Angulo Balduzzi, Gustavo Francisco

Linares Vicente, Jose Antonio

Mayoral Campos, Victoria

English

Via Medica Journals

Address for correspondence: Dr. Jose Antonio Linares Vicente, MD PhD, Cardiology Department, Interventional Cardiology Unit, “Lozano Blesa” University Hospital, Avda. San Juan Bosco, 15, 50009, Zaragoza, Spain, tel: +34 620140664,  e-mail: joselinares1979@hotmail.comReceived: 06.02.2017 Accepted: 08.07.2017Multimodal imaging diagnosis and characterization of quadricuspid aortic valveJose Antonio Linares Vicente1, Victoria Mayoral Campos2,  Gustavo Francisco Angulo Balduzzi31Cardiology Department, Interventional Cardiology Unit,  “Lozano Blesa” University Hospital, Zaragoza, Spain 2Radiology Department, “Lozano Blesa” University Hospital, Zaragoza, Spain 3Cardiology Department, Barbastro Hospital, Huesca, SpainA 46-year-old woman was sent for cardiac catheterization due to symptomatic severe aortic regurgitation (AR) with preserved systolic left ventricular function on transthoracic echocardio-gram. Aortography showed an atypical aortic valve morphology, and a remarkable absence of cusps coaptation (Fig. 1A, white arrow). Transesopha-geal echocardiogram showed a severe AR due to a dysfunctional aortic valve with 4 cusps (Fig. 1B). Retrospective electrocardiography-gated multi-detector cardiac computed tomography (MDCCT) confirmed a quadricuspid aortic valve morphology (Fig. 1C), with a smaller anomalous fourth cusp in the anterior position between right and left aortic cusps (type B according to Hurwitz and Robert’s classification), without further aortic root or ascending aortic abnormalities: aortic valve ring area diameters were 18 × 26.6 mm (area 415 mm2), 36.7 × 32.4 mm for aortic root, 31.5 mm for sinotubular junction, and 34 mm for ascending aorta. No other structural cardiac defect was found.Quadricuspid aortic valve is a rare congenital cardiac defect with an estimated frequency of 0.05%. Approximately 75% present with significant AR due to the absence of central cusps coaptation, typically without other aortic root abnormalities but coexisting congenital cardiac defect in up to 30% of cases. Ascending aortic dilatation has been recently reported also in 30% of cases. Most patients develop symptoms at middle-age (40–60 years old). Surgical aortic valve replacement is the preferred treatment, although in selected cases valve repair (surgical excision of the small fourth cusp and resuspension of the other cusps achieving tricuspid functionality) can be considered. MDCCT provides clear images and definite anatomical infor-mation for classification or surgical considerations.Conflict of interest: None declaredFigure 1. Atypical angiographic aortic valve morphology and absence of cusps coaptation (A, white arrow). Dysfunc-tional aortic valve with 4 cusps in transesophageal echocardiogram (B). Confirmed type B quadricuspid aortic valve by multidetector cardiac computed tomography (C).A B Cclinical cardiologyCardiology Journal 2017, Vol. 24, No. 5, 580DOI: 10.5603/CJ.2017.0117 Copyright © 2017 Via MedicaISSN 1897–5593580 www.cardiologyjournal.orgIMAGE IN CARDIOVASCULAR MEDICINE

Multimodal imaging diagnosis and characterization of quadricuspid aortic valve

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Multimodal imaging diagnosis and characterization of quadricuspid aortic valve

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