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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 [
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).
(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.
(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.
Congenital mitral insufficiency caused by anomalous mitral arcade in an elderly patient: use of echocardiography and multidetector computed tomography for diagnosis.
