Maria Fedchenko, Zacharias Mandalenakis, Helena Dellborg, Görel Hultsberg‐Olsson, Anna Björk, Peter Eriksson, Mikael Dellborg

Congenital Heart Disease, Vol.14, No.4, pp. 549-558, 2019, DOI:10.1111/chd.12785

Abstract Background: The aging patient with adult congenital heart disease (ACHD) faces the risk of developing atherosclerotic disease. Patients with coarctation of the aorta (CoA) are especially vulnerable because of an inherent high risk of developing hy‐ pertension. However, data on the prevalence of other cardiovascular risk factors are scarce. Therefore, this study aimed to describe the prevalence of traditional cardio‐ vascular risk factors (diabetes, hypertension, hyperlipidemia, smoking, obesity, and sedentary lifestyle) in adult patients with CoA.
Methods: Patients with CoA who were registered at the ACHD clinic in Gothenburg were asked to participate in a comprehensive cardiovascular… More >

Jennifer Gerardin¹, Cheryl Raskind‐Hood², Fred H. Rodriguez^3,4, Trenton Hoffman², Andreas Kalogeropoulos⁵, Carol Hogue², Wendy Book³

Congenital Heart Disease, Vol.14, No.4, pp. 541-548, 2019, DOI:10.1111/chd.12780

Abstract Objective: Transfer of congenital heart disease care from the pediatric to adult set‐ ting has been identified as a priority and is associated with better outcomes. Our objective is to determine what percentage of patients with congenital heart disease transferred to adult congenital cardiac care.
Design: A retrospective cohort study.
Setting: Referrals to a tertiary referral center for adult congenital heart disease pa‐ tients from its pediatric referral base.
Patients: This resulted in 1514 patients age 16‐30, seen at least once in three pediat‐ ric Georgia health care systems during 2008‐2010.
Interventions: We analyzed for protective factors associated with age‐appropriate… More >

Lisa X. Deng¹, Lacey P. Gleason², Katherine Awh², Abigail May Khan³, David Drajpuch^2,4, Stephanie Fuller⁵, Leah A. Goldberg², Christopher E. Mascio⁵, Sara L. Partington^2,4, Lynda Tobin^2,4, Adrienne H. Kovacs³, Yuli Y. Kim^2,4

Congenital Heart Disease, Vol.14, No.4, pp. 534-540, 2019, DOI:10.1111/chd.12778

Abstract Objective: To investigate the experiences and communication preferences of adult patients with congenital heart disease (CHD) in the domains of employment, insur‐ ance, and family planning.
Design: Patients ≥ 18 years of age completed a questionnaire about experiences and communication preferences regarding employment, health insurance, and family planning.
Results: Of 152 patients (median age = 33 years, 50% female, 35% with CHD of great complexity), one in four reported work‐related problems due to CHD and a quar‐ ter also recalled a previous gap in health insurance. Of females, 29% experienced an unplanned pregnancy. The median importance of discussion… More >

Fatima M. Ezzeddine¹, Tabitha Moe², Georges Ephrem³, William Aaron Kay³

Congenital Heart Disease, Vol.14, No.4, pp. 511-516, 2019, DOI:10.1111/chd.12771

Abstract Background: Delivery of care to the adult congenital heart disease (ACHD) popula‐ tion has been limited by a shortage in the ACHD physician resources. There is limited data regarding the adequacy of the ACHD physician resources in the United States and our population estimates are extrapolated from Canadian data. Therefore, we proposed to evaluate the adequacy of ACHD physician: patient ratios in the United States at both national and regional levels.
Methods: Data from the Adult Congenital Heart Association (ACHA) website along with metropolitan area and statewide population data from 2016 US Census Bureau estimates were analyzed.… More >

Jose A. Silva‐Sepulveda¹, Yudy Fonseca², Irine Vodkin³, Gabrielle Vaughn¹, Robert Newbury⁴, Vera Vavinskaya⁵, Jerry Dwek⁶, James C. Perry¹, Preeti Reshamwala⁷, Cynthia Baehling⁸, James Lyon⁹, Christopher Davis¹, Jesse W. Lee¹, Hannah El‐Sabrout¹⁰, Doaa Shahbah¹¹, Laith Alshawabkeh¹², John W Moore¹, Howaida El‐Said¹

Congenital Heart Disease, Vol.14, No.4, pp. 600-608, 2019, DOI:10.1111/chd.12770

Abstract Introduction: Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics.
Methods: Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic reso‐ nance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modi‐ fied Ishak congestive hepatic fibrosis score.
Results: Median age 17.8 years, median… More >

Michael V. Di Maria¹, Cindy Barrett¹, Carey Rafferty¹, Kelly Wolfe¹, Sarah L. Kelly¹, Deborah R. Liptzin², Dania Brigham³, Adel Younoszai¹

Congenital Heart Disease, Vol.14, No.4, pp. 590-599, 2019, DOI:10.1111/chd.12769

Abstract Background: Children with single ventricle (SV) heart disease who undergo Fontan operation are at risk for developing multiorgan dysfunction. Although survival has improved, significant comorbidities involving multiple organ systems may develop, requiring evaluation and management by many subspecialists. Using data from an internal survey, we documented high care variability for our Fontan population. We then developed a multidisciplinary clinic, designed and implemented a clinical care pathway to decrease variability of patient assessment.
Methods: After creating a multidisciplinary team and a clinical care pathway, we initiated a multidisciplinary clinic (MDC) where patients could see multiple subspecialists during a… More >

Alexander C. Egbe, William R. Miranda, Patricia A. Pellikka, Sorin V. Pislaru, Barry A. Borlaug, Srikanth Kothapalli, Sindhura Ananthaneni, Harigopal Sandhyavenu, Maria Najam, Mohamed Farouk Abdelsamid, Heidi M. Connolly

Congenital Heart Disease, Vol.14, No.4, pp. 657-664, 2019, DOI:10.1111/chd.12768

Abstract Background: We hypothesized that echocardiographic indices of right ventricular to pulmonary artery (RV‐PA) coupling were comparable to cardiac magnetic resonance imaging (CMRI)‐derived RV volumetric indices in predicting disease severity in chronic pulmonary regurgitation (PR).
Methods: Patients with ≥ moderate PR (2003‐2015) with and without prior CMRI scans were enrolled into the study cohort and validation cohort, respectively. Endpoint was to determine the association between noninvasive RV‐PA coupling in‐ dices (tricuspid annular plane systolic excursion/right ventricular systolic pressure [TAPSE/RVSP] and fractional area change [FAC]/RVSP ratio) and markers of disease severity, and compared this association to that of CMRI‐derived… More >

Madalena Coutinho Cruz¹, André Viveiros Monteiro¹, Guilherme Portugal¹, Sérgio Laranjo², Ana Lousinha¹, Bruno Valente¹, Paulo Osório¹, Pedro Silva Cunha¹, Lídia de Sousa¹, José Alberto Oliveira¹, Ana Agapito¹, Mário Martins Oliveira¹, Fátima Pinto², Rui Cruz Ferreira¹

Congenital Heart Disease, Vol.14, No.4, pp. 525-533, 2019, DOI:10.1111/chd.12767

Abstract Objective: Sudden cardiac death is common in the adult congenital heart disease (ACHD) population. Knowledge and experience about the use of implantable cardio‐ verter defibrillators (ICD) in ACHD patients is very limited. We aimed to characterize a cohort of patients with ACHD and ICDs.
Design: Thirty consecutive ACHD patients submitted to an ICD implantation in a single tertiary center were evaluated. Data on baseline clinical features, heart defect, indication for ICD, type of device, appropriate therapies, ICD‐related complication, and mortality during follow‐up were collected.
Results: Of the 30 patients, 56.7% received appropriate therapies due to ventricular tachycardia (VT)… More >

Jenny Alenius Dahlqvist¹, Jan Sunnegårdh², Katarina Hanséus³, Eva Strömvall Larsson², Anders Nygren², Magnus Dalén^4,5, Håkan Berggren⁶, Jens Johansson Ramgren⁷, Urban Wiklund⁸, Annika Rydberg¹

Congenital Heart Disease, Vol.14, No.4, pp. 582-589, 2019, DOI:10.1111/chd.12766

Abstract Objective: Fontan surgery is performed in children with univentricular heart defects. Previous data regarding permanent pacemaker implantation frequency and indica‐ tions in Fontan patients are limited and conflicting. We examined the prevalence of and risk factors for pacemaker treatment in a consecutive national cohort of patients after Fontan surgery in Sweden.
Methods: We retrospectively reviewed all Swedish patients who underwent Fontan surgery from 1982 to 2017 (n = 599).
Results: After a mean follow‐up of 12.2 years, 13% (78/599) of the patients with Fontan circulation had received pacemakers. Patients operated with the extracardiac conduit (EC) type of total… More >

James A. Kuo¹, Tyler Hamby², Maham N. Munawar³, Eldad Erez⁴, Vincent K. H. Tam⁵

Congenital Heart Disease, Vol.14, No.4, pp. 651-656, 2019, DOI:10.1111/chd.12765

Abstract Objective: Various options exist for right ventricular outflow tract (RVOT) recon‐ struction in congenital heart disease. The Freestyle porcine aortic root may be used but its longevity is not well defined.
Design: We performed a retrospective review of all non‐Ross RVOT reconstructions using the Freestyle root in our institution. Survival and reintervention, either by sur‐ gery, transcatheter valve implantation, balloon valvuloplasty, or bare metal stent placement, were recorded. Factors associated with reintervention were assessed using Cox regression.
Results: Between January 2002 and December 2015, there were 182 patients identi‐ fied. Sixteen patients were lost to follow‐up and 3… More >