Echocardiography a case based review 2e (2013)

Contents Normal Images and Values Liver Function Test Abnormalities, Extremity Edema 28 Exertional Shortness of Breath, Bilateral Lower CASE9 Coronary Artery Grafting Extremity Edema, a

Echocardiography

A Case-Based Review

Echocardiography

A Case-Based Review

Garvan C Kane, MD, PhD Co-Director, Echocardiography Laboratory Consultant, Division of Cardiovascular Diseases

Assistant Professor of Medicine

Mayo Clinic Rochester, Minnesota

Jae K Oh, MD Cardiology Co-Director, Integrated Cardiac Imaging Consultant, Division of Cardiovascular Diseases

Professor of Medicine

Mayo Clinic Rochester, Minnesota

Co-Director, Cardiac and Vascular Center

Samsung Medical Center Seoul, South Korea

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Includes bibliographical references and index

ISBN 978 - 1 -45 1 1 -096 1 -0 (alk paper)-ISBN 1 -45 1 1 -096 1 -X (alk paper)

I Oh, Jae K II Oh, Jae K Echo manual III Title

[DNLM: 1 Heart Diseases-ultrasonography-Case Reports 2 Heart Diseases-ultrasonography­ Examination Questions 3 Echocardiography-methods-Case Reports 4 Echocardiography­

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In memory of Mark J Callahan, MD

Pref ace

Echocardiography has become an essential diagnostic

tool across the spectrum of cardiovascular disease

In the era of rapid technology development and the

increasing use of other imaging modalities, echocar­

diography remains the single most useful imaging

technique which couples comprehensive hemody­

namic data with information on cardiac structure and

systolic and diastolic function Strain-based imaging

now provides sensitive incremental quantitation of

myocardial function with 3-dimensional echocardiog­

raphy allowing real-time views of detailed true cardiac

anatomy

We have been very gratified by the success of The

Echo Manual, currently in its 3rd edition, which has

aided the education of physicians and sonographers

since its first publication in 1 994 As its name indi­

cates, the book is first and foremost a manual to pro­

vide instruction to the learner on the various aspects

of echocardiography and their clinical applications,

highlighting the strengths and limitations of the mo­

dality and providing the reader the necessary steps to

accomplish a comprehensive, quantitative diagnostic

examination

Echocardiography is a dynamic modality and The

Echo Manual has not had real-time moving images

or actual clinical cases to utilize comprehensive echo­

cardiographic data including hemodynamic calcula­

tion In this new case-based echocardiography book,

we hope to fill this void, highlighting the importance

of interpreting echocardiographic images in the set­

ting of the clinical scenario This book is designed as a

guide for learners in the use of echocardiography data

in the evaluation of patients through review of 1 00

selected cases from the Mayo Clinic with a variety of

clinical conditions, both commonly and uncommonly

encountered We have placed a particular emphasis on

the assessment of systolic and diastolic function and

quantitative hemodynamics throughout This book needs to be used in tandem with review of the mov­ ing images, available through the on-line site We rec­ ommend each case be reviewed in isolation from start

to finish following the order of the case questions In many cases, further echocardiographic images play an important role as the clinical cases develops, whether transesophageal, intracardiac or supplemental or sub­ sequent transthoracic imaging While we have selected cases that hopefully are useful to the learner, whether new or old to the practice of clinical echocardiogra­ phy, we have tried to keep the cases true to life Images

at times are 'presentation quality' while at other times challenging and we have chosen to portray the cases

in a predominantly random order The focus of this book is on the interpretation of the echocardiographic data to provide guidance in the management of the patient While acting as a stand-alone educational tool, this case-book also serves as a companion to The Echo Manual, 3rd Edition Here we provide an answer and explanation to each question asked and we also include the location of a more detailed discussion of the topic in The Echo Manual 3rd Edition

We have had and continue to have the good for­ tune to learn and practice echocardiography with a wonderful team of physicians and sonographers We thank our colleagues for sharing their expertise and interesting cases with us We would like to thank our families, time from whom was taken to help com­ plete this project Finally we thank our patients for their educational images and we hope that this book will provide the reader with the tools to better diag­ nose and manage their patients through high quality echocardiography

Garvan C Kane ]aeK Oh

v i i

Contents

Normal Images and Values Liver Function Test Abnormalities,

Extremity Edema 28 Exertional Shortness of Breath, Bilateral Lower CASE9 Coronary Artery Grafting Extremity Edema, and Systolic Murmur after 78

CASE24

Increasing Dyspnea and Orthopnea 34

CASE25

CASE26

Systolic Murmur, Asymptomatic 39

CASE27

CASE28

Progressive Dyspnea with Asthma, Rhinosinusitis, Acute Pleuritic Chest Pain and

i x

Progressive Exertional Dyspnea, Atypical Chest

Discomfort, Abdominal and Lower Extremity

of Breath and Lower Extremity Edema

CASE 55

Exertional Shortness of Breath CASE 56

Hyperlipidemia, Hypertension and Carotid Disease

CASE60 Sharp Chest Pain CASE 61 Transient Loss of Vision with Modest Exertional

C O N T E N TS I X I

Diffuse ST-Segment Depression 200 Exertional Shortness of Breath

Functional Class Ill Exertional Dyspnea 202 CASE 81

Murmur with Systemic Hypertension 205

CASE 82

CASE 83

Transient Loss of Motor Function in Right Upper Shortness of Breath 266

CASE 84

Class Ill Shortness of Breath 221 Exertional Shortness of Breath in

Intermittent Dyspnea with Modest

X I I I C O N T E N TS

CASE 96

Exertional Shortness of Breath

with Episode of Atrial Flutter

Contents by Subject

Cardiac Masses and Aortic Diseases CASE 87

CASE 89 CASE 21

Fever and Dyspnea with History of Severe

Hematuria with Large Renal Mass 88 Ventricular Tachycardia with Rapid

Progressive Fatigue and Marked Lower CASE 92

CASE43

Murmur with Systemic Hypertension 205 Three Month History of

Transient Loss of Motor Function in

Exertional Shortness of Breath, Systolic CASE46

Murmur and Lower Extremity Edema 234 Severe lschemic Cardiomyopathy

CASE48

CASE 79

Progressive NY HA Class 11-111 Exertional Dyspnea and

Systolic and Diastolic Murmurs 262 Exertional Shortness of Breath 179

x i i i

X I V I C ONT E NTS BY SUBJE C T

Exertional Dyspnea with Systemic Hypertension 332

Coronary Artery Disease, including Stress Congenital Heart Disease Echocardiography

CASE28

CASE30

CASE 31

CASE32

Exertional Shortness of Breath with

CASE35 Systolic Blood Pressure of 110 mm Hg 218

Exertional Fatigue, Lower Extremity Dyspnea with Modest Exertion 165

CASE63

History of Symptomatic Paroxysmal

CASE69

Laterally Displaced Apical Impulse 291 CASE 73

Murmur in a 38-Year-Old Patient 306

CASE 81

C O N T E N TS BY S U B J E C T I XV

Native and Prosthetic Valvular Disease, CASE 71

including Infective Endocarditis Class I l l Shortness of Breath 221

Acute Severe Dyspnea 25 Three Months of Progressive

Systolic Murmur, Asymptomatic 39

CASE 85

Exertional Shortness of Breath and

CASE 86

CASE 98

CASE20

Progressive NYHA Class I l l Dyspnea 66 Pericardial and Right-sided Disease,

Exertional Shortness of Breath, Bilateral Lower CASE 16

Extremity Edema, and Systolic Murmur after Liver Function Test Abnormalities, Ascites,

Dyspnea and Chest Tightness 110 Harsh Systolic Murmur in Nursery Examination 63

CASE27

CASE 29

CASE 54 Acute Pleuritic Chest Pain and Lightheadedness 99

Congenital Aortic Valve Stenosis Status Post

CASE 39

Transient Loss of Vision with Modest Exertional CASE45

XVI I C ONT E NTS BY SUBJE C T

CASE49

Progressive Exertional Fatigue and Lower

Extremity Edema

CASE 50

Progressive Exertional Shortness of Breath

and Lower Extremity Edema

Systemic Diseases

CASE 15

Progressive Dyspnea with Asthma,

Rhinosinusitis, Weight Loss, and

CASE42 Progressive Exertional Dyspnea, Atypical Chest Discomfort, Abdominal and Lower Extremity

CASE 52

CASE 58 Progressive Exertional Shortness of Breath with Diabetes and Systemic

CASE 80 Exertional Shortness of Breath with

Echocardiography

A Case-Based Review

C A S E 1

Normal Images and Val ues

B values from a comprehensive echocardiographic examination Please review all mov­ efore looking at abnormal studies, we need to be familiar with normal images and ing (V ideos 1-1 to 1-23) and still images (Figs 1 - 1 to 1 - 1 8 ) obtained from an otherwise healthy normal individual who was referred for a transthoracic echocardiogram in the set­ ting of palpitations A comprehensive echocardiography study demonstrated no abnormal findings Also shown are animations demonstrating four standard transthoracic echocar­ diographic views and representative images obtained from each view (Animations 1 - 1 to

1 -4) After your review of all images, please answer the following questions You should be able to answer and understand all the questions to be able to provide diagnostically helpful data to clinicians ordering an echocardiogram

2 I E C H O C A R D I O G RA P H Y: A C A S E - B AS E D R E V I EW

C A S E 1 I 3

··�

f'YI :J V•� 13 �, 1-.'P 1 2 182 tlrutG

' :: :.·

,_

I .- ••

4 I E C H O C A R D I O G RA P H Y: A C A S E - B AS E D R E V I EW

Figure 1 - 1 7

QUESTION 1 Please estimate left ventricular ejection

fraction (L VEF) by your visual subjective reading

Write that ejection fraction (EF) down somewhere

Then, calculate L VEF from left ventricular (L V) end­

diastolic dimension of 45 mm and end-systolic dimen­

sion of 28 mm (This is a Quinones, simple method for

calculating EF, which we still use in our clinical prac­

tice.) If you are not familiar with this equation, please

see pages 1 1 5 to 1 1 6, section on ejection fraction, in

The Echo Manual, 3rd Edition Please compare your

visual EF with the EF from Quinones method You

need to understand the apical factor

QUESTION 2 Which of the following is the modified

QUESTION 3 From this case, calculate right ventric­

ular systolic pressure (RVSP) How would you report

C Deceleration time 1 60 to 240 milliseconds

D Mitral medial annulus e' 1 0 cm per second

QUESTION 6 Which of the following statements is correct regarding left atrial (LA) volume?

A Enlarged LA volume does not always indicate increased L V filling pressure

B Healthy individuals cannot have large LA volume

C LA volume is always increased in patients with atrial fibrillation

QUESTION 7 Which of the following conditions

can be correctly diagnosed from abdominal aortic

pulsed wave Doppler examination?

A Mild to moderate aortic regurgitation (AR)

B Constrictive pericarditis

C Aortic coarctation

D Hypertrophic cardiomyopathy (HCM)

QUESTION 8 What value of diastolic reversal flow

time velocity integral (TVI) in the proximal descend­

ing aorta indicates severe AR?

A 5 cm

B 1 0 cm

C 1 5 cm

D Depends on heart rate

QUESTION 9 Which of the following segments is

not seen from apical three- or five-chamber (long­

useful in all of the following situations except

A Diastolic function assessment

B Aortic stenosis

C Mitral valve regurgitation

D After atrial fibrillation ablation procedure

QUESTION 11 Which of the following situations is best to use V alsalva maneuver?

A To differentiate Grade 2 from Grade 1 diastolic dysfunction

B To differentiate aortic stenosis from HCM

C To evaluate patent foramen ovale

D To differentiate constriction from restriction

tion fraction on pages 1 1 5 to 1 1 6, and Figure 7-1 on

page 1 1 0

ANSWER 2: A The Mod ified Berno u l l i eq uation is 4

x velocity2, which ca n be further s i m p l ified as (2 x ve­

locity)2

The s i m p l ified method is more p ractica l for velocities

such as 3 5 m per second Rather than squaring 3 5 m

per second fi rst before bei n g m u lti p l i ed by 4, (2 x 3 5)

is 7 , which ca n be sq uared to provide the va l ue of 49

tra n sva lvu l a r g ra d i e nts on pages 63 to 66, a n d

Figure 4-8 on page 6 5

ANSWER 3: D Using the mod ified Bernou l l i equation,

the tricuspid reg u rg itation (TR) velocity of 1 8 mis g ives

a tra nstricuspid va lve g rad ient of 1 3 mm H g If you add

a normal right atri a l p ressu re estimate of 5 m m H g ,

RVS P is 2 3 m m H g H owever, t h e TR vel ocity p rofi l e

is i ncom p l ete a n d l i kely u n d e restimated, a n d w e may

j u st say that p u l monary a rtery systol i c pressu re is nor­

m a l rather than g iving a n actual va l u e Right ventri c u l a r

outflow tract velocity also shows ra p i d onset o f systol i c

velocity com pati ble with a n o r m a l RVS P

ANSWER 4: D Stroke volume (SV) is calcu lated from the

left ventricular outflow tract (LVOT) as a prod uct of the

LVOT a rea (LVOT diameter x 0 785) and the LVOT TVI

Cardiac output is the prod uct of SV and hea rt rate

Here SV = (2 3 cm)2 x 2 1 cm = 1 1 1 m l at a heart rate of

69 beats per m i n ute = 7 7 L per m i n ute

See The Echo Manual, 3rd Edition, Fig u re 4- 1 6 on

page 7 1

ANSWER 5: D A l l other parameters co u l d a lso be

consistent with normal myoca rd i a l relaxation but not

specific since a com b i nation of h i g h fi l l i n g pressu re a n d

a b n o r m a l re laxation ca n g ive a s i m i l a r va l u e f o r E/A,

IVRT, a n d deceleration time Early d iasto l i c velocity of

the m itra l a n n u l u s has been fou n d to have a good cor­

relation with ta u which is the gold-sta n d a rd measu re

of myoca rd i a l relaxation by ca rd iac catheterization Al­

most if not a l l of myoca rd i a l d i seases have a bnormal

myoca rd i a l relaxatio n

ANS WER 6: A Any chron ic elevation i n LA pressure

wi l l lead to LA d i latation over time H owever, i n the

a bsence of elevation i n LA pressure, modest degrees of

LA d i l atation may occur in the setting of atria l fibril lation

C h ronic diastolic dysfunction also produces LA enlarge­ment without an increase in fi l l i ng pressure Wel l tra i ned hea lthy i n d ividuals ca n have increased LA vol ume In that situation, you wou l d expect a n increased stroke volume

ANSWER 7: C Abdo m i n a l aortic velocity shows d ia­stolic flow reversal i n severe AR Ascending aorta shows a notched velocity i n patients with hypertrophic obstructive cardiomyopathy, but it is not usually seen i n abdom i n a l aorta Nonobstructive H C M does not have characteristic flow velocity pattern in the aorta Freq uently, character­istic abdom inal aorta pu lse wave Doppler velocity g ives

an i n itia l d iag nostic clue for coa rctatio n

ANS WER 8 : C Descending aorta pu lse wave Doppler

is very helpful i n determ i n i n g the severity of aortic re­

g u rgitation especia l ly when AR jet is eccentric Although there a re not many p u b l i cations regard i n g the param­eter, time velocity i nteg ra l of d iastolic reversa l flow ve­locity from the descending aorta (by placing the sa mple volume away from the inner wa l l of the aorta at the level

of the left su bclavia n artery) of 1 5 cm or g reater ind icates severe AR There a re however several other cond itions which ca n g ive a s i m i la r d iastolic reversal flow velocities

ANS WER 9: A The i nferior septum is seen from the

a pica l fou r-cha m ber view I nferior wa l l is seen from api­cal 2 cham ber view

ANSWER 10: B The p u l monary vei n Doppler profi le

g ives i nsig hts i nto the d iastolic function (systolic blunting suggests elevated LA pressure), presence of severe mitral valve reg urg itation (systolic flow reversals), or the presence

of pulmonary vei n stenosis (a compl ication after a pulmo­nary vei n isolation procedu re) There is no specific pulmo­nary vei n Doppler finding that suggests aortic stenosis

ANSWER 11: C The Valsalva maneuver red uces venous return by i ncreasi n g i ntrathoractic pressure It is often helpful (but not a lways) to differentiate pseudo-normal­ized m itra l i nflow (grade 2) from true normal m itral in­flow However, the disti nction ca n be done now easi ly by tissue Doppler imaging with the early diastolic velocity of the m itral a n n u l us (e') One of most va luable indications for the Va lsa lva maneuver is to assess right to left shunt via the patent foramen ovale Upon release of the ma­neuver, venous return i ncreases to the right atri i u m and aug ment or demonstrate right to left atrial shunt

C A S E 2

Syncopal Episode

M s NE is a 1 9-year-old woman who is referred for a transthoracic echocardiogram after a syncopal episode She was at college basketball practice and felt briefly light­

headed and then passed out, striking her head and sustaining a scalp laceration She has

no known cardiac history and is on no medications On physical examination, her blood

pressure is 1 00/62 mm Hg and heart rate 50 beats per minute with a regular rhythm

Carotid pulses and jugular venous pulse were normal Precordial examination was normal

apart from a 1 16 systolic ejection murmur

QUESTION 1 Concerning measurement of left

ventricular (L V) dimensions (see Video 2-1 and Figs 2- 1

and 2-2 ), which of the following statements is correct?

C Using the apical contractility correction factor (assuming it is normal) , the calculated LV ejec­ tion fraction is 58%

D All of the choices

7

8 I E C H O C A R D I O G RA P H Y: A C A S E - B AS E D R E V I EW

QUESTION 2 The estimated pulmonary artery systolic

(PASP) and diastolic pressures (PADP) (see Video 2-2

(see Video 2-3 and Figs 2-5 to 2-7 ) is:

Figure 2-5

Figure 2-6

Figure 2-7

A Normal diastolic function

B Grade 1 (impaired diastolic dysfunction)

C Grade 2 (delayed relaxation diastolic dysfunc­

tion)

D Grade 3 (restrictive diastolic dysfunction)

QUESTION 4 This pulsed wave flow pattern taken in

the abdominal aorta (see Fig 2-8 ) would be compat­

ible with:

Figure 2-8

A Coarctation of the descending thoracic aorta

B What is seen in the majority of patients with a

bicuspid aortic valve

C Patent ductus arteriosus

D Severe congenital aortic stenosis

C A S E 2 I 9

QUESTION 5 Transthoracic echocardiography can exclude the following potential causes for syncope in

a young women except:

A Critical congenital aortic stenosis

B Dilated cardiomyopathy with reduced ejection

fraction

C Pulmonary arterial hypertension

D Aortic dissection

E Hypertrophic cardiomyopathy

1 0 I E C H O C A R D I O G RA P H Y: A C A S E - B AS E D R EV I EW

ANSWER 1: D As recommended by the America n

Society o f Echoca rd iography, t h e long- a n d short-axis

d i mensions can be obta ined d i rectly from the end-systolic

and end-d iastolic d imensions (ESd and EDd), measu red at

the level of the m itra l tips as the smal lest and largest

diameters, respectively If there a re no reg ional wal l mo­

tion abnormal ities, the LV d i mensions measured from the

level of the pap i l l a ry m uscles can be used to calculate the

left ventricular ejection fraction (LVEF) as fol lows:

U n corrected LVE F = [(E Dd)2 - (ESd)2] I (EDd)2] x 1 00

C orrected LVE F = uLVEF + [( 1 00 - uLVEF)

x 1 5 % ] uLVE F = u ncorrected LVE F

Here,

U n corrected LVE F = [(50)2 - (35)2] I (50)2]

x 1 00 = 5 1 % Corrected LVE F = 5 1 + [( 1 00 - 5 1 ) x 1 5 % ] = 5 8 %

See The Echo Manual, 3rd Edition, page 1 09

ANSWER 2: C I n the a bsence of p u l monary stenosis,

demonstrated here by a p u l monary va lve that opens

n o rm a l ly on two-d i me n s i o n a l (2D) i m a g i n g without

turbu lence on color flow i m a g i n g and a pea k velocity

of only 1 2 m per second, the rig ht ventric u l a r (RV) sys­

tol i c p ress u re is eq u iva lent to the PAS P RV systol i c (a n d

therefore P A systol ic) p ressu re ca n rel ia b ly be estimated

on the basis of Doppler i nterrogation of tricuspid va lve

reg u rg itation RV systol i c p ressu re is ca lculated by add­

ing a n esti mate of rig ht atri a l p ressu re to the pea k g ra­

d ient between the RV and the RA ( i e , fou r times [the

pea k tricuspi d reg u rg ita nt velocity]2)

Here RV systol i c p ress u re = 4 (2 1 )2 + right atria l

p ress u re

= 1 8 + RA p ress u re

page 1 45, and pages 1 44 to 1 46

The d i asto l i c PA p ressu re can be est i mated by a d d ­

i n g a n estimate o f r i g h t atri a l p ress u re t o the g radient

between the PA a n d the RV i n end d iastole (i e , fou r

times [the e n d p u l monary reg u rg ita nt velocity]2)

See The Echo Manual, 3rd Edition, text and Figu re 9-7

= 8 m m H g See The Echo Manual, 3rd Edition, pages 1 43 t o 1 47

ANSWER 3: A The m itra l i nfl ow patte rn (F i g 2 - 5)

d e m o n strates that m ost of LV fi l l i n g occu rs early i n

d i astol e with a n E velocity o f 0 7 m p e r secon d with

a re l atively s h o rt dece l e ration t i m e of 1 6 1 m i l l isec­ond and a d i m i n utive A velocity of 0 3 m per secon d (E/A ratio o f 2 5) D o p p l e r i nte rrogation o f the p u l ­

m o n a ry ve i n s (Fi g 2-6) d e m o n strates a d i asto l i c p re­dom i n a nt pattern This com b i nation of fi n d i ngs wou l d

p o i n t towa rd a restrictive, G ra d e 3 , seve re d i asto l i c dysf u n ction pattern

However, one a lso notes that 2 D i mages demonstrate normal LV size a n d ejection fraction a n d the left atri u m

is a lso o f n o r m a l s i z e F i g u re 2-7 demonstrates t h e med i a l m itra l a n n u l us tissue Doppler with evidence of excel lent myoca rd i a l relaxation with an e prime (e ' ) ve­locity of 1 3 cm per seco n d

I n n o r m a l you n g people, L V rel axation is s o vigorous that the negative p ressu res generated i n the l eft ven ­tricle lead t o a l m ost a l l fi l l i n g t o occ u r early i n d iastole, leavi n g l ittl e for atr i a l contraction to contri bute It is not u ncommon to see d o m i n a nt d iasto l i c forwa rd flow velocity i n p u l monary vei n i n hea lthy you n g i n d ivid u a l Aga i n , the most i m porta nt para m eter for assessing d ia­sto l i c function is the status of myoca rd ial relaxation as­sessed by e' If e ' velocity is normal, d iasto l i c fu nction

is usually norm a l An i m porta nt d i sease condition with normal e' velocity (from med i a l m itra l a n n u l us) is con­strictive perica rditis

See The Echo Manual, 3rd Edition, F i g u re 8-22 on page 1 33, and text on pages 1 32 to 1 36

ANSWER 4: B H e re s h own is an exa m p l e of a n o r­

m a l p u lse wave D o p p l e r assessment of the a b d o m i n a l aorta taken from t h e su bcosta l l o n g itu d i n a l p l a n e Th is

s h o u l d be p a rt of the sta n d a rd echoca rd i o g ra p h i c ex­

a m i nation espec i a l ly in yo u n g patie nts with ca rd i

ovas-c u l a r sym ptoms a n d/o r hyperte n s i o n S h own h e re a re

a b r i s k systo l i c u pstro ke, b r i s k systo l i c d own stro ke,

and a sma l l early d i asto l i c fl ow reversa l (seen below

the base l i n e) N ote a l so the l a c k of forwa rd fl ow The

typ ical fi n d i n g s i n coa rctation of the a o rta a re a p ro­

l o n g e d t i m e to peak ve locity a n d pers i stent fo rwa rd

d i asto l i c fl ow La rge d i asto l i c fl ow reve rsa l s a re a fea­

t u re of a pate nt d u ct u s a rteriosus o r seve re a o rt i c

reg u rg itation Seve re a o rtic ste n osis typ i ca l ly wi l l h ave

a b l u nted pea k systo l i c ve locity with a decreased t i m e

t o pea k Alth o u g h a p a t i e n t w i t h a b i c u s p i d a o rtic

va lve i s at risk for coa rctati o n , a o rt i c va lve ste nosis,

and reg u rg itat i o n-a ny of wh i c h if seve re may g ive

rise to a b no r m a l ities on p u lse wave i nterrog ation of

t h e a bd o m i n a l a o rta-t h e vast m aj o r i ty of patients

with a b i c u s p i d va lve wi l l h ave a n o r m a l fi n d i n g as

t o exc l u d e a rrhyth moge n i c right ve ntri c u l a r dysplasia

wh ich may req u i re ca rd iac com p uted tomography or mag netic resona nce i m ag i n g

di rected echo exam of syncope on pages 396 and 397

C A S E 3

Progressive Exertional Shortness of Breath

M r HM is a 42-year-old man with a 7-month history of progressive exertional short­ ness of breath (NYHA functional class III) He is a prior smoker, but has no other

known cardiovascular disease His family history is notable for sudden unexplained death

of his mother at the age of 40 His only sibling who is 5 5 years of age recently underwent

a normal cardiovascular comprehensive examination with echocardiography

On examination, his blood pressure is 1 25/70 mm Hg and heart rate 60 bpm His

carotid pulses have brisk upstrokes, and his central venous pressure is normal He has a

sustained and localized left ventricular (L V) apical impulse There is a 2/6 systolic ejection

murmur that increases when the patient goes from a squat position to standing

He is referred for transthoracic echocardiography (see Video 3-1 )

A 1 6 mm Hg

associated with an increased risk of sudden cardiac

death in this case?

B 49 mm Hg

C 64 mm Hg

D 1 36 mm Hg

A Septal wall thickness

B Sudden death of his mother at the age of 40

C Severe central mitral valve regurgitation

D Nonsustained ventricular tachycardia on a

Holter

QUESTION 2 Calculate the maximal instantaneous

intracavitatory gradient (see Fig 3- 1 )

A 90 mm Hg

B 1 20 mm Hg

C 1 50 mm Hg

D 200 mm Hg

Q UES TION 4 In hypertrophic cardiomyopathy

(HCM) , what happens to the left ventricular outflow

tract (LVOT) gradient after a PVC (see Fig 3-2 ) ?

Figure 3-2

A It goes up

B It goes down

QUESTION 5 With regard to the management of this

patient, which of the following is true?

A The overall complication rate is greater with

surgical myectomy than with alcohol septal ab­

lation

B The risk of sudden cardiac death would be lower

after septal alcohol ablation than after surgical

myectomy

C Complete heart block is more likely with alco­

hol ablation than with surgical myectomy

D Alcohol septal ablation would obviate the need

for defibrillator to reduce the risk of sudden

A Ventricular septal defect

B Significant aortic valve regurgitation

C Coronary-ventricular fistula

D Residual systolic anterior motion of the mitral valve with mitral valve regurgitation

1 4 I E C H O C A R D I O G RA P H Y: A C A S E - B AS E D R EV I EW

ANS WER 1: C Echoca rd iog ra p hy in this case dem­

onstrates seve re i ncrease in ci rcu mfe renti a l LV wa l l

thickness with massive thicke n i n g of the i ntraventricu­

lar septu m (septa l d i mension of 43 mm) LV ejection

fraction is norma l I n H C M , severa l c l i n i ca l a n d echo­

card i o g ra p h i c factors have been associ ated with a n

i n c reased risk o f sudden ca rd iac death The five most

freq uently cited factors a re as fol l ows :

Sudden C a rdiac Death Risk Stratification i n H C M

I LV wa l l thickness � 3 0 m m (typ ica l ly septum-but

a ny wa l l )

I I A b n o r m a l blood p ressu re response t o exercise

I l l Nonsusta i ned ventric u l a r tachyca rd ia

IV Fa m i ly h i story of SCD

V Recu rrent syncope1-3

sentence i n the right col u m n

ANSWER 2: C 6 4 m m H g Th is conti n uous wave Dop­

pler obta i ned from the a pex i l l u strates two signals: the

LVOT (right) obstruction a n d superim posed i ncomplete

s i g n a l of m itra l reg u rg itation (left) It ca n be someti mes

d i ffi cu lt to d isti n g u ish between the m In H C M , m itra l

reg u rg itation usua lly beg i n s at m idsystole when there

is systo l i c a nterior motion of the m itra l va lve There­

fore, the Doppler spectrum of m itra l reg u rg itation is

often i n com p l ete a n d may su perfi c i a l ly rese m b l e the

LVOT fl ow velocity spectru m H owever, the rising slope

at m i dsystole is usual ly perpe n d i c u l a r to the base l i n e

i n m itra l reg u rg itation, whereas i t is curvi l i near u n t i l it

reaches the h i g hest velocity in the LVOT s i g n a l F u r­

t h e r m o re , t h e m itra l reg u rg itation ve locity s i g n a l

extends beyond ejection a n d c u l m i nates i n m itra l for­

wa rd flow d u ri n g the onset of diastole Remember that

the m itra l reg u rg itation velocity w i l l always be more

(7 m per second) than that of the LVOT jet velocity

(4 m per second) U s i n g the mod ified Bernou l l i equa­

tion, the pea k p ressu re g rad ient may be ca lculated from

the late-peaking dagger-sha ped LVOT Doppler signal as

fol l ows 4v2 = 4(4)2 = 64 m m H g

and Figures 1 5- 1 4 to 1 5-1 7 o n page 261

ANSWER 3: C 1 5 0 m m H g With t h e ava i l a b l e

data, LV systo l i c p ress u re c a n b e ca l c u l ated i n two

ways I n t h e h e m odyn a m i c echoca rd i o g ra p h i c as­

sessment of a patient with H C M , it is good p rac­

tice to d e rive both measu res to e n s u re i ntern a l

consisten cy LV systol i c p ressu re equals t h e pea k g ra­

d ient across the m itra l va lve (4v2; where v is the pea k

m itra l reg u rg ita nt velocity) p l us a n estimate of left atria l

p ress u re I n this case, 4(7)2 + 2 0 = 2 1 6 m m H g

LV p ressu re = systo l i c b l oo d p ress u re +

i ntracavitatory g ra d i ent Systo l i c blood p ress u re = LV p ress u re - i ntracavitatory

g rad ient Systo l i c blood p ress u re = 2 1 6 - 64 = 1 52 m m Hg Alternatively (in the a bsence of aortic va lve d i sease),

LV p ress u re a lso equals systo l i c blood p ressu re p l us the

i ntracavitatory g rad ient

Hence, using this strategy, one can estimate the LVOT

g rad ient i n two sepa rate ways i n H C M patients i n the echo laboratory: the fi rst through d i rectly measu ri n g

t h e pea k velocity o f t h e LVOT conti n uous wave Doppler signal and the second using the pea k velocity of the m itral reg u rg itant contin uous wave Doppler signal and the bra­chia! systolic blood pressure It is advisable to ensure the consistency of these two strategies wherever possi ble See The Echo Manual, 3rd Edition, fi rst paragraph

on page 26 1 See Circulation

ANSWER 4: A It goes u p D u ring the prolonged phase

of LV fi l l i n g associated with a prematu re ventric u l a r complex, there is a n i ncrease i n LV vol u m e that i n turn potentiates a n i ncrease i n LV p ressu re I n this setting, there is a d ifferential physiologic response in the setting of fixed (e g , aortic stenosis) or dynamic (e g , hypertrophic obstructive card iomyopathy) LV obstructio n The i ntensity

of the systolic flow increases i n aortic stenosis and H C M The aortic pressure increases i n fixed obstruction, but de­creases or remains uncha nged in dyna m i c obstruction These findi ngs i n patients with H C M reflect the B rocken­broug h-Brau nwald-Morrow sign where postextrasystolic potentiation resu lts i n a n i ncreased LVOT g radient with decreased or u nchanged aortic pu lse pressure

S e e The Echo Manual, 3rd Edition, F i g u re 1 5-1 5 on page 261

ANSWER 5: C Pati ents with hypertro p h i c obstruc­tive ca rd iomyopathy, who rem a i n sym ptomatic (NYHA class Ill to IV) despite medica l therapy, a re can d idates for

i nvasive thera py with either surgical myectomy or septa l

a blati o n The goal of either thera py is to relief the out­flow tract obstruction through p hysica l remova l (myec­tomy) or t h ro u g h therapeutic i n fa rction of the excess septa l m uscle mass In experienced centers, the i n hos­pita l morta l ity rates with s u rgical myectomy a re l ow a n d

the overa l l success rates h i g h Patients with concomi­

ta nt orga n i c m itra l va lve d isease or obstructive coronary

d i sease a re ca n d idates for com b i ned surg ical corrective

procedures M itra l reg u rg itation related to systol i c an­

terior motion of the m itra l va lve is usua l ly corrected by

myectomy without a m itra l va lve proced u re Both surgi­

ca l septa l myectomy a n d septa l a l cohol a b lation red uce

LVOT obstruction and i m p rove sym ptom g rade To date,

there has been no ra ndomized com pa rison tria l of my­

ectomy versus ablatio n A recent meta-a na lysis indicates

a si m i la r in hospita l morta lity (0 6 % for myectomy and

1 6 % for a blation) Septa l myectomy a p pea rs to have

a lower rate of permanent pacemaker i m p l a ntation for

complete heart block (3 3 % vs 1 8 4%), a h igher suc­

cess rate (req u i red repeat proced u re 0 6 % i n myectomy

patients vs 5 5 % in a blation patients) Published data

point to a poss i b l e red uction i n sudden ca rd iac death

References

1 Spirito P, Bellone P, Harris KM, et al Magnitude of left

ventricular hypertrophy and risk of sudden death in hypertrophic

cardiomyopathy New Engl J Med 2000;342: 1 778-1785

2 McKenna WJ, Behr ER Hypertrophic cardiomyopathy: manage­

ment, risk stratification, and prevention of sudden death Heart

2002;87: 1 69

3 Maron BJ, McKenna WJ, Danielson GK, et al American College

of Cardiology/European Society of Cardiology clinical expert

consensus document on hypertrophic cardiomyopathy A report

of the American College of Cardiology Foundation Task Force

on Clinical Expert Consensus Documents and the European

Society of Cardiology Committee for Practice Guidelines J Am

Coll Cardiol 2003;42: 1 687

4 McLeod CJ, Ommen SR, Ackerman MJ, et al Surgical septal my­

ectomy decreases the risk for appropriate implantable cardioverter

defibrillator discharge in obstructive hypertrophic cardiomyopa­

thy Eur Heart] 2007;28:2583-2588

5 Ommen SR, Maron BJ, Olivotto I, et al Long-term effects of

surgical septal myectomy on survival in patients with obstructive

hypertrophic cardiomyopathy.j Am Coll Cardiol 2005;46:470-476

C A S E 3 I 1 5

and rates of appropriate defi bri l l ator discharges fol l ow­

i n g myectomy This does not appear to be the case fol­lowi ng septa l a blation 4-9

ANS WER 6: C A co m p l i cation that i s re l atively

u n i q ue to surgical myectomy is the u n roofing of a n in­tra m u ra l septa l coro n a ry a rtery at the myectomy site, thus creati n g a coro n a ry to LVOT fistu l a Seen here

is a Doppler flow s i g n a l , below the aortic va lve, a ris­ing from with i n the septa l myoca rd i u m i nto the LVOT Two d i sti n ct cha ra cte rist i cs separati n g a coro n a ry fist u l a from a ve ntric u l a r septa l d efect a re d i rectio n (i nto t h e left ventricle as opposed t o typica l ly i nto t h e right ventricle) a n d ti m i n g (d iasto l i c as opposed t o sys­tol ic) Postmyectomy septa l coronary to LV fistu la is very rarely of any c l i n i ca l sign ifi cance 1 0

6 Qin JX, Shiota T, Lever HM, et al Outcome of patients with hypertrophic obstructive cardiomyopathy after percutaneous transluminal septal myocardial ablation and septa! myectomy surgery J Am Coll Cardiol 200 1 ;38: 1 994-2000

7 Sorajj a P, Valeri U, Nishimura RA, et al Outcome of alcohol septa! ablation for obstructive hypertrophic cardiomyopathy

2005 ; 1 1 1 :2033-2038

1 0 Bax ]], Raphael D, Bernard X, et al Echocardiographic detection and long-term outcome of coronary artery-left ventricle fistula after septal myectomy in hypertrophic obstruc­ tive cardiomyopathy J Am Soc Echocardiogr 200 1 ; 1 4:308-3 1 0

She is referred for transthoracic echocardiography (V ideos 4- 1 to 4-6 and Figs 4- 1

to 4-5 )

QUESTION 3 Doppler-derived aortic valve pressure

gradients are typically slightly lower than that of the

catheter-derived aortic valve pressure gradients

A True

B False

C A S E 4 I 1 7

lead to a disproportionately elevated aortic mean gra­ dient for a given aortic valve area?

A An increase in left ventricular (L V) contractility

B Anemia

C Aortic valve regurgitation

D All of the choices

QUESTION 5 Which of the following is the next best management step?

A Aortic valve replacement

B Coronary angiography and then aortic valve re­ placement

C Treadmill exercise testing

D Hemodynamic catheterization to assess the aor­ tic valve area

QUESTION 6 Which of the following statements

is not correct regarding left ventricular outflow tract (L VOT) time velocity integral (TVl) and aortic valve

1 8 I E C H O C A R D I O G RA P H Y: A C A S E - B AS E D R EV I EW

ANS WER 1: A Aortic va lve a rea = [(LVOT TVI) x

(LVOT a rea)]/Ao TVI

= [(LVOT TVI) x (0 7 8 5 (LVOT D)2]/Ao TVI

= [(2 5) x (3 1 4)]/1 2 0

= 7 8 5/1 2 0

= 0 6 5 cm2

Here two conti n uous wave Doppler signals a re shown,

one from the a pex (F i g 4-4) and one from the right

pa rasternal a rea (Fig 4-5) I n a com p rehensive exa m i ­

nation o f a patient with aortic stenosis, it is critica l to

perform a Doppler eva l uation from a l l ava i la b l e tra ns­

d ucer windows Fifteen to twenty percent of the time,

the pea k signal wi l l be obta i ned from a win dow other

than the apex

See The Echo Manual, 3rd Edition, d i scussion

of Doppler echoca rd iography i n aortic stenosis on

pages 1 90 and 1 9 1

ANS WER 2: D B l ood flow velocity (v) measu red

with D o p p l e r ech oca rd i o g ra p hy re l i a b l y reflects the

p ressu re g ra d ient accord i n g to the mod ified Bernou l l i

equation Accord i n g to t h e equation, p ressu re g radi­

ent = 4v2 H ere the pea k tra nsaortic flow vel ocity is

5 5 m per secon d , which corresponds to a pea k tra ns­

aortic p ressu re g ra d i ent of 1 2 1 mm H g

ANS WER 3 : B False There typ ica l ly is a sma l l d if­

ference between the Doppler-derived a n d cath eter­

derived aortic va lve p ressu re g radients beca use of the

p ressu re recovery phenomeno n Pa rt of the ki netic en­

e rgy lost d u r i n g flow passag e t h ro u g h a sma l l orifice

is recovered Therefore, this p ressu re recovery resu lts

i n a h i g her a bsol ute p ressu re i n the asce n d i n g aorta

away from the ste n otic aortic va lve, expl a i n i n g why

the catheter-derived p ressu re g rad ient i s l ower than

the Doppler-derived p ress u re g rad ient (Doppler echo­

ca rd iogra phy measu res the h i g h est va l ue) Pressu re re­

covery is smaller when the aorta is d i lated However,

Reference

1 Omran H, Schmidt H, Hackenbroch M, et al Silent and appar­

ent cerebral embolism after retrograde catheterization of aortic

valve in valvular stenosis: a prospective, randomized study Lan­

cet 2003;36 1 : 1 24 1 - 1 246

p ress u re recovery may be an i m porta nt factor in caus­ing a d iscrepancy between echo-derived aortic va lve

a rea a n d catheter-derived aortic va lve a rea

ANS WER 4: D When LV systol i c function a n d ca r­diac output a re abnormally h i g h , the fol l owi n g poi nt should be considered : pea k velocity a n d mean aortic

g ra d ient va ry with chan ges i n stroke vol u m e In pa­tients with increased ca rd iac output across the aortic

va lve (as i n aortic reg u rg itation or anem ia), aortic ste­nosis may not be severe even when the pea k velocity is

4 5 m per secon d or g reater a n d the mean g ra d ient is

50 m m H g or h igher Aortic va lve a rea should be more helpf u l i n determ i n i n g the severity of aortic stenosis i n those situations

ANS WER 5: B This woman has sym ptoms a n d signs

of severe aortic va lve stenosis The echoca rd i ogram demonstrates a mea n systol i c g rad ient over 50 m m H g

a n d a va lve a rea <0 7 5 cm2 T h e d iag nosis is clea r, a n d

no fu rther assessment i s req u i red G iven t h e patients age, a coronary a n g iogra m is a p p ropriate to assess for concomitant coronary a rtery d isease However, a he­modyn a m i c l eft heart catheterization is in most cases

u n n ecessa ry a n d should be avoided A su bsta nti a l por­tion of patients develop subcl i n ical as wel l as c l i n ica l embolic lesion after a hemodyn a m i c ca rd iac catheter­ization for aortic stenosis 1

ANS WER 6: C The ratio of the TVI of the LVOT to that of the aortic va lve is a usefu l parameter that does not req u i re the measu rement of the LVOT d i a meter

Th i s " d i mension l ess i n d ex " is h e l pf u l parti c u l a rly in cases of a very heavi ly ca lcified va lve where a n accu­rate meas u rement of the LVOT d i a m eter is not fea­

s i b l e As the severity of the aortic stenosis i ncreases (decreasi n g aortic va lve a rea), the ratio wi l l d ecrease

A cutoff of <0 2 5 corresponds with a severely stenotic aortic va lve

C A S E 5

Systemic Hypertension

A 2 1 -year-old man with systemic hypertension is referred for transthoracic echocardiogram

associated with this aortic valve finding (see Video 5-1 ) ?

A Aortic valve stenosis

B A similar long-term survival to those with a nor­

mal aortic valve anatomy

C Thoracic aortic dissection

D 2: 1 Female:male incidence

QUESTION 2 Which of the following cusps is fused

(V ideo 5-1 and Fig 5- 1 ) ?

Figure 5-1

A Right and left cusps

B Right and noncoronary cusps

C N oncoronary and left cusps

QUESTION 3 Which of the following patients with

bicuspid aortic valve disease requires endocarditis pro­

from the abdominal aorta (Fig 5-2) indicates:

Figure 5-2

A Normal flow

B Contamination of the signal from a mesenteric artery

C Coarctation of the descending thoracic aorta

D Severe aortic valve regurgitation

1 9

2 0 I E C H O C A R D I O G RA P H Y: A C A S E - B AS E D R EV I EW

QUESTION 5 Which of the following findings is un­

likely to be present on clinical examination?

A Radiofemoral pulse delay

B An elevated femoral arterial pressure

C A midsystolic murmur heard over the back

D Rib notching on chest roentography

QUES TION 6 Which of the following statements concerning bicuspid aortic valve and coarctation of the descending thoracic aorta is true?

A Coarctation of the aorta is present in 1 5 % to 20% of patients with bicuspid aortic valve

B Bicuspid aortic valve is present in 5% to 1 0% of patients with coarctation of the aorta

C Bicuspid aortic valve is present in 50% to 75%

of patients with coarctation of the aorta

D Coarctation and bicuspid valve are present to­ gether only in female patients