Double-Inlet Left Ventricle

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Surgery

Background:

• Double-inlet left ventricle (DILV) is the most common univentricular atrioventricular connection.
• It involves a single functioning ventricle receiving both atrioventricular valves, often with transposed great vessels and pulmonary stenosis.

Purpose of the Study:

• To review the diagnosis, pathophysiology, and management of DILV.
• To describe the staged surgical approach, including the Fontan procedure, for DILV patients.

Main Methods:

• Diagnosis primarily relies on echocardiography (echo-Doppler studies).
• Review of current medical, catheter interventional, and surgical treatment practices.

Main Results:

• DILV anatomy and pathophysiology are well-defined by echo-Doppler.
• Staged total cavo-pulmonary connection (Fontan procedure) is the standard surgical treatment.
• Inter-stage mortality and post-Fontan complications are recognized challenges.

Conclusions:

• DILV is accurately diagnosed using echo-Doppler.
• Current treatment strategies, including staged surgical palliation, offer effective management for DILV.