Managing bilateral discontinuous pulmonary arteries of ductal origin in single ventricle anatomy.

BACKGROUND: We describe outcomes and management strategies for single ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus. METHODS: We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995-2023, excluding those with biventricular repair. RESULTS: Median age at centralization was 9 days (range 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n=20, 91%; 2 following bilateral ductal stents) or bidirectional cavopulmonary connection (n=2, 9%) via pericardial roll (n=14, 64%), patch-augmented direct anastomosis (n=7, 32%), and interposition graft (n=1, 5%) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n=11, 50%) was associated with significantly inferior survival (P=.01). Five patients (23%) died at a median of 59 (6-257) days post-centralization, all with non-cardiac TAPVC. At latest follow-up for 17 survivors (median 13.5 [0.5-25.1] years post-centralization), 12 completed Fontan, 4 completed second stage palliation, and 1 was transplanted prior to second stage palliation. Fourteen patients (64%) required PA reintervention, including 3 with reoperations independent of staged palliation. Baseline to pre-second stage echocardiography demonstrated branch PA growth with significantly increased diameters (left P=.0006, right P=.0002); z-scores significantly increased for right (P=.004) but not left (P=.11). CONCLUSIONS: Successful single ventricle palliation is possible, though high-risk, for patients with bilateral discontinuous ductal PAs. Early post-centralization mortality remains substantial, particularly with associated non-cardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation.

Outcomes in adults with congenital heart disease and heterotaxy syndrome: A single-center experience.

BACKGROUND: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with congenital heart disease (CHD). Little is known about the adult HS population with CHD. OBJECTIVE: To describe the outcomes and sociodemographics of the adult CHD population with HS. METHODS: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed. RESULTS: Sixty-two patients met inclusion criteria. Median age was 22.7 [IQR 19.6-30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap in care of >3 years. Median follow-up time in adulthood was 2.9 [IQR 1.3-8.2] years. Forty-three (69%) of patients had single ventricle heart disease, 31 (71%) of whom completed Fontan circulation. A total of 36 interventions occurred in 24 patients which included 16 cardiac catherization interventions, 13 electrophysiology-related procedures, and 18 surgical procedures including 2 orthotopic heart transplants. The median age for death or heart transplant was 45.3 (95%CI 34.3-56.1) years. Heart failure-free survival was 80.8 ± 5.2%, 58.7 ± 11.0%, and 31.1 ± 15.7% at 20, 30, and 40 years old, respectively. Cerebrovascular accident-free survival was 84.3 ± 5.1%, 54.2 ± 11.3%, and 40.6 ± 14.5% at 20, 30, and 40 years old, respectively. Tachyarrhythmia-free survival was 54.0 ± 7.1%, 29.2 ± 8.3%, and 19.5 ± 9.7% at 20, 30, and 40 years old and bradyarrhythmia-free survival was 66.0 ± 6.3%, 41.7 ± 9.4%, and 33.4 ± 10.6% at ages 20, 30, and 40 years, respectively. CONCLUSIONS: At a tertiary referral center, adult patients with CHD and HS have high rates of comorbidities and early death or heart transplant. Longitudinal surveillance and further exploration into factors associated with improved survival in this population are warranted.

Contemporary Outcomes of the Fontan Operation: A Large Single-Institution Cohort.

BACKGROUND: The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short- and midterm outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure. METHODS: All patients undergoing a Fontan operation between 1995 and 2016 were included. Failure was defined as death, transplantation, Fontan takedown or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic failure (after hospital discharge or 30 days postoperatively, or both). RESULTS: The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, nonfenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n = 3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom from failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (odds ratio, 0.30; 95% confidence interval [CI], 0.11 to 0.87). Independent risk factors for chronic failure included genetic syndrome (hazard ratio [HR], 2.54; 95% CI, 1.11 to 5.83), ventricular dysfunction (HR, 3.86; 95% CI, 1.81 to 8.24), cardiopulmonary bypass time in 30-minute intervals (HR, 1.242; 95% CI, 1.100 to 1.402), and persistent pleural effusions (HR, 4.26; 95% CI, 2.25 to 8.07). Moderate or severe atrioventricular valve regurgitation (HR, 2.61; 95% CI, 1.13 to 6.02) and cardiopulmonary bypass time (HR, 1.22; 95% CI, 1.03 to 1.45) were associated with reduced long-term transplant-free survival. CONCLUSIONS: Contemporary midterm outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood.

Genetic architecture of laterality defects revealed by whole exome sequencing.

Aberrant left-right patterning in the developing human embryo can lead to a broad spectrum of congenital malformations. The causes of most laterality defects are not known, with variants in established genes accounting for <20% of cases. We sought to characterize the genetic spectrum of these conditions by performing whole-exome sequencing of 323 unrelated laterality cases. We investigated the role of rare, predicted-damaging variation in 1726 putative laterality candidate genes derived from model organisms, pathway analyses, and human phenotypes. We also evaluated the contribution of homo/hemizygous exon deletions and gene-based burden of rare variation. A total of 28 candidate variants (26 rare predicted-damaging variants and 2 hemizygous deletions) were identified, including variants in genes known to cause heterotaxy and primary ciliary dyskinesia (ACVR2B, NODAL, ZIC3, DNAI1, DNAH5, HYDIN, MMP21), and genes without a human phenotype association, but with prior evidence for a role in embryonic laterality or cardiac development. Sanger validation of the latter variants in probands and their parents revealed no de novo variants, but apparent transmitted heterozygous (ROCK2, ISL1, SMAD2), and hemizygous (RAI2, RIPPLY1) variant patterns. Collectively, these variants account for 7.1% of our study subjects. We also observe evidence for an excess burden of rare, predicted loss-of-function variation in PXDNL and BMS1- two genes relevant to the broader laterality phenotype. These findings highlight potential new genes in the development of laterality defects, and suggest extensive locus heterogeneity and complex genetic models in this class of birth defects.

Pulsatile Glenn as long-term palliation for single ventricle physiology patients.

OBJECTIVE: There are limited studies analyzing pulsatile Glenn as a long-term palliation strategy for single ventricle patients. This study sought to determine their outcomes at a single institution. DESIGN: A retrospective review was performed. SETTING: Study performed at a single pediatric hospital. PATIENTS: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included. OUTCOME MEASURES: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk factors were assessed by Cox multivariable competing risk analyses. RESULTS: Seventy-eight patients underwent pulsatile Glenn at age 9 months (interquartile range, 5-14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time-to-palliation of 4 years (interquartile range, 3-5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five- and 10-year transplant-free survival were 91% and 84%, respectively. At a median follow-up of 6 years (interquartile range, 2-8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3-11], oxygen saturation 83% ± 4%). Preoperative moderate-severe atrioventricular valve regurgitation (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80-33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08-6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for covariates. Reaching further palliation was less likely in patients with preoperative moderate-severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08-0.59; P =.002). CONCLUSION: Pulsatile Glenn can be an effective tool to be used in challenging circumstances, these patients can have a favorable long-term prognosis without reducing their suitability for further palliation.

Heterotaxy syndrome and associated arrhythmias in pediatric patients.

BACKGROUND: Heterotaxy syndrome (HS) is a rare disorder with complex anatomy involving misarrangements of the cardiac conduction system. Arrhythmias may be related to anatomic variations and contribute to morbidity. OBJECTIVE: The purpose of this study was to investigate the associations between arrhythmias, anatomy, and outcomes in a large HS cohort. METHODS: A single-center retrospective review of patients ≤21 years of age diagnosed with HS was performed. RESULTS: A total of 337 patients were included in the study. During median follow-up of 7 years (interquartile range 2-16 years), 129 patients (38%) had ≥1 clinically significant rhythm disturbance: tachyarrhythmias in 75 (22%), bradyarrhythmias in 29 (9%), and both in 25 (7%). Factors associated with tachyarrhythmia by multivariate analysis were at least moderate atrioventricular valve regurgitation (hazard ratio [HR] 1.66; 95% confidence interval [CI] 1.11-2.50), single ventricle anatomy (HR 2.30; 95% CI 1.09-4.85), and pulmonary venous obstruction (HR 2.33; 95% CI 1.45-3.76). Isomerism subtype was not associated with tachyarrhythmias. In adjusted and unadjusted analyses, bradyarrhythmias (symptomatic sinus/atrial bradycardia and high-grade or complete heart block) were associated with left atrial isomerism (LAI) compared to right atrial isomerism (HR 7.12; 95% CI 3.01-16.9). The overall transplant-free survival of the cohort was 66%. Tachyarrhythmias, but not bradyarrhythmias, were associated with mortality or need for transplant (HR 2.24; 95% CI 1.45-3.46). CONCLUSION: Clinically significant arrhythmias are common in HS. Although bradyarrhythmias are associated with LAI, tachyarrhythmia occurrence may depend more on hemodynamic and anatomic factors than isomerism subtype. Tachyarrhythmias, but not bradyarrhythmias, are associated with death or need for transplant.

Evaluating a management strategy for malrotation in heterotaxy patients.

BACKGROUND: There is disagreement over the management of malrotation in children with heterotaxy and congenital heart disease (CHD). We sought to evaluate the outcomes of management with a Ladd procedure compared to observation in this cohort of patients. METHODS: We performed a retrospective review of CHD children with heterotaxy and malrotation identified on radiographs treated from 8/2002 until 4/2014. Primary outcomes evaluated were readmission for small bowel obstruction (SBO) or volvulus. RESULTS: We identified 88 patients with cardiac heterotaxy and malrotation. Sixty-eight (77%) had a Ladd procedure. Eighteen (26%) of the 68 had abdominal symptoms, but only one had an underlying volvulus without ischemia. Twenty (23%) patients died of cardiopulmonary complications, 8 before and 12 after the Ladd procedure. Sixty-eight patients survived to the review date (median: 5years): 56 in the Ladd cohort and 12 observed. Six of the 56 (11%) surviving Ladd patients were readmitted to hospital with an SBO, and 2 required surgical intervention. None of the 12 surviving nonoperative patients developed a volvulus. CONCLUSION: Eleven percent of patients developed SBO after their Ladd procedure. Conversely, no observed patients developed a volvulus. This suggests that complications from a Ladd procedure occur with higher frequency than complications from observing heterotaxy patients with malrotation.

Racial disparities in heterotaxy syndrome.

BACKGROUND: Heterotaxy syndrome (HTX) is a constellation of defects including abnormal organ lateralization and often including congenital heart defects. HTX has widely divergent population-based estimates of prevalence, racial and ethnic predominance, and mortality in current literature. METHODS: The objective of this study was to use a population-based registry to investigate potential racial and ethnic disparities in HTX. Using the Texas Birth Defects Registry, we described clinical features and mortality of HTX among infants delivered from 1999 to 2006. We calculated birth prevalence and crude prevalence (cPR) ratios for infant sex, maternal diabetes, and sociodemographic factors. RESULTS: A total of 353 HTX cases were identified from 2,993,604 births (prevalence ratio = 1.18 per 10,000 live births. HTX prevalence was approximately 70% higher among infants of Hispanic and non-Hispanic black mothers and 28% higher among female infants (cPR = 1.28; 95% confidence interval,1.04-1.59). There was a twofold higher female preponderance for infants of mothers who were non-Hispanic white or black. Mothers with diabetes were three times more likely to have a child with HTX compared with nondiabetics (cPR = 3.13; 95% confidence interval, 2.12-4.45). Among nondiabetics, HTX cases were 86% more likely to have a Hispanic mother and 72% a non-Hispanic black mother. First-year mortality for live born children with HTX was 30.9%. CONCLUSION: This study represents one of the largest population-based studies of HTX to date, with a novel finding of higher rates of HTX among Hispanic infants of mostly Mexican origin, as well as among female infants of only non-Hispanic white and black mothers. These findings warrant further investigation.

The association between brain injury, perioperative anesthetic exposure, and 12-month neurodevelopmental outcomes after neonatal cardiac surgery: a retrospective cohort study.

BACKGROUND: Adverse neurodevelopmental outcomes are observed in up to 50% of infants after complex cardiac surgery. We sought to determine the association of perioperative anesthetic exposure with neurodevelopmental outcomes at age 12 months in neonates undergoing complex cardiac surgery and to determine the effect of brain injury determined by magnetic resonance imaging (MRI). METHODS: Retrospective cohort study of neonates undergoing complex cardiac surgery who had preoperative and 7-day postoperative brain MRI and 12-month neurodevelopmental testing with Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III). Doses of volatile anesthetics (VAA), benzodiazepines, and opioids were determined during the first 12 months of life. RESULTS: From a database of 97 infants, 59 met inclusion criteria. Mean ± sd composite standard scores were as follows: cognitive = 102.1 ± 13.3, language = 87.8 ± 12.5, and motor = 89.6 ± 14.1. After forward stepwise multivariable analysis, new postoperative MRI injury (P = 0.039) and higher VAA exposure (P = 0.028) were associated with lower cognitive scores. ICU length of stay (independent of brain injury) was associated with lower performance on all categories of the Bayley-III (P < 0.02). CONCLUSIONS: After adjustment for multiple relevant covariates, we demonstrated an association between VAA exposure, brain injury, ICU length of stay, and lower neurodevelopmental outcome scores at 12 months of age. These findings support the need for further studies to identify potential modifiable factors in the perioperative care of neonates with CHD to improve neurodevelopmental outcomes.

Erythropoietin neuroprotection in neonatal cardiac surgery: a phase I/II safety and efficacy trial.

OBJECTIVES: Neonates undergoing complex congenital heart surgery have a significant incidence of neurologic problems. Erythropoietin has antiapoptotic, antiexcitatory, and anti-inflammatory properties to prevent neuronal cell death in animal models, and improves neurodevelopmental outcomes in full-term neonates with hypoxic ischemic encephalopathy. We designed a prospective phase I/II trial of erythropoietin neuroprotection in neonatal cardiac surgery to assess safety and indicate efficacy. METHODS: Neonates undergoing surgery for D-transposition of the great vessels, hypoplastic left heart syndrome, or aortic arch reconstruction were randomized to 3 perioperative doses of erythropoietin or placebo. Neurodevelopmental testing using the Bayley Scales of Infant and Toddler Development III was performed at age 12 months. RESULTS: Fifty-nine patients received the study drug. Safety profile, including magnetic resonance imaging brain injury, clinical events, and death, was not different between groups. Three patients in each group died. Forty-two patients (22 in the erythropoietin group and 20 in the placebo group; 79% of survivors) returned for 12-month follow-up. In the group receiving erythropoietin, mean Cognitive Scale scores were 101.1 ± 13.6, Language Scale scores were 88.5 ± 12.8, and Motor Scale scores were 89.9 ± 12.3. In the group receiving placebo, Cognitive Scale scores were 106.3 ± 10.8 (P = .19), Language Scores were 92.4 ± 12.4 (P = .33), and Motor Scale scores were 92.6 ± 14.1 (P = .51). CONCLUSIONS: Safety profile for erythropoietin administration was not different than placebo. Neurodevelopmental outcomes were not different between groups; however, this pilot study was not powered to definitively address this outcome. Lessons learned suggest optimized study design features for a larger prospective trial to definitively address the utility of erythropoietin for neuroprotection in this population.

Neurodevelopmental outcomes after regional cerebral perfusion with neuromonitoring for neonatal aortic arch reconstruction.

BACKGROUND: In this study we report magnetic resonance imaging (MRI) brain injury and 12-month neurodevelopmental outcomes when regional cerebral perfusion (RCP) is used for neonatal aortic arch reconstruction. METHODS: Fifty-seven neonates receiving RCP during aortic arch reconstruction were enrolled in a prospective outcome study. RCP flows were determined by near-infrared spectroscopy and transcranial Doppler monitoring. Brain MRI was performed preoperatively and 7 days postoperatively. Bayley Scales of Infant Development III was performed at 12 months. RESULTS: Mean RCP time was 71 ± 28 minutes (range, 5 to 121 minutes) and mean flow was 56.6 ± 10.6 mL/kg/min. New postoperative MRI brain injury was seen in 40% of patients. For 35 RCP patients at age 12 months, mean Bayley Scales III Composite standard scores were: Cognitive, 100.1 ± 14.6 (range, 75 to 125); Language, 87.2 ± 15.0 (range, 62 to 132); and Motor, 87.9 ± 16.8 (range, 58 to 121). Increasing duration of RCP was not associated with adverse neurodevelopmental outcomes. CONCLUSIONS: Neonatal aortic arch repair with RCP using a neuromonitoring strategy results in 12-month cognitive outcomes that are at reference population norms. Language and motor outcomes are lower than the reference population norms by 0.8 to 0.9 standard deviations. The neurodevelopmental outcomes in this RCP cohort demonstrate that this technique is effective and safe in supporting the brain during neonatal aortic arch reconstruction.

Changing expectations for neurological outcomes after the neonatal arterial switch operation.

BACKGROUND: Expectations for outcomes after the neonatal arterial switch operation (ASO) continue to change. This cohort study describes neurodevelopmental outcomes at age 12 months after neonatal ASO, and analyzes both modifiable and nonmodifiable factors for association with adverse outcomes. METHODS: Patients who underwent an ASO (n=30) were enrolled in a prospective outcome study, with comprehensive clinical data collection during the first 12 months of life. Brain magnetic resonance imaging was done preoperatively and 7 days postoperatively, and the Bayley Scales of Infant Development III was performed at age 12 months. RESULTS: Ten of 30 patients (33%) had preoperative magnetic resonance imaging injury; 13 of 30 patients (43%) had new postoperative magnetic resonance imaging injury. Twenty patients (67%) had Bayley Scales of Infant Development III: Cognitive Composite standard score mean was 104.8±15.0, Language Composite standard score median was 90.0 (25th to 75th percentile, 83 to 94), and Motor Composite standard score mean was 92.3±14.2. Best subsets multivariable analysis found associations between lower preoperative and intraoperative cerebral oxygen saturation, preoperative magnetic resonance imaging brain injury, total bypass time, and total midazolam dose and lower Bayley Scales of Infant Development III scores at age 12 months. CONCLUSIONS: At 12 months after ASO, neurodevelopmental outcome means were within normal population ranges. The new associations reported in this study between potentially modifiable perioperative factors and outcomes require investigations in larger patient cohorts. Beyond survival, which was 100% in this cohort, factors influencing quality of life including neurodevelopmental outcomes should be routinely investigated in studies of ASO patients.

Enteral potassium supplementation in a pediatric cardiac intensive care unit: evaluation of a practice change.

BACKGROUND: Potassium supplementation is a common practice in critically ill children, especially those with heart disease. Intravenous potassium supplementation is the standard route of administration in most intensive care units. Although the enteral route is safer and thus may be a reasonable alternative, data on the efficacy of enteral potassium administration are lacking. METHODS: A change of practice to encourage use of enteral potassium was instituted in the cardiac intensive care unit at Texas Children's Hospital, and a review of this practice change was undertaken. The primary outcome of interest was the comparable efficacy of enteral and intravenous potassium administration. Patient demographic data, including urine output, diuretic use, route of potassium administration, and adverse events were documented and analyzed. RESULTS: Seventy-six patients met inclusion criteria and received 399 bolus doses of potassium (166 intravenous and 233 enteral). No patients became hyperkalemic after either route of administration. The increase in serum potassium was similar in both groups of patients. Side effects of the two routes of administration were not different. CONCLUSIONS: The efficacy of enteral potassium is comparable to intravenous potassium for potassium replacement in pediatric patients after congenital heart surgery.

Electroencephalographic seizures after neonatal cardiac surgery with high-flow cardiopulmonary bypass.

BACKGROUND: Postoperative electroencephalographic (EEG) seizures are reported to occur in 14% to 20% of neonates after cardiac surgery with cardiopulmonary bypass (CPB). EEG seizures are associated with prolonged deep hypothermic circulatory arrest and with adverse long-term neurodevelopmental outcomes. We performed video/EEG monitoring before and for 72 hours after neonatal cardiac surgery, using a high-flow CPB protocol and cerebral oxygenation monitoring, to ascertain incidence, severity, and factors associated with EEG seizures. METHODS: The CPB protocol included 150 mL/kg/min flows, pH stat management, hematocrit >30%, and high-flow antegrade cerebral perfusion. Regional cerebral oxygen saturation (rSo(2)) was monitored, with a treatment protocol for rSo(2) <50%. EEG was assessed for seizures. RESULTS: Sixty-eight patients (36 single ventricle [SV] and 32 2-ventricle [2V]) were monitored for a total of 4824 hours. The total midazolam dose was 2.4 mg/kg (1.5-7.3 mg/kg) (median, 25th-75th percentile) for the SV group and 1.3 mg/kg (1.0-2.7 mg/kg) for the 2V group (P = 0.009). One SV patient experienced 2 brief EEG seizures postoperatively (1.5% incidence; 95% confidence interval: 0.3%-7.9%). The SV patients experienced a significant incidence of cerebral desaturation (rSo(2) <45% for >240 minutes total) perioperatively (18 of 36 SV vs 0 of 32 2V patients, P < 0.001). This difference did not affect electrographic seizure occurrence or other EEG characteristics. CONCLUSIONS: EEG seizures are infrequent in neonates undergoing surgery with high-flow CPB. Cerebral desaturation did not affect EEG seizure occurrence; however, benzodiazepines may play a role in suppressing postoperative seizures caused by cerebral hypoxemia in this patient population. Using this anesthetic and surgical protocol, EEG seizures are a poor surrogate marker for acute neurological injury in this population.

Endocrinal complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease.

A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval. The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the endocrine system. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases. As surgical survival in children with congenital cardiac disease has improved in recent years, focus has necessarily shifted to reducing the morbidity of congenital cardiac malformations and their treatment. A comprehensive list of endocrinal complications is presented. This list is a component of a systems-based compendium of complications that will standardize terminology and thereby allow the study and quantification of morbidity in patients with congenital cardiac malformations. Clinicians caring for patients with congenital cardiac disease will be able to use this list for databases, initiatives to improve quality, reporting of complications, and comparing strategies of treatment.

Worsening renal function in children hospitalized with decompensated heart failure: evidence for a pediatric cardiorenal syndrome?

OBJECTIVES: The purpose of this study was to determine the incidence of renal insufficiency in children hospitalized with acute decompensated heart failure and whether worsening renal function is associated with adverse cardiovascular outcome. DESIGN: Prospective observational cohort study. SETTING: Single-center children's hospital. PATIENTS: All pediatric patients from birth to age 21 yrs admitted to our institution with acute decompensated heart failure from October 2003 to October 2005. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Acute decompensated heart failure was defined as new-onset or acute exacerbation of heart failure signs or symptoms requiring hospitalization and inpatient treatment. We required that heart failure be attributable to ventricular dysfunction only. Worsening renal function was defined as an increase in serum creatinine by > or = 0.3 mg/dL during hospitalization. Sixty-three patients (35 male, 28 female) comprised 73 patient hospitalizations. Median age at admission was 10 yrs (range 0.1-20.3 yrs). Median serum creatinine at admission was 0.6 mg/dL (range 0.2-3.5 mg/dL), and median creatinine clearance was 103 mL/min/1.73 m2 (range 22-431 mL/min/1.73 m2). Serum creatinine increased during 60 of 73 (82%) patient hospitalizations (median increase 0.2 mg/dL, range 0.1-2.7 mg/dL), and worsening renal function occurred in 35 of 73 (48%) patient hospitalizations. Clinical variables associated with worsening renal function included admission serum creatinine (p = .009) and blood urea nitrogen (p = .04) and, during hospitalization, continuous infusions of dopamine (p = .028) or nesiritide (p = .007). Worsening renal function was independently associated with the combined end point of in-hospital death or need for mechanical circulatory support (adjusted odds ratio 10.2; 95% confidence interval 1.7-61.2, p = .011). Worsening renal function was also associated with longer observed length of stay (33 +/- 30 days vs. 18 +/- 25 days, p < .03). CONCLUSIONS: These data suggest that an important cardiorenal interaction occurs in children hospitalized for acute decompensated heart failure. Renal function commonly worsens in such patients and is associated with prolonged hospitalization and in-hospital death or the need for mechanical circulatory assistance.

Glycemic profile in infants who have undergone the arterial switch operation: hyperglycemia is not associated with adverse events.

OBJECTIVE: Tight glycemic control improves outcomes in critically ill adults. There are limited data regarding the effect of glycemic profiles in infants after cardiac operations. The aim of this study was to evaluate the association of hyperglycemia and hypoglycemia on adverse events in infants undergoing the arterial switch operation. METHODS: From 2000 through 2005, 93 infants underwent the arterial switch operation (mean age, 2.5 +/- 5.9 weeks; mean weight, 3.4 +/- 0.8 kg). All serum glucose values during the first 24 postoperative hours were documented. The effect of time spent in specific glycemic bands on adverse events was determined. RESULTS: Twenty-three (25%; group 1) infants spent more than 50% of the time with glucose values between 80 and 110 mg/dL, and 13 (14%; group 2) spent more than 50% of the time with glucose values of greater than 200 mg/dL. A total of 71 adverse events was documented in 45 (48%) of 93 infants. Group 1 infants were more likely to have any adverse event (P = .001) and renal insufficiency (P < .001). Group 2 infants were not more likely to have adverse events. When controlling for preoperative and operative factors, being in group 1 was an independent predictor of postoperative adverse events (P = .004). CONCLUSION: Hyperglycemia does not appear to be detrimental in postoperative infants with congenital heart disease. Infants who spent the majority of the time with glucose values between 80 and 110 mg/dL were at increased risk for adverse events. The ideal glycemic profile in the postoperative cardiac infant has yet to be defined.

Prevalence of obesity and overweight among children and youth in Mississippi: current trends in weight status.

The purpose of this research was to estimate the 2007 prevalence of obesity and overweight among children and youth and to compare these data with previous prevalence data to determine current trends in weight status in Mississippi. Measured height and weight data were collected on a weighted sample of 3,837 public school students in grades K-12. Prevalence estimates of obesity and overweight, as indicated by Body Mass Index, were calculated. Analyses were made by gender, race, and grade level for 2007 data, and these data were compared to the 2005 findings. In 2007, 23.5% of the students were obese. The prevalence was highest among elementary school students, males and nonwhite students. The prevalence of obesity in 2007 was lower than in 2005, and the decline occurred primarily among middle and high school students. While the 2007 prevalence was still high, the decline suggests that recent obesity initiatives may be having a positive impact.

Acetazolamide therapy for hypochloremic metabolic alkalosis in pediatric patients with heart disease.

BACKGROUND: Pediatric patients with heart disease are often treated with high doses of diuretics, which can lead to hypochloremic metabolic alkalosis. There are no data in children regarding the efficacy and safety of acetazolamide to treat hypochloremic metabolic alkalosis. METHODS: Patients from January 2004 to June 2005 who received acetazolamide were identified. Inclusion criteria were: age less than 18 years, being a cardiology patient, diuretics use, and had received a 3-day course of acetazolamide. Demographic information was collected along with serum electrolytes, serum creatinine/blood urea nitrogen, urine output, pH, acid-base excess, concurrent medications, cardiac lesion/surgery, and incidence of adverse effects. Efficacy of acetazolamide was determined by comparing variables before and after the 3-day course. Statistical comparisons were made using Student's t-test. RESULTS: A total of 28 patients were identified, 7 of whom received oral acetazolamide, 21 intravenous acetazolamide. Patients were a median of 2.5 (range, 0.3-20) months of age, and 57% (17/28) were female. Seventy-one percent of the cohort received acetazolamide after cardiac surgery. There was no significant difference in any electrolyte, blood urea nitrogen, or serum creatinine from baseline, except for serum bicarbonate, which decreased (36.2 +/- 4.6 vs. 30.9 +/- 4.5 mmol/L, P < 0.001), and chloride, which increased (91.1 +/- 6.8 vs. 95.4 +/- 6.2, P < 0.03). Acid-base excess values and pH decreased during therapy in patients who had the laboratory values drawn (n = 22). No change in urine output at 8 hours (5.2 +/- 2.3 vs. 4.9 +/- 2.3 mL/kg/hr, P = 0.6) or 24 hours (4.7 +/- 1.5 vs. 4.3 +/- 1.4 mL/kg/hr, P = 0.18) occurred after administration of acetazolamide. CONCLUSION: Acetazolamide was safely used in pediatric patients with heart disease to lower serum bicarbonate and acid-base excess values and raise chloride values in hypochloremic metabolic alkalosis.

Persistence of bilateral arterial ducts in pulmonary atresia despite confluent branch pulmonary arteries: opportunity for two percutaneous therapeutic alternatives.

We present a case of a newborn infant with double inlet left ventricle, pulmonary atresia, confluent pulmonary arteries, and bilateral arterial ducts (AD), to discuss the therapeutic alternatives offered by interventional catheterization techniques in this anatomic arrangement. The infant initially underwent stenting of the right AD to stabilize pulmonary blood flow off of prostaglandin infusion. Three weeks later, she developed left pulmonary artery isolation upon closure of the left arterial duct. An additional stent was placed in the pulmonary artery confluence, restoring blood flow to the left lung and significantly improving her oxygen saturations. At 6 months of age she underwent her first surgical procedure, a successful bidirectional cavopulmonary anastomosis with removal of the left pulmonary artery stent and patch enlargement of the pulmonary artery confluence. She continues to do well in clinical follow-up at 16 months of age.