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

Zentner, Dominica; Celermajer, David S.; Gentles, Thomas L.; d’Udekem, Yves; Ayer, Julian; Blue, Gillian M.; Bridgman, Cameron; Burchill, Luke J.; Cheung, Michael; Cordina, Rachael; Culnane, Evelyn; Davis, Andrew M.; Plessis, Karin du; Eagleson, K.; Finucane, Kirsten; Frank, Belinda; Greenway, Sebastian; Grigg, Leeanne; Hardikar, Winita; Hornung, Tim; Hynson, Jenny; Iyengar, Ajay J.; James, Paul; Justo, Robert; Kalman, Jonathan M.; Kasparian, Nadine A.; Le, Brian; Marshall, K. C.; Mathew, Jacob; McGiffin, David C.; McGuire, Mark A.; Monagle, Paul; Moore, Ben D.; Neilsen, Julie; O'Connor, B.; O’Donnell, Clare; Pflaumer, Andreas; Rice, Kathryn; Sholler, Gary F.; Skinner, Jonathan R.; Sood, Siddharth; Ward, Juliet; Weintraub, Robert G.; Wilson, Tom; Wilson, William; Winlaw, David S.; Wood, Angela

doi:10.1016/j.hlc.2019.09.010

Cited by 47 publications

(43 citation statements)

References 404 publications

(500 reference statements)

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“…[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.…”

Section: Discussioncontrasting

confidence: 93%

“…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.…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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Background: Care of children with functionally univentricular hearts is resource-intensive. Objectives: To analyse pregnancy and early post-natal outcomes of fetuses with functionally univentricular hearts in the setting of a low–middle-income country. Methods: A retrospective study was conducted during the period of January 2008–October 2019. Study variables analysed included gestational age at diagnosis, maternal and fetal comorbidities and cardiac diagnosis including morphologic type of single ventricle. Outcomes analysed included pregnancy outcomes, type of post-natal care and survival status on the last follow-up. Results: A total of 504 fetuses were included. Mean maternal age was 27.5 ± 4.8 years and mean gestational age at diagnosis was 25.6 ± 5.7 weeks. Pregnancy outcomes included non-continued pregnancies (54%), live births (42.7%) and loss to follow-up (3.3%). Gestational age at diagnosis was the only factor that impacted pregnancy outcomes (non-continued pregnancies 22.5 ± 3.5 vs. live births 29.7 ± 5.7 weeks; p < 0.001). Of the 215 live births, intention-to-treat was reported in 119 (55.3%) cases; of these 103 (86.6%) underwent cardiac procedures. Seventy-nine patients (36.7%) opted for comfort care. On follow-up (median 10 (1–120) months), 106 patients (21%) were alive. Parental choice of intention-to-.treat or comfort care was the only factor that impacted survival on follow-up. Conclusions: Prenatal diagnosis of functionally univentricular hearts was associated with overall low survival status on follow-up due to parental decisions on not to continue pregnancy or non-intention-to-treat after birth. Early detection of these complex defects by improved prenatal screening can enhance parental options and reduce resource impact in low-and-middle-income countries.

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Section: Discussioncontrasting

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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“…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.…”

Section: Introductionmentioning

confidence: 99%

Fontan-associated liver disease and hepatocellular carcinoma in adults

Kogiso

Tokushige

2020

Sci Rep

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The Fontan operation creates a unique circulation, and is a palliative therapy for patients with single-ventricle congenital heart disease. Increased venous pressure and decreased cardiac output and hepatic venous drainage result in sinusoidal dilatation around the central veins. This causes congestion and hypoxia in the liver, leading to Fontan-associated liver disease (FALD). Non-invasive and invasive markers enable diagnosis and evaluation of the fibrosis status in chronic liver disease; however, these markers have not been validated in FALD. Additionally, regenerative nodules such as focal nodular hyperplasia (FNH) are frequently found. The severity of fibrosis correlates with the duration of the Fontan procedure and the central venous pressure. Cirrhosis is a risk factor for hepatocellular carcinoma (HCC), the annual risk of which is 1.5–5.0%. HCC is frequently difficult to diagnose and treat because of cardiac complications, coagulopathy, and congenital abnormalities. The mortality rate of FALD with liver cirrhosis and/or FALD-HCC was increased to ~ 29.4% (5/17 cases) in a nationwide survey. Although there is no consensus on the surveillance of patients with FALD, serial monitoring of the alpha fetoprotein level and imaging at 6-month intervals is required in patients with cirrhosis.

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“…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 ].…”

Section: Radiologic Imaging In the Surveillance Of Fontan Patientsmentioning

confidence: 99%

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

Lange

2020

Pediatr Radiol

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The Fontan operation is performed for various cardiac lesions with single-ventricle physiology. The survival rate of Fontan patients is increasing for adolescents and young adults, with an expected 30-year survival of >80%. Medical health care providers including specialists in organ systems and pediatric radiologists need to improve their knowledge about the Fontan circulation and the various organ complications to monitor care. In this review the author explains the basic anatomical and functional features of Fontan palliation and gives an overview of the multiple long-term organ complications that might present in the pediatric population. These include decreased physical capacity, ventricular dysfunction, atrioventricular valve regurgitation and arrhythmia, as well as protein-losing enteropathy, plastic bronchitis, growth/bone composition disturbances, renal dysfunction, and the recently recognized and important liver fibrosis (Fontan-associated liver disease). Neuropsychological and behavioral deficits occur frequently. This review focuses on the key role of radiology in making the diagnosis of these complications, monitoring therapy and predicting outcomes in the pediatric age group. The author discusses how and when radiology is important in Fontan patients, as well as how new techniques enabling quantitative measures in imaging with US, MRI and CT are adapted for pediatric use, and how they contribute to urgently needed surveillance strategies. Electronic supplementary material The online version of this article (10.1007/s00247-020-04682-5) contains supplementary material, which is available to authorized users.

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

Cited by 47 publications

References 404 publications

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

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

Fontan-associated liver disease and hepatocellular carcinoma in adults

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

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