Characterization of the Placenta in the Newborn with Congenital Heart Disease: Distinctions Based on Type of Cardiac Malformation.

The placenta is a complex organ that influences prenatal growth and development, and through fetal programming impacts postnatal health and well-being lifelong. Little information exists on placental pathology in the presence of congenital heart disease (CHD). Our objective is to characterize the placenta in CHD and investigate for distinctions based on type of malformation present. Placental pathology from singleton neonates prenatally diagnosed and delivered at > 37 weeks gestation was analyzed. Placental findings of absolute weight, placental weight-to-newborn birth weight ratio, chorangiosis, villus maturity, thrombosis, and infarction were recorded and analyzed based on four physiological categories of CHD: (1) single ventricle-aortic obstruction, (2) single ventricle-pulmonic obstruction, (3) two-ventricle anomalies, and (4) transposition of the great arteries (TGA). Associations between fetal Doppler assessments of middle cerebral/umbilical arterial flow and placental findings were investigated. A total of 120 cases of complex CHD were analyzed. Overall placental-to-birth weight ratios were < 10th percentile for 77% and < 3rd percentile for 49% with abnormalities of chorangiosis (18%), hypomature villi (15%), thrombosis (41%), and infarction (17%) common. There was no association between fetal Doppler flow measures and placental abnormalities. Newborns with TGA had the greatest degree of placental abnormality. Placentas of newborns with CHD are smaller than expected and manifest a number of vascular abnormalities, with TGA most prominent. Fetal Doppler does not correlate with these abnormalities. Studies investigating the relationship between placental abnormalities and postnatal outcomes may offer insight into the fetal origins of outcome variability in CHD.

The Burdens of Offering: Ethical and Practical Considerations.

We reflect upon highlights of a facilitated panel discussion from the 2016 Pediatric Cardiac Intensive Care Society Meeting. The session was designed to explore challenges, share practical clinical experiences, and review ethical underpinnings surrounding decisions to offer intensive, invasive therapies to patients who have a poor prognosis for survival or are likely to be burdened with multiple residual comorbidities if survival is achieved. The discussion panel was representative of a variety of disciplines including pediatric cardiology, cardiac intensive care, nursing, and cardiovascular surgery as well as different health-care delivery systems. Key issues discussed included patient's best interests, physician obligations, moral distress, and communication in the context of decisions about providing therapy for patients with a poor prognosis.

The Effect of Skin Pigmentation on the Accuracy of Pulse Oximetry in Infants with Hypoxemia.

To compare pulse oximetry measurement bias between infants with hypoxemia with either dark skin or light skin with Masimo Radical 7 and Nellcor Oximax. There was no significant difference in systematic bias based on skin pigment for either oximeter.

Defining pediatric inpatient cardiology care delivery models: A survey of pediatric cardiology programs in the USA and Canada.

BACKGROUND: The treatment of children with cardiac disease is one of the most prevalent and costly pediatric inpatient conditions. The design of inpatient medical services for children admitted to and discharged from noncritical cardiology care units, however, is undefined. North American Pediatric Cardiology Programs were surveyed to define noncritical cardiac care unit models in current practice. METHOD: An online survey that explored institutional and functional domains for noncritical cardiac care unit was crafted. All questions were multi-choice with comment boxes for further explanation. The survey was distributed by email four times over a 5-month period. RESULTS: Most programs (n = 45, 60%) exist in free-standing children's hospitals. Most programs cohort cardiac patients on noncritical cardiac care units that are restricted to cardiac patients in 39 (54%) programs or restricted to cardiac and other subspecialty patients in 23 (32%) programs. The most common frontline providers are categorical pediatric residents (n = 58, 81%) and nurse practitioners (n = 48, 67%). However, nurse practitioners are autonomous providers in only 21 (29%) programs. Only 33% of programs use a postoperative fast-track protocol. When transitioning care to referring physicians, most programs (n = 53, 72%) use facsimile to deliver pertinent patient information. Twenty-two programs (31%) use email to transition care, and eighteen (25%) programs use verbal communication. CONCLUSION: Most programs exist in free-standing children's hospitals in which the noncritical cardiac care units are in some form restricted to cardiac patients. While nurse practitioners are used on most noncritical cardiac care units, they rarely function as autonomous providers. The majority of programs in this survey do not incorporate any postoperative fast-track protocols in their practice. Given the current era of focused handoffs within hospital systems, relatively few programs utilize verbal handoffs to the referring pediatric cardiologist/pediatrician.

Complex Surgical Repair of a Flail Tricuspid Valve After Chest Wall Trauma in a Pediatric Patient.

Traumatic injury to the tricuspid valve can result from severe nonpenetrating chest wall trauma. We present the case of an initially asymptomatic 11-year-old girl who was kicked in the chest by a horse. The trauma resulted in avulsed papillary muscles, ruptured chordae, and right heart failure. She underwent early tricuspid valve reconstruction and annuloplasty. We advocate for routine use of echocardiography to assess for traumatic injury to intracardiac structures. In addition, we believe that early surgical intervention may prevent right-sided heart dysfunction, atrial arrhythmias, and the need for valve replacement.

Outcomes of Single-Ventricle Patients Supported With Extracorporeal Membrane Oxygenation.

OBJECTIVES: Extracorporeal membrane oxygenation is often used in children with single-ventricle anomalies. We aimed to describe extracorporeal membrane oxygenation use in single-ventricle patients to test the hypothesis that despite increasing prevalence, mortality has not improved and overall burden measure by hospital charges and length of stay have increased. DESIGN: Retrospective analysis of the Healthcare Cost and Utilization Project Kids' Inpatient Database was performed with sample weighting to generate national estimates. PATIENTS: Pediatric patients (age ≤ 20) with a diagnosis of single ventricle heart disease requiring extracorporeal membrane oxygenation support from 2000 to 2009. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Seven hundred one children (95% CI, 559-943) with single ventricle were supported with extracorporeal membrane oxygenation in the reporting period. Mortality was 57% and did not improve over time (2000 = 52%, 2003 = 63%, 2006 = 57%, and 2009 = 55%; p = 0.66). Single-ventricle patients who required extracorporeal membrane oxygenation were more likely to have had a cardiac procedure (90% vs 46%; p < 0.001), a diagnosis of arrhythmia (22% vs 13%; p < 0.001), cerebrovascular or neurologic insult (9% vs 1%; p < 0.001), heart failure (24% vs 12%; p < 0.001), acute renal failure (28% vs 3%; p < 0.001), or sepsis (28% vs 8%; p < 0.001). By multivariable analysis, acute renal failure was a risk factor for mortality (adjusted odds ratio, 3.12; 95% CI, 1.95-4.98; p < 0.001). The length of stay for single-ventricle patients with extracorporeal membrane oxygenation increased from 25.2 days in 2000 to 55.6 days in 2009 (p < 0.001). Total inflation-adjusted charges increased from $358,021 (95% CI, $278,658-439,765) in 2000 to $732,349 (95% CI, $671,781-792,917) in 2009 (p < 0.001). CONCLUSIONS: Extracorporeal membrane oxygenation support is uncommon with single-ventricle admissions occurring in 2.3% of all hospitalizations. Among those patients, the mortality rate was 57% with no change over time. Acute renal failure was an independent risk factor for mortality during hospitalization. In addition, length of stay for these patients increased and hospital charges doubled. Further studies are needed to determine suitability and cost-effectiveness of extracorporeal membrane oxygenation in single-ventricle patients.

Unplanned Readmission to the Pediatric Cardiac Intensive Care Unit: Prevalence, Outcomes, and Risk Factors.

BACKGROUND: Factors leading to cardiac intensive care unit (CICU) readmission and the impact on mortality have yet to be well delineated. We sought to define the prevalence and outcome for unscheduled CICU readmission. Secondary objectives were to identify indications and risk factors for unscheduled CICU readmission. METHODS: Retrospective analysis of prospectively collected registry data at a tertiary care children's hospital. Pediatric and adult patients with congenital and acquired heart disease who survived to initial CICU discharge were included. Patients with unexpected return to the CICU for acute change in clinical status were defined as unscheduled readmissions. RESULTS: Of the 645 discharges that met inclusion criteria, 37 resulted in unplanned readmission to the CICU. Patients requiring unscheduled readmission had higher mortality rates (16.2% vs 0.5%, P < .0001). Cardiac symptoms were the most common reason for readmission. On multivariate analysis, genetic anomaly (P = .001) and longer length of stay (LOS) during the index CICU admission (P = .01) were independently associated with readmission. For surgical patients, genetic anomaly (P = .001), single-ventricle anatomy (P = .05), and longer surgical support time (P < .001) were independently associated with readmission. CONCLUSION: Unscheduled readmission to the CICU within the same hospitalization was uncommon but associated with a higher mortality rate. Genetic anomaly and longer initial LOS were important risk factors for the entire cohort. Single-ventricle anatomy and longer intraoperative course were risk factors for surgical readmissions.

The Use of Pediatric Ventricular Assist Devices in Children's Hospitals From 2000 to 2010: Morbidity, Mortality, and Hospital Charges.

OBJECTIVE: The use of ventricular assist devices has increased dramatically in adult heart failure patients. However, the overall use, outcome, comorbidities, and resource utilization of ventricular assist devices in pediatric patients have not been well described. We sought to demonstrate that the use of ventricular assist devices in pediatric patients has increased over time and that mortality has decreased. DESIGN: A retrospective study of the Pediatric Health Information System database was performed for patients 20 years old or younger undergoing ventricular assist device placement from 2000 to 2010. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Four hundred seventy-five pediatric patients were implanted with ventricular assist devices during the study period: 69 in 2000-2003 (era 1), 135 in 2004-2006 (era 2), and 271 in 2007-2010 (era 3). Median age at ventricular assist device implantation was 6.0 years (interquartile range, 0.5-13.8), and the proportion of children who were 1-12 years old increased from 29% in era 1 to 47% in era 3 (p = 0.002). The majority of patients had a diagnosis of cardiomyopathy; this increased from 52% in era 1 to 72% in era 3 (p = 0.003). Comorbidities included arrhythmias (48%), pulmonary hypertension (16%), acute renal failure (34%), cerebrovascular disease (28%), and sepsis/systemic inflammatory response syndrome (34%). Two hundred forty-seven patients (52%) underwent heart transplantation and 327 (69%) survived to hospital discharge. Hospital mortality decreased from 42% in era 1 to 25% in era 3 (p = 0.004). Median hospital length of stay increased (37 d [interquartile range, 12-64 d] in era 1 vs 69 d [interquartile range, 35-130] in era 3; p < 0.001) and median adjusted hospital charges increased ($630,630 [interquartile range, $227,052-$853,318] in era 1 vs $1,577,983 [interquartile range, $874,463-$2,280,435] in era 3; p < 0.001). Factors associated with increased mortality include age less than 1 year (odds ratio, 2.04; 95% CI, 1.01-3.83), acute renal failure (odds ratio, 2.1; 95% CI, 1.26-3.65), cerebrovascular disease (odds ratio, 2.1; 95% CI, 1.25-3.62), and extracorporeal membrane oxygenation (odds ratio, 3.16; 95% CI, 1.79-5.60). Ventricular assist device placement in era 3 (odds ratio, 0.3; 95% CI, 0.15-0.57) and a diagnosis of cardiomyopathy (odds ratio, 0.5; 95% CI, 0.32-0.84), were associated with decreased mortality. Large-volume centers had lower mortality (odds ratio, 0.55; 95% CI, 0.34-0.88), lower use of extracorporeal membrane oxygenation, and higher charges. CONCLUSIONS: The use of ventricular assist devices and survival after ventricular assist device placement in pediatric patients have increased over time, with a concomitant increase in resource utilization. Age under 1 year, certain noncardiac morbidities, and the use of extracorporeal membrane oxygenation are associated with worse outcomes. Lower mortality was seen at larger volume ventricular assist device centers.

Characterization of extracorporeal membrane oxygenation for pediatric cardiac arrest in the United States: analysis of the kids' inpatient database.

To characterize the overall use, cost, and outcomes of extracorporeal membrane oxygenation (ECMO) as an adjunct to cardiopulmonary resuscitation (CPR) among hospitalized infants and children in the United States, retrospective analysis of the 2000, 2003, and 2006 Kids' Inpatient Database (KID) was performed. All CPR episodes were identified; E-CPR was defined as ECMO used on the same day as CPR. Channeling bias was decreased by developing propensity scores representing the likelihood of requiring E-CPR. Univariable, multivariable, and propensity-matched analyses were performed to characterize the influence of E-CPR on survival. There were 8.6 million pediatric hospitalizations and 9,000 CPR events identified in the database. ECMO was used in 82 (0.9 %) of the CPR events. Median hospital charges for E-CPR survivors were $310,824 [interquartile range (IQR) 263,344-477,239] compared with $147,817 (IQR 62,943-317,553) for propensity-matched conventional CPR (C-CPR) survivors. Median LOS for E-CPR survivors (31 days) was considerably greater than that of propensity-matched C-CPR survivors (18 days). Unadjusted E-CPR mortality was higher relative to C-CPR (65.9 vs. 50.9 %; OR 1.9, 95 % confidence interval 1.2-2.9). Neither multivariable analysis nor propensity-matched analysis identified a significant difference in survival between groups. E-CPR is infrequently used for pediatric in-hospital cardiac arrest. Median LOS and charges are considerably greater for E-CPR survivors with C-CPR survivors. In this retrospective administrative database analysis, E-CPR did not significantly influence survival. Further study is needed to improve outcomes and to identify patients most likely to benefit from this resource-intensive therapy.

Risk factors for postoperative acute kidney injury in pediatric cardiac surgery patients receiving angiotensin-converting enzyme inhibitors.

BACKGROUND: Angiotensin-converting enzyme inhibitor therapy is often initiated in pediatric patients who have had cardiac surgery. Acute kidney injury can occur in patients secondary to angiotensin-converting enzyme inhibitor initiation. Risk factors for acute kidney injury after angiotensin-converting enzyme inhibitor initiation have yet to be defined in postoperative pediatric cardiac patients. OBJECTIVES: To identify the frequency of acute kidney injury in patients receiving angiotensin-converting enzyme inhibitor therapy in postoperative pediatric cardiac surgical patients and to identify risk factors for acute kidney injury in this patient population. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The pharmacy and surgery databases were used to identify all patients <18 yrs of age who received angiotensin-converting enzyme inhibitor therapy after cardiac surgery at our institution from January 2006 to December 2007. Patients who did not have a baseline serum creatinine and at least one serum creatinine obtained after angiotensin-converting enzyme inhibitor initiation were excluded. Data collection included demographic information and cardiac pathophysiology/surgery, diuretic and/or nephrotoxic medication use, and angiotensin-converting enzyme inhibitor characteristics and initiation date. Baseline, daily, and maximum serum creatinine values were collected. Acute kidney injury was defined as the maximum change in pediatric-modified RIFLE (Risk, Injury, Failure, Loss, End-stage) acute kidney injury criteria within 48 hrs of initiation or increase in dose of angiotensin-converting enzyme inhibitor. Descriptive statistics were used to characterize the patient population, and a multivariate logistic regression model was developed to identify independent predictors of angiotensin-converting enzyme inhibitor-associated acute kidney injury. The study included 415 patient admissions (386 patients), 57% (n = 239) being male and infants (31 days to 2 yrs) being the most common age group. A functional single ventricle was present in 46% of the patients. Enalapril was initiated in 60% (n = 250) and captopril in 40% (n = 165) of patient admissions. Acute kidney injury occurred in 21% (n = 88) of patients initiated on an angiotensin-converting enzyme inhibitor (pediatric-modified RIFLE categories: R = 15%, I = 3%, F = 4%). Logistic regression identified cyanosis, coadministration of furosemide, and baseline estimated creatinine clearance as independent risk factors for any degree of angiotensin-converting enzyme inhibitor-associated acute kidney injury (p < .05). The hospital lengths of stay of patients with angiotensin-converting enzyme inhibitor-associated acute kidney injury (median 12 days, range 4-298 days) were greater compared to those of patients without angiotensin-converting enzyme inhibitor-associated acute kidney injury (median 10 days, range 3-199 days, p < .05). CONCLUSIONS: Initiation of angiotensin-converting enzyme inhibitor after cardiac surgery in pediatric patients may result in acute kidney injury. The presence of cyanosis and coadministration of furosemide are independent risk factors for acute kidney injury in patients receiving angiotensin-converting enzyme inhibitor.

Safety and efficacy of intravenous labetalol for hypertensive crisis in infants and small children.

OBJECTIVE: To determine the efficacy and safety of labetalol for hypertensive crisis in children ≤ 24 months of age. DESIGN: Retrospective chart review. Statistical analysis utilized analysis of variance for continuous data, chi-square tests for nominal data, and linear regression. SETTING: A 737-bed pediatric teaching institution. PATIENTS: Twenty-seven patients ≤ 24 months of age were treated with 37 intravenous infusions of labetalol, nicardipine, or nitroprusside for hypertensive crisis or hypertensive urgency. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The primary end point consisted of time to 20% reduction in systolic blood pressure. Primary safety end points measured the prevalence of deleterious effects of labetalol. Continuous infusion of labetalol reduced mean systolic blood pressure by at least 20% in < 8 hrs. This effect was similar to nicardipine and nitroprusside infusions. The reported side effects were similar in each group. Patients receiving labetalol and presenting with ischemic or traumatic brain injury were likely to develop hypotension requiring infusion discontinuation. CONCLUSIONS: Continuous intravenous labetalol infusion is efficacious for treatment of hypertensive crisis in children ≤ 24 months of age. Aside from patients presenting with ischemic or traumatic brain injury, labetalol was safe to use in this population for hypertensive emergencies and had a satisfactory adverse effect profile. Labetalol may reach dose saturation at a much lower dose in young children in comparison to adults. Clinicians should use caution when initiating labetalol infusions in young patients with brain injury.

Feeding, growth, and nutrition in children with congenitally malformed hearts.

In the United States of America, approximately 40,000 infants are born annually with congenitally malformed hearts. Children with defects that require complex surgical palliation, or definitive repair, face many challenges in achieving optimal short-term and long-term growth. The presence of associated chromosomal abnormalities, cyanosis, and cardiac failure adds to the complexity and challenge. In this review, we address three themes related to feeding, growth, and nutrition of infants after neonatal cardiac surgery: nutritional challenges after chylothorax; breastfeeding after surgery; and the challenges of feeding after discharge. Chylothorax is a rare complication following cardiothoracic surgery in children. Children with chylothorax have nutritional depletion secondary to protein losses in chylous fluid, hypovolaemia, and electrolyte losses. In spite of the evidence supporting the use of human milk and breastfeeding in preterm infants, barriers to its use appear to persist in infants with critical cardiac disease. Yet, human milk is the preferred form of nutrition for well, preterm, or ill infants. It is well documented that after complex neonatal cardiac surgery medical teams and families struggle with infant feeding problems. Parents have described feeding their children as difficult, time consuming, and anxiety producing. Medical complications such as chylothorax, limited access to human milk, and parental concerns and stress about feeding are but three of the myriad of factors that may contribute to poor outcomes regarding nutrition and growth. Compelling evidence exists that this multi-factorial problem must be addressed with both physiological and behavioural strategies.

Triiodothyronine Supplementation in Infants and Children Undergoing Cardiopulmonary Bypass (TRICC): a multicenter placebo-controlled randomized trial: age analysis.

BACKGROUND: Triiodothyronine levels decrease in infants and children after cardiopulmonary bypass. We tested the primary hypothesis that triiodothyronine (T3) repletion is safe in this population and produces improvements in postoperative clinical outcome. METHODS AND RESULTS: The TRICC study was a prospective, multicenter, double-blind, randomized, placebo-controlled trial in children younger than 2 years old undergoing heart surgery with cardiopulmonary bypass. Enrollment was stratified by surgical diagnosis. Time to extubation (TTE) was the primary outcome. Patients received intravenous T3 as Triostat (n=98) or placebo (n=95), and data were analyzed using Cox proportional hazards. Overall, TTE was similar between groups. There were no differences in adverse event rates, including arrhythmia. Prespecified analyses showed a significant interaction between age and treatment (P=0.0012). For patients younger than 5 months, the hazard ratio (chance of extubation) for Triostat was 1.72. (P=0.0216). Placebo median TTE was 98 hours with 95% confidence interval (CI) of 71 to 142 compared to Triostat TTE at 55 hours with CI of 44 to 92. TTE shortening corresponded to a reduction in inotropic agent use and improvement in cardiac function. For children 5 months of age, or older, Triostat produced a significant delay in median TTE: 16 hours (CI, 7-22) for placebo and 20 hours (CI, 16-45) for Triostat and (hazard ratio, 0.60; P=0.0220). CONCLUSIONS: T3 supplementation is safe. Analyses using age stratification indicate that T3 supplementation provides clinical advantages in patients younger than 5 months and no benefit for those older than 5 months. Clinical Trial Registration-URL: http://www.clinicaltrials.gov. Unique identifier: NCT00027417.

Gentamicin dosing for pediatric patients with congenital heart disease.

Pediatric patients with congenital heart disease can have physiologies that alter the pharmacokinetics of certain medications, such as aminoglycosides. Currently, no literature describes the appropriate dosing of aminoglycoside antibiotics for infants and children with congenital heart disease. Patients were identified through the pharmacy and laboratory computer systems. Patients were included in the study if they were younger than 18 years, received gentamicin on the acute-care (nonintensive care) cardiology floor at the authors' institution, had structural congenital heart disease, and had a peak and trough level obtained at about the third dose or later. Cohort achievement of therapeutic peak and trough concentrations based on standard dosing guidelines was evaluated. The inclusion criteria were met by 48 patients (31 boys). Eight patients (17%) had baseline cyanosis. Cardiac surgery was performed for 23 patients (48%) during the same admission at which aminoglycoside therapy was initiated. A total of 27 patients (56%) received at least one other nephrotoxic medication at the time of aminoglycoside therapy. Six patients had undetectable serum trough levels. A therapeutic peak concentration was not achieved by 16.7% of the cohort, and 7.1% of the cohort did not achieve a therapeutic trough concentration. Pediatric patients with congenital heart disease may require alterations in gentamicin dosing. Close pharmacokinetic monitoring of aminoglycoside therapy for these patients is warranted to ensure attainment of goal concentrations.

Anomalous left coronary artery from the pulmonary artery in a preterm infant: presentation after ligation of ductus arteriosus.

Surgical or medical closure of a patent ductus arteriosus (PDA) is a very common practice in premature infants in the neonatal intensive care unit, but often the coronary arteries are not delineated prior to closure. In this report, a 32-week gestational age premature infant who underwent surgical ligation of a PDA developed new-onset left ventricular systolic dysfunction. A repeat echocardiogram noted an anomalous left coronary artery from the pulmonary artery. Successful left coronary artery reimplantation with rapid normalization of left ventricular systolic function occurred. As previously not reported, ligation of a PDA in a premature infant can unmask anomalous left coronary artery from the pulmonary artery. Surgical left coronary artery reimplantation is the mainstay of therapy and should be performed to establish normal coronary blood flow, even in a premature, low birth weight infant.

Automated external defibrillators and simulated in-hospital cardiac arrests.

OBJECTIVE: To test the hypothesis that pediatric residents would have shorter time to attempted defibrillation using automated external defibrillators (AEDs) compared with manual defibrillators (MDs). STUDY DESIGN: A prospective, randomized, controlled trial of AEDs versus MDs was performed. Pediatric residents responded to a simulated in-hospital ventricular fibrillation cardiac arrest and were randomized to using either an AED or MD. The primary end point was time to attempted defibrillation. RESULTS: Sixty residents, 21 (35%) interns, were randomized to 2 groups (AED = 30, MD = 30). Residents randomized to the AED group had a significantly shorter time to attempted defibrillation [median, 60 seconds (interquartile range, 53 to 71 seconds)] compared with those randomized to the MD group [median, 103 seconds (interquartile range, 68 to 288 seconds)] (P < .001). All residents in the AED group attempted defibrillation at <5 minutes compared with 23 (77%) in the MD group (P = .01). CONCLUSIONS: AEDs improve the time to attempted defibrillation by pediatric residents in simulated cardiac arrests. Further studies are needed to help determine the role of AEDs in pediatric in-hospital cardiac arrests.