Two-ventricle repairs in the unbalanced atrioventricular canal defect spectrum with midterm follow-up John E. Foker, MD, PhD, James M. Berry, RDMS, Jeffrey.

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Two-ventricle repairs in the unbalanced atrioventricular canal defect spectrum with midterm follow-up John E. Foker, MD, PhD, James M. Berry, RDMS, Jeffrey M. Vinocur, MD, Brian A. Harvey, BA, Lee A. Pyles, MD The Journal of Thoracic and Cardiovascular Surgery Volume 146, Issue 4, Pages 854-860.e3 (October 2013) DOI: 10.1016/j.jtcvs.2013.05.013 Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure 1 Schematic depiction of the creation of a second mitral valve from a large tricuspid valve in an unbalanced atrioventricular (AV) canal defect. On the left, the presence of a small (rudimentary) mitral valve means the large valve is a tricuspid valve, rather than a common AV valve. The septal plane, therefore, would be as depicted. With a large inlet ventricular septal defect (VSD), however, the ventricular closure patch can be used to shift the septum and create a second mitral valve. On the right, the new septal plane created by the VSD closure patch creates both a second mitral valve (Mitral 2) and leaves an adequate tricuspid valve. The septal line is also used for the atrial septal closure patch. This repair was straightforward. The Journal of Thoracic and Cardiovascular Surgery 2013 146, 854-860.e3DOI: (10.1016/j.jtcvs.2013.05.013) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure 2 Initial ventricular and atrioventricular (AV) valve hypoplasia in individual patients with unbalanced AV canal (UAVC). Vertical bar, an individual patient; open diamond, the z value of the hypoplastic ventricle (HV); and solid circle, the z value of the hypoplastic AV valve (HAVV). These show that there is little uniformity between the degree of hypoplasia in the individual’s hypoplastic ventricle and AV valve. The Journal of Thoracic and Cardiovascular Surgery 2013 146, 854-860.e3DOI: (10.1016/j.jtcvs.2013.05.013) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure 3 Left ventricular growth induction by staged repair shown by sequential echo studies in a patient with unbalanced atrioventricular canal. A, Preoperative study (aged 2 months) shows a non–apex-forming left ventricle (LV) with an indexed volume of 4.5 mL and a z value of −7.1. B, After a period of growth induction (aged 5 months) and before the second stage of repair, the LV volume was 11.6 mL and the z value was −2.4. C, Follow-up of completed repair (aged 13 years), with an LV volume of 83 mL and a z value of −1.9. RV, Right ventricle; RA, right atrium; LA, left atrium. The Journal of Thoracic and Cardiovascular Surgery 2013 146, 854-860.e3DOI: (10.1016/j.jtcvs.2013.05.013) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure 4 Growth in ventricular volumes for 16 patients with unbalanced atrioventricular canal. The initial and midterm follow-up echo studies show that stable ventricular growth has occurred in these patients. (From the 23 patients originally, 3 deaths, 2 lost to follow-up, and 2 who had an inadequate echo study left the 16 patients depicted.) A, Indexed ventricular volumes. Bar, an individual patient, depicting the change from initial (preoperative) to follow-up indexed volumes after growth induction. B, Ventricular volume z values. Bar, change in the z values of the ventricular volumes with growth for an individual patient. The 2 patients who did not reach a z value of −2.0 had only a single-plane follow-up echo study, a technique that results in lower-volume estimates. The Journal of Thoracic and Cardiovascular Surgery 2013 146, 854-860.e3DOI: (10.1016/j.jtcvs.2013.05.013) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure 5 Rapid ventricular growth induction by staged repair for 5 patients with unbalanced atrioventricular canal. For patients with more severe hypoplasia, a staged repair was used to induce catch-up growth of these ventricles. Bar, an individual patient tracking the initial and postgrowth induction values; and solid diamonds, volume z values at those time points. These patients were followed up more closely, with echo studies within 5 months, and usually much sooner after the growth-inducing procedure. These results show that catch-up growth was relatively rapid and the 2-ventricle repair was completed within a few months. The patients depicted were those who had satisfactory serial echo studies available. The Journal of Thoracic and Cardiovascular Surgery 2013 146, 854-860.e3DOI: (10.1016/j.jtcvs.2013.05.013) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure 6 Growth in valve area for 9 patients with unbalanced atrioventricular canal. Bar, an individual patient with the initial and follow-up z values for the size of the hypoplastic AV valves (solid diamonds). The catch-up growth of the hypoplastic AV valves is somewhat slower than for the growth of the ventricles (data not shown), but they reliably reach and maintain a normal size. The Journal of Thoracic and Cardiovascular Surgery 2013 146, 854-860.e3DOI: (10.1016/j.jtcvs.2013.05.013) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions

Figure E1 Doppler echocardiogram showing inflow through the created mitral valve (mitral 2) into the left ventricle (LV). The red Doppler stream into the LV through the newly created mitral valve (A) appears larger than the stream through the native, but rudimentary, mitral valve (B). Together, they allow a satisfactory output, giving time for the hypoplastic LV to grow. At this point, a small atrial septal defect and a small ventricular septal defect are present, which also increase patient stability. The Journal of Thoracic and Cardiovascular Surgery 2013 146, 854-860.e3DOI: (10.1016/j.jtcvs.2013.05.013) Copyright © 2013 The American Association for Thoracic Surgery Terms and Conditions