Journal of Cardiothoracic and Vascular Anesthesia 34 (2020) 2272 2273

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Journal of Cardiothoracic and Vascular Anesthesia

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Editorial
Congenital Mitral Stenosis

In this issue of the Journal of Cardiothoracic and Vascular  Supramitral Ring. Though described as congenital in the
Anesthesia, Montgomery et al. presented an interesting case of present discussion, others consider this to be an acquired
parachute mitral valve in a female young adult as a cause of lesion resulting from turbulent flow across the mitral valve.
mitral stenosis (MS) and cardiogenic embolic stroke.1 Because It is a circumferential ring of connective tissue that origi-
congenital MS very rarely is identified as a de novo diagnosis nates from the atrial surface of the mitral valve leaflets dis-
in the adult patient, a morphologic review of this lesion may tal to the opening of the left atrial appendage and causes
be useful to the adult echocardiographer. various degrees of mitral inlet obstruction (Fig 4). It fre-
Congenital MS classically has been divided by Van Praagh quently is associated with multilevel obstruction of the left
into 4 anatomic types: typical, hypoplastic, supramitral ring, side of the heart, as in Shone’s complex (supramitral ring,
and parachute mitral valve.2 parachute mitral valve, subaortic stenosis, and coarctation
of the aorta).6 Mild forms of this complex have been
reported as de novo diagnoses in adults. It is important to
 Typical Congenital MS. This form of congenital MS distinguish this lesion from a membrane corresponding to
seems to correspond to the subgroup of dysplastic valve cor triatriatum sinister, which typically is located proximal
described by Anderson as the papillary muscle to commis- to the left atrial appendage.
sure fusion.3 This is the most common form of congenital  Parachute Mitral Valve. Although this is the least com-
MS and shows large phenotypic variability. Morphologi- mon form of congenital mitral stenosis, it is discovered the
cally, it is characterized by thickened leaflets, short and most frequently as a de novo diagnosis in the adult popula-
thickened chords with obliteration of interchordal spaces, tion.7-9 The tendineae chordae are thickened and shortened
and underdeveloped papillary muscles with reduced inter- and insert into a single papillary muscle (typically the post-
papillary distance (Fig 1). In the most severe cases, the pap- eromedial), while the anterolateral is absent or hypoplastic.
illary muscles come together and insert directly into the
leading edge of the anterior mitral leaflet, creating a muscu-
lar arcade when the valve is viewed by the echocardiog-
rapher from the left ventricular aspect (arcade mitral valve)
(Fig 2).4 In other cases, the shortened and thickened chords
insert directly into the muscular mass of the posterior wall,
causing the appearance of a hammock when the valve is
viewed from the left atrium perspective (hammock mitral
valve).5 This particular type of congenital MS typically is
diagnosed early in life and is very unlikely to be found
untreated in the adult population. It commonly is associated
with coarctation of the aorta and aortic stenosis.
 Hypoplastic MS. This is the second most common type of
congenital mitral valve stenosis. All the components of the
hypoplastic valve are a miniature of the normal valve
(Fig 3). It virtually always is associated with severe left ven-
tricular outflow tract abnormalities, most commonly hypo-
plastic left heart syndrome. It is diagnosed in infancy and Fig 1. Typical congenital mitral stenosis in a 2-year-old patient. Midesopha-
never has been reported as a de novo diagnosis in adults. geal 4-chamber view showing thickened mitral valve leaflets, thickened and
shortened chords, and restrictive opening of the mitral valve with diastolic
DOI of original article: http://dx.doi.org/10.1053/j.jvca.2020.01.021. deformity (similar appearance to rheumatic mitral valve disease).

https://doi.org/10.1053/j.jvca.2020.01.041
1053-0770/Ó 2020 Elsevier Inc. All rights reserved.
 Editorial / Journal of Cardiothoracic and Vascular Anesthesia 34 (2020) 2272 2273
Fig 2. Arcade mitral valve. Left, midesophageal mitral commissural view showing the papillary muscle inserting directly into the mitral valve leaflets. Right, trans-
gastric midpapillary short-axis view in pediatric format showing the muscle arcade created by the papillary muscles. ALPM, anterolateral papillary muscle;
PMPM, posteromedial papillary muscle; PML, posterior mitral leaflet.
Fig 3. Hypoplastic mitral stenosis in a 7-day-old patient. Midesophageal 4-
chamber view showing a mildly hypoplastic left ventricle with hypoplasia of
the mitral valve complex (miniature mitral valve). Aortic stenosis, bicuspid
aortic valve, hypoplasia of the ascending aorta, and coarctation of the aorta
were also present.
Fig 4. Supramitral ring. 3-dimensional live acquisition of the mitral valve
from the left atrium perspective showing a complete supramitral ring (inter-
rupted line). Note the location of the membrane distal to the opening of the
LAA. AV, aortic valve; LAA, left atrial appendage; AML, anterior mitral leaf-
let; PML, posterior mitral leaflet.
2273
In parachute-like mitral valve, the hypoplastic papillary
muscle is connected to an underdeveloped commissure,
creating an asymmetric opening of the mitral valve.
In conclusion, congenital MS diagnosed for the first time in
an adult patient most likely corresponds to parachute mitral
valve either in the isolate form or associated with a mild form
of Shone’s complex.
Conflict of Interest
None.
Nelson H. Burbano, MD
Anesthesiology Institute, Cleveland Clinic, Cleveland, OH
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