Approach Considerations

Electrocardiographic findings are rarely diagnostic for double outlet right ventricle (DORV).

Common findings in a child with double outlet right ventricle include right ventricular hypertrophy, right axis deviation, and, occasionally, evidence of left ventricular hypertrophy.

Routine laboratory testing in patients with double outlet right ventricle includes the following:

Complete blood cell (CBC) count
Electrolyte levels
Renal profile
Hepatic function
Coagulation profile
Assessment of nutritional status, if indicated

Echocardiography of the heart and great vessels

Echocardiography generally provides enough information for accurate and adequate diagnosis, and provides the needed information to plan the surgical approach in neonates and young infants.

Sanders et al reported that standard transthoracic echocardiography (TTE) was used to diagnose conotruncal malformation in 109 of 113 infants.^[11]Of the 12 infants in whom double outlet right ventricle (DORV) was diagnosed and confirmed with angiography, 11 previously received a diagnosis based on subxiphoid two-dimensional echocardiography.

Echocardiography can be used to correctly identify the relative position of the great arteries, the degree of subsemilunar narrowing, the position of the ventricular septal defect (VSD), and the status of the mitral valve and left ventricle.

Cardiac angiography

Cardiac catheterization, once the criterion standard for confirming double outlet right ventricle, is now rarely required in the evaluation or preoperative planning of this cardiac disorder.

Angiography has several advantages, when indicated, such as the following:

In the older child with long-standing disease, hemodynamic parameters can be directly measured.
In the setting of possible aberrant coronary anatomy (3-5% of cases), the actual anatomic variation can be defined. This knowledge can affect surgical planning when Rastelli-type repair is contemplated.
In the setting of pulmonary vascular anomalies, angiography can help in defining the main pulmonary branches, the pulmonary vascular tree, and the collateral vessels to the lungs.

Magnetic resonance imaging (MRI)

MRI has been used in the diagnosis of double outlet right ventricle, but it is not yet a routine or well-established diagnostic modality forthis condition. MRI is useful to obtain additional anatomic information, such as the relationship of both ventricles.

Chest radiography

Regardless of the end of the clinical spectrum (tetralogy of Fallot [TOF] or transposition of the great arteries [TGA]) at which double outlet right ventricle occurs, findings on anteroposterior and lateral chest radiography depend on the degree of pulmonary (or subpulmonary) stenosis.

In the setting of severe stenosis, the pulmonary parenchyma is relatively oligemic, whereas in the setting of minimal pulmonary stenosis (especially with a Taussig-Bing heart), findings are likely to be consistent with congestive heart failure. Either way, the chest image shows cardiomegaly.

Computed tomography (CT) scanning

Preoperative CT scanning is potentially useful for identifying coronary artery anatomy in children with TOF or Fallot type of double outlet right ventricle.^[12] A study that assessed the incidence and diagnostic accuracy of preoperative cardiac CT scanning for identifying detailed coronary artery anatomy in 318 children with TOF or Fallot type of double outlet right ventricle found a 95% concordance between cardiac CT scanning and surgical findings, and a 96.9% diagnostic accuracy for cardiac CT scanning.^[12]

Treatment & Management

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Media Gallery

Double Outlet Right Ventricle Surgery. Double outlet right ventricle (DORV) with transposition of the great arteries accounts for 26% of cases of DORV. The aorta (AO) is anterior and to the right of the pulmonary artery (PA), and both arteries arise from the right ventricle (RV). The only outflow from the left ventricle (LV) is a ventricular septal defect (VSD), which diverts blood toward the RV. Pulmonary veins drain into the left atrium (LA) after blood has been oxygenated in the lungs (L). Systemic venous return is to the right atrium (RA).

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