Long-term outcome and quality of life in adult patients after the Fontan operation

doi:10.1016/j.amjcard.2004.01.041

The American Journal of Cardiology

Volume 93, Issue 9, 1 May 2004, Pages 1141-1145

https://doi.org/10.1016/j.amjcard.2004.01.041 Get rights and content

Abstract

The first successful Fontan operation was performed in 1971, and this first cohort of Fontan patients is reaching adulthood with unclear outcome of this palliative procedure. We studied the mortality, morbidity, and quality of life in our adult Fontan patients. We examined all patients (n = 36) who underwent a Fontan procedure and were being seen in an adult outpatient clinic by using electrocardiography, exercise testing, and echocardiography. Quality of life was assessed by the Short Form 36 questionnaire. The mean follow-up period was 15 years (range 0 to 23). Of the initial 36 patients, 10 died (28%) at a mean of 10 years (range 0 to 21) after the Fontan operation and 1 patient underwent cardiac transplantation. Reoperations were performed in 21 patients (58%), and the most common reason was revision of the Fontan connection. Sustained supraventricular tachycardia was observed in 20 patients (56%) with an increased incidence of arrhythmias with longer follow-up. Thromboembolic events were detected in 9 patients (25%), 5 of whom had adequate anticoagulant levels at the time of event. The thromboembolic event was fatal for 3 patients. A total of 195 hospital admissions (mean 3.8 ± 2.7, range 1 to 13) was recorded. Quality-of-life assessment showed physical functioning, mental health, and general health perception to be significantly lower for Fontan patients than for the normal Dutch population. Thus, we found high mortality and very high morbidity in adult patients after the Fontan operation. In particular, reoperations, arrhythmias, and thromboembolic events compromised quality of life.

Section snippets

Patients

All adult patients who underwent a Fontan procedure and regularly attended the outpatient clinic of the Thoraxcenter at the Erasmus Medical Center were included in this study. In 1978, the first Fontan operation was performed in our institute. We studied the long-term follow-up from Fontan operation until last follow-up or death. In 2002, a cross-sectional study of surviving patients was undertaken. All medical and surgical records of the patients were reviewed for reoperations, arrhythmias,

Patients

Thirty-six adult patients with a Fontan procedure were seen in the outpatient clinic and included in the study. There were 18 men (50%). Mean age at the time of Fontan operation was 12 years (range 2 to 34). Twenty-nine patients were operated in childhood and reached adulthood, and 7 patients underwent the Fontan operation at an adult age. The primary cardiac malformation was tricuspid atresia in 21 patients (58%), double-inlet left ventricle in 9 patients (25%), and other complex congenital

Discussion

This study showed a surprisingly high mortality rate in young adults and a high morbidity rate after the Fontan operation. Arrhythmias, reoperations, and thromboembolic events often occurred, and all patients had ≥1 hospital admission during follow-up.

A possible risk factor for late mortality is surgery at older age.13, 14 In our patients who underwent the Fontan operation during adulthood, the mortality rate was as high as 57%. Three of our patients died suddenly after Fontan operation,

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Cited by (145)

Cardiovascular and abdominal flow alterations in adults with morphologic evidence of liver disease post Fontan palliation
2020, International Journal of Cardiology
Although morphologic abnormalities in the liver are commonly encountered post Fontan palliation, the relationships between hepatic morphology, vascular flows, and clinical status remain incompletely understood. We therefore aimed to explore flow characteristics in hepatic and intestinal vessels and to examine cardiovascular associations with liver disease.
This was a retrospective study of adults post Fontan palliation undergoing clinically indicated cardiovascular magnetic resonance imaging (MRI). Patients were included if MRI flow quantification was available for cardiac, hepatic and intestinal vessels; patients were excluded if phase-contrast flow imaging was insufficient for analysis.
Thirty patients were studied (median age at MRI 28.5 years [range 19–47]). Eighteen subjects (60%) were classified as having morphologic liver disease according to validated criteria based on available MRI imaging. Abdominal and cardiovascular flows were quantified. Patients with morphologic liver disease had a 41% reduction in superior mesenteric artery (211 ± 124 versus 358 ± 181 mL/min/m², p = .004), a 36% reduction in hepatic vein (496 ± 247 versus 778 ± 220 mL/min/m², p = .01), a 31% reduction in portal vein (399 ± 133 versus 580 ± 159 mL/min/m², p = .004), and an 18% reduction in Fontan pathway flows (1358 ± 429 versus 1651 ± 270 mL/min/m², p = .04) compared with the remaining population. Adverse cardiovascular events were not associated with morphologic liver disease.
Morphologic liver disease appears to be associated with flow alterations within the heart, liver and intestine post Fontan palliation. These novel observations suggest that a potential relationship exists between morphologic disease and vascular flows thereby providing further insights into the pathophysiology of liver disease in this high-risk population.
Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement
2020, Heart Lung and Circulation
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.
- •
  Lifelong quality medical care with access to multidisciplinary services, is of prime importance. Care includes regular tests for surveillance of health status.
- •
  Transition from paediatric to adult care is an active process that should commence during early adolescence and continue until successful engagement with adult congenital cardiology care.
- •
  Children and adults with a Fontan circulation often have reduced peak exercise capacity (on average, 60–65% of predicted values). Increasingly, evidence suggests exercise training may improve exercise capacity and cardiovascular function.
- •
  People with a Fontan circulation have higher rates of anxiety and behavioural disorders, and there needs to be a low threshold for the provision of mental health care.
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  Pregnancy has increased maternal and fetal risks, and pre-conception multidisciplinary assessment and counselling is essential.
- •
  Atrial arrhythmias are common, often late after Fontan surgical repair and due to intra-atrial re-entry or “flutter” mediated by atrial stretch and scarring. Some anti-arrhythmic agents, most classically the type IC drugs, may allow haemodynamically unstable, life-threatening 1:1 AV conduction.
- •
  Anticoagulation with warfarin is routine care in patients with atrial arrhythmias.
- •
  In patients with recurrent atrial arrhythmias, catheter ablation or surgical conversion may be considered.
- •
  The Fontan circulation is an ideal substrate for thrombus formation and may result in intracardiac or intravascular thrombosis, ischaemic stroke, or other embolic phenomena. Antiplatelet and anticoagulant agents are commonly prescribed for thromboprophylaxis in patients with a Fontan circulation. Evidence suggests that treatment with one of these agents is advantageous, but there is no consensus on which is optimal. Despite treatment, symptomatic thromboembolic events are associated with significant mortality.
- •
  Heart failure is the leading cause of morbidity and mortality. Diuretics provide symptomatic relief, however standard heart failure medical therapy is not of proven benefit.
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  Though not well understood, there is increasing concern regarding progressive liver disease with a long-term risk of hepatocellular carcinoma.
- •
  Despite early higher mortality post heart transplant, these individuals have better long-term survival outcomes compared with many other heart transplant recipients.
Pathophysiology of thrombosis and anticoagulation post Fontan surgery
2018, Thrombosis Research
Citation Excerpt :
Previous reports have ascertained that the total resolution of thrombosis occurs in only 48% of cases [85]. Moreover, thromboembolic events are associated with 25% mortality in pediatric patients [67,85] and 38% in adult patients [86], despite aggressive treatment of thrombi. Other outcomes of thrombosis include pulmonary embolism (which may lead to stroke or Fontan failure); collateral development (which may complicate future chest surgery); post-phlebitic syndrome; and thrombosis reoccurrence [87].
The Fontan procedure has transformed the lives of children born with single-ventricle physiology, previously deemed inoperable. Worldwide, there are an increasing number of children with Fontan circulation, with the potential for survival into adulthood. Due to the abnormal circulation, Fontan patients have an increased risk of thromboembolic (TE) events, with up to 25% of events leading to death. Despite the importance of preventing TE events in this patient population, there is currently no clinical consensus on the optimal monitoring, thromboprophylaxis therapies, and treatment of these events. This paper reviews the available literature regarding anticoagulation in the pediatric and adult Fontan population, including the mechanisms for thrombosis and current antithrombotic therapies.
Maternal Congenital Heart Disease in Pregnancy
2018, Obstetrics and Gynecology Clinics of North America
“How long will I continue to be normal?” Adults with a Fontan circulation's greatest concerns
2018, International Journal of Cardiology
Little is known about adults living with a Fontan circulation's concerns outside the scope of their clinical outcomes. We examined adults with a Fontan circulations' greatest concerns, as well as their concerns around anti-coagulation, pregnancy and finances.
Adults with a Fontan circulation in the Australian and New Zealand Fontan Registry were invited to complete an anonymous online survey, of which 57 participated. A qualitative method approach using thematic analyses was used.
The greatest concerns for adults living with a Fontan circulation were fear of death/uncertainty around life expectancy which for many individuals colored their concerns around physical health, pregnancy and having children, quality of life and finances. Improving information about outcomes to patients with a Fontan circulation might alleviate uncertainties about their future.
Fear of death is the primary concern of adults with a Fontan circulation. It may require improved communication and targeted psychological interventions. Physical exercise incorporated as part of their lifestyle should be encouraged to alleviate physical concerns and also improve psychological well-being.
Bridging the psychological issues of living with the Fontan circulation
2018, International Journal of Cardiology

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Long-term outcome and quality of life in adult patients after the Fontan operation

Abstract

Section snippets

Patients

Patients

Discussion

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Clin Epidemiol

J Am Coll Cardiol

Am J Cardiol

Int J Cardiol

Surgical repair of tricuspid atresia

Thorax

The Fontan procedure for tricuspid atresia

Circulation

Exercise capacity and mid-term survival in patients with tricuspid atresia and complex congenital cardiac malformations after modified Fontan-operation

Eur J Cardiothorac Surg