Keri M. Shafer^1,2, Alexander R. Opotowsky^1,2, Jonathan Rhodes¹

Congenital Heart Disease, Vol.13, No.6, pp. 903-910, 2018, DOI:10.1111/chd.12661

Abstract Objective: Risk prediction using cardiopulmonary exercise testing (CPET) in complex congenital heart disease tends to either focus on single diagnoses or complete cohorts. We aimed to evaluate patients with two distinct anatomies cared for at a single institution over the same time period to determine CPET variables associated with mortality.
Design: All Fontan and tetralogy of Fallot (TOF) subjects with CPET between November 1, 2002 and December 31, 2014 and subsequently died were identified (cases). Cases were matched 1:3 to controls with similar age, underlying anatomy and timing of exercise test.
Results: Of the 42 cases, 27 had… 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 >

Adikan Bhasipol¹, Nopawan Sanjaroensuttikul², Prapaporn Pornsuriyasak³, Sukit Yamwong¹, Tarinee Tangcharoen¹

Congenital Heart Disease, Vol.13, No.6, pp. 952-958, 2018, DOI:10.1111/chd.12659

Abstract Objective: We aimed to study the efficiency and safety of once‐a‐week outpatient rehabilitation followed by home program with tele‐monitoring in patients with com‐ plex cyanotic congenital heart disease.
Design: Prospective nonrandomized study.
Method: Patients who have been diagnosed either Eisenmenger’s syndrome or inop‐ erable complex cyanotic heart disease and able to attend 12‐week cardiac rehabilita‐ tion program were included. Training with treadmill walking and bicycling under supervision at cardiac rehabilitation unit once‐a‐week in the first 6 weeks followed by home‐based exercise program (bicycle and walking) with a target at 40%‐70% of maximum heart rate (HRmax) at pretraining peak… More >

Benjamin M. Moore^1,2, Rachael L. Cordina^1,2, Mark A. McGuire^1,2, David S. Celermajer^1,2

Congenital Heart Disease, Vol.13, No.6, pp. 944-951, 2018, DOI:10.1111/chd.12657

Abstract Objective: Amiodarone is a highly effective antiarrhythmic therapy, however its tox‐ icity profile often limits treatment. This is particularly relevant in adults with congeni‐ tal heart disease (CHD), who are often young and in whom other antiarrhythmic agents commonly fail or are contraindicated. We sought to determine incidence and predictors of adverse effects caused by amiodarone in adult CHD (ACHD).
Design: A retrospective review of patients with moderate to complex ACHD treated with amiodarone at our center between 2000 and 2017 was performed. Incidence and predictors of adverse effects were described. Efficacy of amiodarone therapy in controlling… More >

Fabricio González‐Andrade, Daniel Echeverría, Valeria López, Michaela Arellano

Congenital Heart Disease, Vol.13, No.6, pp. 911-918, 2018, DOI:10.1111/chd.12654

Abstract Objective: To assess the pulse oximetry as a method for screening critical congenital heart disease (CHD) in newborns.
Study design: This is an observational, transversal, descriptive simple study. The pre‐ ductal and postductal saturation were taken in term newborns thatfulfilled the criteria of inclusion and exclusion in the Hospital Gineco‐Obstetrico Isidro Ayora (HGOIA) in Quito. These measurements were performed between the 24 and 48 h after birth. Those new‐ borns that saturated less than 90% on initial pulse oxìmetry underwent 3 successive measurements at 1‐h intervals. Those who saturate less than 90% after 3 measurements or have… More >

Shankar Baskar, Seth B. Gray, Erica L. Del Grippo, Onyekachukwu Osakwe, Adam W. Powell, Justin T. Tretter

Congenital Heart Disease, Vol.13, No.5, pp. 808-810, 2018, DOI:10.1111/chd.12675

Abstract Cardiology fellows‐in‐training, both in adult and pediatric hospitals, need structured education in regards to congenital heart disease (CHD) nomenclature. With improved survival of patients with CHD, it is not uncommon for these patients to seek care in multiple adult and pediatric hospitals. A deep understanding of CHD nomenclature would aid in providing accurate medical and surgical care for these patients. In this forum, we share our experience with such structured education and also comment on recent advances in morphologic imaging that would aid in understanding the nomenclature. More >

Rohit S. Loomba¹, Seth B. Gray², Saul Flores³

Congenital Heart Disease, Vol.13, No.5, pp. 646-654, 2018, DOI:10.1111/chd.12662

Abstract Introduction: Ketamine is a drug often used for procedural sedation or as adjunct agent for general sedation in children with congenital heart disease. In the clinical realm, there is often confusion regarding the effects of ketamine on hemodynamics, particularly pulmonary vascular resistance and systemic vascular resistance. We per‐ formed a meta‐analysis of studies investigating the effects of ketamine on hemodynamics.
Methods: A systematic review was conducted to identify studies characterizing the hemodynamic effects of ketamine in children with congenital heart disease. Studies were assessed for quality and those of satisfactory quality with pre‐ and postketa‐ mine hemodynamics… More >

Varun Aggarwal, Venkatachalam Mulukutla, Athar M. Qureshi, Henri Justino

Congenital Heart Disease, Vol.13, No.5, pp. 782-787, 2018, DOI:10.1111/chd.12653

Abstract Background: Congenital coronary artery fistula is a rare coronary anomaly. Most commonly, such fistulae drain into the right side of the heart or the pulmonary artery. Children with coronary artery fistulae are generally asymptomatic, although they may have left ventricular enlargement in the setting of a moderate sized left to right shunt. Symptoms of congestive heart failure or ischemia are very rare in neonatal period, and suggest the presence of a very large shunt and/or coronary steal.
Methods: Single center retrospective review of transcatheter intervention on coro‐ nary artery fistulae presenting with symptoms in the neonatal period… More >

Michelle Keir¹, Barbara Bailey¹, Angela Lee¹, Adrienne H. Kovacs^1,2, S. Lucy Roche^1,3

Congenital Heart Disease, Vol.13, No.5, pp. 740-747, 2018, DOI:10.1111/chd.12647

Abstract Background: With access to surgical care, >90% of today’s infants with congenital heart disease (CHD) will reach adulthood. During childhood, survivors accrue a wealth of health care experience and develop strategies for navigating life with a chronic disease.
Methods: Seeking to learn from this individualized process, we invited adults with complex CHD to participate in narrative analysis—an established qualitative research method for studying how individuals derive meaning from their personal stories. Audio recordings of 2‐4 hour free‐form interviews were transcribed and iteratively analyzed to identify common themes and detect similarities or differences in language, viewpoint and interpretation.… More >

Ashley Brunmeier^1,2, Michael P Reis³, Michael G. Earing^1,2, Laura Umfleet ⁴, Salil Ginde^1,2, Peter J. Bartz^1,2, Scott Cohen^1,2

Congenital Heart Disease, Vol.13, No.5, pp. 728-733, 2018, DOI:10.1111/chd.12646

Abstract Objective: Children with congenital heart disease (CHD) and adults with acquired heart disease are at an increased risk of neurocognitive impairment. The objective of this study was to determine the prevalence of self‐reported neurocognitive impair‐ ment and its risk factors in the adult congenital heart disease (ACHD) population.
Design: The Wisconsin Adult Congenital Heart Disease Program recently began screening ACHD patients to identify those with significant self‐perceived neurocog‐ nitive impairments. Screening consists of using a validated neuro‐oncology screening instrument that has been modified for the ACHD population. Patients who answer this survey in a predetermined fashion consistent… More >