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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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430
a
b
Fig. 130.2 The apical four-chamber view demonstrates that the ratio
of the functional right ventricle to the anatomic right ventricle is about
16.5/30 = 55 %. The functional right ventricle is measured via the right
ventricular area at the displaced septal leafl et point ( b ), and the ana-
tomic right ventricular area is measured at the annulus ( a )
Fig. 130.3 The apical four-
chamber view reveals the
signifi cant tethering of the
anterior tricuspid valve leafl et
( arrow ) ( a ) and severe tricuspid
regurgitation ( b ). RA right
atrium, RV right ventricle
Case 130 Ebstein’s Anomaly with Severe Tricuspid Regurgitation
431
Fig. 130.5 Right ventricular and
atrial evaluation by tissue
Doppler imaging: the strain
curves are demonstrated in three
points, i.e., the green sample in
the mid-septal segment, yellow
sample at the site of the septal
displacement of the tricuspid
valve (anatomic right atrium),
and the red sample in the
interatrial septum. The green
diagram on the left is in favor
of ventricular strain; the red
diagram is in favor of atrial strain
[ 143 ]; and the yellow diagram,
despite its position in the
anatomic right atrium, shows
ventricular strain
Fig. 130.4 The right ventricular ejection fraction is 40 %, which is in favor of mild right ventricular systolic dysfunction (four-dimensional
reconstruction)
Case 130 Ebstein’s Anomaly with Severe Tricuspid Regurgitation
432
Diagnosis
The patient had Ebstein’s anomaly, severe tricuspid regurgitation, apical displacement of the septal leafl et, tethering of
the anterior leafl et, moderate right ventricular dilation, and
mild right ventricular systolic dysfunction.
Comment
The patient had no cyanosis, good functional class, and no
clinical evidence of right-sided ventricular failure. As a
result, medical therapy and follow-up echocardiography
were recommended.
Lesson
Surgery should be considered with more than moderate
tricuspid regurgitation and NYHA functional class more
than II [ 7 ].
Case 130 Ebstein’s Anomaly with Severe Tricuspid Regurgitation
433
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_131, © Springer International Publishing Switzerland 2015
A young man presented with a history of frequent episodes
of palpitation and exercise intolerance of 3-month duration.
Physical examination showed a blood pressure of
130/80 mmHg, regular pulse rate of 85 beats per minute,
normal jugular venous pressure, widely split S1 with a loud
tricuspid component (sail sound), right-sided third heart
sound, and pansystolic murmur, which increased with inspiration and could be best heard at the lower left sternal
border. The body surface area was equal to 1.7 m 2 . The
patient was a candidate for the radiofrequency ablation of
paroxysmal supraventricular tachycardia. Transthoracic
echocardiography showed a normal left ventricular size
with mild systolic dysfunction and a left ventricular ejection
fraction of about 45 %. The right ventricular systolic function, in terms of the fractional area change (FAC), was
normal (FAC = 66 %).
Ebstein’s Anomaly with Severe
Tricuspid Regurgitation, Severe
Septal Displacement Ratio, and
Grade 3 Right Atrial Cavity Ratio
Case 131
a
b
Fig. 131.1 Severe right ventricular dilation ( a ) and visible tricuspid leafl ets ( b , c ), which are an unusual fi nding in the parasternal long-axis view.
RV right ventricle, TV tricuspid valve 1 posterior tricuspid valve leafl et, 2 anterior tricuspid valve leafl et, 3 septal tricuspid valve leafl e t
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_131 ) contains supplementary material,
which is available to authorized users.
434
a
b
c
Fig. 131.2 The apical four-chamber view reveals the apical displace-
ment and tethering of the septal and anterior leafl ets of the tricuspid valve.
The distance between the septal leafl et and the mitral valve annulus is
about 72 mm. The septal displacement alone is 46 mm (27 mm/m
2
), and
the displacement and tethering of the septal leafl et is approximately
26 mm, which is in favor of Ebstein’s anomaly ( a ). The smaller yellow
arrow shows the apical displacement of the septal tricuspid valve leafl et,
and the larger green arrow depicts the sail-like appearance of the anterior
tricuspid valve leafl et and its displacement in a four-dimensional reconstruction ( b ). (The tethering of the anterior leafl et is more prominent,
while the displacement of the septal leafl et is more evident.) This is a
schematic illustration ( c ) of the right ventricular atrialization, displace-
ment and tethering of the septal leafl et of the tricuspid valve, and tethering and displacement of the anterior leafl et of the tricuspid valve ( c ). LA
left atrium, LV left ventricle, RA right atrium, RV right ventricle
c
Fig. 131.1 (continued)
Case 131 Ebstein’s Anomaly with Severe Tricuspid Regurgitation, Severe Septal Displacement Ratio
435
Fig. 131.3 The apical four-chamber view illustrates the elongated sail-
like appearance and tethering and displacement (33 mm) of the anterior
tricuspid valve leafl et. RA right atrium
a
b
Fig. 131.4 The cavity area ratio is measured as follows: the right atrial area ( a )/left atrial area+ left ventricular area + right ventricular area ( b ) in
the apical four-chamber view = 71.1/48.7 = 1.45 (grade 3)
Fig. 131.5 The septal leafl et attachment ratio is estimated at about
0.62 (severe displacement). This ratio is estimated by dividing the apical displacement of the septal leafl et (from the atrioventricular ring) by
the septal length in the apical four-chamber view (in diastole)
(11.1 – 4.2/11.1 = 62 %) and is a representation of the scoring for the
severity of the tricuspid abnormality in Ebstein’s anomaly. A ratio less
than 0.45 is mild, between 0.5 and 0.6 is moderate, and greater than 0.6
is representative of the severe displacement of the tricuspid valve [ 144 ]
Case 131 Ebstein’s Anomaly with Severe Tricuspid Regurgitation, Severe Septal Displacement Ratio
436
a
b
Fig. 131.6 This is severe tricuspid regurgitation and poor coaptation of the tricuspid valve leafl ets ( b ). ( a , b ) Apical four-chamber view and para-
sternal short-axis view
a
b
Fig. 131.7 The apical four-chamber view depicts a partially aneurysmal interatrial septum. ( a , b ) Apical four-chamber view in systole and
diastole
Diagnosis
The patient was symptomatic and had Ebstein’s anomaly with
severe tricuspid valve regurgitation; severe apical displacement of the septal leafl et of tricuspid valve; apical displacement and tethering of the septal, posterior, and anterior leafl ets
of the tricuspid valve; and severe right ventricular dilation.
Comment
There was no evidence of right-sided failure and no cyanosis but limited functional capacity. Thus, the patient was
referred fi rst for supraventricular tachycardia ablation and
reevaluation after the treatment for arrhythmia. Decision
was made to candidate the patient for surgery if the symptoms of limited functional capacity persisted. Repair would
be possible in an experienced hand and if tethering of anterior tricuspid leafl et is not more than one half. When anterior tricuspid leafl et is mobile and the ratio of functional
right ventricle to anatomic right ventricle is more than one
third, repair is preferable over replacement by creating a
monoleafl et tricuspid valve [ 7 ].
Case 131 Ebstein’s Anomaly with Severe Tricuspid Regurgitation, Severe Septal Displacement Ratio
437
Lesson
1. This is a rare case of Ebstein’s anomaly with the tethering
and displacement of the anterior tricuspid valve leafl et.
(The involvement of the anterior leafl et of the tricuspid
valve is rarely seen in Ebstein’s anomaly [ 146 ].)
2. The indications for surgery in Ebstein’s anomaly include
the following:
(a) Substantial cyanosis
(b) Right-sided heart failure
(c) Poor functional capacity
(d) Perhaps the occurrence of paradoxical emboli and
relative indications include the following:
(i) Recurrent supraventricular arrhythmia uncon-
trolled by medical and ablation
(ii) Substantial cardiomegaly (cardiothoracic ratio
>65 %) [ 2 ]
3. The cavity ratio has been correlated with a long-term
clinical outcome or successful tricuspid valve repair. The
increased severity of the cavity area ratio indicates higher
morbidity and mortality and less likelihood of the success
of surgery (grade 1 ≤ 0.5, grade 2 = 0.5–0.99, grade
3 = 1–1.49, and grade 4 ≥ 1.5) [ 147 ].
4. In Ebstein’s anomaly, right ventricular fractional area
change may be within normal limits, but small functional
right ventricle may produce symptoms and signs of right
ventricular systolic dysfunction.
Fig. 131.8 The multiplane modality of a
four-dimensional probe shows the apical
displacement of the three leafl ets of the
tricuspid valve in the apical four-chamber
view ( arrowhead , septal leafl et, curved
arrow , anterior leafl et), apical two-chamber
view (anterior or septal and posterior
leafl ets), and apical three-chamber view
(septal and posterior leafl ets) [ 145 ]
Case 131 Ebstein’s Anomaly with Severe Tricuspid Regurgitation, Severe Septal Displacement Ratio
439
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_132, © Springer International Publishing Switzerland 2015
A middle-aged man with a history of palpitation was referred
to our echocardiography laboratory. He underwent ablation
for supraventricular arrhythmia, but there was a recurrence
of arrhythmia despite medical treatment. Echocardiography
revealed normal left ventricular size and systolic function
and mild right ventricular dilation with mild right ventricular
systolic dysfunction. His body surface area was equal to
1.6 m
2
.
Ebstein’s Anomaly with Severe
Tricuspid Regurgitation, Mild
Septal Displacement Ratio,
and Grade 4 Right Atrial
Cavity Ratio
Case 132
Fig. 132.1 The apical four-chamber view shows that the apical dis-
placement of the septal leafl et of the tricuspid valve is about 14 mm
(>8 mm/m
2
), 14/1.6 = 8.75. It also demonstrates the severe redundancy
of the anterior leafl et of the tricuspid valve ( arrow ). LA left atrium, LV
left ventricle, RA right atrium, RV right ventricle
Fig. 132.2 The apical four-chamber view reveals a huge right atrium
of about 50 cm
2
as well as grade 4 of the right atrial cavity ratio as calculated via this formula: RA area/LA + LV + RV area (50/28 = 1.8). LA
left atrium, LV left ventricle, RA right atrium, RV right ventricle
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_132 ) contains supplementary material,
which is available to authorized users.
440
a
b
Fig. 132.3 Midesophageal transesophageal echocardiography (TEE)
(10° and 113°) of the tricuspid valve shows severe tricuspid regurgitation. The coaptation of the septal and anterior tricuspid valve leafl ets is
evident in ( a ), while the failure of the coaptation of the anterior and
posterior tricuspid valve leafl ets is evident in ( b ). The arrows in ( b )
point to the tricuspid valve leafl ets in systole. RA right atrium, SVC
superior vena cava
Case 132 Ebstein’s Anomaly with Severe Tricuspid Regurgitation, Mild Septal Displacement Ratio
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