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… 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… 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 More >