Repair Of Congenital Cardiac Defects Research Articles

Lower Partial Mini-Sternotomy for Congenital Cardiac Surgery

With the continuous decrease in morbidity and mortality associated with cardiac surgery, there has been a trend toward the development of operative techniques that are aimed toward improving other measures such as cosmetic outcomes. We have previously reported on such efforts, where a lower partial mini sternotomy is utilized to limit the skin incision and extent of median sternotomy. In this article we detail the operative technique for lower partial mini-sternotomy for the repair of congenital cardiac defects. With the continuous decrease in morbidity and mortality associated with cardiac surgery, there has been a trend toward the development of operative techniques that are aimed toward improving other measures such as cosmetic outcomes. We have previously reported on such efforts, where a lower partial mini sternotomy is utilized to limit the skin incision and extent of median sternotomy. In this article we detail the operative technique for lower partial mini-sternotomy for the repair of congenital cardiac defects.

Equine pericardium: a versatile alternative reconstructive material in congenital cardiac surgery

BackgroundPericardial patches are often used for repair of congenital cardiac defects. The aim of this study was to describe our initial experience with the use of equine pericardium and its safety and advantages and disadvantages compared to bovine pericardium.MethodsWe designed a retrospective cohort study of 111 patients who were surgically treated for congenital heart disease between 2017 and 2020. Equine pericardium was used in 58 patients and bovine pericardium was used in 53 patients. Recorded variables included demographic data, preoperative cardiac pathology, site of patch insertion, morbidity and mortality.ResultsThe overall survival rate was 94.5% and no deaths were related to patch insertion. None of our patients were reoperated on for patch related complications. Postoperative transcatheter intervention was needed in 2 patients (1.8%): one for dilatation of aortic arch stenosis after repair of hypoplastic left heart syndrome with equine pericardium and one for dilatation of pulmonary artery branches after repair of tetralogy of Fallot using bovine pericardium.ConclusionsEquine pericardium is a safe patch material for reconstruction in congenital heart surgery. It may be preferable to bovine pericardium in cases requiring a complex shape or a pliable patch as in in arch reconstruction or for valve reconstruction.

Rise in Angiopoietin-2 Following Neonatal Cardiac Surgery Is Associated With Adverse Clinical Outcomes.

Neonatal cardiac surgery for congenital cardiac defects is associated with significant morbidity and mortality, and there is a need for early identification of patients at highest risk of adverse outcomes. Because vascular endothelial injury mediates damage across organ systems, we measured serum biomarkers of endothelial injury in neonates following cardiopulmonary bypass and examined their associations with short-term outcomes. Prospective cohort study. Pediatric cardiac ICU. Thirty neonates less than 2 weeks old undergoing repair of congenital cardiac defects with cardiopulmonary bypass. None. Biomarkers of endothelial integrity, angiopoietin-1 and angiopoietin-2, were measured preoperatively and at 24 hours postoperatively. A composite adverse outcome was defined as any of the following: stroke, need for renal replacement therapy, extracorporeal membrane oxygenation support, cardiac arrest, or death. Associations of biomarkers with adverse outcomes were examined using Wilcoxon rank-sum test. There was an increase in angiopoietin-2 from preoperatively to 24 hours postoperatively (p < 0.0001) and a decrease in angiopoietin-1 from preoperatively to 24 hours postoperatively (p < 0.0001). Patients with greater rise in angiopoietin-2 from preoperatively to 24 hours postoperatively had greater risk of composite adverse outcome (p = 0.04). They had a trend toward higher Vasoactive-Inotropic Score (p = 0.06) and a higher prevalence of low cardiac output syndrome (p = 0.06). Twenty-four hour postoperative angiopoietin-2 level was associated with the composite adverse outcome (p = 0.03). The rise in angiopoietin-2 level from preoperatively to 24 hours postoperatively directly correlated with cardiopulmonary bypass duration (r = 0.47; p = 0.01). In neonatal cardiac surgery, longer duration of cardiopulmonary bypass is directly associated with greater endothelial injury as measured by increased serum levels of angiopoietin-2. Angiopoietin-2 levels 24 hours postoperatively were significantly associated with a composite adverse outcome. Postoperative angiopoietin-2 level may serve as an early indicator of patients in need of closer monitoring and protective intervention. Further research into endothelial protective strategies is warranted.

Performance of the ADAPT-Treated CardioCel® Scaffold in Pediatric Patients With Congenital Cardiac Anomalies: Medium to Long-Term Outcomes.

Background: A Phase II Clinical Trial reviewed the performance (morbidity and calcification) of the tissue-engineered ADAPT® bovine pericardial scaffold (CardioCel®) in pediatric patients (n = 30) with congenital cardiac defects. In that study, CardioCel® demonstrated no graft-related morbidity and mortality in 25 patients, over 12 months. Five patients died due to non-graft-related events. Echocardiography revealed hemodynamically stable repairs with no calcification of the scaffold. Magnetic resonance imaging (MRI) at 12 months in 10 patients confirmed the absence of calcification. These patients were followed up for further up to 10 years. We present the results of this retrospective review of these patients that were followed for further medium to long-term (median 7.2 years, 25%: 3.6 years 75%: 9.25 years) postoperatively in these patients.Methods: Between April 2008 and September 2009, CardioCel® was implanted in 30 patients with congenital cardiac defects. Efficacy measures included graft-related mortality, morbidity and haemodynamic abnormalities. Calcification was assessed by standard 2D-M mode echocardiography and MRI at 12 months. Medium to long-term assessment included routine clinical assessments and echocardiography.Results: Median age at surgery was 18 months (27 days−13 years). Twenty-five patients (142 patient years) were followed for up to 10 years. The 10-year survival rate is estimated as 86.9% (95% CI 71.4–100.0%) over the entire follow-up period. One patient was lost to follow-up. No graft-related mortality was encountered up to a median follow-up of 7.2 years. Two patients died (pacemaker complications >5 years and arrhythmia >7 years postoperatively). No graft failure, thromboembolic events, infections or device-related reinterventions were recorded. Non-significant residual leaks occurred in 3 patients. Echocardiography demonstrated the absence of calcification in all implants.Conclusion: The tissue-engineered ADAPT® bovine pericardial scaffold demonstrated excellent medium to long-term performance (up to 10 years) when used as a scaffold for repair of congenital cardiac defects in children. Durability, acellularity, biostability and non-calcifying potential of CardioCel® makes it a very attractive tissue for congenital cardiac repair procedures.

Transverse Split Sternotomy: A Mini-Invasive Approach for Repair of Congenital Cardiac Defects.

In this article, we aim to review our mini-invasive technique of transverse sternal split (TSS) with or without cervical cannulation for cardiopulmonary bypass (CPB), its usefulness, and efficiency for repair of congenital cardiac defects. Between January 2013 and June 2015, 34 infants and small children were operated through TSS in third or fourth intercostal space [Tetralogy of Fallot 11, perventricular ventricular septal defect (VSD) device closure 23]. Cardiopulmonary bypass was established through cervical (common carotid artery [CCA] and internal juglar vein [IJV]) and inferior vena cava cannulation. In patients operated on CPB, near-infrared spectroscopy was monitored during surgery for cerebral oxygenation and Doppler ultrasonography was performed postoperatively for patency of CCA and IJV. Patients were followed up after discharge with physical examination and transthoracic echocardiography (TTE). Surgical site exposure through TSS was adequate in all patients. There was no significant morbidity, postoperatively or during follow-up except three patients with VSD device closure who required prolonged mechanical ventilation. Cervical cannulation was sufficient for arterial inflow in all patients operated on CPB. There was one mortality and three procedure failures in VSD device closure group. There was no incidence of neurological complication. Both CCA and IJV were patent in all patients operated on CPB. No significant residual defect was found in either group on postoperative transthoracic echocardiography. Transverse sternal split with or without cervical cannulation is a feasible technique for repair of tetralogy of Fallot and perventricular device closure in selected group of infants and small children without compromising the exposure or quality of repair.

Cervical Cannulation for Surgical Repair of Congenital Cardiac Defects in Infants and Small Children.

IntroductionThe biggest challenge faced in minimally invasive pediatric cardiac surgery is cannulation for cardiopulmonary bypass. Our technique and experience of cervical cannulation in infants and small children for repair of congenital cardiac defects is reported in this study.MethodsFrom January 2013 to June 2015, 37 children (22 males) with mean age of 17.97±8.63 months and weight of 8.06±1.59 kg were operated on for congenital cardiac defects through right lateral thoracotomy. The most common diagnosis was ventricular septal defect (18 patients). In all patients, right common carotid artery, right internal jugular vein and inferior vena cava were cannulated for institution of cardiopulmonary bypass and aorta was cross clamped through right 2nd intercostal space.ResultsThere were no deaths or any major complications related to cervical cannulation. Common carotid artery cannulation provided adequate arterial inflow while internal jugular vein with inferior vena cava provided adequate venous return in all patients. No patient required conversion to sternotomy or developed vascular, neurological or wound related complications. Three patients had residual lesions (small leak across ventricular septal defect patch-2, Grade II left atrio-ventricular valve regurgitation-1) and one patient had mild left ventricular dysfunction. At discharge, both common carotid artery and internal jugular vein were patent on color Doppler ultrasonography in all patients. In a mean follow-up period of 11.4±2.85 months, all patients were doing well. No patient had any wound related, neurological or vascular complication. No patient had residual leak across ventricular septal defect patch.ConclusionCervical cannulation of common carotid artery and internal jugular vein is a safe, reliable, efficient and quick method for institution of cardiopulmonary bypass in minimally invasive pediatric cardiac surgery.

Myocardial progenitor cells as an option for cell therapy for congenital heart disease

Myocardial progenitor cells represent a perspective source for cell therapy of cardiovascular disorders and are intensively studied. However, mainly the cells from adult patients are studied whereas the cells derived from children with congenital heart diseases remained poorly investigated. The aim of the present work was to obtain progenitor cells from intraoperatively obtained cardiac tissue from infants and children undergoing repair of congenital cardiac defects. Following isolation, the cells gave rise to a clonogenic, highly proliferative spindle-shaped cell population. The cells expressed markers of cardiogenic origin and were shown to differentiate towards cardiogenic lineage. This resident myocardial progenitor cells obtained from infant myocardial tissue demonstrate similar characteristics to previously described cells derived from adult myocardial tissue. This study confirms the possibility of obtaining a pool of progenitor cells from tiny tissue fragments and opens a new perspective of using these cells in regenerative medicine and further research of congenital heart disease pathogenesis.

Expansion and characterization of neonatal cardiac pericytes provides a novel cellular option for tissue engineering in congenital heart disease.

BackgroundLiving grafts produced by combining autologous heart-resident stem/progenitor cells and tissue engineering could provide a new therapeutic option for definitive correction of congenital heart disease. The aim of the study was to investigate the antigenic profile, expansion/differentiation capacity, paracrine activity, and pro-angiogenic potential of cardiac pericytes and to assess their engrafting capacity in clinically certified prosthetic grafts.Methods and ResultsCD34pos cells, negative for the endothelial markers CD31 and CD146, were identified by immunohistochemistry in cardiac leftovers from infants and children undergoing palliative repair of congenital cardiac defects. Following isolation by immunomagnetic bead-sorting and culture on plastic in EGM-2 medium supplemented with growth factors and serum, CD34pos/CD31neg cells gave rise to a clonogenic, highly proliferative (>20 million at P5), spindle-shape cell population. The following populations were shown to expresses pericyte/mesenchymal and stemness markers. After exposure to differentiation media, the expanded cardiac pericytes acquired markers of vascular smooth muscle cells, but failed to differentiate into endothelial cells or cardiomyocytes. However, in Matrigel, cardiac pericytes form networks and enhance the network capacity of endothelial cells. Moreover, they produce collagen-1 and release chemo-attractants that stimulate the migration of c-Kitpos cardiac stem cells. Cardiac pericytes were then seeded onto clinically approved xenograft scaffolds and cultured in a bioreactor. After 3 weeks, fluorescent microscopy showed that cardiac pericytes had penetrated into and colonized the graft.ConclusionsThese findings open new avenues for cellular functionalization of prosthetic grafts to be applied in reconstructive surgery of congenital heart disease.

MULTICENTER VALIDATION OF TECHNICAL PERFORMANCE SCORE AS A QUALITY ASSESSMENT TOOL IN CONGENITAL CARDIAC SURGERY

Technical Performance Score (TPS), a novel tool for assessing adequacy of repair of congenital cardiac defects based on clinical and echocardiographic data, has been validated and correlates with outcomes in single center studies. This study aims to validate TPS in a multicenter environment.

Concomitant Slide Tracheoplasty and Cardiac Operation for Congenital Tracheal Stenosis Associated With VACTERL

The association of congenital tracheal stenosis and tracheoesophageal (TE) fistula is rare. Here, we report 2 patients with tracheobronchial stenosis (complete cartilage ring) involving the lower trachea and right bronchus. Both patients had associated VACTERL (vertebral anomalies, anal atresia, cardiovascular anomalies, TE, renal, and limb defects) congenital cardiac defects and tracheal diverticula after repair of the TE fistula in type C esophageal atresia. The stenotic segment began at the orifice of the TE fistula, which became diverticula after the TE fistula was repaired. Concomitant repair of congenital cardiac defects and a slide tracheoplasty with elimination of the diverticula were performed successfully.

Resuscitation and Extracorporeal Life Support during Cardiopulmonary Resuscitation following the Norwood (Stage 1) operation

The success of extracorporeal support in providing cardiopulmonary support for a variety of patients has led to use of Extracorporeal Life Support, also known as ECLS, as a rescue for patients failing conventional resuscitation. The use of Extracorporeal Life Support in circumstances of cardiac arrest has come to be termed "Extracorporeal Life Support during Cardiopulmonary Resuscitation" or "ECPR". Although Extracorporeal Life Support during Cardiopulmonary Resuscitation was originally described in patients following repair of congenital cardiac defects who suffered a sudden arrest, it has now been used in a variety of circumstances for patients both with and without primary cardiac disease. Multiple centres have reported successful use of Extracorporeal Life Support during Cardiopulmonary Resuscitation in adults and children. However, because of the cost, the complexity of the technique, and the resources required, Extracorporeal Life Support during Cardiopulmonary Resuscitation is not offered in all centres for paediatric patients with refractory cardiac arrest. The increasing success and availability of Extracorporeal Life Support during Cardiopulmonary Resuscitation in post-operative cardiac patients, coupled with the fact that patients undergoing the Norwood (Stage 1) operation can have rapid, unpredictable cardiac deterioration and arrest, has led to a steady increase in the use of Extracorporeal Life Support during Cardiopulmonary Resuscitation in this population. For Extracorporeal Life Support during Cardiopulmonary Resuscitation to be most successful, it must be deployed rapidly while the patient is undergoing excellent cardiopulmonary resuscitation. Early activation of the team that will perform cannulation could possibly shorten the duration of cardiopulmonary resuscitation and might improve survival and outcome. More research needs to be done to refine the populations and circumstances that offer the best outcome with Extracorporeal Life Support during Cardiopulmonary Resuscitation, to evaluate the ratios of cost to benefit, and establish the long-term neurodevelopmental outcomes in survivors.

Coming from behind to win - A Qualitative research about psychological conditions of adolescents who have undergone open-heart surgery for single ventricle between the ages 0-5

Early recognition of congenital cardiac pathologies and their treatment by means of palliative or corrective surgery at birth or infancy has vital importance. Successful repair of congenital cardiac defects by surgical methods has gained importance especially during the last twenty years. As the scope of the surveillance increased so did the interest in the outcomes of these treatments when the patients had reached puberty and adulthood. The purpose of our research was to study the psychological framework of the adolescents who had experienced these surgeries by listening both the children and the parents talk about their feelings and experiences. Our data was accumulated through interviews with 17 adolescents and their families, using qualitative methods. The main theme at the end of the analysis was "to be strong and resistive". We reached the conclusion that this condition was not a pathological build up but an attitude of coping, as it did not cause loss of functionality. The defensive psychological mechanisms used by these adolescents consisted of repression, compensation and reaction formation. We believe that this information is important to understand the real meaning of the manners displayed when these adolescents and their families pursue their daily lives, communicate and make relationships with their environment and especially professionals in the health services.

Impact of Postoperative Hyperglycemia following Surgical Repair of Congenital Cardiac Defects

The objective of this study was to determine the prevalence of postoperative hyperglycemia in pediatric patients following surgery for congenital cardiac defects and its impact on morbidity and mortality. It was designed as a retrospective cohort study in a pediatric intensive care unit of a university-affiliated free-standing children's hospital. A cohort of 213 patients who underwent 237 surgical procedures for repair or palliation of congenital cardiac defects comprised the study. Postoperative blood glucose measurements and all clinical and laboratory data were compiled for the first 10 days after surgery. The intensity and duration of hyperglycemia were analyzed for association with hospital morbidities and mortality. Mild and severe hyperglycemia were highly prevalent in our cohort (97% and 78%, respectively). Survivors had significantly lower peak (289.7 +/- 180.77 mg/dl vs. 386 +/- 147.95 mg/dl), mean (110.13 +/- 36.22 mg/dl vs. 146.75 +/- 57.12 mg/dl), and duration (2.59 +/- 2.3 days vs. 5.35 +/- 2.8 days) of hyperglycemia compared to nonsurvivors. Duration of hyperglycemia was independently associated with morbidity [odds ratio (OR): 1.95; p < 0.001] and mortality (OR: 1.41; p = 0.03) by multivariate logistic regression. Hyperglycemia is common in children following surgical repair or palliation of congenital cardiac defects. Postoperative hyperglycemia is associated with increased morbidity and mortality in these patients.

Complement Factor 1 Inhibitor Improves Cardiopulmonary Function in Neonatal Cardiopulmonary Bypass

The inflammatory insult associated with cardiopulmonary bypass (CPB) continues to result in morbidity for neonates undergoing complex repair of congenital cardiac defects. Complement and contact activation are important mediating processes involved in this injury. Complement factor 1 esterase inhibitor (C1-inh), a natural inhibitor of complement, kallikrein, and coagulation pathways, may be decreased in children undergoing cardiac operations requiring CPB. We tested the hypothesis that C1-inh supplementation will ameliorate the cardiac and pulmonary dysfunction in a model of neonatal CPB. Fifty-two neonatal pigs were randomly assigned to receive 0 IU (n = 22), 500 IU (n = 15), 1,000 IU (n = 8), or 1,500 IU (n = 7) of C1-inh. Doses were delivered 5 minutes before starting 90 minutes of normothermic CPB. Pulmonary and cardiovascular measures were taken before and 5, 30, and 60 minutes after CPB. Five animals did not survive CPB. The C1-inh concentration post-CPB increased monotonically with increasing dose (p < 0.001). Weight gain was significantly less in the 1,500 IU group (0.24 +/- 0.10 kg versus 0.38 +/- 0.09 kg, p = 0.001). Dynamic compliance increased with C1-inh dose from 0 to 500 IU by 23% +/- 4% (p < 0.001), but the increase leveled off at the higher doses. Alveolar-arterial O2 gradient decreased with C1-inh dose (p = 0.009). Time derivative of left ventricular pressure (dP/dt(max)) increased significantly with increasing dose (p = 0.016). At the highest dose of C1-inh, the time constant of isovolumic relaxation was increased (p = 0.018). The C1-inh supplementation results in improved pulmonary and systolic cardiac function in a model of neonatal CPB. The negative effect on diastolic function requires further investigation.

Factors influencing the outcome of paediatric cardiac surgical patients during extracorporeal circulatory support.

BackgroundVeno-arterial extracorporeal membrane oxygenation (ECMO) is a common modality of circulatory assist device used in children. We assessed the outcome of children who had ECMO following repair of congenital cardiac defects (CCD) and identified the risk factors associated with hospital mortality.MethodsFrom April 1990 to December 2003, 53 patients required ECMO following surgical correction of CCD. Retrospectively collected data was analyzed with univariate and multivariate logistic regression analysis.ResultsMedian age and weight of the patients were 150 days and 5.4 kgs respectively. The indications for ECMO were low cardiac output in 16, failure to wean cardiopulmonary bypass in 13, cardiac arrest in 10 and cardio-respiratory failure in 14 patients. The mean duration of ECMO was 143 hours. Weaning off from ECMO was successful in 66% and of these 83% were survival to hospital-discharge. 37.7% of patients were alive for the mean follow-up period of 75 months. On univariate analysis, arrhythmias, ECMO duration >168 hours, bleeding complications, renal replacement therapy on ECMO, arrhythmias and cardiac arrest after ECMO were associated with hospital mortality.On multivariate analysis, abnormal neurology, bleeding complications and arrhythmias after ECMO were associated with hospital mortality. Extra and intra-thoracic cannulations were used in 79% and 21% of patients respectively and extra-thoracic cannulation had significantly less bleeding complications (p = 0.031).ConclusionECMO provides an effective circulatory support following surgical repair of CCD in children. Extra-thoracic cannulation is associated with less bleeding complications. Abnormal neurology, bleeding complications on ECMO and arrhythmias after ECMO are poor prognostic indicators for hospital survival.

Aprotinin and Sevoflurane Do Not Affect Renal Function During Single-Ventricle Palliative Surgery

To the Editor: A recent study of aprotinin in adult cardiac surgery is rekindling interest in its renal toxicity (1). Single-ventricle, palliative surgery entails low oxygen delivery and bleeding, which are attenuated by aprotinin (2,3). After University IRB approval, we retrospectively reviewed medical records of 18 children having Sano-Norwood, 28 bidirection cavopulmonary Glenn shunts, and 15 Fontans from August 2002 to April 2004, roughly corresponding to the time that aprotinin use became standard. We gave a test dose of aprotinin, 700 U/kg, at prebypass, a pump prime bolus of 30,000 U/kg, and an infusion during bypass of 10,000 U/kg/h. The demographics of the children are summarized in Table 1.Table 1: Preoperative and Intraoperative DemographicsThe outcomes were peak creatinine during the first 7 postoperative days, postbypass partial pressure of oxygen (inspired oxygen 100%), liver function tests, and time spent in the operating room. Table 2 shows the outcome variables, including those groups whose means differed at P < 0.05, uncorrected for the number of statistical tests. Although several P values did reach statistical significance, as expected with this many comparisons without Bonferroni correction, in no case was worse outcome associated with use of aprotinin. Table 3 shows the relationship between increase in serum exposure and sevoflurane exposure, stratified by operation. Again, the P tests were not corrected for repeated measures, and as expected, a single test identified a relationship between sevoflurane exposure and postoperative serum creatinine increase. As was the case with aprotinin, the data weakly suggest that sevoflurane exposure is associated with reduced risk of postoperative renal dysfunction, as patients with evidence of renal dysfunction are those who had lower, not higher, exposure to sevoflurane.Table 2: Outcomes Mean Listed ± sd, with P Values <0.05 in ParenthesesTable 3: Relationship of Sevoflurane Dose to a Rise in Creatinine of 50%Although this is a very small study, peak creatinine and amounts of transfusions were not related to administration of aprotinin or sevoflurane. In general, oxygen partial pressure was improved with aprotinin in Fontans, as reported by Tweddell et al. who described an improved transpulmonary pressure gradient (3). Many studies show the potential for thrombosis with aprotinin, but unfortunately data are sparse regarding renal dysfunction (4). The key point of these data is that we did not see evidence of clinical concern in this population of children with either aprotinin or sevoflurane. If anything, our data support the safety of these drugs for use in children undergoing repair of congenital cardiac defects. Kimberly L. Skidmore, MD Anthony Azakie, MD University of California San Francisco, CA [email protected]

Genetic variation in the mitochondrial enzyme carbamyl-phosphate synthetase I predisposes children to increased pulmonary artery pressure following surgical repair of congenital heart defects: A validated genetic association study

Increased pulmonary artery pressure (PAP) can complicate the postoperative care of children undergoing surgical repair of congenital heart defects. Endogenous NO regulates PAP and is derived from arginine supplied by the urea cycle. The rate-limiting step in the urea cycle is catalyzed by a mitochondrial enzyme, carbamoyl-phosphate synthetase I (CPSI). A well-characterized polymorphism in the gene encoding CPSI (T1405N) has previously been implicated in neonatal pulmonary hypertension. A consecutive modeling cohort of children ( N = 131) with congenital heart defects requiring surgery was prospectively evaluated to determine key factors associated with increased postoperative PAP, defined as a mean PAP > 20 mmHg for at least 1 h during the 48 h following surgery measured by an indwelling pulmonary artery catheter. Multiple dimensionality reduction (MDR) was used to both internally validate observations and develop optimal two-variable through five-variable models that were tested prospectively in a validation cohort ( N = 41). Unconditional logistic regression analysis of the modeling cohort revealed that age (OR = 0.92, p = 0.01), CPSI T1405N genotype (AC vs. AA: OR = 4.08, p = 0.04, CC vs. AA: OR = 5.96, p = 0.01), and Down syndrome (OR = 5.25, p = 0.04) were independent predictors of this complex phenotype. MDR predicted that the best two-variable model consisted of age and CPSI T1405N genotype ( p < 0.001). This two-variable model correctly predicted 73% of the outcomes from the validation cohort. A five-variable model that added race, gender and Down’s syndrome was not significantly better than the two-variable model. In conclusion, the CPSI T1405N genotype appears to be an important new factor in predicting susceptibility to increased PAP following surgical repair of congenital cardiac defects in children.

2006 William W. L. Glenn Lecture–Rebuilding the Heart: New Horizons for Cardiac Surgeons

Cardiac surgery is changing today, as it was during the time of Dr William Glenn. In those days, cardiac surgeons were trained to do thoracic and vascular surgery.Today, cardiac surgeons are retraining to do intravascular and minimally invasive cardiac and vascular procedures. However, today’s cardiac surgeons should also retrain to employ gene-enhanced cell therapy to modify both the heart and the vasculature to improve the outcomes of their interventions. Cell transplantation has come of age and is undergoing extensive clinical trials. The implantation of precursor cells induces angiogenesis, improves regional and global function, and enhances the recruitment of reparative cells to the heart. In addition to correcting anatomic cardiac lesions, surgeons may be able to restore function to the heart by a combination of cardiac regeneration and rejuvenation of the response to injury. Tissue engineering may restore heart function without synthetic materials, provided the surgeon employs the right combination of cells and biodegradable scaffolds. These grafts may be ideal for the surgical repair of congenital cardiac defects, and clinical trials are underway. The implanted grafts grow and remodel as the child becomes an adult. Cardiac surgeons are retraining to acquire new skills to correct cardiovascular defects. In addition, surgeons should acquire the knowledge required to regenerate and rebuild the heart and vasculature.

Laryngotracheal Consequences of Pediatric Cardiac Surgery

To determine the incidence and character of clinically significant laryngotracheal anomalies in pediatric patients undergoing surgical repair of congenital cardiac defects at a tertiary care center. Single-center retrospective review. The charts of pediatric patients who required surgical treatment for congenital heart disease over a 4-year period were reviewed. Forty-eight of 1957 patients were seen in inpatient consultation by the otolaryngology service. The parameters studied included cardiac diagnosis, reason for consultation, findings on examination, and follow-up. There were 16 (33%) cases of subglottic stenosis, which were graded according to the Cotton-Myer classification system as follows: grade 1 (n=8); grade 2 (n=3); and grade 3 (n=5). Three of the 16 patients with subglottic stenosis required tracheotomy and 4 required laryngotracheal reconstruction. Nine (19%) of the 48 patients were diagnosed as having unilateral true vocal cord paralysis and 3 (6%) as having bilateral paralysis. With the exception of 1 patient, all patients with true vocal cord paralysis on the left side had undergone repair of the aortic arch. Pediatric patients with congenital cardiac disease are predisposed to laryngeal anomalies owing to (1) frequent intubation, (2) prolonged ventilatory support, and (3) recurrent laryngeal nerve injury. In our patients, subglottic stenosis was the most common laryngeal abnormality. When recognized early, in the eschar phase, most of these cases can be managed with sequential endoscopic debridement, which is conceptually similar to debridement that is performed after functional endoscopic sinus surgery. Established stenosis requires more vigorous intervention, the invasive degree of which depends on the length and circumference of the narrowing. Unilateral vocal paralysis tends to be a self-limited problem, while an elegant solution to bilateral paralysis remains elusive.

Body Weight-Related ionized hypomagnesemia in pediatric patients undergoing cardiopulmonary bypass for surgical repair of congenital cardiac defects

Study objectives To examine the serial time course of perioperative plasma ionized magnesium (iMg 2+ ) concentrations and to analyze the plasma iMg 2+ concentrations in children with different body mass who were undergoing open-heart surgery. Design Randomized, single-blinded study. Setting University-affiliated hospital of an academic medical institution. Patients 38 children undergoing open-heart surgery. Interventions Patients were divided into three groups according to their body mass: Group 1 (n = 12) <10 kg, Group 2 (n = 13) 10 kg to 20 kg, and Group 3 (n = 13) >20 kg. Measurements The relationship of iMg 2+ among the three groups of different body mass were analyzed at five different time intervals during the operation: induction of anesthesia, 5 minutes and 30 minutes after the onset of cardiopulmonary bypass (CPB), the beginning of rewarming, and the end of surgery. Main results iMg 2+ levels at 5 minutes after onset of CPB in patients weighing less than 20 kg (Groups 1 and 2) differed with those weighing more than 20 kg (Group 3) (p = 0.007 and 0.013). However, there was no difference in the iMg 2+ levels between Groups 1 and 2 (p = 0.993). In addition, iMg 2+ levels at 5 minutes after onset of bypass correlated well (r 2 = 0.66) in children with body mass less than 20 kg. Conclusions Low levels of ionized magnesium is an important finding in patients at the onset of CPB, which correlates well with the body mass of patients weighing less than 20 kg, and could be predicted by the regression curve. Based on these findings, hypomagnesemia can be prevented during CPB.