Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Abstract

The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival.

Pregnancy and early post-natal outcomes of fetuses with functionally univentricular heart in a low-and-middle-income country

“…[18][19][20][21] In the setting of a low-and-middle-income country, besides the gestational age at diagnosis, factors like economic burden, concerns about the long-term health and quality of life of a child with complex CHD and the potential impact of the affected child on the future of the family and other children might have influenced the pregnancy outcomes reported in this study. 5,6,8,9 The overall low utilisation of cardiac care in the live-born babies in this study is different from reports from the developed world where the majority receive specialised cardiac care. 21,22 This is despite the fact that all palliative surgeries for univentricular heart were being performed in our centre from the inception of the study.…”

“…33,34 The challenges of providing long-term care for the survivors of these complex CHDs may prove overwhelming for low-and-middle-income countries with an ever-increasing number of patients surviving tertiary cardiac care into adult life. [8][9][10][11][33][34][35] Policymakers and funding agencies need to develop strategies for providing extended support for follow-up care of these patients and not merely fund the cost of the surgical procedures in order to take care of complex CHDs feasible for affected families. 36 The results of this study highlight the importance of early pregnancy screening of the fetal heart, especially in the low-andmiddle-income countries.…”

“…7 Lifelong specialist cardiac review is required for these patients and additional neurodevelopmental and psychological issues cause further concern for longer-term survivors. 8,9 The medical and socio-economic challenges involved in the care of these complex CHDs may be very challenging for patients and their families in the setting of low-and-middle-income countries. 10,11 Screening of the fetal heart is routinely offered as a part of the mid-trimester anomaly scan in all pregnancies in developed countries.…”

Fontan-associated liver disease and hepatocellular carcinoma in adults

“…Medical therapies specific for FALD are not available. Nevertheless, preventive, medical, surgical, and transplant strategies beneficial for similar disease processes may be applicable in FALD 14 , 16 , 48 . Prior to initiating a treatment strategy, it is important to improve cardiac output and/or raise the Fontan pressure (Fig.…”

Imaging of complications following Fontan circulation in children — diagnosis and surveillance

“…When a pediatric cardiologist follows a Fontan patient, his or her personal knowledge of how the other organs are affected will guide the surveillance by other clinical specialties. When these children reach early adolescence, their transition and follow-up from pediatric to adult care regarding both cardiology and radiology needs to be assured until successful commitment [ 9 ].…”