Surgical Correction of Congenital Supravalvular Aortic Stenosis

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Presentation transcript:

Surgical Correction of Congenital Supravalvular Aortic Stenosis Julia S. Donald, MD, Tyson A. Fricke, MBBS, Sophie Griffiths, BSc, Igor E. Konstantinov, MD, PhD Operative Techniques in Thoracic and Cardiovascular Surgery Volume 21, Issue 3, Pages 202-210 (September 2016) DOI: 10.1053/j.optechstcvs.2017.05.003 Copyright © 2017 Terms and Conditions

Figure 1 (A) One should keep in mind that supravalvular aortic stenosis is almost always associated with an excessive thickness of the aortic wall typically in the proximal ascending aorta at the level of sinotubular junction. RCA = right coronary artery. (B) However, the same process may involve the entire aortic arch and the aortic arch branches. Thus, preoperative evaluation of the extent of supravalvular aortic stenosis is crucial as the concomitant patching of the aortic arch and the aortic arch branches may be required. Operative Techniques in Thoracic and Cardiovascular Surgery 2016 21, 202-210DOI: (10.1053/j.optechstcvs.2017.05.003) Copyright © 2017 Terms and Conditions

Figure 2 The aorta is cannulated at the level of the brachiocephalic artery, so that the entire ascending aorta can be enlarged, if required, and the ascending aorta is cross-clamped as distal as possible. If a concomitant aortic arch repair is needed, we would advance the aortic cannula into the brachiocephalic artery and patch the entire aortic arch and arch branches with hypothermia at 25°C and cerebral perfusion. The importance of assessing the arch and planning the arch operation ahead of time cannot be overemphasized, as the small aortic cannula and additional femoral artery cannulation may be required in case of significant narrowing of the brachiocephalic artery. Operative Techniques in Thoracic and Cardiovascular Surgery 2016 21, 202-210DOI: (10.1053/j.optechstcvs.2017.05.003) Copyright © 2017 Terms and Conditions

Figure 3 (A) The aorta is cross-clamped and cardioplegia is administered into the ascending aorta. In patients with an excessive thickness of the aortic wall that extends onto the coronary orifices, a higher-than-normal cardioplegia may be required to arrest the heart. The left ventricle is vented in a standard fashion via the right upper pulmonary vein. (B) The ascending aorta is transected above the sinotubular junction, and the initial incisions are symmetrically made as indicated by dashed lines. Operative Techniques in Thoracic and Cardiovascular Surgery 2016 21, 202-210DOI: (10.1053/j.optechstcvs.2017.05.003) Copyright © 2017 Terms and Conditions

Figure 4 Three symmetrical incisions (A - longitudinal view, B - cross-sectional view) are extended into the aortic sinuses. We start with an incision into the noncoronary sinus, so that the aortic valve and both coronary orifices can be inspected. Then 2 incisions are made into the right and left coronary sinuses as shown. Care must be taken to ensure that the aortic root is not enlarged excessively so that it results in a malcoaptation of the cusps of the aortic valve. Operative Techniques in Thoracic and Cardiovascular Surgery 2016 21, 202-210DOI: (10.1053/j.optechstcvs.2017.05.003) Copyright © 2017 Terms and Conditions

Figure 5 Three previously prepared glutaraldehyde-treated autologous pericardial patches are fashioned to achieve a symmetrical enlargement of the aorta at the sinotubular junction (A - longitudinal view with arrow representing points of approximation of the native aorta, B - cross-sectional view with arrow demonstrating the anterior aspect of the aorta). The patch from the noncoronary sinus is typically extended onto the anterior wall of the ascending aorta. Operative Techniques in Thoracic and Cardiovascular Surgery 2016 21, 202-210DOI: (10.1053/j.optechstcvs.2017.05.003) Copyright © 2017 Terms and Conditions

Figure 6 After the completion of the aortic reconstruction and the appropriate deairing of the heart, the aortic cross clamp is removed. Good hemostasis is important at this stage, as a higher-than-normal perfusion pressure is sometimes required to ensure good blood flow via the thickened coronary arteries in the hypertrophied myocardium. Operative Techniques in Thoracic and Cardiovascular Surgery 2016 21, 202-210DOI: (10.1053/j.optechstcvs.2017.05.003) Copyright © 2017 Terms and Conditions

Figure 7 (A) In patients with an extensive involvement of the aortic arch and the aortic arch branches, as sometimes seen in patients with Williams syndrome, the aortic cannula is introduced into the brachiocephalic artery. (B) A concomitant reconstruction of the aortic arch and its branches is achieved with autologous pericardium. We prefer to use autologous or homograft pericardium as this allows for superb hemostasis of the suture lines. Operative Techniques in Thoracic and Cardiovascular Surgery 2016 21, 202-210DOI: (10.1053/j.optechstcvs.2017.05.003) Copyright © 2017 Terms and Conditions