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Patients with hypoplastic left heart syndrome (HLHS) can have associated genetic abnormalities. This study evaluated the incidence of genetic abnormalities among infants with HLHS and the short-term outcomes of this population during the first hospitalization. This is a retrospective analysis of the multi-center Pediatric Heath Information System database of infants with HLHS who underwent Stage I Norwood, Hybrid, or heart transplant during their first hospitalization from 2004 through 2013. We compared clinical data between infants with and without genetic abnormality, among the three most common chromosomal abnormalities, and between survivors and non-survivors. Multivariable analysis was completed to evaluate predictors of mortality among patients with genetic abnormalities. A total of 5721 infants with HLHS were identified; 282 (5%) had associated genetic abnormalities. The three most common chromosomal abnormalities were Turner (25%), DiGeorge (22%), and Downs (12.7%) syndromes. Over the study period, the number of patients with genetic abnormalities undergoing cardiac operations increased without any significant increases in mortality. Infants with genetic abnormalities compared to those without abnormalities had longer hospital length of stay and higher morbidity and mortality. Variables associated with mortality were lower gestational age, longer duration of vasopressor therapy, need for dialysis, and cardiopulmonary resuscitation; and complicated clinical course as suggested by necrotizing enterocolitis, septicemia. Presence of any genetic abnormality in infants with HLHS undergoing cardiac surgery is associated with increased mortality and morbidity. Timely genetic testing, appropriate family counseling, and thorough preoperative case selection are suggested for these patients for any operative intervention.

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease, making up 10% of all congenital heart defects. Annual follow-up echocardiograms are recommended in patients with repaired ToF, but evidence-based guidelines do not exist. We hypothesized that most echocardiograms performed in asymptomatic patients with repaired ToF and no physical exam change do not result in an actionable change (AC) in management. We retrospectively reviewed records of all patients with ToF and prior complete repair at our institution between January 2000 and September 2015. Changes in echocardiograms resulting in hospital admission, medication addition/change, cardiac catheterization, or surgical procedure were identified via chart review. These changes were referred to as an AC. A total of 1135 echocardiograms were reviewed from 233 patients (160 with initial complete repair, 70 with prior shunt, and 3 with other initial surgery). The median number of echocardiograms per patient was 5. Of the 1135 echocardiograms, 15 (1.3%) were associated with AC. Of the 15 patients with AC echocardiograms, 9 underwent a shunt prior to complete repair (9/70, 12.9%) and 6 had undergone an initial complete repair (6/160, 3.8%). The median age at AC was 6.3 years (IQR 4.4, 6.8) in the shunt group and 0.90 years (IQR 0.87, 1.1) in the initial complete repair group. In asymptomatic patients with repaired ToF and no physical exam change, echocardiograms rarely lead to a change in clinical management. In conclusion, the likelihood and timing of AC echocardiograms and reinterventions vary based on the type of initial surgery.

Pulmonary artery (PA) stenosis is the most common late sequela following arterial switch for d-transposition of the great arteries. The purpose of this study was to assess the effectiveness of transthoracic echocardiography in evaluating the pulmonary arteries following repair. This was a retrospective, cross-sectional analysis of all echocardiograms performed on patients following arterial switch operation. A numerical scoring system was devised and used to quantify PA visualization based on 2D images, color mapping, and spectral Doppler. The study cohort included 150 patients. The ability to visualize at least one PA was poorer in patients who were older [> 10 years (47%) vs ≤ 10 years (89%) (p 1.25 m2 (40%) vs ≤ 1.25 m2 (90%) (p

Little is known about the causes and risks of non-postoperative pericardial effusion (PCE) in pediatric patients. We sought to assess the diagnoses most frequently associated with admissions for PCE, and to determine if certain conditions were associated with higher in-hospital mortality and rates of readmission. Nationally distributed data from 44 pediatric hospitals in the 2004–2015 Pediatric Health Information System database were used to identify patients with hospital admissions for International Classification of Disease, Ninth Revision (ICD-9) codes for PCE and/or cardiac tamponade. Children with congenital heart disease were excluded. ICD-9 codes for conditions associated with PCE were grouped into eight categories: neoplastic, renal, autoimmune/inflammatory, pneumonia, viral, bacterial, hypothyroidism, and idiopathic. Multivariable models were used to evaluate odds of in-hospital mortality and readmission within 30 and 90 days. There were 9902 patients who met inclusion criteria. Total in-hospital mortality was 8.2% (n = 813); of those without a neoplastic diagnosis, mortality was 6.5% (n = 493/7543). Idiopathic PCE accounted for the most admissions (36%), followed by neoplasms (24%), pneumonia (20%), and autoimmune/inflammatory disease (19%). In multivariable models, odds of death were highest for neoplasms (adjusted odds ratio 3.83, p

Myocardial dysfunction and heart failure are common in pediatric patients with congenital and acquired heart disease. Alkaline phosphatase (AP) has been suggested as a biomarker for myocardial dysfunction after Fontan operation. We hypothesized that pediatric patients with myocardial dysfunction requiring orthotopic heart transplant (OHT) have diminished AP compared to normal. A retrospective review was performed in all patients who underwent OHT at Arkansas Children’s Hospital between January 2007 and October 2012. Anatomic diagnoses, therapeutic interventions, and ventricular ejection fraction (EF) were recorded. Z scores for AP levels in the study group were determined by comparing the observed AP levels to age- and gender-matched normative values. T tests were performed to compare the mean AP Z score prior to and after OHT. p values

Multiple reports have shown that echocardiograms are neither cost-effective nor of high diagnostic yield for a number of indications. This study sought to evaluate the impact of indications and provider type on the diagnostic yield of first-time outpatient pediatric echocardiograms. All initial echocardiograms interpreted at our institution from February 2009 to December 2014 were reviewed retrospectively. Positive findings were defined as any abnormality of structure or function. Ordering physicians were grouped as Primary Care, Subspecialist, or Cardiologist. A cost analysis of cardiac consultation versus direct echocardiogram ordering was performed using 2014 Arkansas Medicaid office-based allowables. A total of 7854 echocardiograms had complete data and were included in the study. Median age was 7.2 years (range 2 days to 18.9 years). There were 1179 (15%) abnormal first-time echocardiograms. Diagnostic yields were particularly low for the indications of chest pain (4.9%), syncope (5.3%), and palpitations (9.1%). When ordered by the Cardiology group, echocardiographic yields were increased 35% for all indications (p

Stenotrophomonas maltophilia can present as bacteremia, respiratory tract infection, urinary tract infection, soft tissue and wound infections, bone and joint infections, meningitis, and endocarditis especially in immunosuppressed patients and those with underlying medical conditions. The incidence and impact of S. maltophilia in young children with heart disease are poorly defined. A single center retrospective observational study was conducted in infants

Our aim was to evaluate postoperative morbidity and mortality following initial intervention, comparing primary repair versus palliative shunt in the setting of ductal-dependent tetralogy of Fallot. When neonatal surgical intervention is required, controversy and cross-center variability exists with regard to surgical strategy. The multicenter Pediatric Health Information System database was queried to identify patients with TOF and ductal-dependent physiology, excluding pulmonary atresia. Eight hundred forty-five patients were included-349 (41.3%) underwent primary complete repair, while 496 (58.7%) underwent initial palliation. Palliated patients had significantly higher comorbid diagnoses of genetic syndrome and coronary artery anomalies. Primary complete repair patients had significantly increased morbidity across a number of variables compared to shunt palliation, but mortality rate was equal (6%). Second-stage complete repair was analyzed for 285 of palliated patients, with median inter-stage duration of 231 days (175-322 days). In comparison to primary complete repairs, second-stage repairs had significantly decreased morbidity and mortality. However, cumulative morbidity was higher for the staged patients. Median adjusted billed charges were lower for primary complete repair ($363,554) compared to staged repair ($428,109). For ductal-dependent TOF, there is no difference in postoperative mortality following the initial surgery (6%) whether management involves primary repair or palliative shunt. Although delaying complete repair by performing a palliative shunt is associated with a shift of much of the morbidity burden to outside of the newborn period, there is greater total postoperative morbidity and resource utilization associated with the staged approach.

Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder affecting 1 in 3,000 people. Cardiovascular involvement is a prominent feature of MFS, with aortic dissection and/or rupture being the leading cause of death. Advances in the medical and surgical care of patients with MFS have improved survival. Hospital resource utilization and outcomes have not been evaluated in a large population of patients with MFS. We sought to analyze pediatric hospital resource utilization and outcomes in patients with MFS. Nationally distributed data from 43 pediatric hospitals in the 2004–2011 Pediatric Health Information System database were used to identify patients admitted to the hospital with International Classification of Diseases-9th Revision codes for a diagnosis of MFS. Aortic aneurysm (AA) with or without dissection, length of stay (LOS), and hospital charges were determined. During the study period, there were 1,978 admissions in 1,228 patients with MFS. AA was present in 217 (11 %) admissions in 188 (15 %) patients (63 % male). Mean age of patients with AA was 13.8 ± 5.9 years. Aortic dissection or rupture was present in 15 (7 % with AA) admissions in 15 (8 % with AA) patients (mean age 15.7 ± 5.2 years). Other cardiac diagnoses occurred more commonly in the AA cohort (p < 0.0001), regardless of the reason for admission. Cardiothoracic surgical procedures were performed in 116 AA admissions (53 %). Mean LOS, hospital charges per admission, and charges per day were significantly higher in AA cohort compared to those without AA. In-hospital mortality for AA was 2 %. The presence of AA in patients with MFS increases hospital resource utilization. Cardiothoracic surgeries are commonly performed in this cohort. Other cardiovascular diagnoses are more prevalent in patients with AA suggesting a more severe phenotype.