Review
Role of comprehensive care in Fontan survivors: A multidisciplinary team approach

https://doi.org/10.1016/j.ppedcard.2018.02.002Get rights and content

Highlights

  • Fontan operation remains the current standard management strategy for patients born with single ventricle heart disease.

  • Following Fontan, there is increasing evidence for the broad range of pathology spanning nearly every organ system.

  • The natural history and progression of Fontan associated multisystem disease remains unclear and will need further investigation.

  • A multidisciplinary standardized approach is critical in surveillance and management of Fontan survivors.

Abstract

The current standard management strategy for patients born with single ventricle heart disease includes a series of palliative surgeries, culminating in the Fontan operation. With the recognition that survivors of Fontan palliation are at risk for developing a wide breadth of complications, spanning multiple different organ systems, interest in developing multidisciplinary teams dedicated to the care of these patients has emerged. The key element of this approach is the recognition that the care paradigm in which Fontan patients simply see their cardiologist annually for an echocardiogram is no longer adequate or appropriate. Leaving behind the passive, reactive approach, screening for pathology in the early stages will likely afford an opportunity for intervention in some cases, or at minimum closer surveillance in situations where therapies lag behind diagnostic testing. A multidisciplinary standardized approach to screening and evaluation by subspecialists is critical in surveillance and management of Fontan associated multisystem aberrations.

Introduction

The current standard management strategy for patients born with single ventricle heart disease includes a series of palliative surgeries, culminating in the Fontan operation [1]. This procedure was first described by François Fontan in 1971 [1], yet more than four decades later we continue to uncover a variety of comorbidities in Fontan survivors involving multiple organ systems. Although the Fontan operation is successful in eliminating the mixing of desaturated systemic venous return from ventricular output, thus eliminating cyanosis, the desaturated systemic venous blood has to flow passively through the lungs, resulting in an obligate elevation in central venous pressure (CVP), typically in the range of 10–15 mmHg [2,3]. Apart from this elevation in CVP, in patients who have undergone Fontan operation, relative pulmonary hypertension, lack of pulsatile pulmonary blood flow and chronic low cardiac output state contribute to an unfavorable hemodynamic milieu. The comorbidities resulting from this disadvantageous physiology, including cardiac, pulmonary, gastrointestinal, hepatic and renal issues are thought to be related in large part to the chronic elevation in CVP [4]. In the early years of Fontan palliation, the primary focus of medical community was on optimizing the flow dynamics of the Fontan circuit by modifying the surgical approach. Currently, the extracardiac conduit Fontan is the most common variation of the Fontan surgery performed. More recently, the medical community has shifted its focus to long-term evaluation and management of late, multisystem complications associated with Fontan physiology. Many of these complications develop in the second decade of life [5], which has been unmasked in recent years due to a decrease in early mortality [[5], [6], [7], [8], [9]]. With the recognition that survivors of Fontan palliation are at risk for developing a wide breadth of complications, spanning multiple different organ systems, interest in developing multidisciplinary teams dedicated to the care of these patients has emerged [9].

Section snippets

Cardiac

Ventricular pump function has been demonstrated to be strong predictor of outcomes after Fontan operation [10]. Although ventricular systolic function is usually well preserved, relaxation abnormalities of the ventricular myocardium and diastolic dysfunction are thought to be common and often progressive. Atrioventricular valve regurgitation is another common issue that requires close attention and follow-up in Fontan patients. A dominant right ventricular morphology is more commonly associated

Protein-losing Enteropathy

Protein-losing enteropathy (PLE) is one of the dreaded complications following Fontan operation. The development of PLE, similar to plastic bronchitis, results from a breakdown in the integrity of the intestinal mucosa and leakage of protein-rich fluid into the bowel lumen, resulting in significant loss of protein [16]. An association with resistive indices in the superior mesenteric artery has been demonstrated [17], suggesting a role for gut hypoperfusion as well. The pathophysiology of PLE

Pulmonary

Plastic bronchitis, the formation of exudative airway casts [27,28], is a rare complication seen after Fontan operation. The casts can partially or completely obstruct the lower airways and can lead to life threatening hypoxemia. The cause of plastic bronchitis remains uncertain, but the commonly accepted working theory involves high lymphatic pressures leading to fibrin-rich fluid entering the airspace and solidifying to form casts. The incidence is 4–16% in post-Fontan patients [29]. The link

Bone Health, Growth and Puberty

Fontan survivors have multiple risk factors for abnormal growth and bone abnormalities. Compromised somatic growth and bone demineralization have both been documented after Fontan operation [[33], [34], [35]]. These problems are compounded in the setting of other comorbidities, such as PLE, where therapies often include systemic corticosteroids [36]. Chronic heart failure, nutritional deficiencies, vitamin D deficiencies, malabsorption, increase protein loss, chronic medication use (diuretics,

Development and Mental Health

With the increase in survival after staged palliation, developmental challenges have been widely recognized and phenotyped as patients with Fontan circulation interact more frequently with the school system [[38], [39], [40]]. Common abnormalities identified on neuropsychological assessment include problems with attention and executive functions, such as processing speed and impulsivity [39,41]. Patients with cyanotic heart disease are at risk for stroke [42], which adds an element of possible

Other Organ Systems

Children who have undergone serial operations involving cardiopulmonary bypass are also at risk for the development of acute kidney injury [45]. The etiologies of acute kidney injury after cardiac surgery include hemodynamic, inflammatory and nephrotoxic insults [46]. These episodes are a risk factor for the development of chronic kidney disease [45,47].

The Multidisciplinary Approach

Given the increasing evidence for the broad range of pathology spanning nearly every organ system, it seems less a question of whether or not a multidisciplinary approach is warranted for Fontan patients, but what testing and sub-specialists referrals are most vital to include in a multidisciplinary approach to long-term care of Fontan survivors [2]. The key element of this approach at the outset is recognition that the care paradigm in which Fontan patients simply see their cardiologist

References (48)

  • L. Mertens et al.

    Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group

    J Thorac Cardiovasc Surg

    (1998)
  • M.C. Schwartz et al.

    Hepatic pathology may develop before the Fontan operation in children with functional single ventricle: an autopsy study

    J Thorac Cardiovasc Surg

    (2012)
  • F.M. Wu et al.

    Liver health in adults with Fontan circulation: a multicenter cross-sectional study

    J Thorac Cardiovasc Surg

    (2017)
  • S.A. Hollander et al.

    Intermediate-term outcomes after combined heart-liver transplantation in children with a univentricular heart

    J Heart Lung Transplant

    (2013)
  • S.C. Greenway et al.

    Fontan-associated liver disease: implications for heart transplantation

    J Heart Lung Transplant

    (2016)
  • K.R. Schumacher et al.

    Fontan-associated protein-losing enteropathy and plastic bronchitis

    J Pediatr

    (2015)
  • C.M. Avitabile et al.

    A multifaceted approach to the management of plastic bronchitis after cavopulmonary palliation

    Ann Thorac Surg

    (2014)
  • C.M. Avitabile et al.

    Deficits in bone density and structure in children and young adults following Fontan palliation

    Bone

    (2015)
  • J. Rychik et al.

    Usefulness of corticosteroid therapy for protein-losing enteropathy after the Fontan procedure

    Am J Cardiol

    (1991)
  • A. Sarajuuri et al.

    Neurodevelopmental and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: related risk factor analysis

    J Thorac Cardiovasc Surg

    (2007)
  • A.J. Sinclair et al.

    Stroke in children with cardiac disease: report from the International Pediatric Stroke Study Group Symposium

    Pediatr Neurol

    (2015)
  • J.J. Blinder et al.

    Congenital heart surgery in infants: effects of acute kidney injury on outcomes

    J Thorac Cardiovasc Surg

    (2012)
  • F. Fontan et al.

    Surgical repair of tricuspid atresia

    Thorax

    (1971)
  • M. Gewillig et al.

    Pathophysiological aspects after cavopulmonary anastomosis

    Thorac Cardiovasc Surg

    (2000)
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    Authors declare there is no conflict of interest.

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