The neonate with congenital heart disease: what the cardiac surgeon needs to know from the neonatologist and the cardiologist.

To plan and accomplish a successful operation for a neonate with congenital heart disease, the cardiac surgeon requires a complete anatomic description of the cardiovascular malformation. For optimum outcome, this information must be supplemented by a complete report of the prenatal and postnatal course of the newborn as well as by a thorough summary of any noncardiac congenital or acquired abnormalities. In the most favorable circumstance, the neonate arrives in the operating room completely diagnosed, fully resuscitated, well nourished, and with appropriate monitoring devices in place.

Systemic venous oxygen saturation after the Norwood procedure and childhood neurodevelopmental outcome.

Objective: Neonates with hypoplastic left heart syndrome have impaired systemic oxygen delivery and also have a high risk of hypoxic ischemic brain injury with resultant neurodevelopmental impairment. We hypothesized that decreased postoperative oxygen delivery, as measured on the basis of systemic venous oxyhemoglobin saturation, would be related to persistent neurodevelopmental abnormality assessed in childhood.

Methods: Early perioperative hemodynamic data, prospectively acquired from neonates undergoing staged palliation of hypoplastic left heart syndrome by using deep hypothermic circulatory arrest with uniform perioperative management, were tested for relationship to later neurodevelopmental outcome assessed at age 4 years.

A medical strategy to reduce persistent chest tube drainage after the fontan operation.

A standardized medical regimen aimed at reducing pleural effusions after the Fontan operation was compared with a randomly selected retrospective cohort. The duration of chest tube drainage, hospital stay, and the need for pleural sclerosis were significantly reduced, indicating that postoperative management plays an important role in reducing this morbidity.

Aortic valve repair.

Aortic valve replacement options are limited in children, and all of them have disadvantages. Aortic valve repair techniques have evolved slowly and have not gained wide acceptance; however, large series using a variety of techniques demonstrate that valve repair is possible with excellent early hemodynamics and satisfactory intermediate durability. The results of aortic valve repair at the Children's Hospital of Wisconsin are presented.

Noninvasive assessment of cardiac output.

Improved outcome from shock depends on early detection and correction of circulatory abnormalities. Global cardiac output and oxygen delivery must be adequate and distributed appropriately to meet metabolic demands to prevent the development of multiple organ system dysfunction, prolonged morbidity, and death. Circulatory assessment using standard monitors gives incomplete and sometimes misleading information.

Complex aortic valve repair as a durable and effective alternative to valve replacement in children with aortic valve disease.

Objective: This study was undertaken to determine the utility of aortic valve repair in children.

Methods: A retrospective analysis was conducted on aortic valve surgery from 1973 to 2004 at Children's Hospital of Wisconsin.

Results: Procedures were classified as simple repairs (blunt valvotomy, commissurotomy with or without thinning, n = 147), repair of aortic insufficiency with ventricular septal defect (n = 22), complex repairs (any combination of additional procedures including suspension of prolapsed leaflets, leaflet extensions, repair of torn or perforated leaflets, annuloplasty, reduction of sinus of Valsalva plasty, and concomitant repair of supravalvular or subvalvular stenosis, n = 57), and replacements (n = 57, 20 mechanical, 2 porcine, and 35 human valves).

Erythropoietin protects the infant heart against ischemia-reperfusion injury by triggering multiple signaling pathways.

The immediate protective effect of erythropoietin (EPO) against ischemia in heart suggests a role beyond hematopoiesis and the treatment of anemia. We determined the role of JAK/STAT and Ras/Rac/MAPK in the protective effect of EPO against ischemia-reperfusion injury in infant rabbit heart. EPO (1.

Newborn heart surgery: reasonable expectations and outcomes.

This article discusses the necessity for standards for reasonable expectations and outcomes for neonatal treatment of congenital heart disease and the databases that allow such standards to be measured and to be compared among treatment centers.

Delayed cardioprotection with isoflurane: role of reactive oxygen and nitrogen.

We determined whether isoflurane can confer delayed cardioprotection in the adult rat by triggering increased production of reactive oxygen (ROS) and nitrogen species (RNS). Our objectives were to determine 1) the concentration of isoflurane that confers delayed cardioprotection in the adult rat, 2) the role of ROS and RNS in the induction of delayed cardioprotection, and 3) the cellular sources of ROS and RNS responsible for induction of delayed cardioprotection by isoflurane. Male Sprague-Dawley rats at 8 wk of age (n = 8 rats/group) were exposed to 0.

Surgical therapy for sudden cardiac death in children.

Sudden cardiac death (SCD) in children is the result of multiple etiologies and treatment (prophylaxis) must be tailored accordingly. In children who do not have congenital heart disease, surgical therapy of SCD typically consists of implantation of an internal defibrillator, with specific attention to the small size of the patient. In children who have unrepaired congenital heart disease, therapy of SCD is primarily repair of the congenital anomaly.

Cardioprotection in chronically hypoxic rabbits persists on exposure to normoxia: role of NOS and KATP channels.

Hypoxia from birth increases resistance to myocardial ischemia in infant rabbits. We hypothesized that increased cardioprotection in hearts chronically hypoxic from birth persists following development in a normoxic environment and involves increased activation of nitric oxide synthase (NOS) and ATP-dependent K (K(ATP)) channels. Resistance to myocardial ischemia was determined in rabbits raised from birth to 10 days of age in a normoxic (Fi(O(2)) = 0.

Home monitoring of infants after stage one palliation for hypoplastic left heart syndrome.

Despite improved early results with the Norwood procedure (stage one palliation), patients remain with at-risk anatomy and interstage mortality continues to be a limitation of staged single ventricle palliation. Retrospective analyses have implicated residual or recurrent anatomic lesions as well as intercurrent illness as causes of interstage mortality. We hypothesized that potentially life-threatening anatomic lesions and illnesses would be manifest before serious physiologic impact by alteration in arterial saturation, failure to gain weight or in the case of dehydration, acute weight loss.

Orthotopic heart transplantation in a child with histiocytoid cardiomyopathy.

We report a case of histiocytoid cardiomyopathy in a 30-month-old child. This rare disorder has been identified in <100 patients worldwide and no previous reports of cardiac transplantation with this condition have been identified. We reviewed the clinical and pathologic findings and compared them to previous studies.

Acute cardioprotective effects of erythropoietin in infant rabbits are mediated by activation of protein kinases and potassium channels.

Erythropoietin is protective against cardiac ischemia, but the underlying mechanisms are unknown. We determined whether erythropoietin (0.5 - 10.

Early cavopulmonary anastomosis in very young infants after the Norwood procedure: impact on oxygenation, resource utilization, and mortality.

Background: The optimal timing of second-stage palliation after Norwood operations remains undefined. Advantages of early cavopulmonary anastomosis are early elimination of volume load and shortening the high-risk interstage period. Potential disadvantages include severe cyanosis, prolonged pleural drainage and hospitalization, and excess mortality.

Alteration of the critical arteriovenous oxygen saturation relationship by sustained afterload reduction after the Norwood procedure.

Objectives: Hemodynamic vulnerability after the Norwood procedure for hypoplastic left heart syndrome results from impaired myocardial function, and critical inefficiency of parallel circulation. Traditional management strategies have attempted to optimize circulatory efficiency by using arterial oxygen saturation (SaO(2)) as an index of pulmonary/systemic flow balance, attempting to maintain SaO(2) within a theoretically optimal critical range of 75% to 80%. This optimal range of SaO(2) has not been verified clinically, and strategies targeting SaO(2) may be limited by the fact that SaO(2) is a poor predictor of systemic oxygen delivery.

Changes in cerebral and somatic oxygenation during stage 1 palliation of hypoplastic left heart syndrome using continuous regional cerebral perfusion.

Objectives: Stage 1 palliation of hypoplastic left heart syndrome requires the interruption of whole-body perfusion. Delayed reflow in the cerebral circulation secondary to prolonged elevation in vascular resistance occurs in neonates after deep hypothermic circulatory arrest. We examined relative changes in cerebral and somatic oxygenation with near-infrared spectroscopy while using a modified perfusion strategy that allowed continuous cerebral perfusion.

Home surveillance program prevents interstage mortality after the Norwood procedure.

Objective: To determine whether early identification of physiologic variances associated with interstage death would reduce mortality, we developed a home surveillance program.

Methods: Patients discharged before initiation of home surveillance (group A, n = 63) were compared with patients discharged with an infant scale and pulse oximeter (group B, n = 24). Parents maintained a daily log of weight and arterial oxygen saturation according to pulse oximetry and were instructed to contact their physician in case of an arterial oxygen saturation less than 70% according to pulse oximetry, an acute weight loss of more than 30 g in 24 hours, or failure to gain at least 20 g during a 3-day period.

Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course.

Objectives: In this study, we sought to describe the mode of presentation, anatomic features, diagnostic techniques, and surgical outcome in a group of patients with anomalous origin of a coronary artery from the opposite sinus with an interarterial course between the great arteries (AOCA).

Background: Anomalous origin of a coronary artery from the opposite sinus with an interarterial course is associated with myocardial ischemia and sudden cardiac death, particularly in adolescents and young adults.

Methods: The cardiology database at Children's Hospital of Wisconsin was reviewed to identify all patients diagnosed with AOCA.

Complete staged palliation of hypoplastic left heart syndrome in a child with cystic fibrosis.

In recent years, the palliative treatment of Hypoplastic Left Heart Syndrome (HLHS) with a three-staged surgical repair has gained widespread acceptance in North America and elsewhere and has significantly improved the life-expectancy of these children. We report on a child in whom the diagnosis of cystic fibrosis (CF) was made shortly after the first-stage palliation (modified Norwood procedure), and in which surgical palliation was successfully completed with second- (bidirectional Glenn) and third-stage (fenestrated Fontan) procedures. During this period, the child suffered several CF-related complications, i.

Safety of aprotinin use and re-use in pediatric cardiothoracic surgery.

Background: Hypersensitivity reactions to aprotinin have been reported in adult cardiac surgical patients undergoing initial and re-exposure to the medication. This study describes the incidence and impact of aprotinin hypersensitivity reactions in children undergoing cardiothoracic surgery.

Methods And Results: In this retrospective review of our entire experience with aprotinin (n=865), 681 first exposures, 150 second exposures, and 34 third or higher exposures were examined.

Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients.

Background: Outcome of stage 1 palliation (S1P) for hypoplastic left heart syndrome (HLHS) has improved coincident with application of treatment strategies including continuous superior vena cava oximetry (SvO2), phenoxybenzamine (POB), strategies to minimize the duration of deep hypothermic circulatory arrest (DHCA) and efforts to ameliorate the inflammatory response to cardiopulmonary bypass (CPB) using aprotinin and modified ultrafiltration.

Methods And Results: Analysis of a consecutive series of 115 patients undergoing S1P was done to identify the risk factors for mortality and the impact of new treatment strategies. For the current era, July 1996 to October 2001, hospital survival was 93% (75/81) compared with 53% (18/34) for the time period, January 1992 to June 1996, P<0.