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Ventricular Inversion Treatment & Management

  • Author: Ira H Gessner, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Jan 16, 2015
 

Medical Care

Management of heart defects associated with ventricular inversion dictates the requirement for inpatient care.

Pharmacologic therapy in ventricular inversion depends on the type of associated heart defect and its effects on the patient. Treatment of an infant with a cyanotic lesion or congestive heart failure does not differ from treatment of a patient without ventricular inversion.

Indications to observe precautions against bacterial endocarditis depend on the patient's operative status.

Do not presume stability in patients without a clinically significant associated defect because almost all have at least minor degrees of tricuspid valve abnormality.

Patients with complete heart block may require a pacemaker.

Transcatheter therapy for associated defects is indicated less frequently in ventricular inversion than in analogous lesions in the noninverted heart.

Consultations

A pediatric cardiologist should evaluate the patient. If surgery is contemplated, a cardiovascular surgeon experienced in surgery of congenital heart defects should also examine the patient.

Transfer

Evaluation and treatment should occur at a center specializing in congenital heart disease.

Diet and activity

No special diet is required except as dictated by an associated defect.

The type of associated defect dictates activity restriction, if any.

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Surgical Care

The surgical care of associated defects in patients with ventricular inversion includes careful assessment of the risks and benefits.[4, 5, 6]

Elective correction may not be recommended for some defects when they occur with ventricular inversion in contrast to the same defect in a patient without ventricular inversion.

The management of clinically significant subpulmonic stenosis in patients with a large ventricular septal defect and ventricular inversion often is difficult, with a high probability of complete heart block and of a clinically significant residual obstruction. Some patients with these conditions can be treated with a combination of atrial rerouting of venous return by using the Senning technique, redirection of left ventricular outflow through the ventricular septal defect (VSD) to the anterior aorta (analogous to the Rastelli operation), and placement of a conduit from the right ventricle to the pulmonary artery to bypass the pulmonic obstruction.

Closure of a large VSD is also difficult because of the location of the defect and the problems encountered in approaching it.

For some patients, particularly those with a large VSD but without clinically significant pulmonic stenosis, combining an atrial switch (ie, Senning operation) and an arterial switch (ie, Jatene operation) is now increasingly recommended. This procedure results in the left ventricle pumping pulmonary venous blood to the aorta and the right ventricle pumping systemic venous blood to the lungs. Several institutions report highly successful results with this double-switch procedure. Indeed, some patients with right ventricular dysfunction due to tricuspid-valve regurgitation with no clinically significant additional abnormality have undergone successful double-switch surgery.[7]

A patient with right ventricular dysfunction, with or without significant tricuspid regurgitation, presents special problems. Tricuspid valve repair or replacement is more difficult to accomplish with good hemodynamic results. If left ventricle pressure is low, it may not tolerate the sudden requirement of systemic perfusion following the double switch procedure.[8] In such patients, preparation of the left ventricle has been successfully accomplished by first performing pulmonary artery banding. This may have detrimental effects on the left ventricle that appear many years later.

Ventricular inversion associated with an aorta arising from the left ventricle and an pulmonary artery arising from the right ventricle, thereby creating the physiology of simple transposition, can be managed by means of an atrial switch alone. This situation is one in which definitive surgery for ventricular inversion should be electively performed in the young infant.

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Contributor Information and Disclosures
Author

Ira H Gessner, MD Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine

Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Hugh D Allen, MD Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, Western Society for Pediatric Research, American College of Cardiology, American Heart Association, American Pediatric Society

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Jeffrey Allen Towbin, MD, MSc FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital

Jeffrey Allen Towbin, MD, MSc is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, Texas Pediatric Society, Cardiac Electrophysiology Society

Disclosure: Nothing to disclose.

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This right ventricular angiogram shows a patient with transposition of the great arteries. The aorta arises directly from the right-sided anterior right ventricle (10° left anterior oblique [LAO]).
 
 
 
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