David P. Bichell

Improved results with selective management in pulmonary atresia with intact ventricular septum

OBJECTIVE Late outcome of neonatal pulmonary atresia with intact ventricular septum remains poor in most reported series. We have followed a selective approach toward either single ventricle repair versus complete or partial biventricular repair based on the presence of right ventricle-dependent coronary circulation and growth of the right ventricle. METHODS A retrospective chart review was conducted of 47 patients who underwent surgery between January 1991 and September 1998. RESULTS Sixteen (34%) patients had a right ventricle-dependent coronary circulation, with a tricuspid valve Z-score of -3.0 +/- 0.66 versus -2.0 +/- 0.95 (P =.002) for those without a right ventricle-dependent coronary circulation. A systemic-pulmonary artery shunt only was performed in all patients with a right ventricle-dependent coronary circulation, with 1 death. Fourteen of 16 patients with a right ventricle-dependent coronary circulation underwent a bidirectional Glenn shunt at a median of 9 months after their first operation, 9 of whom have had a Fontan procedure (no deaths). In the 31 (66%) patients without a right ventricle-dependent coronary circulation, 6 patients underwent only a systemic-pulmonary artery shunt, 23 had a shunt and right ventricular decompression, and 2 had only a transannular patch. In this group, 10 patients received a 2-ventricle repair, 6 a 1. 5-ventricle repair, and 8 patients had a Fontan procedure. There was 1 early death and the overall survival was 98% at 1 year, 5 years, and 7 years. CONCLUSIONS If patients are stratified well, excellent survival can be achieved in the treatment of pulmonary atresia with intact ventricular septum. This result may be at the price of achieving a 1-ventricle as opposed to a 2-ventricle repair.

Standardized postoperative handover process improves outcomes in the intensive care unit: a model for operational sustainability and improved team performance*.

Objective:To determine whether structured handover tool from operating room to pediatric cardiac intensive care unit following cardiac surgery is associated with a reduction in the loss of information transfer and an improvement in the quality of communication exchange. In addition, whether this tool is associated with a decrease in postoperative complications and an improvement in patient outcomes in the first 24 hrs of pediatric cardiac intensive care unit stay. Design:Prospective observational clinical study. Setting:Pediatric cardiac intensive care unit of an academic medical center. Patients:Pediatric cardiac surgery patients over a 3-yr period. Evaluation of communication and patients studied for two time periods: verbal handover (July 2007–June 2009) and structured handover (July 2009–June 2010). Interventions:None. Measurements and Main Results:Two anonymous surveys administered to the entire clinical team of the pediatric cardiac intensive care unit evaluated loss of information transfer for each of the two handover processes. Quality of structured handover tool was evaluated by Likert scale (1–5) responses in the second survey. Patient complications including cardiopulmonary resuscitation, mediastinal reexploration, placement on extracorporeal membrane oxygenation, development of severe metabolic acidosis, and number of early extubations in the first 24-hr pediatric cardiac intensive care unit stay were compared for the two time periods. Survey results showed the general opinion that the structured handover tool was of excellent quality to enhance communication (Likert scale: 4.4 ± 0.7). In addition, the tool was associated with a significant reduction (p < .001) in loss of information for every category of patient clinical care including patient, preoperative, anesthesia, operative, and postoperative details and laboratory values. Patient data revealed significant decrease (p < .05) for three of the four major complications studied and a significant increase (p < .04) in the number of early extubations following introduction of our standardized handover tool. Conclusions:In this setting, a standardized handover tool is associated with a decrease in the loss of patient information, an improvement in the quality of communication during postoperative transfer, a decrease in postoperative complications, and an improvement in 24-hr patient outcomes.

Minimal sternotomy approach for congenital heart operations

BACKGROUND In recent years, minimal access cardiac operations have increased in application in both the adult and pediatric population. As our experience has grown with these approaches to atrial septal defect closure, we have expanded the same approach to the repair of more complex congenital heart disease. METHODS At the Childrens Hospital in Boston, from August 1996 to November 1999, a minimal sternotomy approach was used to surgically correct 104 children with congenital heart defects other than atrial septal defect. The approach, in most patients, consisted of a skin incision based over the xiphisternum, 3.5 to 5 cm in length, with division of the xiphoid only and elevation of the sternum by fixed retractor. All patients underwent cannulation for cardiopulmonary bypass through the great vessels in the chest using this same incision. The lesions corrected included ventricular septal defect in 41 patients, tetralogy of Fallot in 27, common atrioventricular canal in 15, mitral valve operation in 3.5, and other defects in 18 patients. There were 53 male and 51 female patients. Mean age at operation was 1.4 years (range, 2 weeks to 11 years). RESULTS There were no deaths. The mean cardiopulmonary bypass time was 71 minutes (standard deviation, 19 minutes), mean cross-clamp times 40.8 minutes (standard deviation, 13 minutes), and length of stay 4.5 days (standard deviation, 1.9 days). Complications included transient atrioventricular block in 2 patients, pleural effusion requiring drainage in 4, and pericardial effusion in 3 patients. When compared to similar lesions repaired using a full sternotomy approach there was no difference in operating times and length of stay tended to be shorter in the minimal sternotomy group. CONCLUSIONS A minimal sternotomy approach can be used to repair congenital cardiac lesions other than atrial septal defects. It gives good exposure, particularly for transatrial repairs, does not prolong ischemic times, and may lead to shorter hospital stay.

Minimal access approach for the repair of atrial septal defect: the initial 135 patients

BACKGROUND From May 1996 to August 1998 a minimal access approach was used for 135 of 200 consecutive surgical atrial septal defects closures in children through young adults ranging in age from 6 months to 25 years (median 5 years). METHODS A 3.5- to 5-cm midline incision was centered over the xiphoid with division of the xiphoid alone (transxiphoid) or of the lower sternum (ministernotomy); both groups underwent bicaval venous cannulation through the incision. Cardioplegia and aortic cross-clamping were administered through the incision. Cephalad retraction of the sternum with a fixed-arm retractor aided exposure. RESULTS There have been no early or late deaths and no bleeding or wound complications. No procedure required conversion to a full sternotomy, and no cannulation attempt was abandoned for an alternate site. Cross-clamp and cardiopulmonary bypass times were equivalent to those in the full sternotomy group. The mean length of hospital stay in the ministernotomy group was 2.7 days. CONCLUSIONS The closure of atrial septal defects can be performed through a transxiphoid or ministernotomy approach, conferring a satisfactory cosmetic result without compromising the safety or accuracy of the repair.

Postoperative recovery in children after minimum versus full-length sternotomy.

BACKGROUND Minimal access incisions for pediatric cardiac surgery have been reported to hasten postoperative recovery. This prospective study compared recovery after a minimum versus full-length sternotomy for repair of atrial septal defects in children. METHODS We studied 35 children undergoing atrial septal defect repair using a full-length sternotomy (n = 18) or ministernotomy (n = 17) according to the surgeons preference. All children were managed according to an established clinical practice guideline. Intraoperative comparisons included patient demographics, bypass and cross-clamp times, and, as a measure of stress response, epinephrine, norepinephrine, and lactate levels at six time intervals throughout the surgical procedure. Postoperative comparisons included pain scores at 6, 12, and 24 hours, frequency of emesis, analgesic requirements, respiratory rate and gas exchange, and length of intensive care unit and total hospital stay. Nurse and parent assessment scores of overall recovery were constructed using visual analog and Likert scales. RESULTS No significant differences between mini- versus full-length sternotomy were detected for the measured outcome variables. No adverse outcomes were detected. CONCLUSIONS In this prospective study, a ministernotomy did not enhance postoperative recovery, and the primary advantage appears to be an improved cosmetic result.

B-type Natriuretic Peptide: Perioperative Patterns in Congenital Heart Disease

OBJECTIVE B-type natriuretic peptide (BNP) has diagnostic, prognostic, and therapeutic roles in adults with heart failure. BNP levels in children undergoing surgical repair of congenital heart disease (CHD) were characterized broadly, and distinguishable subgroup patterns delineated. DESIGN Prospective, blinded, observational case series. SETTING Academic, tertiary care, free-standing pediatric hospital. PATIENTS Children with CHD; controls without cardiopulmonary disease. Interventions. None. MEASUREMENTS Preoperative cardiac medications/doses, CHD lesion types, perioperative BNP levels, intraoperative variables (lengths of surgery, bypass, cross-clamp), postoperative outcomes (lengths of ventilation, hospitalization, open chest; averages of inotropic support, central venous pressure, perfusion, urine output; death, low cardiac output syndrome (LCOS), cardiac arrest; readmission; and discharge medications). RESULTS Median BNP levels for 102 neonatal and non-neonatal controls were 27 and 7 pg/mL, respectively. Serial BNP measures from 105 patients undergoing CHD repair demonstrated a median postoperative peak at 12 hours. The median and interquartile postoperative 24-hour average BNP levels for neonates were 1506 (782-3784) pg/mL vs. 286 (169-578) pg/mL for non-neonates (P < 0.001). Postoperative BNP correlated with inotropic requirement, durations of open chest, ventilation, intensive care unit stay, and hospitalization (r = 0.33-0.65, all P < 0.001). Compared with biventricular CHD, Fontan palliations demonstrated lower postoperative BNP (median 150 vs. 306 pg/mL, P < 0.001), a 3-fold higher incidence of LCOS (P < 0.01), and longer length of hospitalization (median 6.0 vs. 4.5 days, P= 0.01). CONCLUSIONS Perioperative BNP correlates to severity of illness and lengths of therapy in the CHD population, overall. Substantial variation in BNP across time as well as within and between CHD lesions limits its practical utility as an isolated point-of-care measure. BNP commonly peaks 6-12 hours postoperatively, but the timing and magnitude of BNP elevation demonstrates notable age-dependency, peaking earlier and rising an order of magnitude higher in neonates. In spite of higher clinical acuity, non-neonatal univentricular CHD paradoxically demonstrates lower BNP levels compared with biventricular physiologies.

Minimal-Access Surgery for Congenital Heart Defects

Improved results with lower operative mortality and morbidity for corrective surgery for many congenital cardiac defects has stimulated a renewed interest in the use of surgical approaches other than a full midline sternotomy. In an effort to decrease pain and discomfort, shorten the recovery period, and improve the cosmetic result, several alternative approaches have been proposed and implemented, with varying results. Anterior thoracotomy in the inframammary area has been the most widely used incision and is most applicable to females patients past puberty, in whom the extent of breast tissue can be assessed more accurately. Complications with this approach including phrenic nerve injury and breast and chest wall deformities have been reported, although most reports describe satisfactory cosmetic results. We have used a midline approach limiting the incision over the xyphoid process either without a sternal incision (infants) or with division of the lower segment (patients younger than 3 to 4 years) with cephalad retraction to expose the heart and great vessels. From May 1996 to June 1997, 54 children had repair of a secundum-type atrial septal defect using a transxyphoid or ministernotomy approach. In 29, arterial cannulation was performed through the ascending aorta, and in 25 via the femoral artery. There were no instances in which conversion to full sternotomy was required, and complete repair with comparable ischemic and bypass time to full sternotomy was achieved in all patients. We have also used the same technique for repair of other congenital cardiac lesions, including ventricular septal defect and partial or complete atrioventricular canal defects, and in selected infants with tetralogy of Fallot. With this approach, cardioplegia for myocardial protection and left ventricular venting to prevent distention and to remove air from the heart can be used routinely. The adaptability of this technique to various cardiac defects and the ability to extend the incision if necessary make it an attractive alternative to other approaches for minimal-access cardiac surgery for congenital defects. Copyright 1998 by W.B. Saunders Company

New approaches to neuroprotection in infant heart surgery

Advances in surgical techniques and perioperative management have led to dramatic improvements in outcomes for children with complex congenital heart disease (CHD). As the number of survivors continues to grow, clinicians are becoming increasingly aware that adverse neurodevelopmental outcomes after surgical repair of CHD represent a significant cause of morbidity, with widespread neuropsychologic deficits in as many as 50% of these children by the time they reach school age. Modifications of intraoperative management have yet to measurably impact long-term neurologic outcomes. However, exciting advances in our understanding of the underlying mechanisms of cellular injury and of the events that mediate endogenous cellular protection have provided a variety of new potential targets for the assessment, prevention, and treatment of neurologic injury in patients with CHD. In this review, we will discuss the unique challenges to developing neuroprotective strategies in children with CHD and consider how multisystem approaches to neuroprotection, such as ischemic preconditioning, will be the focus of ongoing efforts to develop new diagnostic tools and therapies. Although significant challenges remain, tremendous opportunity exists for the development of diagnostic and therapeutic interventions that can serve to limit neurologic injury and ultimately improve outcomes for infants and children with CHD.

Acetaminophen attenuates lipid peroxidation in children undergoing cardiopulmonary bypass.

Objective: Hemolysis, occurring during cardiopulmonary bypass, is associated with lipid peroxidation and postoperative acute kidney injury. Acetaminophen inhibits lipid peroxidation catalyzed by hemeproteins and in an animal model attenuated rhabdomyolysis-induced acute kidney injury. This pilot study tests the hypothesis that acetaminophen attenuates lipid peroxidation in children undergoing cardiopulmonary bypass. Design: Single-center prospective randomized double-blinded study. Setting: University-affiliated pediatric hospital. Patients: Thirty children undergoing elective surgical correction of a congenital heart defect. Interventions: Patients were randomized to acetaminophen (OFIRMEV [acetaminophen] injection; Cadence Pharmaceuticals, San Diego, CA) or placebo every 6 hours for four doses starting before the onset of cardiopulmonary bypass. Measurement and Main Results: Markers of hemolysis, lipid peroxidation (isofurans and F2-isoprostanes), and acute kidney injury were measured throughout the perioperative period. Cardiopulmonary bypass was associated with a significant increase in free hemoglobin (from a prebypass level of 9.8 ± 6.2 mg/dL to a peak of 201.5 ± 42.6 mg/dL postbypass). Plasma and urine isofuran and F2-isoprostane concentrations increased significantly during surgery. The magnitude of increase in plasma isofurans was greater than the magnitude in increase in plasma F2-isoprostanes. Acetaminophen attenuated the increase in plasma isofurans compared with placebo (p = 0.02 for effect of study drug). There was no significant effect of acetaminophen on plasma F2-isoprostanes or urinary makers of lipid peroxidation. Acetaminophen did not affect postoperative creatinine, urinary neutrophil gelatinase-associated lipocalin, or prevalence of acute kidney injury. Conclusion: Cardiopulmonary bypass in children is associated with hemolysis and lipid peroxidation. Acetaminophen attenuated the increase in plasma isofuran concentrations. Future studies are needed to establish whether other therapies that attenuate or prevent the effects of free hemoglobin result in more effective inhibition of lipid peroxidation in patients undergoing cardiopulmonary bypass.

Angiotensin-converting enzyme inhibition alters the inflammatory and fibrinolytic response to cardiopulmonary bypass in children.

Objective: Many children with a congenital heart defect undergo surgical correction requiring cardiopulmonary bypass. One-sixth of these patients take an angiotensin-converting enzyme inhibitor for heart failure treatment. The effect of angiotensin-converting enzyme inhibition on the fibrinolytic and inflammatory response in children undergoing cardiopulmonary bypass is unknown. In adults, angiotensin-converting enzyme inhibition attenuates the increase in plasminogen activator inhibitor-1 after cardiopulmonary bypass, whereas the effect on the interleukin-6 response is uncertain. This study tests the hypothesis that preoperative angiotensin-converting enzyme inhibition attenuates postoperative plasminogen activator inhibitor-1 and interleukin-6 expression after cardiopulmonary bypass in children. Design: Single-center prospective, randomized, nonblinded study. Setting: University-affiliated pediatric hospital. Patients: Children undergoing elective surgical correction of a congenital heart defect requiring cardiopulmonary bypass and taking an angiotensin-converting enzyme inhibitor. Interventions: Children were randomized to continue angiotensin-converting enzyme inhibitor until the morning of surgery (angiotensin-converting enzyme inhibitor group, n = 11) or to discontinue therapy 72 hrs before surgery (no angiotensin-converting enzyme inhibitor group, n = 9). Measurement and Main Results: Blood samples were collected at baseline before cardiopulmonary bypass, at 30 mins of cardiopulmonary bypass, on arrival to the intensive care unit, and on postoperative day 1. Baseline bradykinin concentrations were significantly higher and angiotensin-converting enzyme activity significantly lower in the angiotensin-converting enzyme inhibitor group compared with the no angiotensin-converting enzyme inhibitor group (p = .04 and .001, respectively). Plasminogen activator inhibitor-1 antigen increased 15-fold after cardiopulmonary bypass and peaked on postoperative day 1 (from 4.6 ± 1.2 to 67.7 ± 9.5 ng/mL; p < .001). Postoperative day 1 plasminogen activator inhibitor-1 antigen correlated significantly with cardiopulmonary bypass time (r2 = 0.40, p = .03) and was significantly lower in the angiotensin-converting enzyme inhibitor group compared with the no angiotensin-converting enzyme inhibitor group (p = .03). The proinflammatory markers interleukin-6 and interleukin-8 as well as the anti-inflammatory marker interleukin-10 increased significantly after cardiopulmonary bypass (all p < .001). Interleukin-6 concentrations were significantly higher in the angiotensin-converting enzyme inhibitor group after cardiopulmonary bypass (p = .02) even after controlling for potential confounding factors such as age, cardiopulmonary bypass time, and transfusion volume. Conclusion: Angiotensin-converting enzyme inhibition attenuates the increase in postoperative plasminogen activator inhibitor-1 but enhances the interleukin-6 response in children undergoing cardiopulmonary bypass.