Table 2

Failed Fontan circulation

Early failure3%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 castsUnknown; associated with leakage of proteinaceous material into the airways resulting in bronchial castsUrgent bronchial lavagePulmonary vasodilatorsCardiac transplantation
Primary ventricular dysfunction∼7–10%Progressive exercise intolerance, AV valve insufficiency, hepatomegaly, ascitesChronic 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 resistanceUnknownHypoxaemiaPulmonary arteriovenous malformations, inadequate hepatic vein effluent, lack of pulsatile flowPulmonary vasodilators; stenting of pulmonary arterial narrowing
Hepato-renal insufficiencyLow cardiac output, sepsisSupportive care, optimise cardiac output, high mortality
Hepatic failureUnknownHepatomegaly, ascites; hepatocellular carcinomaProgression of chronic cardiac cirrhosisCardiac and liver transplantation