Mohd Nizam Mat Bah¹, Mohd Hanafi Sapian¹, Mohammad Tamim Jamil¹, Nisah Abdullah¹, Emieliyuza Yusnita Alias¹, Norazah Zahari²

Congenital Heart Disease, Vol.13, No.6, pp. 1012-1027, 2018, DOI:10.1111/chd.12672

Abstract Objectives: There is limited data on congenital heart disease (CHD) from the lower‐ and middle‐income country. We aim to study the epidemiology of CHD with the specific objective to estimate the birth prevalence, severity, and its trend over time.
Design: A population‐based study with data retrieved from the Pediatric Cardiology Clinical Information System, a clinical registry of acquired and congenital heart dis‐ ease for children.
Setting: State of Johor, Malaysia.
Patients: All children (0‐12 years of age) born in the state of Johor between January 2006 and December 2015.
Intervention: None.
Outcome measure: The birth prevalence, severity, and temporal trend over time.
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Shiraz A. Maskatia¹, Christopher J. Petit^2,3, Curtis D. Travers², David J. Goldberg⁴, Lindsay S. Rogers⁴, Andrew C. Glatz⁴, Athar M. Qureshi⁶, Bryan H. Goldstein⁵, Jingning Ao^2,3, Ritu Sachdeva^2,3

Congenital Heart Disease, Vol.13, No.6, pp. 892-902, 2018, DOI:10.1111/chd.12671

Abstract Background: In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echo‐ cardiographic parameters associated with 2V circulation, (2) identify echocardio‐ graphic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions.
Methods: We performed a retrospective analysis of patients who underwent RV de‐ compression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1‐year or prior to Glenn… More >

Joshua Saef¹, Sandeep Sodhi¹, Kristen M. Tecson², Vanessa al Rashida¹, Jong Mi Ko², Kamila S. White³, Philip A. Ludbrook¹, Ari M. Cedars⁴

Congenital Heart Disease, Vol.13, No.6, pp. 967-977, 2018, DOI:10.1111/chd.12668

Abstract Objective: Growth in the adults with congenital heart disease (ACHD) population represents a challenge to the health care infrastructure. As patients with chronic disease are increasingly held accountable for their own care, contributors to disease‐ specific health knowledge, which are known to correlate with patients’ participation in care, merit investigation to design patient‐focused interventions.
Design: We conducted a single‐site, cross‐sectional study of ACHD patients. Investigators retrospectively gathered clinical data as well as psychometric and health status assessments completed at the time of enrollment.
Outcome Measures: We investigated the impact of clinical and psychological varia‐ bles on Leuven Knowledge… More >

Phong Teck Lee, Felix Maverick Uy, Jie Sheng Foo, Ju Le Tan

Congenital Heart Disease, Vol.13, No.6, pp. 1005-1011, 2018, DOI:10.1111/chd.12667

Abstract Background: Ventricular septal defect (VSD) is one of the most common congenital heart anomalies in childhood and there is an increasing prevalence of VSDs in the adult population. The long‐term risk of infective endocarditis (IE) is of concern. The aim of this study was to clarify and compare the incidence of IE in adults with re‐ paired and unrepaired VSDs.
Methods: Patients with VSDs were identified using the Adult Congenital Heart Disease registry at the National Heart Centre Singapore. Patients were divided into Group 1 (repaired VSD) and Group 2 (unrepaired VSDs). The electronic medical re‐ cords… More >

Martina Nassif¹, Reindert P. van Steenwijk², Jacqueline M. Hogenhout², Huangling Lu¹, Rianne H.A.C.M. de Bruin‐Bon B Health¹, Alexander Hirsch^1,3, Peter J. Sterk MD, PhD2 | Berto J. Bouma MD, PhD1 | Bart Straver MD, PhD4 | Jan G.P. Tijssen¹, Barbara J.M. Mulder^1,5, Robbert J. de Winter¹

Congenital Heart Disease, Vol.13, No.6, pp. 959-966, 2018, DOI:10.1111/chd.12665

Abstract Objective: The association between secundum atrial septal defects (ASD) and asthma‐ like dyspnea with consequent long‐term pulmonary inhalant use, is poorly understood in adult ASD patients. Airway hyperresponsiveness is suggested to be the underlying mechanism of cardiac asthma from mitral valve disease and ischemic cardiomyopathy. We hypothesized that airway hyperresponsiveness may also be found in adult ASD patients. Our aim was to study airway responsiveness in adult ASD patients before percutaneous closure and at short‐and long‐term postprocedural follow‐up.
Methods: This prospective study included 31 ASD patients (65% female, mean age 49 ± 15y) who underwent spirometry and… More >

Martin A. Chacon‐Portillo^1,2, Rodrigo Zea‐Vera^1,2, Huirong Zhu³, Heather A. Dickerson^4,5, Iki Adachi^1,2, Jeffrey S. Heinle^1,2, Charles D. Fraser^1,2, Carlos M. Mery^1,2

Congenital Heart Disease, Vol.13, No.6, pp. 927-934, 2018, DOI:10.1111/chd.12664

Abstract Objective: There are limited studies analyzing pulsatile Glenn as a long‐term pallia‐ tion strategy for single ventricle patients. This study sought to determine their out‐ comes at a single institution.
Design: A retrospective review was performed.
Setting: Study performed at a single pediatric hospital.
Patients: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included.
Outcome measures: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk fac‐ tors were assessed by Cox multivariable competing risk analyses.
Results: Seventy‐eight patients underwent pulsatile Glenn at age 9… More >

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 >