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Cristiano Amarelli, Gemma Salerno, Gianpaolo Romano, Ciro Maiello, Margherita Borrelli,

Low Output Syndrome masking aortic regurgitation in a Marfan patient Usefulness of 3D TTE Echocardiography and Heart Team. Cristiano Amarelli, Gemma Salerno, Gianpaolo Romano, Ciro Maiello, Margherita Borrelli, Giuseppe Limongelli, Giuseppe Pacileo , Michelangelo Scardone

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Cristiano Amarelli, Gemma Salerno, Gianpaolo Romano, Ciro Maiello, Margherita Borrelli,

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  1. Low Output Syndrome masking aortic regurgitation in a Marfan patient Usefulness of 3D TTE Echocardiography and Heart Team Cristiano Amarelli, Gemma Salerno, Gianpaolo Romano, Ciro Maiello, Margherita Borrelli, Giuseppe Limongelli, Giuseppe Pacileo, Michelangelo Scardone Monaldi Hospital - Naples(IT)

  2. Low Output Syndrome masking aortic regurgitation in a Marfan patient Clinicalpresentation • 32 y old male, feverfollowed by acute heartfailure NT-pro-BNP=2573 pg/mL Clinical examination • sinus tachycardia, rales and peripheral swelling Echo assessment • low LVEF 10-15%, severe dilatation of LV (LVDd=9,2cm) • mild AR, dilated aortic root (AR 1,39), • severe mitral regurgitation Levosimendanand endovenous diuretics were prescribed

  3. Low Output Syndrome masking aortic regurgitation in a Marfan patient LVEF promptly increased and Mechanical Circulatory Support or Emergent HT were not indicated Next Echo evaluation • 2D-TEE excluded aortic emergencies or endocarditis • 3D-TTE disclosed normal mobility of cusps, mildly redundant with aortic root dilation resulting in inadequate cusp coaptation with central AR jet (Type I AR)

  4. Low Output Syndrome masking aortic regurgitation in a Marfan patient

  5. Low Output Syndrome masking aortic regurgitation in a Marfan patient Unacceptably high risk for mitro-aortic surgery AICD/CRT implantation and neuro-hormonal blockade therapy was planned from the Heart Team as a bridge to decision on the optimal therapeutic choice

  6. Low Output Syndrome masking aortic regurgitation in a Marfan patient Clinical status after 1 month of optimal medical therapy • NYHA I • NT-pro BNP=1444 pg/mL • LVEF 35% • Moderate mitral valve regurgitation Clinical status after 1 year of optimal medical therapy • NT-pro-BNP was 52pg/mL • 2D-TTE: Aortic Ratio 1,46 • Moderate AR (vena contracta width=3 mm, PHT=327ms) • LVEF 50%; Eccentric LV-hypertrophy (LVDd:6.1cm,LVDd/BSA:2,78cm/mq;LVMI=126gr/mq,RWT=0.3) • Absence of mitral valve regurgitation • 2D TT-speckle-tracking :global longitudinal strain (GLS average=-14,8 %)

  7. Low Output Syndrome masking aortic regurgitation in a Marfan patient The patient was candidate to isolate aortic root surgery • Considering the previous myocardial dysfunction and ventricular dilatation, a modified Bentall operation was preferred to David operation to warrant an uneventful postoperative course At 1-year follow-up the patient is in NYHA class I, with normal indexed-LV and no neuro-hormonal activation (NT-pro-BNP<100 pg/mL).

  8. Low Output Syndrome masking aortic regurgitation in a Marfan patient 3D-TE-ECHO permits • Better anatomic visualization of the aortic root • Optimal surgical planning An Heart Team permits • A multidisciplinary approach to borderline clinical situations • An help to choose the best diagnostic tools • Tailor the most appropriate and effective strategy to the single patient HT has been avoided and surgery has been carried out in the ideal clinical situation

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