Profound Aortopathy: A Rare Case of Massive Ascending and Descending Aortic Aneurysms, Type B Aortic Dissection, and Severe Aortic Valve Regurgitation.

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Presentation transcript:

Profound Aortopathy: A Rare Case of Massive Ascending and Descending Aortic Aneurysms, Type B Aortic Dissection, and Severe Aortic Valve Regurgitation in an Adult Patient With Uncorrected Congenital Aortic Coarctation  Rashad J. Belin, MD, PhD, Jessie Aw Zoretic, MD, James Carr, MD, Mark D. Huffman, MD, MPH, FAHA, FACC, Hyde Russell, MD, James Thomas, MD, FACC, FASE  CASE  DOI: 10.1016/j.case.2017.06.004 Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 1 Surface-rendered CT angiogram revealing massive ascending and descending TAAs and the aortic coarctation just proximal to the descending TAA. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 2 ECG-gated thoracic aorta CT angiogram, sagittal view of the aortic root, ascending aorta, and descending TAAs; also shown are the dissection flaps. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 3 MRA, sagittal view revealing ascending and descending TAAs as well as dissection flaps within descending TAA. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 4 ECG-gated thoracic aorta CT angiogram, axial view of the ascending and descending TAA. Also shown is compression of the right pulmonary artery by the ascending TAA. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 5 ECG-gated thoracic aorta CT angiogram, coronal view illustrating aortic coarctation just proximal to the descending TAA. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 6 ECG-gated thoracic aorta CT angiogram, axial view illustrating descending TAA compressing the left pulmonary artery. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 7 ECG-gated thoracic aorta CT angiogram, sagittal view illustrating descending TAA compressing the left pulmonary artery. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 8 Continuous-wave Doppler revealing aortic valve regurgitant jet deceleration and very short pressure half-time consistent with severe aortic valve regurgitation. AR, Aortic regurgitation; Dec, deceleration; maxPG, maximum pressure gradient; PHT, pressure half time; Vmax, maximum velocity. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 9 Pulse-wave Doppler at suprasternal notch view showing holodiastolic flow reversal consistent with severe aortic valve regurgitation. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 10 Cardiac MRI/MRA 4D flow analysis prior to surgery. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 11 TEE revealing type B aortic dissection flap at 25 cm distal to the incisors. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 12 TEE, short-axis view of the aortic valve with color flow after surgery. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 13 TEE, long-axis view of the aortic valve and aortic graft with color flow after surgery. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 14 Cardiac MRI/MRA 4D flow analysis after the first surgery. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 15 Intraoperative photograph of massive descending thoracic aneurysm at the time of surgery. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

Figure 16 Cardiac MRI/MRA 4D flow analysis after the second surgery. CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions

CASE DOI: (10.1016/j.case.2017.06.004) Copyright © 2017 American Society of Echocardiography Terms and Conditions