Extracardiac Conduit Subocclusion in an Adult Patient With a Fontan Circulation
Emanuela C D'Angelo1, Rosalinda Palmieri1, Veronica Lisignoli1
1Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Insights
Extracardiac conduit obstruction can cause Fontan failure. Multimodality imaging is crucial for diagnosing calcific masses in Fontan patients presenting with heart failure symptoms.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Cardiac Surgery
Background:
- Fontan circulation is a complex surgical repair for single-ventricle congenital heart defects.
- Conduit obstruction is a known complication that can lead to Fontan failure.
Observation:
- A Fontan patient presented with exertional dyspnea and peripheral edema, indicative of heart failure.
- Echocardiography revealed a hyperechogenic mass within the extracardiac conduit.
Findings:
- Computed tomography and cardiac MRI confirmed severe conduit obstruction due to a calcific mass.
- Extensive collateral circulation was identified, suggesting chronic obstruction.
Implications:
- Prompt diagnosis and surgical intervention are vital for managing Fontan failure.
- Multimodality imaging is essential for accurate assessment and surgical planning in Fontan patients.
- Suspecting conduit obstruction in Fontan patients with heart failure symptoms is critical.
Background:
Conduit obstruction is a complication of patients with Fontan circulation.
Case Summary:
This case report describes a Fontan patient who presented with exertional dyspnea and bilateral peripheral edema. Echocardiography identified a hyperechogenic structure within the extracardiac conduit, leading to further investigations with computed tomography and cardiac magnetic resonance imaging. These studies confirmed severe conduit obstruction due to a calcific mass and demonstrated extensive collateral circulation.
Management:
After a multidisciplinary team discussion, surgical extracardiac conduit replacement was performed. Postoperatively, the patient developed a pleural effusion, managed with surgical drainage and octreotide treatment.
Discussion:
Fontan failure could be due to many etiologies. Extracardiac conduit obstruction should be suspected in Fontan patients with heart failure symptoms and investigated with multimodality imaging techniques.
Take-Home Message:
This case highlights the necessity of a differential diagnosis in Fontan failure patients and the need of multimodal imaging to reach definitive information necessary for a correct surgical planning.
