Hypoplastic Left Heart Syndrome in an Adult: CT Findings After Fontan Palliation
A radiologist-focused review of HLHS, Fontan anatomy, absent IVC with azygos continuation, and Fontan-associated liver disease. A 25-Year-Old Man With Persistent Chest Pain Edited by ScholarGen MediAI Team Executive Answer A 25-year-old man presented with persistent chest pain, and contrast-enhanced cardiac CT demonstrated the characteristic anatomy of hypoplastic left heart syndrome (HLHS) after Fontan palliation. The key findings include a severely underdeveloped left-sided cardiac system, mitral stenosis, a lateral-tunnel Fontan pathway, a widely patent atrial septal communication, and marked right ventricular enlargement. The CT also shows a prominent systemic venous anomaly consistent with an absent inferior vena cava (IVC) with azygos continuation . An additional upper-abdominal image shows an irregular hepatic contour consistent with cirrhosis, illustrating an important long-term complication of Fontan physiology. Why This Case Matters Hypoplastic left h...