Management of Double Outlet Right Ventricle (DORV)

Anatomic Classification and Surgical Strategy

The American College of Cardiology recommends that management of DORV must be individualized based on the specific anatomic variant, with surgical approaches including VSD closure with intraventricular tunneling, right ventricle-to-pulmonary artery conduit placement, Rastelli-type repair, or single-ventricle palliation depending on the underlying anatomy 1, 2, 3, 4, 5
The surgical approach to DORV is fundamentally determined by the relationship between the ventricular septal defect (VSD) and the great arteries, with the American College of Cardiology suggesting that this relationship guides the choice of surgical strategy 3, 4, 5
When the aorta is closely related to the VSD, management follows principles similar to tetralogy of Fallot repair, according to the American Heart Association 1, 2, 6, 3
If right ventricular outflow tract obstruction exists, augmentation of the RVOT or conduit placement from RV to pulmonary artery is necessary, as recommended by the American College of Cardiology 3
DORV patients have a higher risk of residual left ventricular outflow tract obstruction compared to standard TOF, highlighting the need for careful surveillance, as noted by the American Heart Association 1, 2, 3

The American Heart Association recommends that adults with septal or great artery shunts require periodic screening for pulmonary hypertension with TTE (Class I, Level B-NR) 3
Cardiac catheterization to assess pulmonary vascular hemodynamics is recommended for patients with clinical symptoms, signs, or echocardiographic findings suggestive of pulmonary hypertension (Class I, Level B-NR), according to the American College of Cardiology 2
Patients with pulmonary vascular resistance ≥2.5 Wood units (>4 Wood units×m²) should be assessed collaboratively by an ACHD cardiologist and pulmonary hypertension expert (Class I, Level B-NR), as suggested by the American College of Cardiology 7
For Stage C patients, the American Heart Association recommends ACHD cardiologist visits every 6-12 months, ECG every 12 months, transthoracic echocardiography every 12 months, pulse oximetry at each visit, CMR imaging as needed, exercise testing every 6-12 months, and cardiac catheterization as clinically indicated 1, 2, 6
For Stage D patients, the American Heart Association recommends ACHD cardiologist visits every 3-6 months, ECG every 12 months, transthoracic echocardiography every 12 months, pulse oximetry at each visit, CMR imaging as needed, exercise testing every 6-12 months, and cardiac catheterization as clinically indicated 1, 2, 6

Hemodynamic assessment is beneficial before or at the time of shunt closure to assess suitability for closure (Class I, Level B-NR), according to the American College of Cardiology 3
Cardiac catheterization is recommended when clinical symptoms, signs, or echocardiographic findings suggest pulmonary hypertension (Class I, Level B-NR), as noted by the American College of Cardiology 3
BNP, chest x-ray, 6-minute walk test, and cardiac catheterization are useful for initial and follow-up evaluation of ACHD patients with pulmonary arterial hypertension (Class I, Level C-EO), as suggested by the American Heart Association 6

All DORV patients should be managed in specialized ACHD centers with access to congenital cardiac surgeons, ACHD cardiologists, cardiac anesthesiologists with CHD expertise, and advanced imaging capabilities including CMR and CCT, as recommended by the American College of Cardiology 7