Overview
Double inlet ventricle (DIV) is a congenital heart defect characterized by two functionally univentricular circulations receiving blood from separate atrioventricular valves, often requiring surgical palliation such as the Fontan procedure to establish a functional single ventricle pathway.Diagnosis
Echocardiography: Essential for initial diagnosis, assessing ventricular morphology, and evaluating ventricular function 1.
Cardiac Catheterization: Provides detailed hemodynamic assessment and is crucial post-Fontan for evaluating ventricular performance 1.
Radionuclide Studies: Useful for assessing diastolic filling and ventricular function post-operatively 1.Management
Fontan Procedure: Primary surgical intervention to establish a total cavopulmonary connection, optimizing systemic venous drainage 1.
Medical Management: Focus on managing symptoms and complications such as arrhythmias, heart failure, and thromboembolic risk 1.
Regular Monitoring: Includes echocardiograms and clinical assessments to monitor ventricular function and overall cardiac status 1.Special Populations
Pediatrics: Early surgical intervention is critical; postoperative monitoring focuses on ventricular performance and growth 1.
Morphology Impact: Patients with dominant left ventricular morphology post-Fontan may exhibit altered diastolic filling characteristics compared to those with dominant right ventricular morphology 1.Key Recommendations
Post-Fontan Evaluation: Regular assessment of ventricular diastolic filling and mass/volume ratio is crucial for patients with DIV, particularly noting differences based on ventricular dominance 1 (Evidence: Moderate).
Surgical Timing: Early surgical palliation (Fontan procedure) is recommended to optimize long-term outcomes in pediatric patients with DIV 1 (Evidence: Moderate).
Morphological Considerations: Clinicians should be aware of the potential for elevated mass/volume ratios in patients with dominant left ventricular morphology post-Fontan, guiding tailored follow-up strategies 1 (Evidence: Moderate).References
1 Akagi T, Benson LN, Gilday DL, Ash J, Green M, Williams WG et al.. Influence of ventricular morphology on diastolic filling performance in double-inlet ventricle after the Fontan procedure. Journal of the American College of Cardiology 1993. link90784-x)