Repair Of Congenital Heart Lesions Research Articles

Elevated preoperative von Willebrand factor is associated with perioperative thrombosis in infants and neonates with congenital heart disease

Essentials Perioperative thrombosis is a major cause of morbidity and mortality in congenital heart disease. Neonates and infants undergoing repair of congenital heart lesions were prospectively followed. Elevated von Willebrand factor (VWF) to ADAMTS-13 activity ratios typified the postoperative period. Thrombosis was associated with preoperative VWF activity and cryoprecipitate transfusion SUMMARY: Background The surgical repair of congenital heart malformations is frequently complicated by perioperative thrombosis of unclear etiology. An imbalance between von Willebrand factor (VWF) and ADAMTS-13 is an emerging variable in thrombosis. Objectives To describe perioperative changes to VWF, ADAMTS-13 and NETosis, and evaluate clinical and biochemical associations with postoperative thrombosis. Methods Neonates and infants undergoing palliation or definitive surgical repair of congenital heart malformations were recruited (n=133). Preoperative and postoperative plasma levels of VWF, ADAMTS-13 and markers of NETosis were determined. Patients were followed for up to 30days for the occurrence of thrombosis. Univariate and multivariate logistic regression analyses were conducted to identify variables associated with thrombosis. Results We identified significant postoperative increases in VWF activity, VWF level, DNA-histone complexes and cell-free DNA with an overall decrease in ADAMTS-13 activity. Patients experiencing postoperative thrombotic events (9%) were characterized by surgery performed at a lower intraoperative temperature, higher preoperative lactic acid levels, and higher preoperative VWF activity and level. A multivariate logistic regression model identified preoperative VWF activity (odds ratio (OR)8.39 per IUmL-1 , 95% confidence interval [CI]1.73-40.55) and transfusion of cryoprecipitate (OR1.10 per mLkg-1 , 95%CI1.03-1.17) as being associated with thrombosis. Conclusions Pediatric patients undergoing surgical repair of congenital heart malformations are exposed to high levels of VWF with diminished or minimal change to ADAMTS-13 in the immediate postoperative period. Elevated preoperative VWF activity is associated with postoperative thrombosis in pediatric congenital heart disease.

Debate on congenital heart disease. Con: early total repair is not always preferable to palliative surgery in congenital heart disease.

While early one-stage repair of congenital heart lesions can now be done with low operative mortality, there are reasons to believe that sometimes a palliative procedure, followed later by complete repair, has some advantages. Some of the drawbacks to early repair such as presumably be abolished as better valves are produced, and better understanding of cardiopulmonary bypass may reduce operative myocardial damage. However, problems related to the very small size of some outflow tracts and pulmonary arteries may not be so easily overcome.

Surgery for congenital diseases of the aorta

Congenital diseases of the aorta tend to be obstructive when they present early in life, and aneurysmal when they present later in life. The latter group also tends to be associated with connective tissue disorders and with repaired conotruncal lesions. The indications for intervention in the aneurysm group are still in evolution but are clearly age- and lesion-dependant. Disorders such as Loeys-Dietz syndrome and Turner syndrome may deserve aggressive prophylactic surgery, as well as Marfan syndrome to a lesser extent. The natural history of the dilated aorta after repair of congenital heart lesions is probably more benign than de novo aneurysms and therefore should be treated conservatively.

Functional Assessment for Congenital Heart Disease

Significant improvement in survival of children with congenital cardiac malformations has resulted in an increasing population of adolescent and adult patients with congenital heart disease. Of the long-term cardiac problems, ventricular dysfunction remains an important issue of concern. Despite corrective or palliative repair of congenital heart lesions, the right ventricle, which may be the subpulmonary or systemic ventricular chamber, and the functional single ventricle are particularly vulnerable to functional impairment. Regular assessment of cardiac function constitutes an important aspect in the long-term follow up of patients with congenital heart disease. Echocardiography remains the most useful imaging modality for longitudinal monitoring of cardiac function. Conventional echocardiographic assessment has focused primarily on quantification of changes in ventricular size and blood flow velocities during the cardiac cycles. Advances in echocardiographic technologies including tissue Doppler imaging and speckle tracking echocardiography have enabled direct interrogation of myocardial deformation. In this review, the issues of ventricular dysfunction in congenital heart disease, conventional echocardiographic and novel myocardial deformation imaging techniques, and clinical applications of these techniques in the functional assessment of congenital heart disease are discussed.

Cerebral oximetry for pediatric anesthesia: why do intelligent clinicians disagree?

Reflectance near-infrared spectroscopy has been used to measure cortical tissue oximetry for more than 30 years. In that time, many centers have adopted the routine use of the cerebral oximeter for children having repair of congenital heart lesions, while some prominent academic centers have resisted routine use of these monitors citing lack of definitive evidence for outcome benefit. In this review, we provide an overview of the method used to measure cerebral oximetry, as well as validation and clinical outcome data that have accrued from the use of cerebral oximeters. We discuss the peculiarities of evidentiary review for monitoring devices, and the confounding errors that occur when a monitor is evaluated as a therapeutic intervention. We outline the physiologic basis of cerebral desaturation and the shifts in practice that have occurred with implementation of NIRS monitoring.

Macrophage Migration Inhibitory Factor in Pediatric Patients Undergoing Surgery for Congenital Heart Repair

Macrophage migration inhibitory factor (MIF), a proinflammatory mediator, has been shown to be elevated following heart surgery in adults and may be associated with several postoperative complications, including cardiac and pulmonary dysfunction. In this study, we aimed to measure perioperative plasma MIF, interleukin (IL)-8, and free T4 in 20 children age <4 years undergoing surgical repair of congenital heart lesions with left ventricular volume overload, and to determine whether the response of these mediators determined postoperative outcomes. Plasma samples were obtained preoperatively, immediately on arrival in the pediatric intensive care unit (PICU), and at 12, 24, and 48 h. Patients were continuously monitored in the PICU, and data were recorded daily for therapeutic and monitoring procedures that reflected the invasiveness, intensity, and complexity of care rendered (therapeutic interventional scoring system, TISS). Preoperative plasma MIF, IL-8, and free T4 were not different from age-matched healthy children. However, plasma MIF and IL-8 increased significantly 2 h after completion of cardiopulmonary bypass, and then normalized within 24 h. Peak postoperative levels of MIF (48 +/- 24 ng/mL) and IL-8 (79 +/- 57 pg/mL) correlated significantly with duration of cardiopulmonary bypass. The magnitude of the postoperative increase in plasma MIF was associated with increased number of days required for mechanical ventilation (r = 0.553; P = 0.012), and peak plasma IL-8 correlated significantly with the fraction of inhaled oxygen (FiO(2)) required immediately after surgery (r = 0.510; P = 0.02). Higher circulating MIF levels correlated significantly with increased inotropic support requirements on the second postoperative day, whereas higher postoperative IL-8 levels were associated with higher TISS scores, suggesting increased need for postoperative medical care. These data suggest a potential negative effect of high circulating levels of MIF in the immediate postoperative period on respiratory and cardiovascular functions, and support the development of therapeutic strategies targeting MIF function in this clinical setting.

N-terminal B-type natriuretic peptide levels in pediatric patients with congestive heart failure undergoing cardiac surgery

The objectives of this study were to measure circulating N-terminal B-type natriuretic peptide levels in pediatric patients undergoing surgical repair of congenital heart lesions with left ventricular volume overload and to determine whether presurgical and immediate postoperative N-terminal B-type natriuretic peptide levels could predict patient outcomes after surgical intervention. Thirty-eight children aged 1 to 36 months undergoing surgical repair of cardiac lesions with left ventricular volume overload were studied. Plasma N-terminal B-type natriuretic peptide levels were measured preoperatively and at 2, 12, 24, 48, and 72 hours after surgical intervention and were assessed for their predictive value of postoperative outcomes. Plasma N-terminal B-type natriuretic peptide levels were also measured in 34 similarly aged healthy children. Patient preoperative N-terminal B-type natriuretic peptide levels were significantly higher than those of healthy control subjects (3085 +/- 4046 vs 105 +/- 78 pg/mL). Preoperative N-terminal B-type natriuretic peptide levels correlated with the complexity of surgical repair, as measured by cardiopulmonary bypass time (r = 0.529, P < .001), and with postoperative measures, including fractional inhaled oxygen requirements registered at 12 hours (r = 0.443, P = .005) and duration of mechanical ventilation (r = 0.445, P = .005). Plasma N-terminal B-type natriuretic peptide levels increased 5-fold within 12 hours after cardiopulmonary bypass (14,685 +/- 14,317 pg/mL). Multivariable regression analysis showed that the preoperative N-terminal B-type natriuretic peptide level was a significant predictor of duration of intensive care unit stay (P = .02) and that the peak postoperative N-terminal B-type natriuretic peptide level was a significant predictor of the intensity of overall medical management, as assessed by using the therapeutic intervention scoring system (P = .01). Plasma N-terminal B-type natriuretic peptide levels measured preoperatively and postoperatively can be a prognostic indicator in the management of the pediatric patient after surgical intervention for congenital heart repair.

Aristotle Score Predicts Outcome in Patients Requiring Extracorporeal Circulatory Support Following Repair of Congenital Heart Disease

Extracorporeal membrane oxygenation (ECMO) has become the standard technique of mechanical support for the failing circulation following repair of congenital heart lesions. The objective of this study was to identify predictors of survival in patients requiring postcardiotomy ECMO. The Aristotle score, a method developed to evaluate quality of care based on complexity, was investigated as a potential predictor of outcome. Between 2003 and 2005, 37 patients required ECMO following corrective surgery for congenital heart disease. Records were reviewed retrospectively with emphasis on factors affecting survival to discharge. The comprehensive Aristotle complexity score was calculated for each patient. Overall, 28 patients (76%) survived to decannulation and 17 patients (46%) survived to discharge. There were 24 (65%) neonates and 10 patients (27%) with single ventricle physiology, with a hospital survival of 42% (10 of 24) and 50% (5 of 10), respectively. Univariate factors associated with survival included Aristotle score, duration of support, reexploration, multiple organ failure, and number of complications. Age, weight, and single-ventricle physiology were not significant. In a logistic regression model, an Aristotle score < 14 was identified as a predictor of survival (OR 0.12, CI 0.02-0.87). The Aristotle score is predictive of outcome in patients requiring postcardiotomy ECMO and may serve as a uniform criterion when comparing and evaluating quality of care and performance in this complex patient population.

Repair of Congenital Heart Lesions Combined With Lung Transplantation for the Treatment of Severe Pulmonary Hypertension: A 13-Year Experience

Repair of Congenital Heart Lesions Combined With Lung Transplantation for the Treatment of Severe Pulmonary Hypertension: A 13-Year Experience

The Postoperative Cardiac Patient

The number of children surviving medical management and surgical repair of congenital heart lesions has steadily increased. Children with complex heart defects are therefore seen with increasing frequency in emergency departments. This article reviews the epidemiology of congenital heart disease, some common types of surgical repair, and a few postoperative complications and their management.

Clinical assessment of cardiac performance in infants and children following cardiac surgery

To compare clinical assessment of cardiac performance with an invasive method of haemodynamic monitoring. Prospective observational study in a 16-bed tertiary paediatric intensive care unit. Infants and children undergoing cardiopulmonary bypass and surgical repair of congenital heart lesions. Based on physical examination and routinely available haemodynamic monitoring in the paediatric intensive care unit, medical and nursing staff assessed cardiac index, systemic vascular resistance index and volume status. Clinical assessment was compared with cardiac index, systemic vascular resistance index and global end diastolic volume index, obtained by femoral artery thermodilution. A total of 76 clinical estimations of the three parameters were made in 16 infants and children undergoing biventricular repair of congenital heart lesions. Agreement was poor between clinical and invasive methods of determining all three studied parameters of cardiac performance. Cardiac index was significantly underestimated clinically; mean difference was 0.71 l min(-1) m(-2) (95% range of agreement +/-2.7). Clinical estimates of systemic vascular resistance (weighted kappa=0.15) and volume status (weighted kappa=0.04) showed poor levels of agreement with measured values and were overestimated clinically. There was one complication related to a femoral arterial catheter and one device failure. Routine clinical assessment of parameters of cardiac performance agreed poorly with invasive determinations of these indices. Management decisions based on inaccurate clinical assessments may be detrimental to patients. Invasive haemodynamic monitoring using femoral artery thermodilution warrants cautious further evaluation as there is little agreement with clinical assessment which is presently standard accepted care in this patient population.

Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: A 13-year experience

In patients with severe pulmonary hypertension associated with congenital heart disease, we prefer to perform repair of the congenital heart disease and lung transplantation whenever feasible so as to augment the donor pool and avoid the cardiac complications associated with heart transplantation. We report our experience with repair of congenital heart disease and lung transplantation and compare the results with those of patients who underwent heart-lung transplantation during the same period. The records of patients who had repair of congenital heart disease and lung transplantation (n = 35) and heart-lung transplantation (n = 16) between 1990 and 2003 were reviewed. The underlying congenital heart disease in the repair of congenital heart disease and lung transplantation group included transposition of great vessels (n = 2), atrioventricular canal defect (n = 2), ventricular septal defect (n = 9), pulmonary venous obstruction (n = 7), scimitar syndrome (n = 2), pulmonary arterial atresia or stenosis (n = 5), and others (n = 8). Thirteen of the patients undergoing repair of congenital heart disease and lung transplantation (37.1%) had the congenital heart disease repaired before lung transplantation; the remaining congenital heart disease repairs were performed concurrently with transplantation. Sixteen patients underwent heart-lung transplantation because of poor left ventricular function or single-ventricle anatomy. Freedoms from bronchiolitis obliterans at 1, 3, and 5 years were 72.9%, 54.7%, and 54.7% for the repair of congenital heart disease and lung transplantation group and 77.8%, 51.9%, and 38.9% for the heart-lung transplantation group, respectively. Survivals at 1, 3, and 5 years were 62.9%, 51.4%, and 51.4% for the repair of congenital heart disease and lung transplantation group and 66.5%, 66.5%, and 60% for the heart-lung transplantation group, respectively. Repair of congenital heart disease and lung transplantation is a feasible treatment option. Long-term outcome is determined by associated complications related to lung transplantation. Despite the complexity of combined congenital heart disease repair with lung transplantation and the resulting perioperative morbidity, the patients had similar outcomes to those of patients who underwent heart-lung transplantation.

Effectiveness of cardiac surgery in trisomies 13 and 18 (from the Pediatric Cardiac Care Consortium)

Because of severely reduced lifespan in children with trisomies 13 and 18, surgical repair of congenital heart lesions has rarely been offered. With data from a multicenter registry, we report 35 cases of cardiac surgery in infants and children with trisomy 13 or 18 with a 91% hospital survival rate. Those patients without an extended preoperative ventilatory requirement did not require prolonged mechanical ventilation after surgery.

Assisted venous drainage cardiopulmonary bypass in congenital heart surgery

Background. A novel active venous drainage perfusion circuit was designed to achieve effective venous return through small venous cannulas. The efficacy and safety of this new system was investigated and compared with a conventional gravity drainage system. Methods. Four hundred consecutive patients undergoing open heart repair of congenital heart lesions by one surgeon were studied. The first 200 patients were supported by gravity drainage and the next 200 patients were supported by assisted venous drainage. No patient in the time period was excluded from the study. Results. The two groups did not differ significantly in weight, bypass time, or cross-clamp time. Priming volumes were less in the assisted group than in the gravity group (576 ± 232 mL versus 693 ± 221 mL, p < 0.001). Venous cannula size was smaller in the assisted group when compared with the gravity group (33.2F ± 7.4F versus 38.5F ± 7.1F, p < 0.001). There was a trend to lower operative mortality in the assisted drainage group (5 of 200, 2.5% versus 11 of 200, 5.5%; p = 0.10). Hospital stay and pulmonary, infectious, and neurologic complications were comparable in both groups. Cardiac complications were less common in the assisted group than in gravity group (22 of 200, 11% versus 38 of 200, 19%; p = 0.017). Hematologic complications were less common in the assisted group than the gravity group (6 of 200, 3% versus 19 of 200, 9.5%; p < 0.01). Conclusions. These findings suggest that assisted venous drainage is safe in congenital heart operations and facilitates the use of smaller venous cannulas.

Role of functional block extension in lesion-related atrial flutter.

A line of block in the right atrium (RA) between the venae cavae is necessary to obtain classic atrial flutter (AFL). We tested the hypothesis that the location of that line of block would determine whether the AFL reentrant circuit would be due to single-loop reentry or figure-of-8 reentry. Simultaneous mapping from 392 sites (both atria and the atrial septum) was performed in 13 normal dogs before and after creating a linear lesion on the RA free wall. The lesion was 1 to 1.5 cm anterior and parallel to the crista terminalis (7 dogs) or posterior and close to the crista terminalis region (6 dogs). Sustained AFL (>2 minutes) was then induced. In 4 dogs with an anterior lesion, the AFL reentrant circuit traveled around the lesion (lesion reentry). In 9 dogs (3 with anterior lesions and 6 with posterior lesions), the AFL reentrant circuit included the anterior RA free wall, the atrial septum, and Bachmann's bundle (single-loop reentry). In these 9 dogs, the fixed line of block was extended to the superior and/or inferior vena cava by a functional line of block, thereby preventing lesion reentry. No figure-of-8 reentry was induced. In this model, the location of a fixed line of block and its functional extension determine the type of AFL reentry. These data provide an explanation for the chronic AFL that occurs in some patients after surgical repair of congenital heart lesions.

Perioperative determinants and outcome of cardiopulmonary arrest in children after heart surgery.

To identify perioperative factors associated with postoperative cardiopulmonary arrest (CA) in the pediatric intensive care unit (PICU) in children undergoing cardiovascular surgery, and to report the outcome of cardiopulmonary resuscitation (CPR) in these patients. We reviewed the medical records of all patients under 16 years of age who had undergone cardiovascular surgery and sustained CA in PICU in an urban, tertiary care children's hospital over a 5-year period. We used two control groups of patients who recovered without CA. (1) Sixty-five patients, who were operated under deep hypothermic circulatory arrest (DHCA) during the study period. (2) All patients who underwent repair of congenital heart lesions without DHCA in 1994 (n=278). Eighty-two children experienced CA during postoperative care in PICU, mainly from cardiovascular causes. Thirty-four (41%) were declared dead without attempted resuscitation, CPR was initiated in 48 (59%). The primary survival rate was 56% and 1 year survival rate was 19%. The incidence of CA was 3.6% for closed heart operations, 4.9% for intra-cardiac surgery without DHCA, and 27% for operations involving DHCA. Thirty-three per cent of patients with CA arrested during the first 24 postoperative h. Preoperative mechanical ventilation (P=0.03), prostaglandin E1 (P=0.001) and inotropic support (P=0.04) were given significantly more frequently to patients who postoperatively required CPR, compared to control groups. Patients in whom CPR was attempted were younger than the 1994 controls (0.4 vs. 1.2 years; P<0.04), had longer mean aortic-cross-clamp times (76 vs. 51 min; P<0.0001) and cardiopulmonary bypass times (124 vs. 85 min; P<0.0002), and required more inotropic support upon leaving the operating room (P<0.0001). Patients who received CPR had significantly longer DHCA times (53 vs. 32 min; P<0.0002) and required more inotropic support than patients in the DHCA control group (P<0.002). CA after pediatric cardiac surgery is associated with repair of complex congenital heart anomalies in patients who require preoperative mechanical ventilation and vasoactive agents, prolonged aortic cross-clamp, circulatory arrest; and heavy postoperative inotropic support.

Delay of extubation in neonates and children after cardiac surgery: impact of ventilator-associated pneumonia.

This study was undertaken to determine the delay of extubation attributable to ventilator-associated pneumonia (VAP) in comparison to other complications and complexity of surgery after repair of congenital heart lesions in neonates and children. Cohort study in a pediatric intensive care unit of a tertiary referral center. All patients who had cardiac operations during a 22-month period and who survived surgery were eligible (n = 272, median age 1.3 years). Primary outcome was time to successful extubation. Primary variable of interest was VAP Surgical procedures were classified according to complexity. Cox proportional hazards models were calculated to adjust for confounding. Potential confounders comprised other known risk factors for delayed extubation. Median time to extubation was 3 days. VAP occurred in 26 patients (9.6%). The rate of VAP was not associated with complexity of surgery (P = 0.22), or cardiopulmonary bypass (P = 0.23). The adjusted analysis revealed as further factors associated with delayed extubation: other respiratory complications (n = 28, chylothorax, airway stenosis, diaphragm paresis), prolonged inotropic support (n = 48, 17.6%), and the need for secondary surgery (n = 51, 18.8%; e.g., re-operation, secondary closure of thorax). Older age promoted early extubation. The median delay of extubation attributable to VAP was 3.7 days (hazards ratio HR = 0.29, 95% CI 0.18-0.49), exceeding the effect size of secondary surgery (HR = 0.48) and other respiratory complications (HR = 0.50). VAP accounts for a major delay of extubation in pediatric cardiac surgery.

Sternal wound and mediastinal infections in infants with congenital heart disease.

The objective was to describe the epidemiologic, clinical, bacteriologic and therapeutic features of seven infants who developed sternal wound and mediastinal infections following palliation and/or repair procedures for congenital heart disease. A retrospective chart review was used. All infants with sternal wound and mediastinal infections were < 30 days of age at the initial operative procedure. Six of the infants had hypoplastic left heart syndrome, and one had complete transposition. Two infants required delayed closure of their chest wound. Three infants had superficial sternal infections and presented at a mean of 12 days postoperatively. Four infants had infection of the deep mediastinal structures: they were all asymptomatic and had purulent collections in their mediastinum at their second palliative operation, which was performed at a mean of 120 days after the initial surgery. Staphylococcus aureus, or coagulase-negative Staphylococcus, was isolated from the wound and/or blood of six infants. All infants with mediastinal infections were managed with operative debridement. Infants with superficial infections underwent local debridement. All infants received long-term intravenous antibiotics. Mediastinal infections in infants undergoing palliative staged procedures for congenital heart lesions may be chronic and indolent, resulting in delayed repair of congenital heart lesions.

Intraoperative transesophageal echocardiography for pediatric patients with congenital heart disease.

Intraoperative transesophageal echocardiography for pediatric patients with congenital heart disease.

Sinus Venosus Atrial Septal Defect?

Sinus Venosus Atrial Septal Defect?