Novel pathogenic GATA6 variant associated with congenital heart disease, diabetes mellitus and necrotizing enterocolitis.

BACKGROUND: Pathogenic GATA6 variants have been associated with congenital heart disease (CHD) and a spectrum of extracardiac abnormalities, including pancreatic agenesis, congenital diaphragmatic hernia, and developmental delay. However, the comprehensive genotype-phenotype correlation of pathogenic GATA6 variation in humans remains to be fully understood. METHODS: Exome sequencing was performed in a family where four members had CHD. In vitro functional analysis of the GATA6 variant was performed using immunofluorescence, western blot, and dual-luciferase reporter assay. RESULTS: A novel, heterozygous missense variant in GATA6 (c.1403 G > A; p.Cys468Tyr) segregated with affected members in a family with CHD, including three with persistent truncus arteriosus. In addition, one member had childhood onset diabetes mellitus (DM), and another had necrotizing enterocolitis (NEC) with intestinal perforation. The p.Cys468Tyr variant was located in the c-terminal zinc finger domain encoded by exon 4. The mutant protein demonstrated an abnormal nuclear localization pattern with protein aggregation and decreased transcriptional activity. CONCLUSIONS: We report a novel, familial GATA6 likely pathogenic variant associated with CHD, DM, and NEC with intestinal perforation. These findings expand the phenotypic spectrum of pathologic GATA6 variation to include intestinal abnormalities. IMPACT: Exome sequencing identified a novel heterozygous GATA6 variant (p.Cys468Tyr) that segregated in a family with CHD including persistent truncus arteriosus, atrial septal defects and bicuspid aortic valve. Additionally, affected members displayed extracardiac findings including childhood-onset diabetes mellitus, and uniquely, necrotizing enterocolitis with intestinal perforation in the first four days of life. In vitro functional assays demonstrated that GATA6 p.Cys468Tyr variant leads to cellular localization defects and decreased transactivation activity. This work supports the importance of GATA6 as a causative gene for CHD and expands the phenotypic spectrum of pathogenic GATA6 variation, highlighting neonatal intestinal perforation as a novel extracardiac phenotype.

Utility of the NEONATE Score at an Institution that Routinely Performs the Hybrid Procedure for Hypoplastic Left Heart Syndrome.

NEONATE score > 17 has been proposed as a risk factor for interstage mortality/cardiac transplant (IM/T) for patients with single ventricle physiology. Hybrid procedure is assigned 6 points, the highest possible score for that surgical variable. Most centers reserve the hybrid procedure for high-risk patients. Goal of this study was to evaluate the NEONATE score at a center that routinely performs the hybrid procedure. Retrospective chart review of patients undergoing the hybrid procedure was performed (2008-2021). Demographics and variables used for the NEONATE score were collected. Maximization of Youden's J Statistic used to determine cohort-specific optimal threshold for patients undergoing comprehensive Stage II procedure (H-CSII) versus those with IM/T (H-IM/T). Total of 120 patients met inclusion criteria (H-CSII = 105, H-IM/T = 15). Gestational age was median 39 weeks (IQR 38, 39) and birth weight was 3.18 kg (2.91, 3.57). No patient was discharged with opiates or required post-operative extracorporeal circulatory support. Optimal threshold, as selected by maximizing Youden's J Statistic, was 22. Score > 22 had a positive predictive value of 0.33 (95% CI 0.12-0.62), negative predictive values of 0.90 (95% CI 0.83-0.95), and accuracy of 0.83 (95% CI 0.75-0.90) for IM/T. At a center that routinely performs the hybrid procedure, value of > 22 had the highest accuracy. This suggests that the hybrid procedure is not necessarily intrinsically a risk-factor for IM/T, but rather patient selection for the hybrid procedure may play a larger role at centers that do not routinely perform this procedure.

A ROTEM-guided algorithm aimed to reduce blood product utilization during neonatal and infant cardiac surgery.

BACKGROUND: Neonates and infants undergoing cardiac surgery tend to receive high volumes of blood products. The use of rotational thromboelastometry (ROTEM®) has been shown to reduce the administration of blood products in adults after cardiac surgery. We sought to develop a targeted administration of blood products based on ROTEM® to reduce blood product utilization during and after neonatal and infant cardiac surgery. METHODS: We conducted a retrospective review of data from a single center for neonates and infants undergoing congenital cardiac surgery using cardiopulmonary bypass (CPB) from September 2018-April 2019 (control group). Then, using a ROTEM® algorithm, we collected data prospectively between April-November 2021 (ROTEM group). Data collected included age, weight, gender, procedure, STAT score, CPB time, aortic cross-clamp time, volume, and type of blood products administered in the operating room and cardiothoracic intensive care unit (CTICU). In addition, ROTEM® data, coagulation profile in CTICU, chest tube output at 6 and 24 hours, use of factors concentrate, and thromboembolic complications were recorded. RESULTS: The final cohort of patients included 28 patients in the control group and 40 patients in the ROTEM group. The cohort included neonates and infants undergoing the following procedures: arterial switch, aortic arch augmentation, Norwood procedure, and comprehensive stage II procedure. There were no differences in the demographics or procedure complexity between the two groups. Patients in the ROTEM® group received fewer platelets (36 ± 12 vs. 49 ± 27 mL/kg, p 0.028) and cryoprecipitate (8 ± 3 vs. 15 ± 10 mL/kg, p 0.001) intraoperatively when compared to the control group. CONCLUSION: The utilization of ROTEM® may have contributed to a significant reduction in some blood product administration during cardiac surgery for infants and neonates. ROTEM® data may play a role in reducing blood product administration in neonatal and infant cardiac surgery.

Management of Hypertrophic Cardiomyopathy in a Newborn with Dextro-Transposition of the Great Arteries.

Impaired maternal glucose metabolism during pregnancy can have significant effects on the cardiovascular system of the developing fetus. Early in pregnancy the teratogenic effects may lead to structural heart defects, while later in gestation a form of hypertrophic cardiomyopathy can develop due to overgrowth driven by fetal hyperinsulinism. We describe an infant with the uncommon combination of both dextro-transposition of the great arteries and hypertrophic cardiomyopathy. We emphasize the importance of a longitudinal multi-disciplinary approach, from fetal diagnosis to post-operative management, that allowed for an excellent outcome in this rare combination of severe cardiac malformations.

Post-operative Anticoagulation Strategy Following Comprehensive Stage 2 Procedure for Single Ventricle Physiology.

Thrombosis, especially thrombosis of the pulmonary artery, is a large contributor to morbidity and mortality following comprehensive stage 2 procedure for single ventricle cardiac physiology. A peri-operative management protocol was implemented at our institution in March 2010. It includes 6 weeks of therapeutic anticoagulation post-operatively to mitigate the thrombotic risks in this patient population. This is a retrospective study of hospitalized children who received post-operative anticoagulation following a comprehensive stage 2 procedure for single ventricle cardiac physiology at a free-standing children's hospital. The primary objectives are to describe our institution's anticoagulation strategy and report on the number of thromboses and major bleeding episodes in the 6 weeks post-operatively. Secondary objectives include the dose of enoxaparin required to obtain a therapeutic low-molecular weight anti-factor-Xa (AFXaLMWH) level, and the number of patients outside of the therapeutic range. A total of 71 infants were included in the final analysis. Four patients experienced a thrombosis episode and three patients experienced clinically significant bleeding. The mean dose of enoxaparin required to obtain a therapeutic AFXaLMWH level between 0.5-1 unit/mL was 1.23 mg/kg SQ every 12 h and 37% of patients achieved goal AFXaLMWH levels with the initial starting dose of enoxaparin 1 mg/kg SQ every 12 h. We describe a 9-year experience of anticoagulation after single ventricle palliation. Anticoagulation with therapeutic AFXaLMWH goals of 0.5-1 unit/mL may reduce the rates of clinically significant thrombosis post-operatively in this population and appears safe without increase in significant bleeding episodes when compared to a historical cohort. Further studies comparing this population to those who do not receive post-operative anticoagulation are warranted.

Incidence of and Risk Factors for Aortic Arch Interventions After the Comprehensive Stage II Procedure for Hypoplastic Left Heart Syndrome.

Minimal data exist about the incidence and risk factors for arch intervention after comprehensive stage II (CSII). Goal of this study was to document incidence of arch interventions after CSII and determine if any differences existed between those who underwent an arch intervention (aiCSII) versus those did not have an intervention. Single-center retrospective chart review of all hypoplastic left heart syndrome patients who underwent a CSII between 6/1/2005 and 2/1/2020 was performed. Univariate analysis was conducted in addition to principal components analysis (PCA). One hundred patients were evaluated. Sixteen patients underwent 24 arch interventions. Age at initial arch reintervention was 1.3 ± 1.2 years (median 1.0 years, range 0.5-2.2 years). Univariate analysis showed that the aiCSII group were more likely to be female, to have had a retrograde arch intervention post-hybrid procedure, and to be younger at time of CSII. On echocardiograms, aiCSII group had significantly higher pre-CSII patent ductus arteriosus velocities, arch velocities on their 1st post-operative and discharge study post-CSII, and arch velocities pre-Fontan. Gradients were higher in the aiCSII via pre-Fontan catheterization. With PCA, echocardiographic and catheterization data remained significantly associated with aiCSII versus those who did not undergo an arch intervention (OR = 4.5 (1.9, 19.8), p = 0.008). Incidence of arch intervention post-CSII was 16%. Echocardiographic arch velocities during the CSII hospitalization were the strongest predictors for subsequent aortic arch interventions. Further studies are needed to determine any modifiable variables that may reduce the incidence of arch interventions.

Impact of Viral PCR Positive Nasal Swabs (Non Covid-19) on Outcomes Following Cardiac Surgery.

Viral bronchiolitis is a relative contraindication to elective pediatric cardiac surgery. Nasopharyngeal swab utilizing polymerase chain reaction (PCR) screening for viruses known to cause bronchiolitis are commonly available. The objective of this study was to evaluate clinical outcomes in patients with nasopharyngeal viral PCR positive findings at the time of cardiac surgery. Retrospective review from January 2013 to May 2019 for patients with virus detected by PCR on nasopharyngeal swabs at the time of cardiac surgery. Single ventricle and two ventricle patients were compared to control group of age and procedure matched patients viral negative at the time of surgery. Outcome measures included OR extubation, reintubation, hospital length of stay, and mortality. For two ventricle patients (n = 81; control group = 165), there was no statistical difference in any outcome variable (OR extubation 74% vs 72%; p = 0.9; reintubation 9% vs 11% vs; p = 0.7; hospital length of stay 5 days (1-46) vs 4 days (2-131); p = 0.4; mortality 2 vs 1; p = 0.3). For single ventricle patients, there was no statistical difference in any outcome variable (OR extubation 81% vs 76%; p = 0.6; reintubation 14% vs 21% vs; p = 0.5; hospital length of stay 9.5 days (3-116) vs 15 days (2-241); p = 0.1; mortality 0 vs 3; (p = 0.6)). PCR is a sensitive test that fails to predict which patients will proceed to have a clinically significant infection. Viral bronchiolitis remains a relative risk factor for cardiac surgery; presence of detectable virus via nasopharyngeal swab with limited clinical symptoms may not be a contraindication to cardiac surgery.

Hybrid Procedures: A Surgeon's Viewpoint on the Next 10 Years.

Many forces are once again bringing the congenital heart surgeon and interventional cardiologist, and the teams that support them, ever closer together in what has been deemed Hybrid Approaches to congenital heart disease. The goal of these hybrid approaches is to improve the quantity and quality of life for the patients we serve.

Bloodless Arterial Switch Operation in a 2.7-kg Jehovah's Witness Patient.

Bloodless pediatric cardiac surgery requiring the use of cardiopulmonary bypass (CPB) remains a challenge for the entire operating room (OR) team. The amount of circulating blood volume to pump prime volume mismatch of small patients results in hemodilution that frequently results in transfusion of allogeneic blood products. Patients of families of the Jehovah's Witness (JW) faith reject the use of these products because of religious beliefs. Our institution is a referral center for children of JW families because we have developed techniques to minimize blood loss with the hope of performing bloodless pediatric cardiac surgery whenever possible. These techniques include preoperative treatment with erythropoietin, intraoperative acute normovolemic hemodilution, CPB circuit miniaturization, ultrafiltration during and after CPB, limiting blood gas analyses or other unnecessary blood draws, and using hemostatic agents during and after CPB. We present the case of a 4-day-old patient of the JW faith weighing 2.7 kg with transposition of the great arteries and an intact ventricular septum who underwent an arterial switch operation. The patient received no allogeneic blood product administration throughout the entire hospitalization. The patient's first hematocrit in the OR was 43%, lowest hematocrit on bypass was 15%, and first hematocrit in the cardiothoracic intensive care unit post-procedure was 21%. The patient was discharged on post-op day nine with a hematocrit of 36%.

The Correlation of Two Cerebral Saturation Monitors With Jugular Bulb Oxygen Saturation in Children Undergoing Cardiopulmonary Bypass for Congenital Heart Surgery.

BACKGROUND: Near-infrared spectroscopy (NIRS) is a noninvasive monitoring technique that measures regional cerebral oxygen saturation (rSO2). OBJECTIVES: The primary aim was to compare the output of 2 NIRS-based cerebral oximetry devices, FORESIGHT (CAS Medical Systems Inc, Branford, Connecticut) and INVOS (Covidien, Boulder, Colorado), to venous oxygen saturations from the jugular venous bulb at cannulation and decannulation of the superior vena cava (SVC). Secondary objectives included evaluating correlations of cerebral saturation, as measured by the NIRS devices, with mean arterial blood pressure (MAP), measured by an invasive arterial line, and end-tidal CO2 (ETCO2). METHODS: Near-infrared spectroscopy, MAP, and ETCO2 data were collected at 13 defined events during each case when hemodynamic instability was expected. At SVC cannulation and decannulation, a 0.1 mL sample of blood was collected from the jugular bulb by the surgeon using a long angiocatheter. The oxygen saturation of these blood samples was measured using an AVOX device and compared with contemporaneous readings from the NIRS probes. Mixed-effects linear regression was used to correlate MAP or ETCO2 with cerebral oxygen saturation (by NIRS) at each time point. RESULTS: Children undergoing cardiopulmonary bypass for congenital heart surgery (n = 34) were enrolled in the study. At SVC cannulation, both INVOS ( r = .78) and FORESIGHT ( r = .59) were correlated with AVOX data at P < .001, although the correlation with INVOS was significantly stronger ( P = .003). At SVC decannulation, INVOS ( r = .68; P < .001) and FORESIGHT ( r = .60; P < .001) were similarly correlated with jugular venous rSO2. Correlations of rSO2 (by NIRS) with MAP and ETCO2 levels were stronger than correlations between rSO2 change and change in MAP or ETCO2. CONCLUSION: INVOS correlated more strongly than FORESIGHT with the jugular bulb rSO2 at SVC cannulation but may have underestimated oxygen saturation at low rSO2 values. Data from both NIRS devices were correlated with MAP and ETCO2 over the case duration.

Extracorporeal Membrane Oxygenation Outcomes After the Comprehensive Stage II Procedure in Patients With Single Ventricles.

Outcomes for extracorporeal membrane oxygenation (ECMO) have been described for patients with single ventricle physiology (SVP) undergoing cavopulmonary connection (Glenn procedure). An alternative surgical pathway for patients with SVP consists of an initial hybrid procedure followed by a comprehensive Stage II procedure. No data exist describing the outcomes of patients requiring ECMO after the comprehensive Stage II procedure. The goal of this study is to describe the outcomes for patients who required ECMO after the comprehensive Stage II procedure. Data from the Extracorporeal Life Support Organization (ELSO) registry from 2001 to 2015 for children undergoing the comprehensive Stage II procedure older than 3 months of age were retrospectively analyzed. Demographics and ECMO characteristics were recorded. A total of six children required ECMO support after the comprehensive Stage II procedure (2 males, 4 females). Four patients had the diagnosis of hypoplastic left heart syndrome and two patients had the diagnosis of an unbalanced atrioventricular septal defect. Bypass time was 242.8 ± 110.9 min and cross-clamp time was 91.2 ± 46.2 min for the surgical procedure. Weight was 5.8 ± 1.3 kg and age was 150.2 + 37.9 days at time of ECMO. ECMO duration was 276.0 ± 218.1 h. Complications during the ECMO run included hemorrhage in four patients (67%), renal dysfunction in two patients (33%), and neurologic injury in two patients (33%). Four patients (67%) were discharged alive after ECMO decannulation. Despite being a much more extensive surgical procedure, the morbidity and mortality after ECMO in patients undergoing the comprehensive Stage II procedure are similar to those in patients undergoing the Glenn procedure. If needed, ECMO support is reasonable for patients after the comprehensive Stage II procedure.

Revisiting acute normovolemic hemodilution and blood transfusion during pediatric cardiac surgery: a prospective observational study.

BACKGROUND: The majority of allogeneic transfusions occur in the perioperative setting, especially during cardiac surgery. In addition to the economic implications, there is emerging evidence that blood transfusion may increase both morbidity and mortality. Acute normovolemic hemodilution (ANH) may limit the need for blood products. OBJECTIVES: The primary objective of this study was to determine if the method of blood collection (syringe or bag) during the ANH process impacted the platelet count and function. The secondary objectives included the need for perioperative blood transfusions during the procedure and in the intensive care unit. In addition, we assessed these outcomes' associations with ANH parameters including the method of collection, time of storage, and volume removed. METHODS: Data were collected prospectively from 50 patients undergoing cardiac surgery on cardiopulmonary bypass over a 6-month period. Platelet count and function were measured for the ANH blood immediately after collection and again prior to transfusing to the patient at the end of cardiopulmonary bypass. Other data collected included ANH volume, length of storage, and the quantity of all blood products given throughout the perioperative period. RESULTS: No change in platelet count or function was noted regardless of the length of time or collection method for the ANH blood. Twenty-three patients received blood or blood products in the operating room or the intensive care unit, while 27 patients received no blood transfusion during their entire hospitalization. Higher ANH volume (ml·kg-1 ) and longer storage time were associated with a greater need for intraoperative transfusions. CONCLUSION: Acute normovolemic hemodilution protects the platelets from the untoward effects of cardiopulmonary bypass and offers an important autologous blood product that improves hemostasis at the conclusion of surgery. Platelet count and function are preserved regardless of the method of collection or the length of storage. The volume of ANH removed appears to be an important determinant of blood product use and further understanding of the impact of this variable is a future direction of upcoming prospective research.

Influence of Transplant Center Procedural Volume on Survival Outcomes of Heart Transplantation for Children Bridged with Mechanical Circulatory Support.

Transplant center expertise improves survival after heart transplant (HTx) but it is unknown whether center expertise ameliorates risk associated with mechanical circulatory support (MCS) bridge to transplantation. This study investigated whether center HTx volume reduced survival disparities among pediatric HTx patients bridged with extracorporeal membrane oxygenation (ECMO), left ventricular assist device (LVAD), or no MCS. Patients ≤18 years of age receiving first-time HTx between 2005 and 2015 were identified in the United Network of Organ Sharing registry. Center volume was the total number of HTx during the study period, classified into tertiles. The primary outcome was 1 year post-transplant survival, and MCS type was interacted with center volume in Cox proportional hazards regression. The study cohort included 4131 patients, of whom 719 were supported with LVAD and 230 with ECMO. In small centers (≤133 HTx over study period), patients bridged with ECMO had increased post-transplant mortality hazard compared to patients bridged with LVAD (HR 0.29, 95% CI 0.12, 0.71; p = 0.006) and patients with no MCS (HR 0.33, 95% CI 0.19, 0.57; p < 0.001). Interactions of MCS type with medium or large center volume were not statistically significant, and the same differences in survival by MCS type were observed in medium- or large-volume centers (136-208 or ≥214 HTx over the study period). Post-HTx survival disadvantage of pediatric patients bridged with ECMO persisted regardless of transplant program volume. The role of institutional ECMO expertise outside the transplant setting for improving outcomes of ECMO bridge to HTx should be explored.

The Effect of Autologus Blood Priming on Cerebral Oximetry in Congenital Cardiac Surgery Patients.

Hemodilution is one of the sequelae of cardiopulmonary bypass (CPB). Autologous blood priming (retrograde autologous priming [RAP]/venous antegrade priming [VAP]) and acute normovolemic hemodilution (ANH) may be effective techniques to minimize hemodilution. The primary objective of this study is to investigate the impact of RAP/VAP combined with ANH on changes in cerebral saturations. A retrospective analysis of 52 patients undergoing congenital cardiac surgery requiring CPB between July 2014 and March 2015 was performed. Bivariate analysis correlated RAP/VAP and ANH volumes. SrO2 change scores were regressed on all covariates using multivariable least-squares models. The average percent of circulating blood volume (CBV) removed during RAP/VAP was 21 ± 10% in the cyanotic group and 15 ± 5% in the acyanotic group (p =.006). There was a decrease in SrO2 from 70 ± 11% at baseline to 55 ± 13% at CPB initiation, although this decrease did not differ by cyanosis (p = .668) or use of ANH (p = .566). Bivariate correlation and multivariable regression analysis of the SrO2 change score further demonstrated no statistically significant correlation between percent of CBV removed during RAP/VAP or ANH and the magnitude of the decline in SrO2. RAP and VAP help minimize hemodilution at the onset of CPB. This study further supports the use of these techniques in a pediatric population by demonstrating declines in SrO2 during RAP/VAP were consistent among cyanotic and acyanotic, including those who underwent ANH.

Bloodless Repair for a 3.6 Kilogram Transposition of the Great Arteries with Jehovah's Witness Faith.

Achieving pediatric cardiac surgery using cardiopulmonary bypass (CPB) without allogeneic blood transfusion is challenging. There are many clinical and economic factors that point to the importance of avoiding blood transfusions. In some instances, honoring patients or parents beliefs may be the reason for avoiding blood transfusions. For example, patients or parents of the Jehovah's Witness faith refuse blood transfusion based on their religious beliefs. Over the last decade, our institution has seen a steady increase in our pediatric Jehovah's Witness patient population. Caring for these patients have allowed us to develop specific protocols that enable us to safely provide bloodless CPB in all of our patient populations. The success of such an approach to minimize the need for blood transfusions should not start in the operating room; it must include the preoperative period and the postoperative care by the critical care team in the cardiac intensive care unit (CICU). A multidisciplinary team approach has to be in place with clear communication between the cardiologist, anesthesiologist, cardiac surgeon, perfusionist, and the cardiac intensivist. We present a case of a 7 day old male (3.6 kg) with a preoperative diagnosis of Transposition of the Great Arteries and intact ventricular septum who underwent an arterial switch procedure without the transfusion of any blood products throughout his entire hospital stay.

Induction immunosuppression for combined heart-lung transplantation.

BACKGROUND: Research on induction immunosuppression in patients undergoing combined heart-lung transplantation (HLTx) is limited. METHODS: The United Network for Organ Sharing database was queried from 2000 to 2013 to examine the influence of induction immunosuppression for combined HLTx in adult (≥18 years) and adolescent (≥12 and <18 years) recipients. RESULTS: Of 394 eligible combined HLTx cases (361 adults, 33 adolescents), 384 were included in univariate Cox analysis and 116 in the multivariate Cox model. Univariate analysis demonstrated no differences in survival by induction medication and no difference among the most common maintenance immunosuppression regimens. Adjusting for use of corticosteroids, multivariate analysis demonstrated no benefit of basiliximab (HR=3.582; 95% CI: 0.966, 13.279; P=.056), thymoglobulin/antilymphocyte globulin (ALG)/antithymocyte globulin (ATG) (HR=0.808; 95% CI: 0.134, 4.888; P=.817), alemtuzumab (HR=0.369; 95% CI: 0.087, 1.563; P=.176), or other induction medications (HR=1.511; 95% CI: 0.146, 15.610; P=.729), compared to no induction medication, with respect to mortality hazard post-HLTx. There were also no differences in treated acute rejection episodes by type of induction immunosuppression. CONCLUSIONS: Induction immunosuppression with contemporary agents does not improve survival after combined HLTx.

Learning From Experience: Improving Early Tracheal Extubation Success After Congenital Cardiac Surgery.

OBJECTIVES: The many advantages of early tracheal extubation following congenital cardiac surgery in young infants and children are now widely recognized. Benefits include avoiding the morbidity associated with prolonged intubation and the consequences of sedation and positive pressure ventilation in the setting of altered cardiopulmonary physiology. Our practice of tracheal extubation of young infants in the operating room following cardiac surgery has evolved and new challenges in the arena of postoperative sedation and pain management have appeared. DESIGN: Review our institutional outcomes associated with early tracheal extubation following congenital cardiac surgery. PATIENTS: Inclusion criteria included all children less than 1 year old who underwent congenital cardiac surgery between October 1, 2010, and October 24, 2013. MEASUREMENTS AND MAIN RESULTS: A total of 416 patients less than 1 year old were included. Of the 416 patients, 234 underwent tracheal extubation in the operating room (56%) with 25 requiring reintubation (10.7%), either immediately or following admission to the cardiothoracic ICU. Of the 25 patients extubated in the operating room who required reintubation, 22 failed within 24 hours of cardiothoracic ICU admission; 10 failures were directly related to narcotic doses that resulted in respiratory depression. CONCLUSIONS: As a result of this review, we have instituted changes in our cardiothoracic ICU postoperative care plans. We have developed a neonatal delirium score, and have adopted the "Kangaroo Care" approach that was first popularized in neonatal ICUs. This provision allows for the early parental holding of infants following admission to the cardiothoracic ICU and allows for appropriately selected parents to sleep in the same beds alongside their postoperative children.

Results of a Feeding Protocol in Patients Undergoing the Hybrid Procedure.

Neonates with single-ventricle physiology are at increased risk of developing gastrointestinal morbidities. Feeding protocols in this patient population have been shown to decrease feeding complications after the Norwood procedure, but no data exist to determine the effectiveness of a feeding protocol in patients undergoing the hybrid procedure. Goal of this study was to examine the impact of a standardized feeding protocol on the incidence of overall postoperative gastrointestinal morbidity after the hybrid procedure. Retrospective chart review was performed on neonates undergoing the hybrid procedure. Neonates were divided into two groups, pre-feeding protocol (pre-FP), which encompassed the years 2002-2008, and post-feeding protocol (post-FP), which encompassed the years 2011-2014. Preoperative, operative, and postoperative data were collected. T test or Fisher's exact test was used for analysis. p < 0.05 was considered significant. Seventy-three neonates were in the pre-FP and 52 neonates were in the post-FP. There were no significant differences between the pre-FP and the post-FP in cardiac diagnosis (62 HLHS, 11 other vs. 39 HLHS, 13 other, respectively). Pre-FP underwent hybrid procedure later than the post-FP (9.1 ± 5.8 vs. 5.7 ± 3.4 days, respectively, p < 0.01) and achieved full enteral feeds earlier than the post-FP (3.2 + 2.9 vs. 7.8 + 3.9 days, respectively, p < 0.01). The incidence of necrotizing enterocolitis was higher in the pre-FP versus post-FP [11.0 % (8/65) vs. 5.8 % (3/49), respectively, p = 0.36]. Though not significant, the incidence of necrotizing enterocolitis decreased by almost 50 % after initiating a feeding protocol in patients undergoing the hybrid procedure. This is consistent with previous studies showing beneficial results of a feeding protocol in this complex patient population.

Arrhythmias After Stage I Hybrid Palliation in Single-Ventricle Patients.

The hybrid procedure is an alternative palliative strategy for patients with single-ventricle physiology. No data exist documenting the incidence of arrhythmias after the hybrid procedure. Goal of this study was to determine the incidence and type of arrhythmias in patients undergoing the hybrid procedure. A retrospective chart review was performed including all patients undergoing the hybrid procedure between January of 2010 through December of 2013. Sixty-five patients underwent the hybrid procedure during this time period (43 HLHS, 22 other). Average gestational age at admission was 37.7 weeks. Average age at time of procedure was 7.6 days. Five patients had documented arrhythmias (7.7 %). Four were supraventricular tachycardias, and 1 was a sinus bradycardia. One patient with arrhythmia died during hospitalization, and another patient with arrhythmia died during the interstage period. Hybrid palliation for patients with single-ventricle physiology has a low incidence of arrhythmias. In this cohort of patients, arrhythmias did not contribute to mortality. There was a trend toward association between arrhythmias and longer total length of hospital stay.

Arrhythmias Following Comprehensive Stage II Surgical Palliation in Single Ventricle Patients.

Post-operative arrhythmias are common in pediatric patients following cardiac surgery. Following hybrid palliation in single ventricle patients, a comprehensive stage II palliation is performed. The incidence of arrhythmias in patients following comprehensive stage II palliation is unknown. The purpose of this study is to determine the incidence of arrhythmias following comprehensive stage II palliation. A single-center retrospective chart review was performed on all single ventricle patients undergoing a comprehensive stage II palliation from January 2010 to May 2014. Pre-operative, operative, and post-operative data were collected. A clinically significant arrhythmia was defined as an arrhythmia which led to cardiopulmonary resuscitation or required treatment with either pacing or antiarrhythmic medication. Statistical analysis was performed with Wilcoxon rank-sum test and Fisher's exact test with p < 0.05 significant. Forty-eight single ventricle patients were reviewed (32 hypoplastic left heart syndrome, 16 other single ventricle variants). Age at surgery was 185 ± 56 days. Cardiopulmonary bypass time was 259 ± 45 min. Average vasoactive-inotropic score was 5.97 ± 7.58. Six patients (12.5 %) had clinically significant arrhythmias: four sinus bradycardia, one 2:1 atrioventricular block, and one slow junctional rhythm. No tachyarrhythmias were documented for this patient population. Presence of arrhythmia was associated with elevated lactate (p = 0.04) and cardiac arrest (p = 0.002). Following comprehensive stage II palliation, single ventricle patients are at low risk for development of tachyarrhythmias. The most frequent arrhythmia seen in these patients was sinus bradycardia associated with respiratory compromise.