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  • Open Access

    ARTICLE

    Monocusp valve placement in children with tetralogy of Fallot undergoing repair with transannular patch: A functioning pulmonary valve does not improve immediate postsurgical outcomes

    Nikki M. Singh1, Rohit S. Loomba2, Todd M. Gudausky1, Michael E. Mitchell3

    Congenital Heart Disease, Vol.13, No.6, pp. 935-943, 2018, DOI:10.1111/chd.12670

    Abstract Introduction: In patients with tetralogy of Fallot (TOF), use of transannular patch (TAP) may be required in order to relieve significant right ventricular outflow tract obstruction, subsequently resulting in pulmonary insufficiency (PI). The monocusp valve has been used to temporarily reduce insufficiency in hopes to improve short and midterm outcomes. The purpose of this study was to assess for potential benefits of the monocusp valve in this subset of patients.
    Design: Between 2005 and 2016, 119 patients with TOF with pulmonary stenosis who underwent repair with TAP were analyzed, 43 (36.1%) had a monocusp valve placed. Immediate… More >

  • Open Access

    ARTICLE

    Contributors to disease‐specific health knowledge in adults with congenital heart disease: A correlational study

    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 >

  • Open Access

    ARTICLE

    Increased incidence of infective endocarditis in patients with ventricular septal defect

    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 >

  • Open Access

    ARTICLE

    Complete atrioventricular canal repair with a decellularized porcine small intestinal submucosa patch

    Eliana Al Haddad1*, Damien J. LaPar1*, Jeffrey Dayton2, Elizabeth H. Stephens1, Emile Bacha1

    Congenital Heart Disease, Vol.13, No.6, pp. 997-1004, 2018, DOI:10.1111/chd.12666

    Abstract Background: Congenital heart defects affect nearly 1% of all children born per year in the United States, and complete atrioventricular canal (CAVC) accounts for 2%‐9%. While several patch materials have been used for septal defect closure during CAVC repair, clear superiority of one material over another has yet to be established.
    Methods: A retrospective review of clinical outcomes following CAVC repair at Morgan Stanley Children’s Hospital/Columbia University was performed on opera‐ tions conducted from March 2010 to September 2017. Univariate and Kaplan‐Meir survival analyses were utilized to evaluate primary outcomes of interest following CAVC repair in the… More >

  • Open Access

    ARTICLE

    Atrial septal defect in adults is associated with airway hyperresponsiveness

    Martina Nassif1, Reindert P. van Steenwijk2, Jacqueline M. Hogenhout2, Huangling Lu1, Rianne H.A.C.M. de Bruin‐Bon B Health1, Alexander Hirsch1,3, Peter J. Sterk MD, PhD2 | Berto J. Bouma MD, PhD1 | Bart Straver MD, PhD4 | Jan G.P. Tijssen1, Barbara J.M. Mulder1,5, 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 >

  • Open Access

    ARTICLE

    Pulsatile Glenn as long‐term palliation for single ventricle physiology patients

    Martin A. Chacon‐Portillo1,2, Rodrigo Zea‐Vera1,2, Huirong Zhu3, Heather A. Dickerson4,5, Iki Adachi1,2, Jeffrey S. Heinle1,2, Charles D. Fraser1,2, Carlos M. Mery1,2

    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 >

  • Open Access

    ARTICLE

    Screening for thoracic aortic pathology: Clinical practice in a single tertiary center

    Lidia R. Bons1, Lucas Uchoa de Assis1, Silvy Dekker1, Robert M. Kauling1, Judith A.A.E. Cuypers1, Hence J.M. Verhagen2, Ricardo P.J. Budde3, Jolien W. Roos‐Hesselink1

    Congenital Heart Disease, Vol.13, No.6, pp. 988-996, 2018, DOI:10.1111/chd.12663

    Abstract Objective: The aim is to present current clinical practice of thoracic aorta screening in a tertiary referral center. We identified how often imaging techniques were used for screening and established the value of transthoracic echocardiography (TTE) in com‐ parison with computed tomography (CT) to detect aortic dilation. We also investigated which additional abnormalities of the heart, aorta or smaller arteries were discovered.
    Design: All patients ≥15 years who visited our tertiary center in 2012‐2016 for first thoracic aortic screening were retrospectively included. Diameters of the sinus of Valsalva (SoV) and maximum ascending aorta (AA) were compared between… More >

  • Open Access

    ARTICLE

    Exercise testing and spirometry as predictors of mortality in congenital heart disease: Contrasting Fontan physiology with repaired tetralogy of Fallot

    Keri M. Shafer1,2, Alexander R. Opotowsky1,2, 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 >

  • Open Access

    ARTICLE

    Evaluation of systemic microvascular reactivity in adults with congenital heart disease

    Pablo Marino1,2, Gabriella de Oliveira Lopes1,3, 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 >

  • Open Access

    ARTICLE

    Efficiency of the home cardiac rehabilitation program for adults with complex congenital heart disease

    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 >

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