Managing adult Fontan patients: where do we stand?

Abstract

The Fontan operation is performed as a palliative procedure to improve survival in infants born with a functionally univentricular circulation. The success of the operation is demonstrated by a growing adult Fontan population that exists with this unique physiology. Late follow-up has demonstrated expected and unexpected sequelae, and has shown multisystem effects of this circulation. This review discusses the challenges of managing the late complications in terms of understanding this unique physiology and the innovative therapeutic interventions that are being investigated. The challenge remains to maintain quality of life for adult survivors, as well as extending life expectancy.

Successful Treatment of Protein-Losing Enteropathy and Plastic Bronchitis by Biphasic Cuirass Ventilation in a Patient with Failing Fontan Circulation

“…2) Although PLE may successfully resolve after cardiac transplantation, early survival rate is lower in patients with PLE than those without PLE. 2,5) Medical therapies include unfractionated heparin, high-dose anti-aldosterone therapy, oral budesonide, loperamide, and targeted pulmonary vasodilators (nitric oxide, prostacyclin, and endothelin), but none are reliably effective. 2,5) Although the precise mechanisms of PLE onset remain unclear, high CVP is thought to be closely associated with the onset.…”

“…2,5) Medical therapies include unfractionated heparin, high-dose anti-aldosterone therapy, oral budesonide, loperamide, and targeted pulmonary vasodilators (nitric oxide, prostacyclin, and endothelin), but none are reliably effective. 2,5) Although the precise mechanisms of PLE onset remain unclear, high CVP is thought to be closely associated with the onset. 2) It is now well known that spontaneous inspiration enhances pulmonary blood flow and stroke volume in Fontan circulation.…”

“…1,2,4) Protein-losing enteropathy (PLE) is a characteristic feature of Fontan failure, leading to peripheral edema, ascites, pleural effusions, malabsorption, and loss of immunoglobulins. 5) Various therapies have been tried for the treatment of PLE, but the management strategy for this complex pathophysiology is not yet established. 2,5) Biphasic cuirass ventilation (BCV), a form of noninvasive extrathoracic mechanical ventilation, is an advanced model of the iron lung.…”

“…5) Various therapies have been tried for the treatment of PLE, but the management strategy for this complex pathophysiology is not yet established. 2,5) Biphasic cuirass ventilation (BCV), a form of noninvasive extrathoracic mechanical ventilation, is an advanced model of the iron lung. 6) It consists of a cuirass and a pressure source capable of providing both positive and negative pressure, which supports spontaneous breathing in a more physiological manner than does positive pressure ventilation.…”

The revolution of pulmonary arterial hypertension

“…RAINA and HUMBERT [8] discuss the importance of risk factors in PAH development, while SIMONNEAU et al [9] offer an extraordinary overview of future research prospects, outlining the potential targets for new therapeutic approaches to PAH including mitochondrial dysfunction, iron deficiency, pulmonary artery denervation and others. Two further papers address special conditions such as the management of adult patients after the Fontan procedure [10] and the risks and difficulties of managing PAH in pregnant women [11]. This series of cutting-edge papers begins with an article by MEHTA and VACHIÉRY [12] emphasising the importance of making the right diagnosis of pulmonary hypertension, and discussing illustrative cases of great educational value (figure 2).…”