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 propensity score matching… 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 a web-based survey… 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 were… 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 recorded. The t… More >

Andrew S. Kim¹, Colleen M. Witzenburg², Mark Conaway³, Jeffrey E. Vergales¹, Jeffrey W. Holmes^2,4, Thomas J. L’Ecuyer¹, Peter N. Dean¹

Congenital Heart Disease, Vol.14, No.6, pp. 1185-1192, 2019, DOI:10.1111/chd.12834

Abstract Background: Children with hypoplastic left heart syndrome (HLHS) have risk for mortality and/or transplantation. Previous studies have associated right ventricular (RV) indices in a single echocardiogram with survival, but none have related serial measurements to outcomes. This study sought to determine whether the trajectory of RV indices in the first year of life was associated with transplant‐free survival to stage 3 palliation (S3P).
Methods: HLHS patients at a single center who underwent stage 1 palliation (S1P) between 2000 and 2015 were reviewed. Echocardiographic indices of RV size and function were obtained before and following S1P and stage 2 palliation (S2P).… More >

Alan F. Riley¹, Elena C. Ocampo¹, Joseph Hagan², M. Regina Lantin‐Hermoso¹

Congenital Heart Disease, Vol.14, No.5, pp. 706-712, 2019, DOI:10.1111/chd.12774

Abstract Background: When performed by cardiologists, hand‐held echocardiography (HHE) can assess ventricular systolic function and valve disease in adults, but its accuracy and utility in congenital heart disease is unknown. In hypoplastic left heart syndrome (HLHS), the echocardiographic detection of depressed right ventricular (RV) systolic function and higher grade tricuspid regurgitation (TR) can identify patients who are at increased risk of morbidity and mortality and who may benefit from additional imaging or medical therapies.
Methods: Children with HLHS after Stage I or II surgical palliation (Norwood or Glenn procedures) were prospectively enrolled. Subjects underwent HHE by a pediatric cardiologist on the… More >

Jeannine M. Hoch¹, Oluwatosin Fatusin², Gayane Yenokyan³, W. Reid Thompson², Maureen A. Lefton‐Greif⁴

Congenital Heart Disease, Vol.14, No.3, pp. 438-445, 2019, DOI:10.1111/chd.12742

Abstract Background: Tube feedings are often needed to achieve the growth and nutrition goals associated with decreased morbidity and mortality in patients with single ventricle anat‐ omy. Variability in feeding method through the interstage period has been previously described, however, comparable information following stage 2 palliation is lacking.
Objectives: To identify types of feeding methods following stage 2 palliation and their influence on length of stay.
Design: Secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative registry was performed on 932 patients. Demographic data, medical characteristics, postoperative complications, type of feeding method, and length of stay for stage 2 palliation… More >

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… 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 or genetic… More >