Double Outlet Right Ventricle With Transposition Differential Diagnoses

Updated: Jan 29, 2019
  • Author: M Silvana Horenstein, MD; Chief Editor: Syamasundar Rao Patnana, MD  more...
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Diagnostic Considerations

Other problems to consider

Distinguish double outlet right ventricle (DORV), with or without transposition of the great arteries, and subaortic ventricular septal defect (VSD) from isolated VSD.

DORV with pulmonary valve stenosis (PS) may have a presentation similar to that of tetralogy of Fallot (TOF).

DORV with subpulmonary VSD but without PS may have a presentation similar to that of transposition of the great arteries with VSD.

Important considerations

Medicolegal pitfalls in caring for patients with double outlet right ventricle and transposition of the great arteries are similar to those for any patient with congenital heart disease (CHD).

Failure to make the correct diagnosis is of paramount importance. The correct treatment plan can be determined only if all anatomic details are known. Misdiagnosis can lead to inappropriate care.

Another issue is surgery. Because most of these patients do well when medical/surgical care is administered at a center with considerable experience in caring for infants with CHD, referral to such a center provides the best opportunity for a good long-term outcome.

The physician must be familiar with the possible complications that may result from surgery and be able to treat complications resulting from surgery appropriately.

Prenatal diagnosis can be made with detail in most cases of DORV [11] and was found to be useful in providing parents adequate counseling regarding the type of surgery required and the chances of survival of the fetus with DORV (survival is poor in patients with extracardiac or chromosomal anomalies). [12]

Special concerns

Subaortic or subpulmonary VSD without PS

If left unrepaired, these infants develop congestive heart failure (CHF) from pulmonary overcirculation, which evolves into pulmonary vascular obstructive disease.

Subaortic or subpulmonary VSD with PS

If left untreated, complications develop, including cyanosis (leading to polycythemia) and pardoxical embolism, which can lead to stroke.

Differential Diagnoses