Charis Tan^1,2 , Diana Zannino³, Carley Clendenning³, Sophie Offen⁴, Thomas L. Gentles⁵, Julian Ayer⁶, David Tanous⁷, Vishva Wijesekera⁸, Leeanne Grigg⁹, David Celermajer^2,4,10, Mark McGuire^2,4 , Yves d’Udekem^3,11,12, Rachael Cordina^2,4,10,*

Congenital Heart Disease, Vol.18, No.5, pp. 507-523, 2023, DOI:10.32604/chd.2023.028829

Abstract Objective: Sudden cardiac death (SCD) and malignant ventricular arrhythmia (VA) are increasingly recognized as important issues for people living with a Fontan circulation, but data are lacking. We sought to characterize the cohort who had sudden cardiac death, most likely related to VA and/or documented VA in the Australia and New Zealand Fontan Registry including risk factors and clinical outcomes. Methods: A retrospective cohort study was performed. Inclusion criteria were documented non-sustained ventricular tachycardia, sustained ventricular tachycardia, ventricular fibrillation, resuscitated cardiac arrest or SCD > 30 days post-Fontan completion. Results: Of 1611 patients, 20 (1.2%) had VA; 14 (1.0%) had… More >

Yuki Kawasaki^1,*, Takeshi Sasaki¹, Daisuke Kobayashi²

Congenital Heart Disease, Vol.18, No.4, pp. 431-445, 2023, DOI:10.32604/chd.2023.030910

Abstract Background: Elevated Fontan pressure (FP) alone cannot fully predict clinical outcomes. We hypothesized that hemodynamic profiling using a cardiac index (CI)-systemic vascular resistance (SVR) plot could characterize clinical features and predict the prognosis of post-Fontan patients. Methods: We included post-Fontan patients who underwent cardiac catheterization at age < 10 years. Patients were classified into four categories: A, CI  ≥ 3, SVR index (SVRI) ≥ 20; B, CI < 3, SVRI ≥ 20; C, CI ≥ 3, SVRI < 20; and D, CI < 3, SVRI < 20. The primary outcome was freedom from the combined endpoint: new onset of protein-losing enteropathy or plastic bronchitis, heart transplant, and death. Clinical and hemodynamic variables and freedom from… More > Graphic Abstract

Pietro Paolo Tamborrino^1,2, Corrado Di Mambro^1,2,*, Cecilia Marcolin^1,2, Walter Vignaroli³, Giulia Cafiero⁴, Gianluca Brancaccio³, Sonia Albanese³, Massimo Stefano Silvetti^1,2, Adriano Carotti³, Fabrizio Drago^1,2

Congenital Heart Disease, Vol.16, No.1, pp. 85-94, 2021, DOI:10.32604/CHD.2021.013038

Abstract Introduction: While previous studies only focused on the arrhythmic risk associated with specific correction strategies, this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair. Methods: In this single centre observational cohort study, we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment, patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes. Surgical history and clinical outcomes were reviewed. Results: 86 patients were included in the study (54 male [62.8%], mean age 16.4 ± 6.1 years), with… 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 cavopulmonary connection… More >

Gruschen R. Veldtman¹, Alexander R. Opotowsky², Samuel G. Wittekind¹, Jack Rychik³, Daniel J. Penny⁴, Mark Fogel³, Bradley S. Marino⁵, Marc Gewillig⁶

Congenital Heart Disease, Vol.12, No.6, pp. 699-710, 2017, DOI:10.1111/chd.12526

Abstract Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations. The overarching aim is to provide… More >

Christopher R. Broda¹, Hamsini Sriraman², Devanshi Wadhwa², YunFei Wang¹, Hari Tunuguntla¹, Ayse Akcan-Arikan^3,4, Peter R. Ermis¹, Jack F. Price¹

Congenital Heart Disease, Vol.13, No.4, pp. 602-607, 2018, DOI:10.1111/chd.12617

Abstract Purpose: Elevated central venous pressure (CVP) has deleterious effects on several organ systems in patients with Fontan circulation. However, the relationship between CVP and estimated glomerular filtration rate (eGFR) has not been assessed in patients with Fontan circulation.
Methods: Patients with Fontan circulation whose hemodynamics were assessed by catheterization between 1987 and 2015 and had a serum creatinine measured within 72 hours prior to the procedure were included for analysis. Patients with primary kidney disease were excluded. Renal function was calculated by “bedside Schwartz” equation in children (< 18 years) and Modification of Diet in Renal Disease equation in adults.… More >

Christina E. Holbein¹, Nicholas D. Fogleman^1,2, Kevin Hommel¹, Silke Apers³, Jessica Rassart³, Philip Moons^3,4, Koen Luyckx³, Maayke A. Sluman⁵, Junko Enomoto⁶, Bengt Johansson⁷, Hsiao-Ling Yang⁸, Mikael Dellborg^4,9, Raghavan Subramanyan¹⁰, Jamie L. Jackson¹¹, Werner Budts^3,12, Adrienne H. Kovacs¹³, Stacey Morrison¹, Martha Tomlin¹, Kathy Gosney¹, Alexandra Soufi¹⁴, Katrine Eriksen¹⁵, Corina Thomet^3,16, Malin Berghammer^4,17, Luis Alday¹⁸, Edward Callus¹⁹, Susan M Fernandes²⁰, Maryanne Caruana²¹, Samuel Menahem²², Stephen C. Cook²³, Gwen R. Rempel²⁴, Kamila White²⁵, Paul Khairy²⁶, Shelby Kutty²⁷, Gruschen Veldtman¹

Congenital Heart Disease, Vol.13, No.3, pp. 392-400, 2018, DOI:10.1111/chd.12583

Abstract Objective: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL.
Design: Cross-sectional observational study.
Setting: Twenty-four cardiology centers from 15 countries across five continents.
Patients: Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18-83 years.
Outcome Measures: QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions… More >

Djoeke Wolff¹, Henricus B. M. van de Wiel², Mirthe E. de Muinck Keizer¹, Joost P. van Melle³, Petronella G. Pieper³, Rolf M. F. Berger¹, Tjark Ebels⁴, Willebrord C. M. Weijmar Schultz⁵

Congenital Heart Disease, Vol.13, No.2, pp. 319-326, 2018, DOI:10.1111/chd.12576

Abstract Objective: To get an impression of the quality of life (QOL) and sexual well-being in the Fontan population, and to generate hypotheses for future research.
Methods: For this cross-sectional pilot study, questionnaires regarding health-related QOL, sexual function and fertility/pregnancy were completed by 21 patients with a Fontan circulation >16 years old, followed at the University Medical Center Groningen, the Netherlands. Semi-structured qualitative interviews were conducted in 8 patients.
Results: Fontan patients scored significantly lower on general health than their healthy peers (t (19)=-3.0, P = .008), whereas their scores on other QOL domains and sexual well-being were comparable to normal… More >

Christopher R. Broda, Katherine B. Salciccioli, Keila N. Lopez, Peter R. Ermis, Douglas S. Moodie, Heather A. Dickerson

Congenital Heart Disease, Vol.14, No.6, pp. 885-894, 2019, DOI:10.1111/chd.12856

Abstract Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with con‐ genital heart disease (CHD). Little is known about the adult HS population with CHD.
Objective: To describe the outcomes and sociodemographics of the adult CHD popu‐ lation with HS.
Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.
Results: Sixty‐two patients metinclusion criteria.Median agewas 22.7 [IQR19.6‐30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap… More >

Tim Takken¹, Alyanne Evertse¹, Fleur de Waard¹, Mandy Spoorenburg¹, Martijn Kuijpers¹, Christian Schroer^2,3, Erik H. Hulzebos¹

Congenital Heart Disease, Vol.14, No.6, pp. 1005-1012, 2019, DOI:10.1111/chd.12850

Abstract Background: Traveling to high altitude has become more popular. High‐altitude exposure causes hypobaric hypoxia. Exposure to acute high altitude, during air travel or mountain stays, seems to be safe for most patients with congenital heart disorders (CHD). Still, current guidelines for CHD patients express concerns regarding safety of altitude exposure for patients with a Fontan circulation. Therefore, investigating hemodynamic and pulmonary responses of acute high‐altitude exposure (±2500 m) at rest and during maximal exercise in patients with Fontan circulation can provide clarity in this dispute and may contribute to improvement of clinical counseling.
Methods: Twenty‐one Fontan patients with 21 age‐matched… More >