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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5761_Библиотеки_им_академика_М_И_Перельмана
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343
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_106, © Springer International Publishing Switzerland 2015
A 33-year-old man was referred to our echocardiography
laboratory for the evaluation of hypertension. He was a
known case of uncontrolled hypertension and an interrupted
aortic arch. His leg pulses were weak (1+), and his arm
pulses were 4+.
Parachute Mitral Valve
and Interrupted Aortic Arch
Case 106
ab
Fig. 106.1 The parasternal long-axis view shows the thickening of the
mitral valve leafl ets and the reduced orifi ce of the opening of the mitral valve
leafl ets ( arrow ) by two-dimensional echocardiography ( a ). A diastolic
turbulency across the mitral valve is evident in this view by color Doppler
echocardiography ( arrow ) ( b ). LA left atrium, LV left ventricle, RV right
ventricle, AO aorta
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_106 ) contains supplementary material,
which is available to authorized users.
344
ba
Fig. 106.2 The parasternal short-axis view reveals mitral stenosis ( arrow ) ( a ). The mitral valve area is about 0.9 cm 2 by planimetry ( b )
Fig. 106.3 An abnormal chorda is evident in the apical four-chamber
view ( arrow ). LA left atrium, LV left ventricle, RA right atrium, RV right
ventricle
ab
Fig. 106.4 The peak and mean gradients across the mitral valve are 12 and 6 mmHg, respectively ( a ). The mitral valve area is about 0.9 cm 2 by
pressure half time ( b )
Case 106 Parachute Mitral Valve and Interrupted Aortic Arch
345
Fig. 106.5 A diastolic turbulency is also evident in the apical four-
chamber view ( arrow ). LA left atrium, LV left ventricle, RA right atrium,
RV right ventricle
Fig. 106.6 No papillary muscle is found in the apical two-chamber
view on the anterior and inferior walls ( arrow ). LA left atrium, LV left
ventricle
ab
Fig. 106.7 There is only one papillary muscle, which is attached to the posterior ( arrow ) ( a ) and lateral ( arrow ) ( b ) walls. LA left atrium, LV left
ventricle, RA right atrium, RV right ventricle, AO aorta
Case 106 Parachute Mitral Valve and Interrupted Aortic Arch
346
ab
Fig. 106.9 The interrupted aortic arch is seen in the suprasternal long-axis view ( arrow ) ( a , b ), aortic arch branches are also evident in these views
( arrowheads )
Fig. 106.8 No papillary muscle is seen on the anterior and inferior
walls ( arrows ). There is only one papillary muscle, which is attached to
the lateral and posterior walls ( arrowheads ). LV left ventricle
Case 106 Parachute Mitral Valve and Interrupted Aortic Arch
347
Diagnosis
The patient was diagnosed with a parachute mitral valve and
an interrupted aortic arch.
Comment
The patient was referred for surgery at the fi rst stage for
interrupted aortic arch and then for parachute mitral valve.
Lesson
Parachute mitral valve is a rare congenital anomaly of
mitral valve. In parachute mitral valve, there is a single
papillary muscle in the left ventricle instead of two papillary muscles; this papillary muscle has a central location,
chordae tendineae of both mitral leafl ets are attached to
this single papillary muscle, and there is mitral stenosis of
varying severity. In any congenital disease that a small
amount of fl ow passes across the left atrium and left ventricle, there is a tendency for developing coarctation of
aorta later in life. Association between parachute mitral
valve and interrupted aortic arch is rare. It has been
hypothesized that left persistent superior vena cava which
connects to the coronary sinus may have a pressure effect
on the left atrium and left ventricle and produces parachute mitral valve or other forms of hypoplasia of the left
system [ 2 ]. Bicuspid aortic valve is a common fi nding
with parachute mitral valve [ 120 – 122 ].
Fig. 106.10 A bicuspid aortic valve is seen in the parasternal short- axis
view. LA left atrium, AO aorta, 1 fi rst aortic leafl et, 2 second aortic
leafl et
Fig. 106.11 There is a mild increased gradient across the aortic valve
(peak and mean gradients of 14 and 7 mmHg, respectively)
Case 106 Parachute Mitral Valve and Interrupted Aortic Arch
349
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_107, © Springer International Publishing Switzerland 2015
A 13-year-old girl with an 11-year history of valvular disease
presented with dyspnea on exertion (functional class III) and
two episodes of pulmonary edema. Physical examination
revealed a diastolic rumble (grade III/VI) in the mitral area.
Parachute Mitral Valve
Case 107
ab
Fig. 107.1 The apical four-chamber view shows a dilated left atrium,
bowing of the interatrial septum toward the right atrium, dysplastic and
underdeveloped mitral valve leafl ets ( a ), and signifi cant diastolic
turbulency in the color Doppler fl ow study ( b ), suggestive of mitral
stenosis. RA right atrium, LA left atrium, LV left ventricle, RV right
ventricle
Fig. 107.2 The continuous wave study across the mitral valve shows a
peak gradient of about 39 mmHg and a mean gradient of about
24 mmHg, denoting severe mitral stenosis
Fig. 107.3 The mitral valve annulus is approximately 18 mm in the
parasternal long-axis view. LV left ventricle, RV right ventricle, AO
aortic valve, LA left atrium
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_107 ) contains supplementary material,
which is available to authorized users.
350
Diagnosis
The patient was diagnosed with a parachute mitral valve and
severe mitral stenosis.
Comment
The patient was referred for mitral valve repair surgery.
Lesson
This patient had an isolated dysplastic mitral valve (parachute mitral valve) with a single papillary muscle and an
abnormal insertion site. Although the parachute mitral
valve is usually accompanied by the coarctation of the
aorta or the interrupted aortic arch, the isolated form may
exist [ 123 ].
Fig. 107.6 The suprasternal long-axis view by pulse wave Doppler
echocardiography reveals a normal ascending aorta fl ow, indicative of
no valvular or supravalvular aortic stenosis
ab
Fig. 107.4 The short-axis view demonstrates a prominent central
papillary muscle ( arrow ) ( a ) and the absence of another papillary muscle.
There is one single papillary muscle. The mitral valve seems to originate
from the prominent papillary muscle, and it leans asymmetrically in the
left ventricle ( b ). RV right ventricle
ab
Fig. 107.5 The suprasternal long-axis view shows normal aortic arch, ascending aorta, and descending aorta ( a ), (no supravalvular aortic stenosis
or coarctation of the aorta) via the color Doppler study ( b ). RPA right pulmonary artery
Case 107 Parachute Mitral Valve
351
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_108, © Springer International Publishing Switzerland 2015
A 12-year-old girl presented with a history of easy fatigability
and cyanosis with clubbing, which was more prominent in the
upper extremities (differential cyanosis). Echocardiography
revealed that the inferior vena cava drained into the right
atrium, pulmonary vein drained into the left atrium, left
atrium was connected to the left ventricle, right ventricle was
connected to the right atrium (atrioventricular concordance),
left ventricle leaned to the left side, and right ventricle was on
the right side (D-loop).
Interrupted Aortic Arch,
Transposition of Great Arteries,
Atrial Septal Defect, Ventricular
Septal Defect, and Patent
Ductus Arteriosus
Case 108
Fig. 108.1 The subcostal fi ve-chamber view shows that a dilated pul-
monary artery (bifurcated great artery) ( arrowheads ) is connected to
the left ventricle. PA pulmonary artery, LV left ventricle, RV right
ventricle
Fig. 108.2 A relatively small and underdeveloped aorta is connected
to a hypertrabeculated right ventricle (ventriculoarterial discordance) in
the subcostal fi ve-chamber view, with a full anterior tilting of the probe.
RV right ventricle, AO aorta
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_108 ) contains supplementary material,
which is available to authorized users.
352
Fig. 108.3 The subcostal long-axis view reveals that the two great
arteries are parallel to each other. AO aorta, PA pulmonary artery
a b
Fig. 108.4 The suprasternal long-axis view indicates the interrup-
tion of the aorta in the distal portion of the aortic arch ( arrowhead )
after the subclavian artery ( a ). This schematic illustration of the
suprasternal long-axis view depicts the interruption of the aortic
arch after the subclavian artery and the connection between the right
pulmonary artery and the descending aorta via a large patent ductus
arteriosus (PDA) ( b ). DAO descending aorta, RPA right pulmonary
artery
Case 108 Interrupted Aortic Arch, Transposition of Great Arteries, Atrial Septal Defect, Ventricular Septal Defect
353
ab
c
Fig. 108.5 The subcostal descending aorta view reveals that the pul-
monary valve has mild insuffi ciency ( a ) and that the descending aorta is
connected to the pulmonary artery across a PDA tunnel ( head arrow in
a and b ). This is a schematic illustration of the PDA in this view ( c ).
PDA patent ductus arteriosus, DAO descending aorta, PA pulmonary
artery, AO ascending aorta
ab
Fig. 108.6 The transposition of the great arteries: the coronary artery
( arrow ) originates from the anterior great artery ( a ), denoting that the
ascending aorta is anterior and left sided to the pulmonary artery. The
great artery which is located posteriorly is divided; it is, therefore, the
pulmonary artery ( b ). ( a , b ) Short-axis view. AO aorta, PA pulmonary
artery
Case 108 Interrupted Aortic Arch, Transposition of Great Arteries, Atrial Septal Defect, Ventricular Septal Defect
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