Table 3

Long term Fontan surveillance

ParametersMonitoring
Constitutional

  • Adequate growth in childhood

  • Weight or muscle loss in adulthood

  • Avoid overweight

  • Blood pressure: normotensive

  • Functional classification,

  • Aerobic activity

  • Daily napping

  • Aerobic activity

  • Exercise testing

Cardiac haemodynamics

  • Heart size

  • Atrial dilatation

  • Atriopulmonary anastomosis

  • Pulmonary narrowing, distortion

  • Pulmonary venous or atrioventricular (AV) inflow obstruction

  • AV valve regurgitation or stenosis

  • Aortic insufficiency

  • Ventricular outflow obstruction

  • Arch obstruction

  • Ventricular function

  • Echocardiogram

  • Periodic chest radiograph

  • Cardiac MRI or CT

Rhythm

  • Presence of atrial versus junctional rhythm

  • Resting heart rate

  • Chronotropic response to exertion

  • Arrhythmia development

Periodic 24 h ambulatory monitoring; event monitoring
PulmonaryHypoxaemiaVascular resistanceOxygen saturation, resting and exertional
EndocrineThyroid functionThyroid stimulating hormone (TSH), thyroxine (T4)
RenalBlood urea nitrogen, creatinine
Hepatic

  • Hepatomegaly

  • Cardiac cirrhosis

  • Synthetic function

  • Liver function: enzymes, coagulation

  • Abdominal ultrasound, CT or MR

  • Liver biopsy

  • Serum α-fetoprotein

GastrointestinalBloating, distension, diarrhoea, ascites, gallstonesStool α-1 anti-trypsin
HaematologicAnaemia, polycythaemia, thrombocytopeniaBlood count, platelets
MetabolicB-type natriuretic peptide, albumin, alkaline phosphatase
Neurologic

  • Cerebrovascular accident

  • Depression