Eleni P. Asimacopoulos^*, Steven J. Staffa, Peter C. Laussen, Kirsten C. Odegard

Congenital Heart Disease, Vol.17, No.5, pp. 579-590, 2022, DOI:10.32604/chd.2022.020334

Abstract Background: Thromboembolic events are a cause of significant morbidity and mortality in the Fontan population. We previously reported on coagulation profile changes in a cohort of patients with hypoplastic left heart syndrome (HLHS) from Stage I through Fontan completion. In this report, we examine their clinical status, anticoagulation and incidence of thromboembolic events up to 20 years post Fontan. Methods: A retrospective chart review was conducted for twenty (20) surviving patients, from 1998 through December 2020. Patients who underwent orthotopic heart transplantation (OTx) were followed until their transplant. Patients who were found in the original study to have a factor… More >

Alyssia Venna¹, Kathleen Reid², Sarah Davis², Jiaxiang Gai³, Yves d’Udekem¹, Sarah Clauss^2,*

Congenital Heart Disease, Vol.17, No.5, pp. 505-518, 2022, DOI:10.32604/chd.2022.021571

Abstract Background: Patients with single ventricle anatomy are at increased risk of growth failure and malnutrition. Amongst cardiac centers, there is little standardization of feeding practices in this complex population. We hypothesized that initiation of our center’s preoperative feeding protocol would result in decreased gastrostomy tube (G-tube) use, decreased length of stay and would not result in increased Necrotizing Enterocolitis (NEC) rates. Methods: A single institution review of 52 patients who had undergone stage I single ventricle palliative repair was performed. Patient diagnoses were hypoplastic left heart syndrome (39%), atrioventricular canal (15%), and other (46%). Postoperative parameters such as time to… More > Graphic Abstract

Benjamin Zielonka^1,2,*, David M. Harrild^1,2, Sunil J. Ghelani^1,2, Eleni G. Elia^1,2, Christopher W. Baird^3,4, Andrew J. Powell^1,2, Rahul H. Rathod^1,2

Congenital Heart Disease, Vol.17, No.5, pp. 591-603, 2022, DOI:10.32604/chd.2022.021509

Abstract Background: The right ventricle to pulmonary artery conduit (RVPAC) may impair right ventricular (RV) function in patients with functional single right ventricles. Modification of the RVPAC using a ring-reinforced end with dunked insertion into the RV through a limited ventriculotomy may reduce the impact on RV function. We compared RV segmental strain between patients with a traditional RVPAC and ring-reinforced RVPAC using feature tracking cardiovascular magnetic resonance (CMR) imaging. Methods: Patients with CMR examinations after Stage I operation with RVPAC between 2000 and 2018 were reviewed. Ventricular mass, volumes, late gadolinium enhancement (LGE), and peak radial and circumferential strain of… More >

Vitaliy Suvorov^1,*, Vladimir Zaitcev¹, Karolina Andrzejczyk²

Congenital Heart Disease, Vol.17, No.3, pp. 365-374, 2022, DOI:10.32604/chd.2022.019126

Abstract Background: Bilateral banding of the branches of the pulmonary artery in patients with hypoplastic left heart syndrome (HLHS) and other duct dependent critical neonatal heart malformations can significantly reduce the incidence of severe complications in the postoperative period, especially in severely unstable patients. In our study we compared different surgical techniques of bilateral pulmonary artery banding (PAB) in respect to their success in balancing systemic and pulmonary blood flow. Methods: We included 44 neonates with a HLHS and congenital heart diseases (CHD) with a functional single ventricle underwent a hybrid operation: bilateral PAB and patent ductus arteriosus stenting. The hybrid… More >

Nunzia Borrelli^1,2, Jolanda Sabatino^1,3, Martina Avesani^1,4, Josefa Paredes¹, Manjit Josen¹, Alain Fraisse¹, Paolo Guccione², Guido Michielon¹, Giovanni Di Salvo^1,4,*

Congenital Heart Disease, Vol.16, No.1, pp. 27-37, 2021, DOI:10.32604/CHD.2021.012526

Abstract Background: Still little is known about the impact on right ventricle function of the 2 main approaches to Norwood palliation in Hypoplastic left heart syndrome, the right ventricle-pulmonary artery shunt (RVPAS) and the modified Blalock-Taussig shunt (mBTS). Methods: The cohort included 27 patients with Hypoplastic left heart syndrome (10 in the mBTS group, 17 in the RVPAS group). Longitudinal strain, tricuspid annulus peak systolic excursion and fractional area change were evaluated before Norwood and in four different breakpoints in the steady-state after Norwood procedure (30-days, 90-days, 140-days and 200-days after Norwood). Results: No significant differences were found in all standard… More >

Clifford L. Cua¹, Melissa Moore‐Clingenpeel¹, Nazia Husain², Ralf Holzer³, John P. Cheatham¹, Janaki Gokhale⁴

Congenital Heart Disease, Vol.14, No.4, pp. 609-613, 2019, DOI:10.1111/chd.12755

Abstract Background: Increased ventricular end‐diastolic pressure (VEDP) is a known risk fac‐ tor for morbidity and mortality in patients with single right ventricle (RV) physiology. Previous studies have shown mixed results correlating echocardiographic measure‐ ments with catheter‐derived VEDP in this population. Goal of this study was to eval‐ uate if echocardiographic systolic/diastolic ratio (S/D) correlated with VEDP.
Methods: Patients with single RV physiology who underwent simultaneous echocar‐ diography and catheterization were evaluated. Systolic and diastolic durations were measured using tricuspid inflow durations from Doppler analysis to calculate the S/D ratio. VEDP was obtained from the catheterization report.
Results: Twenty‐seven studies 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 of RVNC… 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 based on… 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 >

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 underwent the… More >