Letter to the Editor Low output syndrome masking aortic regurgitation in a Marfan patient. Usefulness of 3D Transthoracic Echocardiography and Heart Team Cristiano Amarelli a, 1 , Gemma Salerno b, ⁎ , 1 , Gianpaolo Romano a, 1 , Ciro Maiello a, 1 , Margherita Borrelli a, 1 , Giuseppe Limongelli b, 1 , Giuseppe Pacileo b, 1 , Michelangelo Scardone a, 1 a Department of Cardiovascular Surgery and Transplant, V. Monaldi Hospital, Azienda Ospedaliera dei Colli, Naples, Italy b Department of Cardiology, Second University of Naples, V. Monaldi Hospital, Azienda Ospedaliera dei Colli, Naples, Italy article info Article history: Received 25 October 2012 Accepted 3 November 2012 Available online xxxx Keywords: 3-D Transthoracic Echocardiography Aortic regurgitation Marfan Syndrome Multidisciplinary heart team The echocardiographic evaluation of aortic valve (AoV) regurgita- tion (AR) during low-output syndrome with tachycardia is dampened by increased left ventricular (LV) end-diastolic pressure and low sys- temic pressure, thus underestimating the grade of AoV dysfunction [1,2]. Indication to surgery may be difficult and surgery may carry a high hospital mortality. 3-D Transthoracic Echocardiography (3D-TTE) may warrant optimal visualization of the valve anatomy guiding surgi- cal choices and should be considered in the assessment of patients with AR. The Heart Team may help to plan the best treatment for every patient. A 32 years old male Marfan patient was referred to our Outpatient Clinic for heart transplant evaluation after a febrile episode followed by acute heart failure. The patient was admitted in severely symptomatic stage, New York Heart Association (NYHA) classes III–IV, on sinus tachycardia (140 bpm), his clinical examination evidenced rales and peripheral swelling. Low ejection fraction (LVEF 10–15%) with a severely dilated left ventricle (LVDd=9.2cm, LVDd/BSA= 4.1 cm/mq), mild aortic regurgitation with a dilated aortic root (aortic ratio, AR, was 1,39) and severe mitral regurgitation were disclosed at 2D-transthoracic echocardiogram (2D-TTE). Levosimendan and endo- venous diuretics were prescribed; Mechanical Circulatory Support was evaluated. LV function promptly increased and 2D-transesophagel echocardiography (2D-TEE) was performed to exclude aortic emergen- cies or endocarditis; the aortic valve was also evaluated with live 3D-TTE reconstruction (Fig. 1) followed by full-volume acquisition that showed annular dilation with central incompetence (Type 1 fol- lowing the El Khoury classification) [3,4]. 3D vena contracta cross sec- tional area method showed a mildly regurgitation [5]. Emergent heart transplantation was not indicated and risk for surgery of the aortic root was considered too high. Cardiac defibrillator incorporating car- diac resynchronization therapy (AICD/CRT implantation) and neuro- hormonal blockade therapy was planned from the Heart Team as a bridge to the optimal choice. After 1 month of optimal medical therapy (OMT) clinical status improved (NYHA class II) and NT-pro-BNP de- creased from 2573 pg/mL to 1444 pg/mL. LVEF recovered until 35% and the grade of mitral regurgitation reduced to moderate. After 1 year of OMT the NT-pro-BNP was 52 pg/mL, the patient was re-evaluated for aortic dilatation. 2D-TTE showed an aortic ratio of 1,46, moderate AR (vena contracta width=0.3 cm, PHT=327 ms), good mitral function, eccentric LV-hypertrophy with mildly abnormal LV-mass indexed (LVDd=6.1 cm, LVDd/BSA=2.78 cm/mq, LVMI= 126 gr/mq, RWT=0.3) and with 50% LVEF. In spite of this finding, 2D-speckle-tracking echocardiography (2D-STE) showed a decrease of global longitudinal strain (Fig. 2) (GLS =-14.8%), indicating the persis- tence of cardiac dysfunction. The patient was successively candidate to isolate aortic root surgery. Considering the previous severe myocardial dysfunction without a complete resolution and the ventricular dilata- tion, 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 slight dilated left ventricle and no neuro- hormonal activation (NT-pro-BNP b 100 pg/mL). The optimal visualiza- tion of the AoV provided by 3D-TTE may warrant the choose of the best surgical therapy, giving to the surgeon information on the valve cusps coaptation, the aortic root, the mechanism of the AR and the left ventric- ular function and size [6]. Actually, the intraoperative funding showed a slightly abnormal AoV resulting in an incomplete coaptation of cusps as- sociated to annular dilatation. In this case the previous ventricular dila- tation pushed us to replace the AoV despite that the central aortic regurgitation could be easily corrected with a valve sparing operation. The data of De Kerchove L. et al. suggest, in fact that a ventricular dilata- tion is the most important determinant of recurrence of AR [7]. A stable result in terms of AoV function had to be assured to a so difficult patient also considering the consistent risk of reoperation for aortic complica- tion related to its primary diagnosis [8]. We confirmed the data about long-term outcome after AoV surgery that is significantly better in pa- tients who are operated in an asymptomatic or mildly symptomatic International Journal of Cardiology xxx (2012) xxx–xxx ⁎ Corresponding author at: Department of Cardiology, Second University of Naples, V.Monaldi Hospital, Italy, Via L. Bianchi, 80100 Naples, Italy. Tel.:+39 0817062865, +39 0817064234. E-mail address: gemma.salerno@hotmail.it (G. Salerno). 1 These authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation. IJCA-15542; No of Pages 2 0167-5273/$ – see front matter © 2012 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2012.11.043 Contents lists available at SciVerse ScienceDirect International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard Please cite this article as: Amarelli C, et al, Low output syndrome masking aortic regurgitation in a Marfan patient. Usefulness of 3D Transthoracic Echocardiography and Heart Team, Int J Cardiol (2012), http://dx.doi.org/10.1016/j.ijcard.2012.11.043