Double Outlet Right Ventricle, With Transposition Follow-up

  • Author: M Silvana Horenstein, MD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Aug 11, 2010
 

Further Inpatient Care

Maintain patency of the ductus arteriosus with prostaglandin E1 in newborns with markedly diminished pulmonary blood flow (PBF) from severe pulmonary valve stenosis (PS). In newborns with double outlet right ventricle (DORV) and transposition of the great arteries who have subpulmonic ventricular septal defect (VSD), performing balloon atrial septostomy to enhance mixing of systemic and pulmonary circulations until surgery can be performed may be necessary.

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Further Outpatient Care

Provide follow-up care every 6-12 months for the first few years after surgery to detect complications of surgery that may include arrhythmias (eg, persistent atrial tachycardias, complex ventricular ectopy) and stenosis or partial obstruction, or both, of the interventricular tunnel.

Because arrhythmias result in morbidity, mortality, or both, patients may require long-term antiarrhythmic medication or may be candidates for radiofrequency ablation of an arrhythmogenic focus or circuit.

Interventricular tunnel obstructions may occur without clinical manifestations. In patients with severe left ventricular outflow obstruction, patients with tunnel obstruction may present with left ventricular failure. As many as 20% of patients who have undergone surgery for DORV require reoperation.

In all patients, subacute bacterial endocarditis prophylaxis is required.

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Transfer

Because surgery in these patients often is technically demanding, strongly consider referring these patients to a center with a large pediatric cardiac surgical program.

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Prognosis

Improvement in surgical techniques in recent years has resulted in good outcomes for most patients born with congenital heart disease (CHD). Prognosis for infants born with DORV and transposition of the great arteries is generally good, although it is dependent on specific anatomy. For example, patients with DORV and transposition of the great arteries with a subaortic VSD and no other anatomic abnormalities (eg, left ventricular hypoplasia) are likely to do well after surgery. Patients with restrictive VSD may not do as well because this is a particularly difficult problem. Enlargement of VSD is difficult and likely to result in complications, such as conduction abnormalities (atrioventricular [AV] block).

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Patient Education

For excellent patient education materials, please refer to eMedicine's Heart Center. All these materials may be printed free of charge.

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Contributor Information and Disclosures
Author

M Silvana Horenstein, MD  Assistant Professor, Department of Pediatrics, University of Texas Medical School Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc

M Silvana Horenstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Michael L Epstein, MD  Director, Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Michigan; Professor, Wayne State University School of Medicine

Michael L Epstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Juan Carlos Alejos, MD  Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation

Disclosure: Actelion Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Julian M Stewart, MD, PhD  Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College

Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM  Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine

Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

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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).
This is an angiogram obtained during catheterization of a patient with double outlet right ventricle (DORV) with transposition of the great arteries. Injection of contrast though the catheter (arrow) into the left ventricle (LV) shows that blood is directed toward the right ventricle (RV) through a remote or doubly committed ventricular septal defect (VSD). The aorta (AO) is anterior to the pulmonary artery (PA) and both clearly arise from the RV.
This is an angiogram obtained during catheterization of a patient with double outlet right ventricle (DORV) with transposition of the great arteries (see Media file 2). Blood fills the aorta (AO) and pulmonary artery (PA) almost simultaneously, which is another indicator of a remote or doubly committed ventricular septal defect (VSD) (curved arrow). LV indicates the left ventricle, RV indicates the right ventricle, and the small arrow to the left indicates the catheter.
 
 
 
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