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 of RVNC… More >

Wen-Yi Luo¹, Zhuo-Ming Xu¹, Li Hong², Qian-Yue Wu¹, Yue-Yue Zhang¹

Congenital Heart Disease, Vol.12, No.6, pp. 777-782, 2017, DOI:10.1111/chd.12489

Abstract Background: Food allergy is a rapidly growing public health concern because of its increasing prevalence, as well as life-threatening potential. There is limited knowledge on the nutritional status for the pediatric congenital heart disease (CHD) patients with food allergy.
Objective: This study investigated both clinical and nutritional outcomes according to the CHD infants with food allergy.
Methods: Forty CHD infants with food allergy and 39 controls were recruited in Shanghai, China. The height and weight for age and weight for height were converted to z-scores to evaluate their effects on nutritional status before and after CHD operation.
Results: Cow’s milk… More >

Maryanne Caruana¹, Victor Grech²

Congenital Heart Disease, Vol.12, No.5, pp. 588-595, 2017, DOI: 10.1111/chd.12488

Abstract Objectives: To investigate survival and freedom from reintervention after aortic coarctation repair in Maltese patients and to compare cardiovascular mortality in coarctation repair survivors with that in the general population.
Design: All 72 aortic coarctation patients with any type of repair, born by end-1997 and logged in the local database were included. Trends in timing and type of repair were determined by comparing patients born before and after 1985. Kaplan-Meier analyses of survival and reintervention-free survival were performed on the 59 repair survivors with complete follow-up data (mean follow-up 26.13 ± 9.62 (range 1.05–44.55 years). Cardiovascular mortality in repair survivors… More >

Shankar Baskar¹, Philippa Horne², Samantha Fitzsimmons³, Philip R. Khoury¹, Joseph Vettukattill⁴, Koichiro Niwa⁵, Teiji Agaki⁶, Mark Spence⁷, Hisanori Sakazaki⁸, Gruschen Veldtman¹

Congenital Heart Disease, Vol.12, No.4, pp. 512-519, 2017, DOI:10.1111/chd.12481

Abstract Background: Patients with Eisenmenger syndrome have a shorter lifespan than the general population. A significant proportion develop arrhythmia and some, sudden death.
Objective: The aims of this study were to characterize the frequency, type and effects of arrhythmias in adult patients with Eisenmenger’s syndrome and to identify risk factors for arrhythmias.
Methods: This retrospective study included patients aged ≥18 years of age with Eisenmenger’s syndrome from three institutions. Arrhythmias were noted from electrocardiograms and Holter study reviews.
Results: A total of 167 patients, 96 females, 63 males (gender not available in 9 patients) were included in this study. The mean… More >

Takaya Hoashi¹, Toru Iwasa², Koji Kagisaki¹, Masatoshi Shimada¹, Kenichi Kurosaki², Isao Shiraishi², Hajime Ichikawa¹

Congenital Heart Disease, Vol.12, No.4, pp. 441-447, 2017, DOI:10.1111/chd.12477

Abstract Objective: To review long-term respiratory outcomes for tetralogy of Fallot and absent pulmonary valve (TOF/APV) in respiratory symptomatic populations.
Methods: Of 25 consecutive patients undergoing primary total correction for TOF/APV between 1987 and 2016, Sixteen patients (64%) with a preoperative respiratory disturbance were enrolled. The median age at operation was 1.9 months old, including 4 neonates and 12 infants. Ten patients (62.5%) preoperatively necessitated mechanical ventilator support. During operation, dilated central pulmonary arteries (cPAs) were plicated and retracted anteriorly in all patients, except for the first patient of the study cohort. VSD was completely closed, and the right ventricular outflow… More >

Jennifer K. Peterson¹, Yanjun Chen², Danh V. Nguyen³, Shaun P. Setty¹

Congenital Heart Disease, Vol.12, No.4, pp. 520-532, 2017, DOI:10.1111/chd.12475

Abstract Objective: Despite overall improvements in congenital heart disease outcomes, racial and ethnic disparities have continued. The purpose of this study is to examine the effect of race and ethnicity, as well as other risk factors on congenital heart surgery length of stay and in-hospital mortality.
Design: From the 2012 Healthcare Cost and Utilization Project Kids Inpatient Database (KID), we identified 13 130 records with Risk Adjustment in Congenital Heart Surgery complexity scoreeligible procedures. Multivariate logistic and linear regression modeling with survey weights, stratification and clustering was used to examine the relationships between predictor variables and length of stay as well… More >

Tarek Alsaied¹, Jouke P. Bokma², Mark E. Engel³, Joey M. Kuijpers², Samuel P. Hanke¹, Liesl Zuhlke⁴, Bin Zhang⁵, Gruschen R. Veldtman⁶

Congenital Heart Disease, Vol.12, No.4, pp. 393-398, 2017, DOI:10.1111/chd.12468

Abstract Background: Reported long-term outcome measures vary greatly between studies in Fontan patients making comprehensive appraisal of mortality hazard challenging. We sought to create a clinical risk score to assist monitoring of Fontan patients in the outpatient setting.
Methods: A systematic review was conducted to evaluate risk factors for long-term (beyond the first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90 patients were included or ≥20 long-term mortalities we reported. Risk factors for long-term mortality were determined. The pooled hazard ratios were used to create components of a clinical score for long-term mortality using meta-analysis techniques.
… 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 risk factor… More >

Maryanne Caruana¹, Victor Grech²

Congenital Heart Disease, Vol.12, No.3, pp. 301-308, 2017, DOI:10.1111/chd.12439

Abstract Objectives: To determine overall and reintervention-free survival for repaired Maltese tetralogy of Fallot patients and to investigate the potential impact of gender, age at repair, genetic syndromes, previous palliation, and type of repair on these outcomes.
Design: All 130 tetralogy of Fallot patients born before the end of 1997 included in the local database were extracted. Surgical repair type, age at repair and operative survival were analyzed among the 103/130 repaired patients. Kaplan–Meier survival analyses were performed on the 75 repair survivors with complete follow-up data (mean follow-up 26.37 ± 9.27 (range 9.95–51.21) years).
Results: Patients born after 1985 were… More >

Jeffrey H. Sacks^1,2, Michael Kelleman², Courtney McCracken², Michelle Glanville¹, Matthew Oster^1,2

Congenital Heart Disease, Vol.12, No.3, pp. 282-288, 2017, DOI:10.1111/chd.12436

Abstract Objective: Hospital readmissions are increasingly becoming a metric for quality in the current landscape of changing and cost effective medicine. However, no 30-d readmission rates have been established for pediatric cardiac medical patients in the United States. Thus, the objective was to determine 30 d readmission rates and risk factors associated with readmission for pediatric cardiac patients, hypothesizing that pediatric cardiac patients would have significantly higher readmission rates than their general pediatric counterparts.
Design: This was a retrospective cohort study.
Setting: The study took place at a large urban academic children’s hospital.
Patients: The 1124 included patients were discharged from… More >