Intensive Care Unit and Acute Care Unit Length of Stay After Congenital Heart Surgery.

Background: Postoperative length of stay (LOS) is an important quality metric and is known to vary widely across hospitals after congenital heart surgery. Whether this variability is explained by factors associated with the intensive care unit (ICU) or acute care unit (ACU) remains unclear. We evaluated the relationship between ICU and ACU LOS and the impact of ACU characteristics on postoperative LOS.

Is Clinic Visit Frequency Associated with Weight Gain During the Interstage Period? A Report from the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative (JCCHD-NPCQIC).

Growth problems are prevalent among infants with congenital heart disease. We sought to determine whether frequency of outpatient clinic visits correlated with weight gain in patients with hypoplastic left heart syndrome or variant during the interstage period between discharge from stage I palliation and presentation for stage II palliation (SIIP). Using prospectively collected data from the JCCHD NPC-QIC database from June 2008 to July 2013, we performed a retrospective cohort study assessing the association of days between clinic visits (DBV) with the change in weight-for-age z-score (WAZ) during the interstage period.

Longitudinal assessment of growth in hypoplastic left heart syndrome: results from the single ventricle reconstruction trial.

Background: We sought to characterize growth between birth and age 3 years in infants with hypoplastic left heart syndrome who underwent the Norwood procedure.

Methods And Results: We performed a secondary analysis using the Single Ventricle Reconstruction Trial database after excluding patients <37 weeks gestation (N=498). We determined length-for-age z score (LAZ) and weight-for-age z score (WAZ) at birth and age 3 years and change in WAZ over 4 clinically relevant time periods.

Heterotaxy syndrome and malrotation: does isomerism influence risk and decision to treat.

Purpose: Controversy remains regarding the management of the asymptomatic heterotaxy syndrome (HS) patient with suspected intestinal rotational abnormalities. We evaluated the outcomes for our HS population to identify frequency of malrotation and identify characteristics of children who might benefit from expectant management.

Methods: After IRB approval, a retrospective review of all patients treated for HS at a large tertiary care children's hospital between January 2008 and June 2012 was performed.

Is there a benefit to postoperative fluid restriction following infant surgery?

Objective: Fluid restriction is often employed immediately following cardiac surgery in children. The goal of this approach is to achieve an early negative fluid balance, which theoretically should lead to less interstitial edema and earlier extubation. The purpose of this study was to determine whether time to negative fluid balance in infants after undergoing systemic-to-pulmonary artery shunt palliation impacts duration of mechanical ventilation and hospital length of stay.

Variation in feeding practices following the Norwood procedure.

Objectives: To assess variation in feeding practice at hospital discharge after the Norwood procedure, factors associated with tube feeding, and associations among site, feeding mode, and growth before stage II.

Study Design: From May 2005 to July 2008, 555 subjects from 15 centers were enrolled in the Pediatric Heart Network Single Ventricle Reconstruction Trial; 432 survivors with feeding data at hospital discharge after the Norwood procedure were analyzed.

Results: Demographic and clinical variables were compared among 4 feeding modes: oral only (n = 140), oral/tube (n = 195), nasogastric tube (N-tube) only (n = 40), and gastrostomy tube (G-tube) only (n = 57).

Patient and practice factors affecting growth of infants with systemic-to-pulmonary shunt.

Background: On recognising poor growth following neonatal palliation with a systemic-to-pulmonary shunt, we sought to determine how patient- and procedure-related factors impact growth, paying attention to the role of the primary cardiologist in this process.

Methods: In a retrospective review, neonates (133 patients) receiving modified systemic-to-pulmonary artery shunts from 2002 to 2009 were studied and outpatient visits were reviewed. Patients with single- and two-ventricle circulations after shunt takedown were compared using weight-for-age z-score.

Coarctation of the aorta in the setting of tetralogy of Fallot: an uncommon cause of myocardial dysfunction.

The combination of both right and left heart obstruction has only rarely been described in the medical literature. We present three cases of coarctation of the aorta in patients with variants of tetralogy of Fallot and hypothesise that this condition may be more common than previously suspected and could represent a hidden cause of morbidity in patients with pulmonary atresia/ventricular septal defect.

Caloric intake during the perioperative period and growth failure in infants with congenital heart disease.

Infants with congenital heart disease have impaired weight gain during the first several months of life. Efforts have focused on improving weight gain and nutritional status during the first months of life. Close examination of the data suggests that the immediate postoperative period is problematic.

Interstage mortality after the Norwood procedure: Results of the multicenter Single Ventricle Reconstruction trial.

Objective: For infants with single ventricle malformations undergoing staged repair, interstage mortality is reported at 2% to 20%. The Single Ventricle Reconstruction trial randomized subjects with a single morphologic right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt (MBTS) or a right ventricle-to-pulmonary artery shunt (RVPAS). The aim of this analysis was to explore the associations of interstage mortality and shunt type, and demographic, anatomic, and perioperative factors.

Association of feeding modality with interstage mortality after single-ventricle palliation.

Objective: Interstage mortality has been reported in 10% to 25% of hospital survivors after single-ventricle palliation. The purpose of this study was to examine the impact of feeding modality at discharge after single-ventricle palliation on interstage mortality.

Methods: We conducted a retrospective review of all neonates undergoing single-ventricle palliation from January 2003 to January 2010.

Freedom from neoaortic insufficiency: a comparison of classic Norwood and Norwood-Sano procedures.

Objectives: To investigate the incidence of neoaortic insufficiency in patients with hypoplastic left heart syndrome treated with the Norwood-Sano palliation and to compare it with that occurring after the classic Norwood procedure.

Design, Setting, Patients, Interventions: This was a retrospective review of all echocardiograms of patients diagnosed with hypoplastic left heart syndrome (concomitant presence of left ventricular and aortic and mitral severe hypoplasia or atresia) who underwent staged palliation of the Norwood or Norwood-Sano type at a single academic institution between September 1999 and February 2005 and who survived a minimum of 3 months.

Outcome Measures: Neoaortic insufficiency was categorized as absent or mild <1 mm jet width, moderate 1-3 mm jet width, or severe >3 mm jet width.

Gastric fundoplication is effective in promoting weight gain in children with severe congenital heart defects.

Aim: The aim of this study was to determine outcomes, including weight gain, morbidity, and mortality, of children with severe congenital heart disease who underwent fundoplication (FP) for gastroesophageal reflux disease.

Methods: An institutional review board-approved retrospective review was conducted on all children with congenital heart disease who underwent FP from 1999 to 2005. Preoperative age, weight, cardiac procedures, postoperative weight, and mortality were extracted from medical records.

Routine immunizations and adverse events in infants with single-ventricle physiology.

Background: Infants with single-ventricle congenital heart defects are at risk of sudden unexpected death. In an effort to decrease the risk of sudden death, some centers have advocated that routine immunizations be deferred in this population. However, it is not known if an association exists between immunizations and adverse events.

Retinoid X receptor alpha represses GATA-4-mediated transcription via a retinoid-dependent interaction with the cardiac-enriched repressor FOG-2.

Dietary vitamin A and its derivatives, retinoids, regulate cardiac growth and development. To delineate mechanisms involved in retinoid-mediated control of cardiac gene expression, the regulatory effects of the retinoid X receptor alpha (RXR alpha) on atrial naturietic factor (ANF) gene transcription was investigated. The transcriptional activity of an ANF promoter-reporter in rat neonatal ventricular myocytes was repressed by RXR alpha in the presence of 9-cis-RA and by the constitutively active mutant RXR alpha F318A indicating that liganded RXR confers the regulatory effect.