indian heart journal 68 (2016) 639–645

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Original Article

Spectrum of congenital mitral valve abnormalities

associated with solitary undifferentiated papillary
muscle in adults

Jagdish C. Mohan *, Madhu Shukla, Vishwas Mohan, Arvind Sethi

Fortis Institute of Cardiac Sciences, Fortis Hospital, Shalimar Bagh, New Delhi 110088, India

article info abstract

Article history: Background: Congenital anomaly wherein the mitral valve leaflets are directly attached to
Received 26 October 2015 the papillary muscle(s) (PM) with or without short under-developed chords is rarely reported
Accepted 21 December 2015 in adults. Patients with two PMs with an intervening fibrous bridge have also been included
Available online 11 January 2016 under this head in previous studies.
Methods: Echocardiography enables accurate evaluation of the morphology and function of
Keywords: valve leaflets, chordae tendineae, and PM. This report describes a series of six patients aged 56–
Arcade mitral valve 84 years who had abnormal mitral valve with a large solitary and anomalously inserted PM seen
Undifferentiated papillary muscle over a period of 3 years. Only those patients who had a single pillar or bridge-like PM and either
Parachute mitral valve absent tendinous chords or small under-developed chords were included in the analysis.
Congenital mitral stenosis Results: Among 9600 consecutive echocardiograms performed, six patients met the criteria
of an abnormal mitral valve with solitary large PM. Two patients underwent mitral valve
replacement with partial excision of the PM wherein echocardiographic observations were
confirmed. The patients were previously followed with the diagnosis of hypertrophic
cardiomyopathy (3) and rheumatic mitral valve disease (3). Multi-planar reconstruction
of 3D echocardiographic images provided incremental value in assessing the detailed patho-
anatomy of PMs in these cases.
Conclusion: In adult patients, a high index of suspicion is required to detect congenital mitral
stenosis/regurgitation with large solitary PM (resembling a parachute mitral valve) which
may masquerade as hypertrophic cardiomyopathy or rheumatic mitral valve disease.
# 2015 Cardiological Society of India. Published by Elsevier B.V. This is an open access
article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

leaflets and tensor apparatus produces anomalies of the mitral

1. Introduction
valve which may resemble an arcade, a hammock, or a
parachute. The majority of mitral valve malformations are not
Congenital malformations of the mitral valve are often classified, and descriptive terms (parachute, hammock, or
complex and affect multiple segments of the valve apparatus. arcade) often have overlapping patho-anatomy. The malfor-
These may occur in isolation or in association with other mation of mitral valve in which the chordal apparatus is
congenital heart defects. Incomplete differentiation of the inserted into a single papillary muscle (PM) or a muscle group

* Corresponding author.
E-mail address: [email protected] (J.C. Mohan).
http://dx.doi.org/10.1016/j.ihj.2015.12.014
0019-4832/# 2015 Cardiological Society of India. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
640 indian heart journal 68 (2016) 639–645

is called parachute deformity.1 The ‘‘parachute mitral valve’’

has the usual two mitral valvular leaflets and commissures,
but the chordae, instead of diverging to insert into two PM,
converge into one major PM. In ‘‘true’’ parachute mitral valve
(PMV), mitral valve chordae insert into a solitary central PM.
According to Tandon et al., PMV is associated with other
congenital anomalies in >95% cases.2 Anomalous mitral
arcade was first described by Layman and Edwards in 1967
as ‘‘an anomaly of the mitral valve that consisted of
connection of the left ventricular (LV) papillary muscles to
the anterior mitral valve leaflet, either directly or through the
interposition of unusually short tendinous chords’’.3 The two
PMs resemble two pillars, and the bridging fibrous tissue in
between the PM muscles resembles the arch of an arcade.
Fig. 1 – 2D-echocardiographic short axis view showing
Undifferentiated PM has been defined as a PM with club-like
dumbbell-shaped anomalous papillary muscle in the left
thickening, often attached to the leaflets directly due to a
panel. The right panel shows mid-ventricular short axis
defect or marked under-development of chordae tendineae
after excision of the muscle bundle.
that leads to compromised flexibility of PM/chordae tendi-
neae/valve leaflet assembly.4 Others have called this anomaly
of single PM with, often short and fused tendinous chords and
narrow interchordal spaces seen in adults also as the PMV.5
However, a true PMV mitral valve has a single central PM
muscle with unifocal attachment of chords, regardless of the
anomalies of the chords and is mostly associated with other
congenital anomalies. Mitral valve abnormalities with short
thick chords or direct attachment of PM to the anterior mitral
leaflet have been also reported in the hypertrophic cardiomy-
opathy6,7; however, single or undifferentiated PM has not been
described. Carpentier et al. classified the congenital mitral
valve abnormalities into four types depending upon the
presence and type of mitral regurgitation and/or mitral
stenosis regardless of the morphology of the tensor apparatus
and refrained from using descriptive names.8
This report deals with clinical and echocardiographic
spectrum of significant mitral valve dysfunction with undif-
Fig. 2 – Modified apical 4-chamber view showing
ferentiated solitary PM in the adults seen over a period of 3
anomalous papillary muscle with attachment to the
years in a busy echocardiographic laboratory.
interventricular septum (yellow arrow). The right panel
shows the residual portion of the muscle bundle (yellow
2. Case 1 arrow) after excision.

A 56-year-old man presented with exertional dyspnea of 4

years duration. He had been previously diagnosed as a case
hypertrophic cardiomyopathy and alcohol septal ablation was
also suggested. The 12-lead electrocardiogram showed left
atrial overload and complete right bundle branch block. The
chest skiagram showed cardiomegaly with pulmonary venous
congestion. His coronary angiogram revealed mild stenosis of
the distal left circumflex artery. Echocardiographic examina-
tion showed dilated inferior vena cava, right atrium, and the
right ventricle with severe tricuspid regurgitation. Doppler-
estimated pulmonary artery systolic pressure was 110 mmHg.
The left atrium was enlarged; there was a solitary large PM
attached to the lateral and the posterior wall and appeared like
a cantilever bridge in the short axis (Fig. 1).
This anomalous muscle bundle also had fibromuscular
attachments to the interventricular septum (Fig. 2).
Both mitral valve leaflets were directly attached to this Fig. 3 – Apical 4-chamber view in diastole (left) and 5-
solitary anomalous muscle bundle with no intervening chamber view in systole (right panel) showing direct
tendinous chords (Fig. 3). attachment of the mitral leaflets to the papillary muscle.
 indian heart journal 68 (2016) 639–645 641

Fig. 5 – Apical 4-chamber (A) and parasternal long axis (B)

views showing thick mitral valve leaflets, short tendinous
Fig. 4 – Left panel shows anomalous papillary muscle in the chords, and a single anomalous papillary muscle. PM,
modified short axis view. The right upper panel shows papillary muscle; LA, left atrium; LV, left ventricle; RA, right
continuous-wave Doppler spectrum of mitral regurgitation atrium; RV, right ventricle.
and mid-cavity obstruction (red arrow). The right lower
panel shows continuous-wave Doppler spectrum across
the mitral funnel.

Mitral valve area by planimetry and the pressure half-time

method was 1.2 cm2 with a mean gradient of 6 mmHg.
Moderate mitral regurgitation (vena contracta = 5 mm) and
an intra-ventricular peak gradient of 30 mmHg were recorded
(Fig. 4).
The patient underwent cardiac catheterization which
showed hardly any gradient between pulmonary wedge and
LV diastolic pressures although pulmonary artery pressure
was recorded as 115/40 mmHg. The patient was subjected to
excision of the muscle bundle along with valve leaflets and
was implanted with a #27 St Jude's prosthesis in mitral
position. The echocardiographic observations were confirmed
Fig. 6 – Reduced opening of the mitral orifice in diastole (A)
on gross pathology.
and narrow inter-chordal spaces. Short chords are seen in
panel B during systole. Panel C shows severe eccentric
3. Case 2 mitral regurgitation.

This 59-year-old female was being followed as a case of

hypertrophic cardiomyopathy for the last 7 years. She had
class III exertional dyspnea. Physical examination was
conspicuous by a loud parasternal ejection systolic murmur,
supine blood pressure of 130/90 mmHg, and no jugular venous
distension. The 12-lead ECG showed sinus rhythm, left atrial
overload, and LV hypertrophy with strain. The chest skiagram
was unremarkable. 2D-echocardiographic examination
revealed uniformly thick walls of the left ventricle (14 mm),
solitary anomalous PM attached to the apicolateral segment,
and both mitral leaflets were thick and club-like and attached
to the PM through small chords. Severe eccentric mitral
regurgitation and a peak gradient of 50 mmHg were recorded Fig. 7 – Parasternal long axis view (left panel) and the apical
in the LV outflow tract. Trans-mitral peak and mean gradients 5-chamber view (right panel) showing narrow left
were 8 and 3 mmHg respectively. The patient underwent ventricular outflow tract due to anteriorly inserted
extended myomectomy, excision of the PM, and mitral valve papillary muscle and the abnormal orientation of the
replacement (Figs. 5–7). thickened anterior leaflet.
642 indian heart journal 68 (2016) 639–645

4. Case 3

An 84-year-old female was followed as a case of rheumatic

mitral valve disease with atrial fibrillation for the last 30 years.
She had class II dyspnea and declined any surgery. Physical
examination revealed a thin-built elderly lady, BP 130/
86 mmHg, irregularly irregular heart rate of 68 BPM, and a
mid-diastolic murmur at the apex. The 12-lead electrocardio-
gram revealed atrial fibrillation and non-specific ST-T
changes. The chest skiagram showed mild cardiomegaly
and pulmonary venous congestion. Her trans-thoracic 2D
echocardiographic examination revealed markedly enlarged
left atrium, reduced motion of the mitral leaflets with
restricted opening, single large PM attached to the inferolateral
and anterolateral walls, short chordae, and mitral valve area of
1.4 cm2 (Figs. 8–10). There was no calcification of the mitral
valve leaflets. Moderate mitral regurgitation was observed on
Fig. 10 – Left lower panel shows multi-plane 3D
color Doppler interrogation.
reconstruction of the left ventricular mid-cavity showing a
large anomalous papillary muscle (arrow) attached from 1
o'clock till 6 o'clock position. Right panel shows continuous
wave Doppler interrogation of the mitral funnel and a peak
gradient of 8 mmHg.

5. Case 4

A 69-year-old female presented with exertional dyspnea of 2

years duration. She had long-standing systemic hypertension
and was recently diagnosed with type II diabetes mellitus. Her
previous echocardiogram was reported as showing mitral
annular calcification and she had the clinical diagnosis of heart
failure with preserved ejection fraction and atrial fibrillation.
Her echocardiographic examination revealed calcification of the
posterior mitral annulus, reduced mitral valve opening with
Fig. 8 – 2D-echocardiographic parasternal long axis view in
normal thickness of the leaflets (mitral valve area of 1.3 cm2),
systole (A) and in diastole (B). Note reduced opening of the
short tendinous chords, and single large PM attached to the LV
mitral orifice with short chords.
free wall. Color Doppler interrogation of the mitral valve
revealed flame-shaped ante-grade flow spectrum and moderate
mitral regurgitation. Continuous-wave Doppler examination
showed trans-mitral peak and mean gradients of 15 and
7 mmHg respectively (Figs. 11 and 12).

6. Case 5

A 63-year-old male was diagnosed as hypertrophic cardiomy-

opathy 13 years back and had class II dyspnea. Detailed
echocardiographic examination showed thick LV walls,
asymmetric septal hypertrophy, solitary PM, and short chords
with systolic anterior motion. Peak gradient across mitral
valve in diastole was 7 mmHg and moderately severe mitral
regurgitation was noted (Figs. 13 and 14).

7. Case 6
Fig. 9 – Parasternal biplane views (long axis + short axis)
showing near direct attachment of the papillary muscle to
the leaflets and reduced oval mitral valve orifice (arrow, A 69-year-old female was a known case of mitral stenosis with
right panel). exertional dyspnea. She was unwilling for any intervention
 indian heart journal 68 (2016) 639–645 643

Fig. 11 – Left panel (A) shows single large papillary muscle

in short axis view (red arrow) while right panel (B) shows
short chords, calcification of the posterior annulus, and a
large, thick club-like papillary muscle in apical 4-chamber
view. Fig. 14 – Continuous-wave Doppler interrogation of the
mitral valve showing spectrum of mitral regurgitation
(upper panel). The lower panel shows pulsed-wave
Doppler spectrum with a peak A velocity of 1.6 m/s.

Fig. 12 – Diastolic frames in figures A and B showing normal

anterior mitral leaflet, calcified posterior annulus. Figure C
shows solitary papillary muscle in short axis. Figure D
reveals typical Doppler spectrum of mitral stenosis and Fig. 15 – Upper panel shows continuous wave Doppler
regurgitation. spectrum of mitral stenosis with a mean gradient of
10 mmHg. Lower left panel shows narrow jet of mitral
diastolic flow and the right lower panel in short axis view
shows narrow orifice of the mitral valve with no
commissural fusion or calcification.

and had been followed as a case of rheumatic heart disease for

a very long time. Recent echocardiogram revised the etiology
of mitral stenosis. Current echocardiogram shows thickened
mitral valve leaflets with no commissural fusion, short chords,
and a single large PM placed centrally (Figs. 15 and 16). There
Fig. 13 – Left panel shows parasternal long axis view in was no involvement of other valves and there was no
systole with two heads of a single papillary muscle and calcification. Mitral valve area by planimetry was 1.1 cm2
short chords. Right panel shows single papillary muscle in and mean diastolic gradient across the mitral valve was
short axis view. 10 mmHg.
644 indian heart journal 68 (2016) 639–645

In most instances, PMV is associated with other congenital

anomalies of the heart, in particular obstructive lesions of
the mitral inflow (mitral valve ring) and LV outflow tract
(subaortic stenosis), and coarctation of aorta and is referred
to as Shone's complex or Shone's anomaly.2,11 Others later
observed that in the setting of isolated PM, the tendinous
chords are often short and fused with interchordal spaces
partially or completely obliterated.5 PMV is commonly
classified as a malformation of the PMs. However, that is
too simplistic. It is often the associated lesions of mitral
valve segments (e.g., commissural underdevelopment, dys-
plastic leaflets, shortened, and fused tendinous chords) that
determine the severity of valvar dysfunction (stenosis and
regurgitation) and hence the need for cardiac intervention.
Fig. 16 – Multi-planar 3D reconstruction showing single
The combination of these lesions can give rise to a funnel
papillary muscle in panel A (short axis) and narrow mitral
rather than parachute-like appearance as seen in six of the
valve orifice as viewed from the left ventricular side (panel B).
cases reported in the present work. A series of six such cases
presenting as mitral regurgitation and confirmed on surgery
have been recently reported.4 Despite two series of ten and
8. Discussion
six cases,4,5 sketchy information is available about PMV or
undifferentiated PM with mitral valve dysplasia in adults as
The abnormalities of PM/chordae tendineae of the mitral valve an isolated anomaly or in association with other congenital
are a rare form of congenital mitral valve dysplasia which may cardiac anomalies.12–18 The clinical course of isolated PMV is
remain asymptomatic in life-time or may present with mitral varied depending on the presence and severity of mitral
valve stenosis or regurgitation or both. In the developmental stenosis and regurgitation. Four of our patients presented
process of mitral valve, chordae tendineae and PM, a with predominant mitral stenosis while two had significant
prominent horseshoe-shaped myocardial ridge appears from mitral regurgitation. It has been reported that many patients
the anterior wall of the LV and extends to the posterior wall of may remain asymptomatic even in adulthood.19,20
the LV approximately on the 33rd fetal day, and this ridge
connects with the endocardial cushion tissue in the atrio-
9. Conclusions
ventricular region.9 Afterwards, the myocardial ridge trans-
forms into two PMs and part of the endocardial cushion tissue
differentiates into valve leaflets and chordae tendineae.9 Mitral stenosis or mitral regurgitation associated with undif-
Disturbed delamination of the anterior or posterior part of ferentiated PM results from tethering and impaired flexibility
the trabecular ridge from the ventricular wall, combined with of leaflets. It is possible to successfully treat the patients by
underdevelopment of chordae, seems to be the cause of mitral valve replacement and excision of the PM wholly or in
asymmetric mitral valves. PMV, however, develops when the part, unless complex congenital cardiac malformations coex-
connection between the posterior and anterior part of the ridge ist. Detailed examination of the insertion and bridging of the
condenses to form one single PM. Oosthoek et al.10 hypothe- PM by echocardiography and intraoperative inspection is
sized that the myocardial ridge of the LV wall connects with necessary and surgical techniques should be selected appro-
the endocardial cushion tissue at the atrio-ventricular septum priately in each case. From the aspect of long-term prognosis,
and differentiates into PM and chordae tendineae at 5–19 fatal it is well known that mitral valve repair is superior to mitral
weeks. PM are found attached to valve leaflets without the valve replacement. However, the results of the mitral
involvement of chordae tendineae in cases of poor differenti- valvuloplasty for congenital mitral valve disease in the adult
ation of endocardial cushion into chordae tendineae. They are not incompletely clarified. Repair is feasible in case there is
also reported 28 cases of PM–chordae tendineae abnormalities predominant regurgitation with well-developed chords. Re-
in which one PM was developed from the high level of the LV cently, repair in one of such cases has been reported.14 It is
and attached to AML directly or with slight mediation of prudent to follow Carpentier's approach in all such cases.8
chordae tendineae and called them asymmetric parachute-
like mitral valves. This report does not include such cases. The
Conflicts of interest
spectrum of PMs in four of our cases (cases 1–4) resembles non-
delamination and non-separation of the embryonic trabecular
ridge. The authors have none to declare.
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