Vanessa Marie Hormaza¹, Mark Conaway², Daniel Scott Schneider¹, Jeffrey Eric Vergales¹

Congenital Heart Disease, Vol.14, No.2, pp. 274-279, 2019, DOI:10.1111/chd.12722

Abstract Objective: Limited information is known on how right ventricular function affects outcomes after stage 2 palliation. We evaluated the impact of different right ventricular indices prior to stage 2 palliation on morbidity and mortality.
Design: Retrospective study design.
Setting: Pediatric Heart Network Single Ventricle Reconstruction Trial Public Data Set.
Patient: Any variant of stage 1 palliation and all anatomic hypoplastic left heart syndrome variants in the trial were evaluated. Echocardiograms prior to stage 2 palliation were analyzed and compared between those who failed and those who survived.
Intervention: None.
Outcome measures: Mortality was defined as death, listed for transplant, or transplanted… More >

Jennifer P. Lundine^1,2, Robert Dempster^3,4, Kirby Carpenito⁵, Holly Miller‐Tate⁵, Wendelin Burdo‐Hartman^4,6, Elizabeth Halpin², Omar Khalid^4,5

Congenital Heart Disease, Vol.13, No.5, pp. 706-712, 2018, DOI:10.1111/chd.12636

Abstract Background: Swallowing dysfunction is a known complication for infants with complex congenital heart disease (CHD), but few studies have examined swallowing outcomes following the hybrid procedure for stage 1 palliation in children with single ventricle physiology.
Objectives: (1) Identify the incidence of aspiration in all infants with single ventricle physiology who underwent the hybrid procedure and (2) Compare results of clinical bedside and instrumental swallowing evaluations to examine the predictive value of a less invasive swallowing assessment for this population of high‐risk infants.
Methods: This was a retrospective cohort chart review study. All patients with single‐ventricle physiology who… More >

Doris P. Yimgang¹, John D. Sorkin², Charles F. Evans³, Danielle S. Abraham¹, Geoffrey L. Rosenthal^1,4

Congenital Heart Disease, Vol.13, No.4, pp. 533-540, 2018, DOI:10.1111/chd.12622

Abstract Introduction: Angiotensin converting enzyme inhibitors are commonly prescribed medications after the Norwood procedure. There are little data that can be used to determine if angiotensin converting enzyme inhibitors improve interstage outcomes in children with single ventricle defects. The objective of this study was to investigate the relationship between angiotensin converting enzyme inhibitors and interstage failure among infants born with hypoplastic left heart syndrome.
Methods: We conducted a retrospective cohort study using data from the National Pediatric Cardiology Quality Improvement Collaborative database (collected between 2008 and 2015). We used logistic regression models to assess the exposure-outcome associations and… More >

Elizabeth C. Caris¹, Nicole Dempster², Gil Wernovsky³, Yongjie Miao², Melissa Moore-Clingenpeel², Trent Neely², Rachel Fonseca², Holly Miller-Tate², Robin Allen², Samantha Fichtner², Jamie Stewart², Clifford L. Cua²

Congenital Heart Disease, Vol.13, No.4, pp. 528-532, 2018, DOI:10.1111/chd.12619

Abstract Objectives: Siblings of children with chronic medical conditions endorse a lower quality of life compared to age-matched peers. Caregiver and sibling-self report of adjustment are often discordant. Congenital heart disease significantly affects family life. To date, there have been no studies addressing the functioning of siblings of children with hypoplastic left heart syndrome, one of the most severe forms of congenital heart disease. The goal of this study was to assess the impact of hypoplastic left heart syndrome on sibling’s quality of life as well as the caregiver’s perception of this effect.
Study Design: Cross-sectional study using… More >

Parthak Prodhan¹, Xinyu Tang², Jeffrey Gossett², Brandon Beam³, Janet Simsic⁴, Nancy Ghanayem⁵, Nahed O. ElHassan⁶

Congenital Heart Disease, Vol.13, No.4, pp. 519-527, 2018, DOI:10.1111/chd.12610

Abstract Objective: Different feeding strategies have been suggested to improve growth and survival of infants with hypoplastic left heart syndrome following stage 1 palliation. The study objective was to assess hospital mortality following stage 1 palliation among infants with hypoplastic left heart syndrome who had two feeding modalities, gastrostomy tube vs no gastrostomy tube.
Design: Retrospective study design.
Setting: Multicenter pediatric heath information system database.
Patient: About 4287 patients with hypoplastic left heart syndrome who underwent stage 1 Norwood procedure from 2004 through 2013. Infants who had gastrostomy tube with or without fundoplication procedure were identified and their clinical characteristics… More >

Natasha L. Lopez, Charitha Gowda, Carl H. Backes, Deipanjan Nandi, Holly Miller-Tate, Samantha Fichtner, Robin Allen, Jamie Stewart, Clifford L. Cua

Congenital Heart Disease, Vol.13, No.4, pp. 512-518, 2018, DOI:10.1111/chd.12602

Abstract Introduction: Neonates with hypoplastic left heart syndrome (HLHS) are at increased risk for necrotizing enterocolitis (NEC). Initial hospital outcomes are well described, but minimal midterm data exist. Goal of this study was to compare outcomes of HLHS infants with NEC (HLHS-NEC) to HLHS without NEC (HLHS-nNEC) during the interstage period.
Methods: Data were reviewed from 55 centers using the NPC-QIC database. Case-control study with one HLHS-NEC matched to HLHS-nNEC neonates in a 1:3 ratio based on institutional site, type of surgical repair, and gestational age ±1 week was performed. Baseline demographics as well as outcome data were… More >

Monique M. Gardner, Meryl S. Cohen

Congenital Heart Disease, Vol.12, No.6, pp. 783-786, 2017, DOI:10.1111/chd.12506

Abstract Background: Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) may occur when the left ventricle is affected but is rarely seen in isolation. RVNC may have clinical significance affecting surgical and long-term outcomes. We describe the diagnosis and clinical course in three patients at our institution.
Methods: We performed a retrospective review of patients diagnosed with RVNC over a 12- month period at our institution and reviewed their imaging and clinical course.
Results: Three patients were identified. All had diagnosis… More >

Katlyn Elizabeth McGrattan^1,2,3,4, Heather McGhee^2,3, Allan DeToma⁵, Elizabeth G. Hill⁵, Sinai C. Zyblewski⁶, Maureen Lefton-Greif^7,8,9, Lucinda Halstead^1,2, Scott M. Bradley¹⁰, Bonnie Martin-Harris^1,2,3,4

Congenital Heart Disease, Vol.12, No.3, pp. 382-388, 2017, DOI:10.1111/chd.12456

Abstract Background: Deficits in swallowing physiology are a leading morbidity for infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliation. Despite the high prevalence of this condition, the underlying deficits that cause this post-operative impairment remain poorly understood.
Objective: Identify the physiologic correlates of dysphagia in infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliative surgery.
Methods: Postoperative fiberoptic laryngoscopies and videofluoroscopic swallow studies (VFSS) were conducted sequentially on infants with functional single ventricles following stage 1 palliative surgery. Infants were dichotomized as having normal or impaired laryngeal function… More >

Waldemar F. Carlo¹, James F. Cnota², Robert J. Dabal³, Jeffrey B. Anderson²

Congenital Heart Disease, Vol.12, No.3, pp. 315-321, 2017, DOI:10.1111/chd.12442

Abstract Objective: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) was established in 2008 to improve outcomes of hypoplastic left heart syndrome (HLHS) during the interstage period. They evaluated changes in patient variables and practice variation between early and late eras.
Design: Data including demographic, operative, discharge, and follow-up variables from the first 100 patients (6/2008–1/2010) representing 18 centers were compared with the most recent 100 patients (1/2014–11/2014) from these same centers.
Results: Prenatal diagnosis increased from 69% to 82% (P = .05). There were no differences in gestational age or weight at Norwood. A composite of any preoperative… More >

Diego A. Lara¹, Mary K. Ethen², Mark A. Canfield², Wendy N. Nembhard³, Shaine A. Morris¹

Congenital Heart Disease, Vol.12, No.1, pp. 105-112, 2017

Abstract Background: Hypoplastic left heart syndrome (HLHS) is strongly associated with Turner syndrome (TS); outcome data when these conditions coexist is sparse. We aimed to investigate long-term survival and causes of death in this population.
Methods: The Texas Birth Defects Registry was queried for all live born infants with HLHS during 1999–2007. We used Kaplan–Meier and Cox regression analyses to compare survival among patients with HLHS with TS (HLHS/TS＋) to patients who had HLHS without genetic disorders or extracardiac birth defects (HLHS/TS–).
Results: Of the 542 patients with HLHS, 11 had TS (2.0%), 71 had other extracardiac birth defects… More >