Ventricular Inversion Treatment & Management

Updated: Jan 28, 2021
  • Author: Ira H Gessner, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Treatment

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. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

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, 7, 8]

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. [9] Indeed, some patients with right ventricular dysfunction due to tricuspid-valve regurgitation with no clinically significant additional abnormality have undergone successful double-switch surgery. [10]

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. [11] 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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Long-Term Monitoring

All patients with ventricular inversion, including those with no clinically significant associated defect, require regular follow-up by a pediatric cardiologist or an adult cardiologist who is thoroughly familiar with congenital heart disease. A permanent risk of developing complete heart block and uncertain long-term stability of both the tricuspid valve and right ventricle dictate this need.

Patients with even mild degrees of clinically apparent tricuspid valve regurgitation require follow-up at regular intervals, no longer than yearly, because this abnormality tends to be progressive.

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