Fontan failure and the role of pulmonary vasodilator therapy
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Journal of Congenital Cardiology
CASE REPORT
Open Access
Fontan failure and the role of pulmonary vasodilator therapy Paul Clift1,2
Abstract Background: The Fontan circulation is a palliative procedure for patients born with a single ventricle physiology. The Fontan circulation is associated with significant late morbidity commonly including atrial arrhythmias. Case presentation: We report a case of an adult Fontan patient who developed protein losing enteropathy (PLE) following persistent atrial arrhythmias. Treatment with a pulmonary vasodilator, sildenafil, resolved the PLE. Conclusions: The importance of a low pulmonary vascular resistance to maintain wellbeing in the Fontan patient is discussed, as is the role of pulmonary vasodilators. Keywords: Fontan circulation, Pulmonary vasodilators, Pulmonary vascular resistance, Protein losing enteropathy
Background It is more than 50 years since Francis Fontan and the Kreutzer brothers performed their initial series of surgical palliation of the single ventricle circulation, now known as the Fontan operation [1, 2]. The concept of bypassing the systemic venous return from the systemic ventricle and allowing passive flow to the pulmonary arteries was simple and remarkably effective. Prior to Fontan surgery the life expectancy for patients with a single ventricle circulation was poor and patients rarely survived beyond childhood years, the challenges of profound cyanosis and a volume loaded single ventricle leading to circulatory failure in early life. Subsequent modifications of the procedure have improved outcomes [3, 4] and patients routinely survive to adult life, even in the most complex forms of single ventricle circulations [5]. The Fontan circulation is unique and poses many challenges (Fig. 1a and b). Patients, whilst overall well, are prone to developing arrhythmias and exercise Correspondence: [email protected] 1 Department of Cardiology, Queen Elizabeth Hospital, Birmingham, United Kingdom 2 Adult Congenital Heart Disease Unit, Queen Elizabeth Hospital, B15 2TH Birmingham, UK
intolerance. Critical to a healthy Fontan circulation is the rapid transit of the systemic venous return through the pulmonary circulation in order to maintain pre-load in the systemic circulation. Therefore, even small increases in the pulmonary vascular resistance may lead to a deterioration in exercise capacity. In the classical atriopulmonary Fontan operation, a rise in pulmonary resistance may lead to increased right atrial chamber pressures and, in combination with multiple surgical scars, predispose to atrial arrhythmia. Such arrhythmias may become life threatening in this population and can lead to rapid clinical deterioration. Moreover, elevation of the systemic venous pressure is an unavoidable feature of the Fontan circulation and is increasingly recognised as a contributory factor to morbidity, including arrhythmia, hepatic fibrosis and the development of protein losing enteropathy (PLE). We report a case in which pulmonary vasodilator therapy was used successfully
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