduit (n = 1). Two patients had preoperative arrhythmias. New onset arrhythmias (ventricular tachycardia and sinus node dysfunction), requiring treatment, occurred in two patients. Fifteen redo-Fontan operations (all nonfenestrated) were performed; lateral tunnel (n = 5) and extracardiac conduit (n = 10). Fifteen patients had preoperative arrhythmias, thirteen of which had intraatrial reentry tachycardia (IART) and required antiarrhythmic medications. Concomitant intraoperative radiofrequency ablation (RFA) (n = 11) and cryoablation (n = 1) procedures were performed. In the immediate postoperative period, there was IART recurrence in five patients (post-RFA [n = 4] and postcryoablation [n = 1]). At latest follow-up, no patient is being treated with antiarrhythmic medications. Two patients had new onset atrial arrhythmias that required treatment. Conclusions The Fontan operation can be performed in adults with minimal morbidity and improved NYHA functional class. New onset arrhythmias requiring treatment are sources of perioperative morbidity. Complete arrhythmia resolution of the preoperative arrhythmia may not be achieved in the immediate postoperative period in redo-Fontan patients. However, modification (intraoperative radiofrequency ablation-right atrial debulking) of the atrial tachycardia circuits in the redo-Fontan patients can result in complete resolution of preoperative atrial tachyarrhythmias at early follow-up.

Adult patients with congenital heart disease are presenting more frequently for cardiac surgery. Frequently, pediatric congenital heart surgeons perform these procedures at pediatric hospitals. Between July 1995 and June 2000, a retrospective review of adult patients (> or = 18 years old) who had undergone cardiothoracic operations was performed. A total of 112 operations were performed and divided into two groups--81 cardiac operations in 79 patients and 31 noncardiac operations in 23 patients. One patient had a cardiac and noncardiac operation performed. The overall early operative mortality was 6% (6/101). There were 3 late deaths. New-onset cardiac arrhythmias requiring treatment were diagnosed after 5/81 (6%) cardiac operations. Six of 79 (7%) patients were diagnosed with postoperative clinical depression. An acceptable mortality can be achieved when adult patients undergo cardiothoracic operations at a pediatric facility. New-onset arrhythmias necessitating treatment are relatively common, and postoperative clinical depression should be anticipated.

Objective: To compare the perioperative outcome of patients ≥13 years old undergoing surgery for congenital heart disease in a children's hospital by a dedicated congenital heart surgery and anesthesia team with procedure-matched younger control patients. Design: Retrospective medical record review study. From October 1997 to July 2000, medical records of all patients >12 years old requiring cardiopulmonary bypass were reviewed. A control group of patients ≤5 years old was reviewed, and 2 patients were matched to each older patient by diagnosis and surgical procedure. Data are reported as mean ± SD. Older (study) patients were compared with younger (control) patients using t -test or chi square, with p ≤ 0.05 significant. Setting: Medical school–affiliated tertiary-care children's hospital. Participants: Patients undergoing congenital heart surgery. Measurements and Main Results: The study group (older patients) comprised 85 patients, and the control group (younger patients) comprised 170 patients. There were no intraoperative deaths. All major complications—cardiopulmonary resuscitation, neurologic injury, massive hemorrhage with sternotomy, femoral cannulation for emergent bypass, and severe episodes of hypotension on induction of anesthesia—occurred in older patients undergoing repeat sternotomy. Conclusion: Mortality and major morbidity were low in both groups; however, all major intraoperative incidents occurred in older repeat sternotomy patients, suggesting increased perioperative risk for adverse outcomes in these patients. Copyright 2002, Elsevier Science (USA). All rights reserved.

Controversy over benefits of pulsatile flow after pediatric cardiopulmonary bypass (CPB) continues. Our study objectives were to first, quantify pressure and flow waveforms in terms of hemodynamic energy, using the energy equivalent (EEP) formula, for direct comparisons, and second, investigate effects of pulsatile versus nonpulsatile flow on cerebral and renal blood flow, and cerebral vascular resistance during and after CPB with deep hypothermic circulatory arrest (DHCA) in a neonatal piglet model. Fourteen piglets underwent perfusion with either an hydraulically driven dual-chamber physiologic pulsatile pump (P, n = 7) or a conventional nonpulsatile roller pump (NP, n = 7). The radiolabeled microsphere technique was used to determine the cerebral and renal blood flow. P produced higher hemodynamic energy (from mean arterial pressure to EEP) compared to NP during normothermic CPB (13 +/- 3% versus 1 +/- 1%, p < 0.0001), hypothermic CPB (15 +/- 4% versus 1 +/- 1%, p < 0.0001) and after rewarming (16 +/- 5% versus 1 +/- 1%, p < 0.0001). Global cerebral blood flow was higher for P compared to NP during CPB (104 +/- 12 ml/100g/min versus 70 +/- 8 ml/100g/min, p < 0.05). In the right and left hemispheres, cerebellum, basal ganglia, and brainstem, blood flow resembled the global cerebral blood flow. Cerebral vascular resistance was lower (p < 0.007) and renal blood flow was improved fourfold (p < 0.05) for P versus NP, after CPB. Pulsatile flow generates higher hemodynamic energy, enhancing cerebral and renal blood flow during and after CPB with DHCA in this model.

Background: Perceived correlation between the coronary arterial anatomy in patients with complete transposition, and the outcome of the arterial switch procedure, has made preoperative identification of their patterns standard practice. Purpose: Our purpose was to assess the accuracy of preoperative echocardiographic identification of coronary arterial patterns, to evaluate the necessity of preoperative imaging by angiography, and to determine the impact of the coronary arterial anatomy on outcome. Methods: We reviewed the medical records of all patients referred for an arterial switch operation between August 1995 and January 2000. The anatomy as described at the time of the operation using the Leiden convention was compared to the preoperative echocardiographic and angiographic findings. Results: The procedure had been performed in 67 patients, at a mean age of 9 days, with a range from 3 days to 15 months. In 42 patients, the ventricular septum was intact, while 21 patients had a ventricular septal defect, and the other four had double outlet right ventricle with the aorta anterior and rightward. In 52 patients, the left coronary artery arose from sinus #1, and the right from sinus #2. In 8 patients, the interventricular branch of the left coronary artery arose from sinus #1, with the circumflex coronary artery arising together with the right coronary artery from sinus #2. In three patients, all three coronary arteries arose from sinus #1, while in the remaining individual patients, a large conal branch arose with the left coronary artery from sinus #1, the right coronary and left anterior descending arteries arose from sinus #1, all three coronary arteries took origin from sinus #2, and the left anterior descending and right coronary artery arose from sinus #1 with no circumflex coronary artery identifiable, respectively. In two patients (4%), we identified an intramural coronary arterial course. Echocardiography and angiography were comparable (81% versus 86%) in delineating the coronary arterial anatomy. Patients with a single arterial orifice, or an atypical coronary arterial anatomy, had a slightly longer stay on the intensive care unit, and in the hospital, but showed no difference in mortality. In fact, there was no early mortality (70% confidence limits; 0–2.9%), while two patients died late (2.9%). Conclusion: We conclude that complex coronary arterial anatomy does not preclude a successful arterial switch procedure, although patients with a single coronary artery, or other arterial patterns, had a slightly longer hospital course. Preoperative echocardiographic evaluation is comparable to non-selective coronary angiography. Irrespective of complexity, nonetheless, the coronary arteries can successfully be translocated, obviating the need for preoperative coronary angiography.

Anomalous origin of the left coronary artery from the pulmonary artery, also known as Garland-Bland-White syndrome, usually occurs as an isolated condition. We report an infant with caudal regression sequence diagnosed with interrupted aortic arch type B and type III aortopulmonary window, who was found to have anomalous origin of the left coronary artery from the right pulmonary artery at surgical repair. Successful repair of the aortopulmonary window and interruption was performed with reimplantation of the left coronary artery into the ascending aorta. This report highlights the importance of closely assessing the coronary ostia in patients undergoing complex aortopulmonary window repair.