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Heart, Lung and Circulation

Mitral Arcade Causing Severe Stenosis in an Adult Patient

Published:October 27, 2021DOI:https://doi.org/10.1016/j.hlc.2021.09.012
      A 23-year-old non-dysmorphic female was admitted for congestive heart failure. She was diagnosed with mitral stenosis, congenital hypopituitarism confirmed by genetic tests (defect of gene encoding growth hormone secretion [GH1]; defect of gene encoding pituitary cell differentiation [PROP1]) and coeliac disease. Echocardiography and cardiac computed tomography (CT) showed a congenital mitral arcade causing severe stenosis [
      • Layman T.E.
      • Edwards J.E.
      Anomalous mitral arcade. A type of congenital mitral insufficiency.
      ,
      • Hakim F.A.
      • Krishnaswamy C.
      • Mookadam F.
      Mitral arcade in adults. A systematic review.
      ,
      • Naeim H.A.
      • Taha E.A.
      • Taha R.A.
      • Alatawi F.O.
      • Amodi O.
      • Abuelatta R.
      Isolated adult congenital uni-leaflet severe mitral valve stenosis, a case report and review of the literature.
      ,
      • Fritz A.V.
      • Phillips S.D.
      • Landolfo K.P.
      • Martin A.K.
      Mitral arcade. A rare cause of valvular disease.
      ,
      • Kim S.J.
      • Shin E.S.
      • Park M.K.
      • Choi S.H.
      • Lee M.G.
      Congenital mitral insufficiency caused by anomalous mitral arcade in an elderly patient: use of echocardiography and multidetector computed tomography for diagnosis.
      ]. Mitral arcade has some anatomical features in common with the parachute valve, such as deformed and restricted leaflet mobility and underdeveloped commissures. On the other hand, the arcade has two thick papillary muscles and elongated chordae tendinae, whereas the parachute valve presents one papillary muscle and short and thick chordae tendinae (Figures 1 and 2).
      Figure thumbnail gr1
      Figure 1TEE: two-chamber view.
      (A) Valve leaflets present fusion of their free margin, doming in the left atrium. Thick papillary muscles without chordae tendinae. Trans-gastric view. (B) Papillary muscles are shaped as a horse-shoe. (C) Accelerated flow with aliasing through the small eccentric single orifice, causing severe mitral stenosis. No separation of leaflets in diastole. (D) Continuous wave Doppler confirms severe stenosis: high trans-valvular gradients and PHT.
      Valve area 0.9 cm2.
      Abbreviations: TEE, transoesophageal echocardiogram; PHT, pulmonary hypertension.
      Figure thumbnail gr2
      Figure 23D TEE: True View modality.
      (A) The left atrium is visualised from the top. There are a small single eccentric orifice and no separation of leaflets in diastole. (B) The valve is bulging into the left atrium. Elongated papillary muscles attach directly to valve leaflets. Cardiac CT: Two-chamber view (C). Abnormal position of the two elongated papillary muscles that attach directly to valve leaflets. No chordae tendinae. (D) Excised mitral arcade: view from left ventricular side. Complete fusion of both leaflets creating a very thick central ridge: the arcade. No primary and secondary chordae tendinae.
      Abbreviations: Co, tertiary chordae tendinae; O, eccentric single orifice; P, tips of papillary muscles; CT, computed tomography; TEE, transoesophageal echocardiography; 3D, three dimensional.

      Keywords

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