Hongyun Ruan¹, Yigang Zhang², Ru Liu³, Xiangjun Yang¹

Congenital Heart Disease, Vol.14, No.4, pp. 645-650, 2019, DOI:10.1111/chd.12764

Abstract Objective: The optimal dose of Fasudil is still controversial in congenital heart disease accompanied with severe pulmonary hypertension (CHD‐PAH). This study aimed to compare acute hemodynamic changes after different doses of Fasudil in 60 consecu‐ tive adult patients with CHD‐PAH.
Design: Prospective randomized controlled trial.
Setting: Tertiary cardiology center.
Patients: Adult patients with CHD‐PAH.
Interventions: Patients were randomized to Fasudil 30 or 60 mg.
Outcome Measures: The hemodynamic parameters were measured at baseline and after 30 minutes of Fasudil through right cardiac catheterization. Blood gas results were obtained from the pulmonary artery, right ventricle, right atrium, superior and inferior vena cava,… More >

Rachel Wong¹, Mohammed Al‐Omary², David Baker², Neil Spratt^1,2, Andrew Boyle^1,2, Natasha Baker Cert¹, Peter Howe¹, Nicholas Collins²

Congenital Heart Disease, Vol.14, No.4, pp. 638-644, 2019, DOI:10.1111/chd.12763

Abstract Objectives: Improvements in the management of complex congenital heart disease, including those with single ventricle physiology, have resulted in increased survival. As this population ages, the recognition of cognitive impairment is increasingly im‐ portant. At present, little is known about the potential mechanisms of cognitive dys‐ function. In this cross‐sectional study, we aimed to characterize the nature of abnormalities in cerebral blood flow and the relationship to cognitive deficits in adults with single ventricular physiology.
Patients: Ten adults with single ventricular physiology (age 18‐40 years) and 12 age‐ and gender‐matched controls underwent transcranial Doppler ultrasound and ac‐… More >

Natasa Karadzov Orlic¹, Amira Egic¹, Barbara Damnjanovic‐Pazin MD2 | Relja Lukic¹, Ivana Joksic³, Zeljko Mikovic¹

Congenital Heart Disease, Vol.14, No.6, pp. 1094-1101, 2019, DOI:10.1111/chd.12852

Abstract Objective: The objective of this study was to analyze if the addition of simple cardiac scan in cases with increased nuchal translucency (NT) and/or abnormal ductus venosus (DV) blood flow, and/or tricuspid regurgitation (TCR) can improve detection of congenital heart defects (CHD) in chromosomally normal fetuses without non‐ cardiac defects at 11‐13 + 6 gestational weeks in a population of singleton pregnancies.
Methods: During the 10 years period, all singleton pregnancies at 11‐13 + 6 weeks were routinely scanned for NT, DV blood flow and TCR assessment and, if a sin‐ gle of these parameters was abnormal,… More >

Christine M. Riley¹, Christopher W. Mastropietro², Peter Sassalos³, Jason R. Buckley⁴, John M. Costello⁴, Ilias Iliopoulos⁵, Aimee Jennings⁶, Katherine Cashen⁷, Sukumar Suguna Narasimhulu⁸, Keshava M. N. Gowda⁹, Arthur J. Smerling¹⁰, Michael Wilhelm¹¹, Aditya Badheka¹², Adnan Bakar^13,14, Elizabeth A. S. Moser¹⁵, Venu Amula¹⁶

Congenital Heart Disease, Vol.14, No.6, pp. 1078-1086, 2019, DOI:10.1111/chd.12849

Abstract Background: Elevated pulmonary vascular resistance (PVR) is common following repair of truncus arteriosus. Inhaled nitric oxide (iNO) is an effective yet costly therapy that is frequently implemented postoperatively to manage elevated PVR.
Objectives: We aimed to describe practice patterns of iNO use in a multicenter cohort of patients who underwent repair of truncus arteriosus, a lesion in which recovery is often complicated by elevated PVR. We also sought to identify patient and center factors that were more commonly associated with the use of iNO in the postoperative period.
Design: Retrospective cohort study.
Setting: 15 tertiary care pediatric referral centers.
Patients:… More >

Sok‐Leng Kang¹, Aimee Armstrong², Gregor Krings³, Lee Benson¹

Congenital Heart Disease, Vol.14, No.6, pp. 1046-1057, 2019, DOI:10.1111/chd.12838

Abstract Three‐dimensional rotational angiography (3D‐RA) enables volumetric imaging through rotation of the C‐arm of an angiographic system and real‐time 3D recon‐ struction during cardiac catheterization procedures. In the field of congenital heart disease (CHD), 3D‐RA has gained considerable traction, owing to its capability for en‐ hanced visualization of spatial relationships in complex cardiac morphologies and real time image guidance in an intricate interventional environment. This review provides an overview of the current applications, strengths, and limitations of 3D‐RA acquisi‐ tion in the management of CHD and potential future directions. In addition, issues of dosimetry, radiation exposure, More >

Jennifer K. Peterson¹, Shaun P. Setty^1,2, Jessica H. Knight³, Amanda S. Thomas⁴, James H. Moller⁵, Lazaros K. Kochilas^4,6

Congenital Heart Disease, Vol.14, No.5, pp. 854-863, 2019, DOI:10.1111/chd.12823

Abstract Objective: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long‐term outcomes in patients with T21 and SV palliation.
Design: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anom‐ aly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI)… More >

Maayke A. Sluman^1,2, Silke Apers^3,4, Judith K. Sluiter^1,*, Karen Nieuwenhuijsen¹, Philip Moons^4,5, Koen Luyckx^6,7, Adrienne H. Kovacs^8,9, Corina Thomet¹⁰, Werner Budts¹¹, Junko Enomoto¹², Hsiao‐Ling Yang¹³, Jamie L. Jackson¹⁴, Paul Khairy¹⁵, Stephen C. Cook¹⁶, Raghavan Subramanyan¹⁷, Luis Alday¹⁸, Katrine Eriksen¹⁹, Mikael Dellborg^20,21, Malin Berghammer^5,22, Eva Mattsson²³, Andrew S. Mackie²⁴, Samuel Menahem²⁵, Maryanne Caruana²⁶, Kathy Gosney²⁷, Alexandra Soufi²⁸, Susan M. Fernandes²⁹, Kamila S. White³⁰, Edward Callus³¹, Shelby Kutty³², Berto J. Bouma³³, Barbara J.M. Mulder³³

Congenital Heart Disease, Vol.14, No.3, pp. 362-371, 2019, DOI:10.1111/chd.12747

Abstract Background: Conflicting results have been reported regarding employment status and work ability in adults with congenital heart disease (CHD). Since this is an impor‐ tant determinant for quality of life, we assessed this in a large international adult CHD cohort.
Methods: Data from 4028 adults with CHD (53% women) from 15 different countries were collected by a uniform survey in the cross‐sectional APPROACH International Study. Predictors for employment and work limitations were studied using general linear mixed models.
Results: Median age was 32 years (IQR 25‐42) and 94% of patients had at least a high school degree. Overall… More >

Shelby C. White¹, Jennifer Sedler², Trahern W. Jones³, Michael Seckeler¹

Congenital Heart Disease, Vol.13, No.6, pp. 1045-1049, 2018, DOI:10.1111/chd.12673

Abstract Objective: Applications of three‐dimensional (3D) printed models in medicine in‐ clude preprocedure planning, patient education, and clinical training. Reproducing complex anatomy as a 3D printed model can be useful for understanding congenital heart defects (CHD). We hypothesized that using 3D printed models during didactic sessions with resident physicians will improve trainees’ understanding of CHD.
Design and intervention: We performed a prospective, randomized educational in‐ tervention for teaching pediatric and pediatric/emergency medicine residents about simple (ventricular septal defect [VSD]) and moderately complex (tetralogy of Fallot [ToF]) CHD. Residents were divided into two groups: intervention and control. Each group… More >

Pablo Marino^1,2, Gabriella de Oliveira Lopes^1,3, Juliana Pereira Borges³, Maria Carolina Terra Cola¹, Daniel Arkader Kopiler¹, Eduardo Tibirica¹

Congenital Heart Disease, Vol.13, No.6, pp. 978-987, 2018, DOI:10.1111/chd.12660

Abstract Objective: Adults with congenital heart disease share some features with those with chronic heart failure. Although microvascular endothelial dysfunction has been de‐ scribed in chronic heart failure, evaluation of the microcirculation in adults with con‐ genital heart disease is lacking. The present study aimed to investigate systemic microvascular reactivity in adults with congenital heart disease.
Interventions: The patients initially underwent cardiopulmonary exercise testing. Then, the cutaneous microvascular reactivity was evaluated in these patients using a laser speckle contrast imaging system coupled with skin iontophoresis of endothelial‐de‐ pendent (acetylcholine) or ‐independent (sodium nitroprusside) vasodilators and pos‐ tocclusive reactive… More >

Alka Anilkumar¹, D. M. Vasudevan², Mahesh Kappanayil³, K. R. Sundaram⁴, R. Krishna Kumar³, Sheela Nampoothiri⁵

Congenital Heart Disease, Vol.13, No.3, pp. 483-487, 2018, DOI:10.1111/chd.12600

Abstract Background and Objectives: The 22q11 deletion syndrome is associated with a wide spectrum of phenotypic features, hence clinical diagnosis is difficult. Individuals with this syndrome are found to have a risk of developing transfusion associated graft-versus-host reaction, if they are given nonirradiated blood. Our aim was to elucidate whether chromosome 22q11 deletion in children with syndromic conotruncal heart defects is associated with platelet abnormality.
Materials and Methods: The genetic analysis was performed by standard cytogenetic and Fluorescence in situ hybridization technique. The platelet parameters in 39 patients with chromosome 22q11 deletion were compared with 154 cases without deletion.
Results:… More >