Abstract
Fontan procedure surgically palliates abnormal anatomy in functional single ventricle congenital heart disease (CHD), a rare, serious condition. Post-surgery, patients may experience exercise intolerance, thrombosis, arrhythmias, and liver disease; clinical worsening (CW) related to pulmonary hypertension can be significant even with small increases in pulmonary vascular resistance (PVR). No therapies are approved to modulate PVR post-Fontan. We explored published evidence on post-Fontan clinical, humanistic, and economic burden.
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