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. 2012 Jul 15;98(14):1098–1104. doi: 10.1136/heartjnl-2011-301133

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