Table 2

Failed Fontan circulation

Condition	Incidence	Manifestations	Aetiologies	Treatments
Early failure	3%	Low cardiac output, pleural effusions, chylothoraces, ascites, hepatomegaly	Pulmonary vasculature abnormalities Incessant/refractory atrial tachycardia Residual obstruction related to surgical technique	Early evaluation to correct obstructions, terminate tachycardia Fontan takedown Recreate systemic to pulmonary blood flow Cardiac transplantation
Late failure Lymphatic dysfunction Protein-losing enteropathy (PLE)	2–13%	Ascites, peripheral oedema, pleural effusions, diarrhoea, malabsorption of fat, hypoalbuminaemia	Unknown, but associated with: Low cardiac output Mesenteric vascular flow abnormalities Intestinal cellular wall damage Autoimmune reactions Intestinal lymphangiectasia Risk factors: prolonged postoperative chest tube drainage, systemic right ventricle	Nutritional support with protein and medium chain triglycerides Optimise cardiac output: atrial rhythm, pulmonary vasodilator therapy, afterload reduction, atrial fenestration Enteric steroids Diuretics Heparin High dose aldactone Intravenous albumin and γ-globulin infusions Immunosuppression Cardiac transplantation
Plastic bronchitis	<2%	Tachypnoea, cough, wheezing, expectoration of bronchial casts	Unknown; associated with leakage of proteinaceous material into the airways resulting in bronchial casts	Urgent bronchial lavagePulmonary vasodilatorsCardiac transplantation
Primary ventricular dysfunction	∼7–10%	Progressive exercise intolerance, AV valve insufficiency, hepatomegaly, ascites	Chronic hypertrophy, abnormal ventricular morphology (systemic right or indeterminate ventricle), older age at repair, prolonged cyanosis or volume overload, myocardial perfusion abnormalities	ACE inhibition Pulmonary vasodilators Calcium channel blockers for diastolic dysfunction β-blockers Multisite pacing Cardiac transplantation
Progressive increase in pulmonary resistance	Unknown	Hypoxaemia	Pulmonary arteriovenous malformations, inadequate hepatic vein effluent, lack of pulsatile flow	Pulmonary vasodilators; stenting of pulmonary arterial narrowing
Hepato-renal insufficiency			Low cardiac output, sepsis	Supportive care, optimise cardiac output, high mortality
Hepatic failure	Unknown	Hepatomegaly, ascites; hepatocellular carcinoma	Progression of chronic cardiac cirrhosis	Cardiac and liver transplantation