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Double outlet right ventricle — Clinical Guidelines

Overview

Double outlet right ventricle (DORV) is a congenital heart defect where both the aorta and pulmonary artery arise predominantly from the right ventricle, necessitating surgical intervention to correct maldistribution of blood flow and ensure adequate systemic and pulmonary circulation 1.

Diagnosis

Imaging Techniques: Cardiac MRI, CT angiography, and echocardiography are essential for detailed anatomical assessment 1 2.

3D Modeling: Utilization of 3D printing enhances preoperative planning by accurately predicting surgical feasibility and technique 1.

Mixed-Reality Holograms: Emerging technology providing dynamic, interactive 3D visualization can improve diagnostic accuracy and surgical planning 2.

Management

Surgical Approaches:

- Biventricular Repair: Aimed for when feasible, involving reconstruction of ventricular septum and outflow tracts 1 5 8. - Transaortic Approach: Considered for better visualization in cases with complex anatomy, such as subaortic VSD 5 8. - Partial Biventricular Repair: Used in cases with left ventricular hypoplasia, incorporating modified biventricular repair techniques 7.

Transcatheter Interventions:

- Ventricular Septal Defect Creation/Enlargement: Transcatheter techniques can palliate restrictive VSDs, reducing surgical risks 3.

Postoperative Management:

- Pacemaker Optimization: Utilize advanced echocardiographic tools like tissue synchronization imaging and speckle-tracking strain analysis for optimizing ventricular function 4.

Special Populations

Pediatrics: Early surgical intervention is critical, with considerations for biventricular versus univentricular repair based on anatomy 1 3 7.

Comorbidities: Subaortic stenosis post-biventricular repair requires vigilant monitoring and potential reoperation 6.

Key Recommendations

Utilize 3D printing for preoperative planning to accurately predict surgical feasibility and technique in DORV (Evidence: Moderate 1).

Consider mixed-reality holograms as a novel tool to enhance diagnostic accuracy and surgical planning (Evidence: Weak 2).

Employ transcatheter techniques for palliation in cases of restrictive VSD to minimize surgical risks (Evidence: Weak 3).

Optimize postoperative care with advanced echocardiographic methods to manage cardiac function and pacemaker settings (Evidence: Moderate 4).

Evaluate the transaortic approach for complex anatomical scenarios where standard ventriculotomy approaches are limited (Evidence: Expert opinion 5 8).

References

1 Hoogerbeets SF, Roest AAW, Valverde I, Gomez-Ciriza G, Kroft L, Hazekamp MG. Printed Models for Better Prediction of Surgery in Patients with Double Outlet Right Ventricle. Pediatric cardiology 2026. link 2 Ye W, Zhang X, Li T, Luo C, Yang L. Mixed-reality hologram for diagnosis and surgical planning of double outlet of the right ventricle: a pilot study. Clinical radiology 2021. link 3 Lin CH, Huddleston C, Balzer DT. Transcatheter ventricular septal defect (VSD) creation for restrictive VSD in double-outlet right ventricle. Pediatric cardiology 2013. link 4 Jeewa A, Potts MT, Sanatani S, Duncan WJ. Echocardiographic tools for pacemaker optimization of ventricular function in an infant following surgical repair for double outlet right ventricle. The Canadian journal of cardiology 2010. link70472-4) 5 Sakamoto K, Charpentier A, Popescu S, De Geeter B, Eisenmann B. Transaortic approach in double-outlet right ventricle with subaortic ventricular septal defect. The Annals of thoracic surgery 1997. link00690-5) 6 Belli E, Serraf A, Lacour-Gayet F, Inamo J, Houyel L, Bruniaux J et al.. Surgical treatment of subaortic stenosis after biventricular repair of double-outlet right ventricle. The Journal of thoracic and cardiovascular surgery 1996. link70016-8) 7 Day R, Laks H, Milgalter E, Billingsley A, Rosengart R, George B. Partial biventricular repair for double-outlet right ventricle with left ventricular hypoplasia. The Annals of thoracic surgery 1990. link90890-i) 8 Harlan BJ, Cooley DA. Transaortic repair of double-outlet right ventricle with situs versus, l-loop, l-malposition (I,L,L), subaortic ventricular septal defect, and associated anomalies. The Journal of thoracic and cardiovascular surgery 1976. link

Double outlet right ventricle