Cardiology Intensive Board Review, 3th Edition

Chapter 2 - Valvular Heart Disease

Dermot Phelan, Maran Thamilarasan

QUESTIONS

Case 1

A 60-year-old man presents to the emergency room with complaints of weakness, lethargy, and severe dyspnea. One week prior, his family notes that he complained of chest pressure that lasted for several hours. On physical examination, he appears to be in respiratory distress. Blood pressure (BP) is 80/50 mmHg. Heart rate is 130 bpm. His oxygen saturation is 87% on room air. Chest examination reveals diffuse crackles. Cardiac examination reveals a nondisplaced point of maximum impulse (PMI). Third and fourth heart sounds are heard, as is an apical systolic murmur. No thrill is present. Electrocardiogram reveals inferior Q waves without ST-segment elevation. He is urgently intubated and pressors are started. An intra-aortic balloon pump is placed. A surface echocardiogram reveals a normal-sized left atrium and a mild jet of mitral regurgitation (MR).

1.What test do you perform first?

a.Cardiac catheterization

b.Transesophageal echocardiography (TEE)

c.Right heart catheterization with an oxygen saturation run

d.Administration of thrombolytic therapy

2.A TEE is performed urgently (Fig. 2.1 shows a 3D view of the mitral valve from above). What is the most likely diagnosis?

a.Endocarditis involving the mitral valve

b.Posterior papillary muscle rupture as it has a single blood supply

c.Anterior papillary muscle rupture as it has a single blood supply

d.Severe mitral valve prolapse secondary to recent myocardial infarction

Case 2

A 65-year-old woman presents to your office for follow-up of a murmur she was told about several years prior. She denies any symptoms, but is not very active. Her past medical history is significant for hypertension and diabetes, both of which have been well controlled. On examination, she is in no acute distress. BP is 125/75 mmHg, with a resting heart rate of 70 bpm. Lungs are clear. Cardiac examination reveals a displaced PMI. S1 is soft. S2 reveals an increased P2 component. There is a right ventricular (RV) lift. An S3 is present. There is a grade III/VI holosystolic murmur heard at the apex radiating to the base. She has no peripheral edema. Chest X-ray demonstrated cardiomegaly with prominence of the central pulmonary vasculature.

Figure 2.1

3.An echocardiogram is performed on this patient (Fig. 2.2). Left ventricular (LV) systolic dimension is 4.7 cm. Ejection fraction is 45%. There is posterior leaflet prolapse. There is a very eccentric jet of MR, which is read out as 2+. Which of the following is most likely?

a.MR is unlikely to account for her presentation.

b.She likely has more severe MR than is evident on the echocardiogram.

c.Her LV function is better than it appears on the echocardiogram.

d.TEE is unlikely to be helpful here.

Figure 2.2

4.What do you recommend next?

a.Stress echo, to assess LV and PA (pulmonary artery) pressures post stress

b.Mitral valve surgery

c.Start an angiotensin-converting enzyme inhibitor (ACEI) and reassess in 3 months

d.Start a β-blocker

Case 3

A 40-year-old woman is referred to your office for evaluation of a murmur heard during a routine physical examination. She is asymptomatic. She used to jog 2 to 3 miles a day without problems but over the past few years has stopped exercising. She had frequent febrile illnesses as a child, but her past medical history is otherwise unremarkable.

Physical Examination

BP 120/70 mmHg, pulse 73 bpm.

She is in no acute distress.

Jugular venous pulse (JVP) is not elevated.

Chest is clear.

Cardiac—PMI not displaced. Regular rate and rhythm. S1 is increased in intensity. S2 is normal. A high-pitched diastolic sound is heard at rest and is heard best between the apex and left sternal border, 0.10 seconds after S2. This is followed by a low-pitch decrescendo murmur with pre-systolic accentuation.

Abdomen—No organomegaly.

Extremities—No edema. Normal distal pulses. Good capillary refill.

An echocardiogram is performed (Fig. 2.3); proximal flow convergence radius (PFCR) using color 3D across the mitral valve indicates an orifice area of 1.2 cm2. Resting PA pressures are 35 mmHg. Splittability score is 5. LV size and function are normal.

Figure 2.3

5.Which of the following would be the most reasonable next step in management?

a.Immediate referral for surgery

b.Immediate referral for percutaneous valvuloplasty

c.Stress echocardiogram, to assess for mitral pressures post stress

d.Follow-up in 2 years

6.A stress echocardiogram is performed. Patient exercises for 6 metabolic equivalents (METs). Right ventricular systolic pressure post stress is estimated at 70 mmHg. Which of the following would be an appropriate next step?

a.Consideration for percutaneous valvuloplasty

b.Mitral valve replacement

c.Start β-blocker and return for follow-up in another 2 years

d.Start digoxin

Case 4

A 50-year-old woman presents to you for evaluation. She complains of easy fatigability, as well as abdominal fullness and right upper quadrant pain. She also notes marked swelling in her legs. She has recently been diagnosed with asthma and is also undergoing evaluation for recurrent diarrhea. On examination, she has a BP of 100/60 mmHg. Heart rate is 96 bpm. There is elevation in jugular venous pressure, with a large a wave and a prominent v wave. Lungs are clear. Cardiac examination reveals a nondisplaced PMI. Rhythm is regular. S1 and S2 (including P2) are normal. A diastolic murmur is heard along the sternal border, which increases with inspiration. A pansystolic murmur is also heard in this area. Hepatomegaly is present, along with ascites and peripheral edema.

7.What is the most likely cause of this patient’s signs and symptoms?

a.Rheumatic heart disease

b.Carcinoid

c.Primary pulmonary hypertension

d.Cirrhosis of the liver secondary to chronic hepatitis

Case 5

A 28-year-old man is referred to your office for a second opinion regarding his hypertension. On physical examination, he is in no acute distress. BP is 160/90 mmHg, symmetric in both arms. Pulse rate is 75 bpm. Cardiac examination reveals a nondisplaced PMI. S1 is normal. It is followed by a high-pitched sound widely transmitted throughout the precordium. A short II/VI systolic ejection murmur is heard. S2 is normal.

8.What is the most important diagnostic test to perform next?

a.Check plasma catecholamines.

b.Check serum potassium level.

c.Check lower extremity BP.

d.Check plasma cortisol levels.

Case 6

A 59-year-old man presents for further evaluation of recurrent congestive heart failure. He appears to be in no acute distress on your evaluation. BP is 100/60 mmHg. Carotid upstrokes are weak, but not delayed. Chest examination shows minimal bibasilar rales. PMI is displaced and sustained. A summation gallop is present. There is an increased P2. There is mild peripheral edema. An echocardiogram reveals a dilated LV with an ejection fraction of 25%. The aortic valve does have some calcification, with restricted leaflet excursion. Peak/mean gradients are 25/15 mmHg. By the continuity equation, the aortic valve area is calculated as 0.7 cm2.

9.What is your next step?

a.Immediate referral for aortic valve replacement (AVR)

b.Referral for cardiac transplant

c.Dobutamine echocardiogram

d.Start an ACEI

10.With dobutamine echocardiography, the gradients across the valve increase to 60/40 mmHg, and the calculated valve area stays at 0.7 cm2. What do you recommend?

a.AVR

b.Continued medical management

c.Cardiac transplant evaluation

d.Balloon aortic valvuloplasty

11.Alternatively, how would you interpret the following results: an increase in stroke volume by 5% and an increase in peak/mean gradients to 30/19 mmHg without a significant change in the aortic valve area?

a.Patient has true severe aortic stenosis (AS) and should proceed to surgery.

b.Patient has pseudo-AS and should be managed with medical therapy alone.

c.Patient has a lack of contractile reserve and should be managed with medical therapy alone.

d.Patient has a lack of contractile reserve but should still be considered for AVR.

Case 7

A 32-year-old man with known bicuspid aortic valve is referred to you for management of aortic insufficiency (AI). He is completely asymptomatic and jogs 3 miles a day as well as doing other aerobic exercise for 30 minutes daily. He has a grade III/VI systolic and diastolic murmur at his left sternal border, a collapsing pulse on examination, and his BP 170/70 mmHg. An echocardiogram reveals a mildly dilated LV (end-diastolic dimension of 6.0 cm) with an ejection fraction of 65%. There is prolapse of the conjoined aortic leaflet with 3 to 4+ insufficiency.

12.What is your recommendation?

a.Referral for surgery

b.Addition of vasodilator therapy

c.Observation for now, return for follow-up in 3 years

d.Cardiac catheterization

13.What do you tell him is his yearly risk of sudden death?

a.<1%

b.2%

c.3% to 5%

d.>5%

14.The above patient undergoes a gated computed tomography angiography of the thorax (Fig. 2.4), what would you recommend?

a.Observation with echocardiography every 6 months

b.Start a β-blocker and reassess in 6 months

c.Refer to computed tomographic (CT) surgery for surgical replacement of his aortic valve

d.Referral for surgical intervention to repair or replace his aortic valve and to replace his ascending aorta

Case 8

A 76-year-old woman has been accepted for AVR for severe symptomatic AS. Your opinion is sought by the cardiothoracic surgeon regarding best management of reported concomitant valvular lesions. On review of the echocardiogram you confirm severe AS. In addition, you note a morphologically normal mitral valve, mild MR, and moderate tricuspid regurgitation (TR) associated with annular dilation (45 mm). There is also mild pulmonary hypertension.

Figure 2.4

15.What do you recommend?

a.AVR alone

b.AVR with mitral and tricuspid valve repair

c.AVR with tricuspid valve replacement

d.AVR with tricuspid valve repair if feasible

e.AVR and mitral valve repair alone

Case 9

A 46-year-old woman with chronic obstructive pulmonary disease is referred by her pulmonologist for evaluation of a murmur and concern that her symptoms of shortness of breath with moderate exertion may be related to severe MR diagnosed on an outside echocardiogram. On examination, her body mass index is 19 kg/m2, BP is 130/75 mmHg, and her heart rate is 75 bpm and regular. Her apex beat is nondisplaced. On auscultation, S1 and S2 are normal; there is a mid-systolic click with a grade IV/VI late systolic murmur heard best at the apex. An echocardiogram is performed (Fig. 2.5A).

16.Assuming an aliasing velocity of 40 cm/s and an MR Vmax of 5 m/s, based on the PFCR seen here, what is the estimated effective regurgitant orifice area (EROA)?

a.0.4 cm2

b.0.45 cm2

c.0.18 cm2

d.Not enough information to calculate an EROA

17.A continuous-wave signal is provided through the mitral valve (Fig. 2.5B); based on the data provided how would you classify this MR?

a.1+, mild

b.2+, moderate

c.3+, moderately severe

d.4+, severe

Figure 2.5A

Figure 2.5B

Case 10

A 45-year-old man with rheumatic mitral stenosis presents for further evaluation. In the past 2 to 3 years, he has noted progressive dyspnea with less than moderate activity. He was started on a β-blocker 1 year ago, but remains symptomatic. Echocardiogram reveals a mean mitral gradient of 4 mmHg with a valve area of 1.6 cm2. As there was a discrepancy between the degree of symptoms and resting hemodynamics you proceed to a stress echocardiogram that revealed a post stress PA pressure of 70 mmHg and a mean transmitral gradient of 17 mmHg. You decide to send this patient for percutaneous intervention.

18.What is the most appropriate test to order at the time of or prior to the valvuloplasty procedure?

a.Transesophageal echocardiogram

b.24-Hour electrocardiographic monitoring to assess for paroxysmal atrial fibrillation

c.Cardiac CT to assess for aortic calcification

d.Stress nuclear perfusion study

Case 11

A 65-year-old man is referred to you for evaluation of a heart murmur. He denies any symptoms at this time. On physical examination, he is in no acute distress. BP is 135/75 mmHg; pulse is 82 bpm and regular. Carotid upstrokes are diminished. The PMI is sustained and displaced. A2 is soft. A late-peaking systolic murmur is heard at the base. You order an echocardiogram. This reveals LV hypertrophy with moderate global impairment of LV function, calculated ejection fraction of 35%. There is severe calcific AS, with peak/mean gradients of 75/45 mmHg. Aortic valve area is 0.5 cm2.

19.What is the role of AVR in this setting?

a.It is absolutely indicated.

b.It is absolutely not recommended.

c.There is some evidence/opinion that would favor valve replacement.

d.Dobutamine echocardiography is needed to determine whether this is truly severe AS.

Case 12

A 76-year-old woman is referred to your clinic with recent onset of exertional chest pain. She has a long-standing history of hypertension and atrial fibrillation. On examination, her body surface area is 2.0 m2, BP is 150/100 mmHg, and heart rate is 80 to 90 bpm and irregular. The carotid upstroke is delayed and diminished. The apex beat is nondisplaced but sustained. S1 is normal, and S2 is soft and paradoxically split. There is a grade II/VI ejection systolic murmur heard best at the right upper sternal border that radiates to the carotids. An echocardiogram reports normal ejection fraction with a stroke volume of 55 mL. The peak and mean gradients across the aortic valve are 44/28 mmHg. The dimensionless index is 0.21 and the calculated aortic valve area is 0.83 cm2. You review the echocardiogram (Fig. 2.6) and confirm the accuracy of the left ventricular outflow tract (LVOT) diameter and are satisfied that multiple windows were used to obtain the gradients.

20.Which of the following statements is true?

a.The patient has moderate AS confirmed by gradients across the valve and should be followed up in 6 months with a repeat echocardiogram.

b.The echocardiogram shows inconsistent data and should be repeated.

c.This is a definite contraindication to AVR.

d.The rate of mortality, for a patient with these findings, is higher compared with patients with severe AS and high gradients across the aortic valve, but aortic valve surgery has resulted in better outcomes in these patients.

Figure 2.6

Case 13

A 75-year-old man is referred to you for evaluation of aortic regurgitation. He has no symptoms at this time. His past medical history is significant only for hypertension. On physical examination, he is in no acute distress. BP is 170/60 mmHg. Arterial pulses are brisk. A bisferiens pulse is noted in the brachial artery. The apical impulse is displaced and hyperdynamic. S1 is not loud, and no opening snap is heard. A high-frequency holodiastolic murmur is heard, loudest along the right sternal border. A late diastolic apical rumble is heard as well.

21.You order an echocardiogram. Which of the following are you most concerned about?

a.Aortic valve commissural anatomy

b.Degree of AI

c.Aortic root dimension

d.Mitral valve

22.The above patient returns for follow-up 6 months later. He now reports symptoms of marked exertional dyspnea. An echocardiogram is read as 2+ central aortic regurgitation, with an LV end-diastolic dimension of 6.9 cm and an ejection fraction of 50%. What do you do next?

a.Cardiac catheterization with aortography

b.Start an ACEI, reassess in 6 months

c.Continue observation

d.Start a β-blocker, reassess in 6 months

Case 14

A 56-year-old man presents to the emergency room with the sudden onset of chest pain. He is tachypneic on presentation. O2 saturation is 82% on room air. BP is 80/60 mmHg. Heart rate is 125 bpm. Lung examination reveals diffuse bilateral crackles. Cardiac examination reveals a nondisplaced PMI. S1 is soft. P2 is loud. An S3 is present. A short decrescendo diastolic murmur is heard at the upper sternal border. Extremities are cool. Electrocardiogram reveals inferior ST-segment elevation. He is promptly intubated, and pressors are started. A brief echocardiogram is performed at the bedside. The study is difficult, but reveals premature closure of the mitral valve. There is hypokinesis of the inferoposterior walls.

23.Which of the following would be your next course of action?

a.Transesophageal echocardiogram, emergent cardiac surgical consultation

b.Intra-aortic balloon pump to stabilize hemodynamics, followed by emergent angiography

c.Administer thrombolytics

d.Send patient for magnetic resonance imaging (MRI)

Case 15

A 77-year-old patient is admitted to the hospital for urosepsis. His past medical history is significant only for having undergone AVR 5 years prior. On examination, he is febrile to 102°F. Heart rate is 106 bpm. Carotid upstrokes are full. Chest examination reveals clear lung fields. Cardiac examination reveals a hyperdynamic apical impulse, which is not displaced. S1 and S2 are normal. An early-peaking systolic murmur is heard at the sternal border. No diastolic murmur is heard. An echocardiogram is performed. Peak/mean gradients are 50/30 mmHg. LVOT VTI (velocity time integral) is 36 cm and aortic valve VTI is 78 cm. The aortic valve itself is not well seen. Flow in the descending thoracic aorta is normal. An echocardiogram 2 years prior had revealed peak/mean gradients of 24/12 mmHg. LVOT VTI was 19 cm and aortic valve VTI 41 cm.

24.What do you conclude about prosthetic aortic valve function?

a.He has prosthetic valve stenosis

b.No evidence for dysfunction

c.He has severe prosthetic valve regurgitation

d.He likely has endocarditis

25.The above patient remains febrile despite 1 week of antibiotic therapy. Electrocardiogram reveals a new long first-degree atrioventricular (AV) block. The patient becomes progressively dyspneic. A short, regurgitant murmur is heard. What do you recommend?

a.TEE with surgical consultation

b.TEE

c.Change antibiotic regimen

d.Monitor closely with daily electrocardiogram

Case 16

A 56-year-old man with mitral stenosis presents for evaluation. He has NYHA class II-III shortness of breath.

Physical Examination

He is in no acute distress.

JVP is mildly elevated.

Pulse is regular at 80 bpm.

Chest is clear.

Cardiac: Nondisplaced PMI. Opening snap heard 0.09 milliseconds after S2. Long diastolic rumble. No peripheral edema.

Echocardiogram reveals a planimetered mitral valve area of 1.2 cm2. Mean gradient 10 mmHg. Pressure half-time of 185 milliseconds.

He undergoes percutaneous valvuloplasty. The following morning, on examination, you note that he is comfortable. His oxygen saturation is 100% on room air. Opening snap is 0.12 milliseconds after S2. A shorter decrescendo diastolic rumble is heard. You obtain a predischarge echocardiogram. The report indicates a pressure half-time of 180 milliseconds.

26.What do you do next based on the echocardiogram?

a.There was a less-than-optimal result from the valvuloplasty. No significant change in mitral valve area was achieved. You plan to send him for another procedure or surgery.

b.There was an error in half-time measurement. You order a repeat assessment of pressure half-time later that day.

c.Repeat echocardiogram with planimetry of mitral valve area.

d.Consider TEE to see the valve opening better.

27.The echocardiogram reveals a small left-to-right shunt at the atrial level by color. What do you recommend?

a.Observation

b.Referral for percutaneous closure

c.Referral for surgical closure

d.Indefinite anticoagulation

Case 17

An 80-year-old man underwent successful AVR with a bioprosthetic valve 4 months ago. He presents to your office for a routine follow-up visit. He is asymptomatic. He is in sinus rhythm. Echocardiogram reveals a normally functioning prosthetic valve. Chamber dimensions are normal with normal biventricular function. He has no clinical history of embolic events.

28.Which of the following should you recommend?

a.Antibiotic prophylaxis, office visits if he feels unwell

b.Antibiotic prophylaxis, with yearly office visits

c.Warfarin therapy indefinitely

d.Clopidogrel therapy indefinitely

Case 18

A 28-year-old 20-week pregnant woman is referred to your clinic after being diagnosed with mitral valve prolapse and severe MR on an echocardiogram ordered by her obstetrician. She reports no symptoms prior to pregnancy but since being told her diagnosis is extremely worried and has noticed some shortness of breath on exertion (New York Heart Association [NYHA] class II). She is clinically euvolemic.

29.What do you recommend?

a.Antibiotics at the time of delivery

b.Commence afterload reduction with an ACEI given her new onset symptoms

c.Refer to an experienced surgeon for consideration for mitral valve repair as there is a high likelihood of successful durable repair

d.Commence afterload reduction with diuretics and hydralazine

e.No therapy at present but follow carefully with serial clinical and echo evaluation

Case 19

A 67-year-old woman is referred to your office for evaluation of a heart murmur. She describes symptoms of significant and limiting exertional dyspnea. On examination, she is normotensive. Pulse rate is 67 bpm and regular. Cardiac examination reveals a sustained but nondisplaced PMI. S1 and S2 are normal. An S4 is present. A loud III/VI systolic ejection murmur is heard throughout the precordium. Carotid upstrokes are delayed and diminished. An echocardiogram is performed (Fig. 2.7); continuous-wave Doppler evaluation reveals a 4.5-m/s jet across the LVOT.

Figure 2.7

30.Which of the following would you do next to arrive at a diagnosis?

a.TEE

b.Repeat echocardiogram with amyl nitrate

c.Stress echocardiogram

d.Dobutamine echocardiogram

e.The Pedoff probe has picked up an MR signal, the MR appears mild on all other views, no need for further investigation

Case 20

A 30-year-old woman presents to your office for a routine physical examination. She is asymptomatic. BP is 95/65 mmHg, with a resting heart rate of 65 bpm. Physical examination is remarkable for a mild pectus deformity. On cardiac auscultation, a mid-systolic click is heard. The click is heard earlier in systole with standing, and later in systole with squatting. No murmur is heard at rest, but a soft systolic murmur becomes audible with dynamic maneuvers.

31.Echocardiography demonstrates no high-risk features. What is the role of aspirin therapy in such patients who have had no evidence of embolic events?

a.Should be prescribed to all patients

b.May play a role, if a murmur is heard

c.There is no clear role for aspirin therapy in such patients

Case 21

A 50-year-old man with severe AI is referred to you for a second opinion. He is asymptomatic. An echocardiogram reveals a mildly dilated LV (end-diastolic dimension of 6.2 cm and end-systolic dimension of 3.5 cm) with a normal ejection fraction. He has already undergone a stress echocardiogram. He exercised for 14 METs. No symptoms or electrocardiographic changes were noted. Resting ejection fraction was calculated at 65%. Post stress, the ejection fraction is 60%. No segmental wall motion abnormalities were seen.

32.What do you recommend?

a.Surgical intervention

b.Continue with vasodilator therapy and reassess in 6 months

c.Cardiac catheterization

d.Stress nuclear ventriculogram

Case 22

A 70-year-old man presents to your office with complaints of exertional dyspnea. He is mildly hypertensive on examination. Carotid upstrokes are brisk, with a secondary upstroke. A loud III/VI systolic murmur is heard along the sternal border radiating to the neck. S1 and S2 are normal. An S4 is heard. The murmur increases in intensity with Valsalva and decreases with handgrip.

33.An echocardiogram reveals a <2-m/s jet across the LVOT. What is your next step?

a.Repeat the echocardiogram, but have Doppler interrogation performed in other views and with a nonimaging transducer. The degree of AS has been underestimated

b.Repeat the echocardiogram with amyl nitrate

c.Transesophageal echocardiogram to better assess the valves

d.Coronary angiography

Case 23

A 26-year-old woman with a history of hypertrophic obstructive cardiomyopathy is referred for consideration for septal myectomy. She has NYHA class III dyspnea on exertion despite maximal medical therapy. On echocardiography, there is severe asymmetric septal hypertrophy with severe systolic anterior motion of the mitral valve. There is a late-peaking gradient across the LVOT of 60 mmHg, which increased to 105 mmHg with Valsalva. She has a structurally normal mitral valve on cardiac MRI with moderately severe posteriorly directed MR (Fig. 2.8).

Figure 2.8

34.What would you advise her regarding surgery?

a.She will probably require mitral valve replacement at the time of surgery.

b.She will probably require mitral valve repair during surgery.

c.She will probably not need surgery on her mitral valve.

d.She may need plication of her papillary muscles.

Case 24

A 62-year-old man with a history of rheumatic heart disease presents to your office with complaints of exertional dyspnea. No constitutional complaints are present. He had undergone a mitral valve replacement with a bileaflet tilting disk mechanical valve 11 years prior. He is normotensive with a heart rate of 73 bpm. On examination, you note a grade II/VI holosystolic murmur at the apex. An echocardiogram is performed, which reveals normal LV and RV function. Peak mitral gradient is 30 mmHg. Mean transmitral gradient is 7 mmHg. Pressure half-time is 80 milliseconds.

35.What is your next diagnostic step?

a.Fluoroscopy of the valve

b.Transesophageal echocardiogram

c.Invasive assessment of hemodynamics

d.Draw blood cultures

36.Which of the following would be the expected physical findings in this patient if the valve were functioning normally?

a.Prominent closing click, soft and brief diastolic rumble

b.Prominent opening and closing clicks, soft and brief diastolic rumble

c.Prominent opening click, long diastolic rumble

d.Prominent closing click, systolic murmur

37.If the patient had a ball-and-cage valve instead, what would you expect to hear?

a.Prominent closing click, soft and brief diastolic rumble

b.Prominent opening and closing clicks, soft and brief diastolic rumble

c.Prominent opening click, long diastolic rumble

d.Prominent closing click, systolic murmur

38.Recommended antithrombotic therapy for a patient with a mechanical mitral valve replacement without a prior thromboembolic event or other high-risk features is

a.Warfarin therapy with a target international normalized ratio (INR) of 3.0 to 4.0

b.Warfarin therapy with a target INR of 2.5 to 3.5

c.Warfarin therapy with a target INR of 2.0 to 3.0 plus aspirin 75 to 100 mg

d.Warfarin therapy with a target INR of 2.5 to 3.5 plus aspirin 300 mg

e.Warfarin therapy with a target INR of 2.5 to 3.5 plus aspirin 75 to 100 mg

Case 25

A 65-year-old man presents to your office for evaluation of valvular heart disease. He is asymptomatic. He walks 5 miles a day without difficulty. An echocardiogram reveals severe AS, with a maximum aortic jet velocity of 4.7 m/s by Doppler echocardiography. LV systolic function is preserved. There is mild LV hypertrophy (wall thickness 1.4 cm). He walks on a treadmill for 9 minutes, with a normal hemodynamic response.

39.Continued observation is recommended. What do you tell him is his yearly risk of sudden death, provided he remains asymptomatic?

a.<2%

b.5%

c.5% to 10%

d.>10%

40.What is the likelihood that he will become symptomatic, or come to surgery, within the next 3 years?

a.10%

b.10% to 25%

c.25% to 50%

d.>50%

Case 26

A 52-year-old man who previously underwent AVR with a tilting disk valve presents to you several months following a documented transient ischemic attack (TIA). He has no symptoms at present. Workup at the time of his TIA included carotid Dopplers, and transthoracic and transesophageal echocardiogram. These were unremarkable. The valve was well seated and was functioning normally. No thrombus was seen. Only minimal aortic atheroma was seen. No intracardiac shunt was identified. He has been on warfarin throughout and has maintained an INR between 2 and 3. INR was 2.2 at the time of his TIA. On examination, he is in no acute distress. BP is 120/80 mmHg; pulse is 68 and regular. Carotid upstrokes are full and not delayed. Crisp valve closure sound is heard along with a short, early-peaking systolic ejection murmur at the base. No S3 is heard. P2 is normal. No peripheral edema is noted.

41.Which of the following would you recommend?

a.Start ASA (acetylsalicylic acid), 325 mg/day.

b.Increase warfarin, to achieve an INR of 3.5 to 4.5.

c.Increase warfarin, to achieve an INR of 4.0 to 5.0.

d.Start ASA, 81 mg/day, and increase warfarin, to achieve an INR of 2.5 to 3.5.

42.If his transesophageal study had revealed a small (1 to 2 mm) echodensity on the valve strut—suggestive of thrombus—but no obstruction to valve function, what should have been done?

a.Intravenous heparin

b.Bolus thrombolytic therapy

c.Reoperation

d.Intravenous IIb/IIIa inhibitors

Case 27

You are following a 50-year-old man with moderate mitral stenosis, who had been asymptomatic. He presents to the emergency room with complaints of mild exertional dyspnea and palpitations, present for the past 3 to 4 days. On arrival, he appears comfortable, with an O2 saturation of 99% on room air. His pulse rate is 140 bpm and irregular. BP is 130/75 mmHg. Electrocardiogram reveals atrial fibrillation.

43.The above patient spontaneously converts to sinus rhythm. Which of the following are you most likely to recommend?

a.Therapy with warfarin

b.Percutaneous valvuloplasty

c.Mitral valve replacement

d.No change in therapy

Case 28

A 34-year-old woman presents to your office for evaluation because she had been on treatment with anorectic agents 5 years ago. She is asymptomatic at this time. She is now at her ideal body weight. On examination, she is in no acute distress. BP is 107/68 mmHg. Jugular venous pulsations appear normal. Chest is clear. Cardiac examination reveals a nondisplaced PMI. S1 and S2 are normal, with an appropriate physiologic split of S2. P2 is not loud. No S3 or S4 is heard. Auscultation is performed with the patient sitting, supine, and in the left lateral decubitus position. No murmur is heard.

44.What do you most likely recommend for this patient?

a.Reassurance, with a repeat physical examination in 6 months

b.Echocardiogram

c.Stress test

d.TEE

Case 29

A 50-year-old man presents for his first physical examination in several years. He notes that a murmur had been documented a number of years ago. He is entirely asymptomatic. On examination, he has a BP of 120/70 mmHg with a pulse rate of 58 bpm. Neck veins are not distended. Carotid upstrokes are brisk. Lungs are clear. Cardiac examination reveals a nondisplaced PMI. S1 is soft; S2 is normal (with a preserved A2). An S3 is heard. A III/VI holosystolic murmur is heard at the apex radiating to the base and carotids, which increases with handgrip.

Echocardiogram reveals myxomatous mitral valve disease with posterior leaflet prolapse and severe MR. The prolapse involves the P2 (middle) segment and is severe. There is no calcification of the valve. End-systolic dimension is 3.0 cm; end-diastolic dimension is 5.6 cm. Ejection fraction is 65%. TR velocity is 2.9 m/s.

45.Which of the following would be most appropriate at this time?

a.Referral for mitral valve replacement

b.Consider elective mitral valve repair at a hospital where repair is performed with a high degree of success or if he wishes to defer surgery, follow up at 6 monthly intervals with echo

c.The addition of an ACEI and follow-up in 2 years

d.The addition of amiodarone to prevent atrial fibrillation

e.Follow-up in 2 years without an echocardiogram

46.The above patient agrees to close medical follow-up. However, he does not present back to your office until 2 years later, now with complaints of dyspnea. A repeat echocardiogram reveals an ejection fraction of 45% with an end-systolic dimension of 4.7 cm. What do you recommend?

a.Referral for mitral valve repair

b.Start an ACEI and reassess in 3 months

c.Mitral valve replacement

d.Start a β-blocker and reassess in 3 months

Case 30

An 80-year-old man with severe AS is turned down for surgical AVR due to significant comorbidities. He is referred to you for consideration for transcatheter AVR.

47.Which of the following findings is considered a contraindication for this procedure?

a.Calcified and tortuous femoral arteries

b.The apex is not accessible

c.Life expectancy <1 year

d.A history of treated endocarditis

e.Annulus size of 20 mm

Case 31

A 35-year-old man presents to your office for evaluation of valvular heart disease. He complains of shortness of breath with only modest amounts of exertion, as well as two-pillow orthopnea. He also complains of easy fatigability, as well as lower extremity edema and abdominal fullness. On examination, he is in no acute distress. He is normotensive. Jugular venous pressure is elevated, with a prominent a wave. The v wave is not easily discerned. S1 is loud. S2 is normal. A sound is heard in diastole, 0.07 milliseconds after S2. A diastolic rumble is heard at the apex. A diastolic murmur is also heard along the left sternal border, which increases with inspiration. Mild hepatomegaly is present. There is 2+ peripheral edema.

48.What is your diagnosis?

a.Mitral stenosis

b.Mitral stenosis with tricuspid insufficiency

c.Mitral and tricuspid stenosis

d.Mitral stenosis and AS

e.Tricuspid stenosis

Case 32

An 80-year-old man presents to your office with complaints of chest tightness when climbing up a flight of stairs. His past medical history is unremarkable. On physical examination, he is in no acute distress. BP is 140/80 mmHg; pulse is 78 bpm and regular. Chest is clear. Carotid upstrokes are diminished. The PMI is sustained, but not displaced. A fourth heart sound is present. The second heart sound is diminished and single. A loud late-peaking systolic murmur is heard, loudest at the second intercostal space, radiating to the neck.

49.Which of the following would be a reasonable next step in this patient’s management?

a.Stress sestamibi

b.Stress electrocardiogram

c.Cardiac catheterization

d.Prescribe prn SL (sublingual) NTG (nitroglycerin) and review back in one week

50.The above patient is found to have an aortic valve area of 0.7 cm2 with a mean gradient of 60 mmHg. Following catheterization, he develops massive upper gastrointestinal bleeding. Endoscopy reveals a gastric ulcer with a bleeding vessel at its base. Cauterization is performed, which temporarily stops the bleeding. However, the bleeding recurs and urgent partial gastrectomy is recommended. He complains of chest pain during these bleeding episodes. What is the best course of action?

a.Proceed to AVR first.

b.Refer for percutaneous balloon valvuloplasty, followed by gastrectomy.

c.Start nitroprusside and proceed with gastric surgery.

d.Proceed with gastric surgery directly.

51.What valve would you recommend to an 80-year-old patient with severe symptomatic AS?

a.Bovine pericardial valve

b.Ball-and-cage mechanical valve

c.Bileaflet mechanical valve

d.Aortic homograft

Case 33

A 28-year-old man presents for evaluation of difficult to control hypertension. He initially denies any symptoms but on further questioning admits to some leg fatigue and weakness and cold feet. On examination his BP is 180/90 mmHg, heart rate is 77 bpm and regular. His radial pulses are easily palpable but his femoral pulses are weak and there is radiofemoral delay. An ejection systolic murmur is heard at the left upper sternal border that radiates to the intrascapular region. In addition, there is a soft continuous murmur heard throughout the precordium.

52.Based on your suspicion you order a CT aorta (Fig. 2.9). What is the most common associated lesion?

a.~5% of cases have a bicuspid aortic valve.

b.~50% of cases have mitral valve prolapse.

c.~50% of cases have a bicuspid aortic valve.

d.~5% of cases have an associated cleft mitral valve.

e.This lesion is rarely associated with concomitant cardiac abnormalities.

Figure 2.9

Case 34

A 65-year-old man with a history of rheumatoid arthritis (well controlled) presents for evaluation of a heart murmur. He notes some increase in fatigue and decrease in activity level over the past 2 years, but denies any specific complaints of dyspnea. He leads a rather sedentary lifestyle. On examination, he is 6-ft, 1-in. tall. BP is 150/50 mmHg. Heart rate is 80 bpm and regular. Carotid upstrokes are brisk with a rapid upstroke and decline. Apical impulse is displaced and hyperdynamic. S1 and S2 are normal. A decrescendo, nearly holodiastolic murmur is heard along the left sternal border, loudest with the patient sitting up. An echocardiogram is performed, which reveals a dilated LV (end-diastolic dimension of 6.8 cm and end-systolic dimension of 3.5 cm). Ejection fraction is 55%. There is significant aortic regurgitation.

53.What do you most likely recommend?

a.Stress test

b.Reassess with repeat echocardiogram in 6 months

c.Start vasodilator therapy and reassess in 2 years

d.Refer to surgery

54.He is started on a vasodilator and is seen back in 6 months. He reports no change in symptoms. A repeat echocardiogram demonstrates an end-diastolic dimension of 7.6 cm. Ejection fraction remains normal. What do you recommend now?

a.Stress test

b.Surgical intervention

c.Increase vasodilators and reassess in 6 months

d.MRI to assess LV volumes

Case 35

A 42-year-old woman, who underwent mitral valve replacement with a bileaflet tilting disk valve for rheumatic disease, presents to the emergency room with complaints of severe dyspnea. On examination, she has a BP of 120/60 mmHg. Heart rate is 83 bpm. Chest reveals bilateral crackles, one-third up. Cardiac examination reveals a nondisplaced PMI. Prosthetic clicks are muffled. A long diastolic rumble is heard at the apex. Her past medical history is otherwise unremarkable.

55.An echocardiogram is ordered on the above patient. Which of the following would you expect to see?

a.Severe MR

b.Mean gradient across the mitral prosthesis of 17 mmHg

c.Pressure half-time of 80 milliseconds

d.Ejection fraction of 20%

Case 36

A 26-year-old woman presents to your office for evaluation. She was told she had a murmur many years ago. She has a history of palpitations, but is otherwise asymptomatic. On examination, she is in no acute distress. Prominent vwaves are noted in the JVP. Carotid upstrokes are normal. Chest is clear to auscultation. Cardiac examination reveals a nondisplaced PMI. Auscultation reveals a widely split first heart sound, with a loud second component that sounds like a click. A holosystolic murmur is heard at the right sternal border, which increases with inspiration. Hepatomegaly is present. An echocardiogram is performed (Fig. 2.10).

Figure 2.10

56.What is the most likely cause for her palpitations?

a.Arrhythmias secondary to an accessory pathway

b.AV nodal reentrant tachycardia

c.Ventricular tachycardia

d.Atrial fibrillation

e.Anxiety

57.No intervention is performed for the above patient. She returns to your clinic 3 months later. She describes an episode of transient word-finding difficulty, which lasted for a number of seconds. This occurred while she was recovering from a fractured tibia. A CT scan was performed, which was negative. She is concerned that she may have a recurrence. What is the most appropriate next test for her?

a.Echocardiography with saline contrast study

b.Carotid Dopplers

c.24-hour ambulatory electrocardiographic monitoring

d.Right heart catheterization with oxygen saturation run

Case 37

A 21-year-old man presents to your office for evaluation. He tells you that a murmur was noted a few days after birth. He is presently asymptomatic. On examination, he is normotensive. Pulse is 65 bpm and regular. Carotid upstrokes are normal. Chest is clear. Cardiac examination reveals a nondisplaced PMI. An RV lift is present. A systolic thrill is present in the suprasternal notch. A high-pitched sound is heard after S1. A crescendo–decrescendo systolic murmur is heard at the left second intercostal space. A2 is normal.

58.Which of the following would you expect to find on echocardiography?

a.Vmax across the aortic valve of 4 m/s

b.Vmax across the pulmonic valve of 4 m/s

c.A wide jet of mitral insufficiency

d.Flow reversal in the hepatic veins

59.The above patient returns 1 year later for follow-up. Which of the following is a definite indication for intervention?

a.He tells you of an episode of syncope

b.No symptoms, but RV to PA peak gradient of 30 to 39 mmHg

c.No symptoms, but RV to PA peak gradient of 20 to 29 mmHg

d.He has occasional feelings that his heart has extra beats

60.If intervention is recommended, what is the preferred treatment approach?

a.Percutaneous valvuloplasty

b.Ross procedure

c.Mechanical valve

d.Bioprosthetic valve

61.In the setting of AS with moderate insufficiency, which of the following methods would provide the most accurate estimate of aortic valve narrowing?

a.Invasive hemodynamics, using the Gorlin formula

b.Doppler echocardiography, using the continuity equation

c.Pressure half-time

d.Bernoulli equation

62.Which of the following valves has the lowest incidence of endocarditis?

a.Mechanical valve

b.Bioprosthetic valve

c.Aortic homograft

d.Stentless mitral valve

63.What is the most common cause of TR in an adult population?

a.Rheumatic tricuspid disease

b.Carcinoid

c.Congenital abnormalities

d.Pulmonary hypertension resulting from primary left-sided disease

e.Myxomatous disease of the tricuspid valve

64.The most common organism seen in native valve endocarditis is what?

a.Streptococcus viridans

b.Enterobacter faecalis

c.Staphylococcus aureus

d.Chlamydia pneumoniae

e.HACEK organisms

65.A patient presents with systemic embolic events 1 month after uncomplicated mitral valve replacement. He has been febrile for the past week. TEE demonstrates multiple echodensities on the valve ring. Blood cultures that are drawn are most likely to grow which of the following?

a.Streptococcus viridans

b.Staphylococcus aureus

c.Staphylococcus epidermidis

d.E. faecalis

e.Candida albicans

ANSWERS

1.b. Transesophageal echocardiography (TEE). The clinical history is of a patient who had an inferior wall myocardial infarction approximately 1 week ago. He now presents in shock with acute congestive heart failure. Mechanical complication of myocardial infarction is first on the differential. The presence of a ventricular gallop and an apical murmur without a thrill makes papillary muscle rupture the leading diagnosis (as opposed to ventricular septal defect). Transthoracic echocardiography may miss eccentric jets in this setting. TEE should be performed to make the diagnosis. He will certainly need a cardiac catheterization (at which time a saturation run may be performed), but a TEE should be done quickly at the bedside to confirm the diagnosis so that the surgical team can be mobilized.

2.b. Posterior papillary muscle rupture as it has a single blood supply. The 3D reconstruction of the mitral valve shown here is orientated in the “surgeons view,” looking down on the mitral valve from the left atrium with the aortic valve situated on top, the anterior mitral valve leaflet adjacent to it, and the posterior mitral valve leaflet inferiorly. We see a bulky mass (the posterior papillary muscle) protruding into the left atrium in systole. The middle panel (early diastole) clearly shows that the mass is attached to the posterior leaflet. The posterior papillary muscle has a single blood supply (usually the right coronary artery), while the anterior papillary muscle often has dual blood supply. For this reason, post infarction rupture of the posterior papillary muscle is more common.

3.b. She likely has more severe MR than is evident on the echocardiogram. Her examination is suggestive of severe MR. The echo confirms LV dilation and mitral leaflet pathology, which could be consistent. The eccentric nature of the jet suggests that it may have been underestimated by transthoracic imaging. A more definitive imaging procedure such as TEE will be helpful here.

4.b. Mitral valve surgery. The presence of mild LV dysfunction with LV dilation is a class I indication for surgery. TEE would be the next test of choice prior to surgery to confirm the severity and mechanism of MR and assess suitability for surgical repair. While exercise echo is reasonable in asymptomatic patients with severe MR to assess functional capacity the patient already has indications for surgery.

5.c. Stress echocardiogram, to assess for mitral pressures post stress. She has moderate mitral stenosis. The fact that she has stopped exercising may be a clue to the onset of symptoms. An assessment of functional capacity and post-stress mitral pressures would be useful in management There are insufficient data for immediate referral for intervention. Follow-up in a short period of time may not be unreasonable; however, 2 years is too long a period.

6.a. Consideration for percutaneous valvuloplasty. Her functional capacity is below average for her age. Her valve is favorable for percutaneous valvuloplasty (splittability score of 6) and she had a significant rise in PA pressures post stress. Ideally, the splittability index should be 8 or less for optimal results post balloon valvuloplasty. A increase in mean valve gradient of 15 mmHg with exercise is a class I indication by the American College of Cardiology (ACC)/American Heart Association (AHA) guidelines. A β-blocker would not be an unreasonable addition, but she should be followed more frequently than every 2 years. In addition, she does have class I indication for intervention. She has normal LV function and is in sinus rhythm—there is no role for digoxin in this setting. Valve replacement is considered only if the valve is deemed unsuitable for percutaneous valvuloplasty or surgical repair.

7.b. Carcinoid. The history and examination are consistent with tricuspid stenosis and regurgitation. (She has symptoms of fatigability from decreased cardiac output, signs, and symptoms of systemic venous congestion—hepatic distension and right upper quadrant pain, peripheral edema, and ascites. There is a diastolic murmur along the sternal border, which increases with inspiration, along with a prominent a wave in the JVP. In addition, she has a pansystolic murmur and a prominent v wave.) However, no evidence for mitral stenosis is noted on examination. Isolated rheumatic tricuspid stenosis is very rare. Thus, other causes for tricuspid stenosis should be considered. The second most common cause of tricuspid stenosis is the carcinoid syndrome. She also has bronchospasm and diarrhea, which go along with this diagnosis. She has a normal P2, making primary pulmonary hypertension unlikely. Liver disease in and of itself would not produce elevation in the JVP.

8.c. Check lower extremity BP. He has a bicuspid aortic valve (an ejection sound is heard, along with a short systolic ejection murmur). There is an association between bicuspid aortic valves and coarctation of the aorta. Therefore, looking for discrepancy between the upper and lower extremity BP would be paramount.

9.c. Dobutamine echocardiogram. This is a patient presenting with low-gradient AS in the setting of LV dysfunction. It may be that the patient has severe AS, but the gradients are now low secondary to decreased stroke volume. However, the degree of AS may not be that significant, but because of decreased cardiac output, the continuity equation overestimates AS severity. In this setting, low-dose dobutamine echocardiography may be useful. With inotropic stimulation, an improvement in stroke volume and cardiac output may help to differentiate true severe AS from what has been labeled pseudo-AS. If true severe AS is not present, then valve area will increase. It would not be prudent to send such a patient to aortic valve surgery without performing such an evaluation. It would be necessary to exclude severe stenosis before proceeding with transplant evaluation. ACEI may be beneficial, but it would be important to proceed with the workup as above first. Afterload reduction would need to be introduced with very careful hemodynamic monitoring if true severe AS were in fact present. The use of dobutamine echocardiographic testing to evaluate low-gradient AS in the setting of LV dysfunction is a class IIa indication by ACC/AHA guidelines.

10.a. AVR. The patient has true, severe AS and although may have a higher potential complication rate with surgery is likely to benefit prognostically and symptomatically from surgery. Balloon aortic valvuloplasty has not been shown to improve survival without the addition of a more definitive procedure such as aortic valve surgery. It is only indicated for palliation or as a bridge to a more definitive procedure such as transcatheter aortic valve replacement (TAVR) or surgery.

11.d. Patient has a lack of contractile reserve but should still be considered for AVR. There are three possible outcomes to a low-dose dobutamine test in this situation: true AS, pseudo-AS, and absence of contractile reserve. As was the case in Question 10, there may be an increase in stroke volume (defined as ≤20% increase from baseline) associated with an increase in transvalvular gradients (mean gradient >40 mmHg) without a significant increase in aortic valve area (AVA) (AVA increase <0.2 cm2) indicative of true AS. Conversely, in pseudo-AS the increase in stroke volume is associated with an increase in AVA without a significant change in gradients. Finally, absence of contractile reserve is defined as failure to increase the stroke volume by ≤20% from baseline. In this case, dobutamine does not help to differentiate between the former two scenarios. While there is a significantly higher mortality during the perioperative period in those with the absence of contractile reserve compared with true AS, for those that survive surgery their 5-year survival is significantly better than those treated with medical therapy alone. Therefore, surgery should be considered on an individual basis.

12.b. Addition of vasodilator therapy. The patient is asymptomatic with good functional capacity. He has a normal ejection fraction with a mildly dilated LV. Surgery is a class III indication (harmful) in this setting. Vasodilator therapy may have some benefit in this asymptomatic population with preserved ejection fraction and LV dilation, although this is not definite. This is a class IIb indication. However, he has systolic hypertension which is likely at least in part related to his aortic regurgitation, and vasodilator therapy is an optimal therapy for this. Observation alone would be reasonable, but such a patient should be followed at 6-month intervals initially and not every 3 years. There is no role for cardiac catheterization at this juncture.

13.a. <1%. From the available published literature, as summarized in the ACC/AHA consensus guidelines, the risk is about 0.2% per year in those asymptomatic patients with preserved LV function.

14.d. Referral for surgical intervention to repair or replace his aortic valve and to replace his ascending aorta. An aortic dimension >5.0 cm (or growth >0.5 cm per year) in a patient with a bicuspid aortic valve is a class I indication for surgery. The valve is often repairable in bicuspid valve associated with predominant aortic regurgitation assuming the mechanism is due to prolapse of the conjoint cusp, and there is no significant stenosis or calcification of the valve.

15.d. AVR with tricuspid valve repair if feasible. The mitral valve appears morphologically normal. After relief of the outflow tract obstruction, the MR will likely improve; therefore, mitral valve repair is not indicated. Tricuspid valve repair for moderate TR at the time of left-sided valve surgery is reasonable in the context of annular dilation and elevated PA pressures. This is a class IIb indication from the ACC/AHA guidelines but receives a class IIa recommendation from the European Society of Cardiology (2012). Tricuspid valve repair is favored over replacement.

16.a. 0.4 cm2. The EROA based on the assumptions above is 0.4 cm2 consistent with severe MR. The EROA is calculated using the abbreviated proximal isovelocity surface area (PISA) method as r2/2 (r = radius of the PFCR). In this case, the radius is 0.9 cm; therefore, the EROA can be estimated as 0.4 cm2.

17.b. 2+, moderate. Using the complete PISA method and calculating the regurgitant volume, the MR is determined to be only moderate in severity which is consistent with the brief duration of MR heard on physical examination. The complete method for calculating the EROA is (2πr2 × AV)/Vmax (AV: aliasing velocity; Vmax: maximum velocity across the mitral valve); therefore, in this case the EROA = (2π(0.9)2 × 38.5)/600 = 0.33 cm2. However, as we see from the continuous-wave Doppler signal, the MR only occurs in late systole consistent with mitral valve prolapse. The regurgitant volume is equal to EROA × VTIMR (VTIMR = velocity time integral of the mitral regurgitation), which in this case is = 0.33 × 100 = 33 mL consistent with 2+ MR. If we used the EROA from the abbreviated PISA method (0.4 cm2), the regurgitant volume is 40 mL, which is still consistent with moderate MR.

18.a. Transesophageal echocardiogram. Left atrial and appendage thrombus should be excluded prior to proceeding with percutaneous valvuloplasty and is recommended by ACC/AHA guidelines to be performed prior to the procedure. Transthoracic echocardiography does not have sufficient sensitivity for this purpose. Documentation of atrial fibrillation by ambulatory monitoring may make the likelihood of finding a thrombus higher, but the transesophageal echocardiogram should be performed regardless. Routine surveillance for aortic calcification has no role in this setting. A nuclear perfusion study would not be necessary here (angiography can be performed if needed at the time of the procedure).

19.a. It is absolutely indicated. Given LV dysfunction (EF < 50%), this is a class I indication for surgery. There is no question as to the severity of the AS given the gradients and the aortic valve area; thus dobutamine echocardiography is not of value here.

20.d. The rate of mortality, for a patient with these findings, is higher compared with patients with severe AS and high gradients across the aortic valve but aortic valve surgery has resulted in better outcomes in these patients. This woman has paradoxical low-gradient, severe AS with preserved ejection fraction. Her clinical history, examination, and 2D imaging of the aortic valve are consistent with severe AS. She has a low indexed stroke volume (<35 mL/m2), resulting in low gradients across the AV but the dimensionless index and AVA both are consistent with severe AS. When there is discordant echocardiographic data the accuracy of measurements should always be looked at again; however, in this case, the low gradients are consistent with low-stroke volume. There are currently no guidelines from the ACC or AHA on how to manage these patients; however, the recent 2012 European Society of Cardiology guidelines for valvular heart disease provide a class IIa recommendation for AVR in symptomatic patients with paradoxical low-gradient, severe AS with preserved ejection fraction. A number of studies have confirmed that the rate of mortality is higher in this cohort when compared with patients with severe AS and high gradients but surgery on the aortic valve is associated with significantly better outcomes.

21.c. Aortic root dimension. The patient clinically has severe AI. The murmur is loudest at the right sternal border, suggesting aortic root dilation as a potential cause of his AI. The presence of root dilation (≤5.5 cm) may lead to earlier surgery, hence is vital to know. The diastolic rumble is most likely an Austin Flint murmur and not concomitant mitral stenosis (no opening snap, S1 not loud).

22.a. Cardiac catheterization with aortography. Clinically, the patient has severe aortic regurgitation. He is symptomatic. Consistent with this, the echocardiogram reveals a dilated LV with low normal systolic function. The degree of aortic regurgitation must be underestimated by this study. When there is such discrepancy, proceed with aortography to confirm aortic regurgitation severity and to assess coronaries prior to surgical referral. As he is symptomatic, continued observation and/or medical therapy is not the preferred treatment approach. β-Blockers, by prolonging the diastolic filling period, could actually increase regurgitant volume.

23.a. Transesophageal echocardiogram, emergent cardiac surgical consultation. The patient has a clinical presentation of severe acute AI (short diastolic murmur, soft S1 from premature mitral valve closure, low output state, and pulmonary edema). In the context of chest pain, this scenario suggests aortic dissection until proven otherwise. The dissection flap likely involves the ostium of the right coronary artery, producing the inferior ST-segment elevation. Thrombolytics should not be used until dissection is ruled out. Even if there is no dissection, an intra-aortic balloon pump should not be used with severe AI. The augmented diastolic pressure worsens the severity of the insufficiency. MRI would also provide the diagnosis, but given the hemodynamic instability of the patient, a bedside TEE would be a safer and quicker option to arrive at the diagnosis.

24.b. No evidence for dysfunction. The physical examination does not suggest either stenosis or insufficiency. He appears to be in a high-output state, secondary to his febrile illness. As a result, the gradients are increased. The LVOT VTI is also increased, secondary to the increased cardiac output. The LVOT/aortic valve VTI ratio is the same in the two echocardiograms, which would speak against any significant obstruction.

25.a. TEE with surgical consultation. The clinical scenario, with a new first-degree AV block and acute aortic regurgitation, is highly suspicious for prosthetic valve abscess and possibly even partial dehiscence. A transesophageal echocardiogram should be performed, but prompt surgical consultation should also be requested given the high suspicion for aortic root abscess and the onset of heart failure symptoms.

26.c. Repeat echocardiogram with planimetry of mitral valve area. With acute changes in atrial and ventricular compliance (as with valvuloplasty), the half-time is unreliable. Usually 72 hours or more is required after the procedure before the half-time can be used with reasonable reliability. Planimetry, if performed correctly, would provide a more reliable estimate of stenosis severity. Clinically, the patient seems to have had a good result (longer S2–OS interval, shorter murmur). TEE rarely provides incremental data on mitral stenosis if the transthoracic images are reasonable.

27.a. Observation. The shunt is secondary to the valvuloplasty procedure where the interventionalist must perform an interatrial septal puncture in order to access the mitral valve. Most of these small shunts will close over the next 6 months without any intervention. The shunt is left to right by color. He has good O2 saturation on room air, making any significant right-to-left shunting unlikely. Anticoagulation with an atrial septal defect/patent foramen ovale may be recommended in certain settings, however not indefinitely, given the good chance that the defect will close.

28.b. Antibiotic prophylaxis, with yearly office visits. He requires antibiotic prophylaxis with a prosthetic valve (by 2007 guidelines, prosthetic cardiac valves are an indication for subacute bacterial endocarditis prophylaxis prior to dental procedures). These patients still require close follow-up with complete evaluation on a yearly basis. Some advocate a 3-month period of warfarin therapy after bioprosthetic valve placement. He is now 4 months out, and has no other indications or a high-risk profile (LV dysfunction, prior embolic event, and atrial fibrillation); thus warfarin is no longer needed at this time. Similarly, there is no specific indication in this man for clopidogrel therapy.

29.e. No therapy at present but follow carefully with serial clinical and echo evaluation. Antibiotics are not recommended routinely for prophylaxis at the time of delivery for patients with valvular heart disease unless infection is suspected. If there is evidence of pulmonary congestion treatment with diuretics and afterload reduction with hydralazine is recommended but ACEIs are teratogenic and are absolutely contraindicated in pregnancy. Your patient is clinically euvolemic and therefore medical therapy need not be initiated. Surgery should only be performed if the mother’s life is threatened due to the associated high fetal mortality rate (20% to 30%). Management will involve close follow-up and monitoring by her cardiologist and obstetrician and treatment only if her clinical situation deteriorates.

30.a. TEE. The echocardiogram reveals a normal-appearing aortic valve. Yet, the profile of the continuous-wave Doppler jet is more consistent with a fixed obstruction, as opposed to the dagger shape of dynamic obstruction. These findings are suggestive of the presence of a subvalvular membrane. TEE would be useful to better delineate this area and identify the membrane. The patient already has a 5-m jet in the absence of systolic anterior motion; therefore, it would not be prudent to use provocation with amyl nitrate. A stress echocardiogram would have no diagnostic value and may have some risk in the setting of symptomatic LVOT obstruction. One should always consider contamination with an MR signal; however, the physical examination is consistent with outflow tract obstruction and the continuous-wave Doppler signal begins after the QRS (after isovolumetric relaxation) consistent with outflow tract obstruction. The MR signal will begin earlier relative to the QRS (through isovolumetric contraction).

31.c. There is no clear role for aspirin therapy in such patients. If there is echocardiographic evidence for high-risk mitral valve prolapse (leaflet thickening, elongated chordae, left atrial enlargement, and LV dilation), aspirin therapy is considered a class IIb indication. Therapy is clearly recommended if there have been documented stroke or transient ischemic events.

32.b. Continue with vasodilator therapy and reassess in 6 months. By ACC/AHA guidelines, decline in ejection fraction following stress echocardiography by itself is not an accepted indication for referral to surgical intervention. Owing to the high afterload and the increase in afterload on exercise, a small-to-modest decline in ejection fraction (<10%) may still be consistent with well-compensated AI. This patient has normal resting ejection fraction and mildly dilated LV with excellent functional capacity.

33.b. Repeat the echocardiogram with amyl nitrate (Fig. 2.11). The physical examination is highly suggestive of hypertrophic cardiomyopathy (brisk, bisferiens carotid pulse, normal S2, and murmur increasing with Valsalva and decreasing with handgrip). The patient may not have a significant resting gradient, but may have a significant provocable gradient. Generally, a transesophageal echocardiogram is not needed to make the diagnosis. Invasive hemodynamics with provocation would be useful, but angiography alone would not be sufficient.

Figure 2.11

34.c. She will probably not need surgery on her mitral valve. MR secondary to systolic anterior motion of the mitral valve is related to hydrodynamic drag and Venturi effects on the anterior mitral valve. Often there are intrinsic mitral valve abnormalities that, in combination with septal hypertrophy, predispose to systolic anterior motion. Abnormal chordal attachments or hypermobile papillary muscles seen best on cardiac MRI may mandate chordae remodeling or papillary muscle reorientation; however, in this case the mitral valve apparatus is noted to be structurally normal. Relief of the hydrodynamic effects of a narrowed LVOT after myectomy will usually result in significant improvement in MR without further surgery. Before chest closure, careful assessment of MR should be done after the myectomy, usually with isoproterenol infusion, to ensure resolution.

35.b. Transesophageal echocardiogram. By examination, the patient has mitral insufficiency. The echocardiogram is consistent with this, with an elevated peak transmitral gradient. Pressure half-time is not prolonged; thus there does not appear to be any significant stenosis (gradients elevated owing to increased flow from regurgitant volume). A TEE would be the most useful to confirm the diagnosis. Fluoroscopy may identify partial dehiscence, but would not be helpful if there were a leak in the setting of a well-seated valve. There is no evidence for stenosis, where fluoroscopic evaluation of leaflet motion could be diagnostic. There is no clinical evidence for endocarditis.

36.a. Prominent closing click, soft and brief diastolic rumble. The bileaflet mechanical valves do not typically produce a loud opening sound, but do have prominent closing sounds. A brief diastolic rumble may be heard in a normally functioning prosthetic valve in the mitral position.

37.b. Prominent opening and closing clicks, soft and brief diastolic rumble. With the ball-and-cage valves, one would expect to hear the opening click as well.

38.e. Warfarin therapy with a target INR of 2.5 to 3.5 plus aspirin 75 to 100 mg. All patients with a mechanical valve require warfarin. The risk of thromboembolic events is higher for prosthetic valves in the mitral position; therefore, the recommended therapeutic range is higher than that for mechanical valves in the aortic position. The addition of low-dose aspirin (75 to 100 mg) further reduces the risk of thromboembolic event and reduces mortality from cardiovascular disease. Therefore, aspirin is recommended for all patients with valvular prostheses. Higher doses of aspirin have not been shown to be beneficial and increase the bleeding risk.

39.a. <2%. In the absence of symptoms, natural history studies would suggest a relatively low risk of sudden death.

40.d. >50%. He has a velocity across the aortic valve of >4 m/s. Observational studies would suggest a high likelihood of symptom development in the next 3 years.

41.d. Start ASA, 81 mg/day, and increase warfarin to achieve an INR of 2.5 to 3.5. ACC/AHA guidelines recommended an INR of 2.5 to 3.5 for patients with bileaflet tilting disk mechanical valves in the aortic position who have had a thromboembolic event, and who have atrial fibrillation, LV dysfunction, or a hypercoagulable state. Addition of low-dose ASA (75 to 100 mg) is a class I indication for all patients with mechanical heart valves, and those with the above risk factors and bioprosthetic valves.

42.a. Intravenous heparin. Since a small clot was present (without any obstruction to valve function) the patient would benefit from increased anticoagulant therapy. If he were to fail this, then the other alternatives could be considered, such as continuous infusion thrombolytic therapy. Such continuous therapy (although not bolus) could also be used as primary treatment. No established indications exist at this time for glycoprotein IIb/IIIa inhibitors in this clinical setting. It would not be advisable to proceed to reoperation just yet, in the absence of a large clot burden or obstruction to inflow. If treatment with heparin leads to clot resolution, then subsequent warfarin dosing should be increased to maintain INR in the 3.0 to 4.0 range.

43.a. Therapy with warfarin. He should be on warfarin. Valvuloplasty in this setting (onset of atrial fibrillation, in an otherwise asymptomatic patient) is a class IIb indication by current guidelines.

44.a. Reassurance, with a repeat physical examination in 6 months. If a thorough physical examination reveals no signs of cardiopulmonary disease and the patient has no symptoms, then reassurance and follow-up are all that are required. Echocardiography should be performed if obesity limits the physical examination or if signs or symptoms are present.

45.b. Consider elective mitral valve repair at a hospital where repair is performed with a high degree of success or if he wishes to defer surgery, follow up at 6 monthly intervals with echo. Referral for surgery is reasonable (class IIa indication) if chance of repair is >95%. There are no data to suggest a beneficial role for the addition of afterload-reducing agents in the absence of systemic hypertension (again by ACC/AHA guidelines). There is absolutely no role for the prophylactic use of amiodarone. Close clinical follow-up is reasonable, but repeat evaluation should not be deferred for 2 years. Guidelines use LV dimensions and ejection fraction to guide surgical intervention, even in the absence of symptoms. As such, these patients should have clinical reevaluation and echo every 6 months.

46.a. Referral for mitral valve repair. He is now symptomatic with depressed ejection fraction and a dilated LV. This is a class I indication for surgery. Valve repair as opposed to replacement is the preferred surgical treatment. Medical therapy may be needed as an adjunct, but is insufficient as the sole treatment.

47.c. Life expectancy <1 year. Life expectancy of <1 year, despite treatment of AS, is an absolute contraindication for TAVR. Severe peripheral artery disease precludes a transfemoral approach; however, the procedure may be done via a transapical approach, a transsubclavian approach, and even a transaortic approach. Severe pulmonary disease and an inaccessible apex preclude a transapical approach but the other approaches remain available. Active endocarditis is a contraindication to the procedure. The available valves are suitable for annular sizes between 19 and 29 mm.

48.c. Mitral and tricuspid stenoses. The loud S1, opening snap, and apical diastolic rumble are features of mitral stenosis. The presence of the diastolic rumble along the sternal border, which increases with inspiration, along with the prominent a wave in the JVP and evidence of systemic venous congestion (hepatomegaly and peripheral edema) suggests that concomitant tricuspid stenosis is present as well.

49.c. Cardiac catheterization. By physical examination, the patient has severe AS (no A2 of second heart sound, late-peaking murmur, and diminished carotid upstrokes). A stress test would not be appropriate in a patient with symptomatic AS. An echocardiogram would usually be the first step, but proceeding directly to catheterization to measure transvalvular gradients and assess coronary anatomy would be reasonable. SL NTG could have disastrous consequences in this setting. By reducing preload, it may precipitate syncope.

50.b. Refer for percutaneous balloon valvuloplasty, followed by gastrectomy. He has symptomatic critical AS. AVR, with concomitant need for anticoagulation while on cardiopulmonary bypass, is not an attractive first option. Proceeding directly to gastric surgery would carry high risk, given the ongoing symptoms. Valvuloplasty would be a reasonable bridge to lower risk from the noncardiac surgery.

51.a. Bovine pericardial valve. He is at an age where there is substantial durability of the bioprosthetic valve. He is at increased risk for anticoagulation; thus, mechanical valves would not be the valve of first choice. By history, he would not appear to need anticoagulation for any other indication. Homograft is not unreasonable, but there would not appear to be any hemodynamic or durability benefits for an 80-year-old patient, and its insertion requires a more difficult operation.

52.c. ∼50% of cases have a bicuspid aortic valve. Coarctation of the aorta is suspected clinically and confirmed on a gated CT angiogram of the thoracic aorta. This lesion is frequently associated with concomitant congenital cardiac anomalies, the most frequent of which is a bicuspid aortic valve (occurs in ∼50% to 85% of cases).

53.a. Stress test. The patient has significant aortic regurgitation with a dilated LV although not yet at the dimension that would be an indication for surgery in the absence of symptoms (his end-systolic dimension is <5.0 cm and end-diastolic dimension is <7.0 cm). He leads a sedentary lifestyle, and although he has no dyspnea, he does relate some equivocal symptom. A stress test would be useful to assess functional capacity and to objectively assess symptoms. If he were to develop symptoms at a low level of exercise, this may be an indication for surgical intervention. A vasodilator may be useful (class IIb indication with dilated LV), but he would need more frequent follow-up, given the LV dilation.

54.b. Surgical intervention. (Refer for surgery.) His ventricle has dilated even further. An end-diastolic dimension of >7.5 cm is a class IIa indication for surgery and is associated with an increased risk of sudden death, even in the absence of symptoms.

55.b. Mean gradient across the mitral prosthesis of 17 mmHg. The clinical presentation and examination are suggestive of prosthetic mitral stenosis (long diastolic rumble, muffled closing click, and clinical heart failure). The PMI is not displaced, so it is unlikely that she has significant LV dysfunction. There are no clinical signs of severe MR. Given the acute onset of symptoms, acute valvular thrombosis leading to valvular obstruction is high on the differential. If this were the case by ACC/AHA guidelines, reoperation would be the preferred treatment approach. If other comorbidities were prohibitive, thrombolytic therapy could be considered.

56.a. Arrhythmias secondary to an accessory pathway. The examination is highly suggestive of Ebstein anomaly (presence of TR, widely split first heart sound). The echocardiogram confirms this. Accessory pathways are frequently associated with this condition.

57.a. Echocardiography with saline contrast study. Ebstein anomaly is frequently associated with cardiac shunts (either patent foramen ovale, or atrial or ventricular septal defect). The setting of a TIA in someone who has been immobilized (such as with a fracture) raises the concern of paradoxical embolism of a venous thrombus to the systemic circulation.

58.b. Vmax across the pulmonic valve of 4 m/s. The physical examination is consistent with pulmonic stenosis (presence of thrill, RV heave, ejection click, and crescendo-decrescendo murmur loudest over the pulmonic area). Normal carotid upstrokes and preserved A2 make significant AS unlikely. The murmur is not consistent with a regurgitant murmur.

59.a. He tells you of an episode of syncope. The presence of exertional dyspnea, angina, syncope, or near-syncope are class I indications for intervention. For gradients between 30 and 39 mmHg, there is some divergence of opinion about the role of intervention (class IIb for gradients 30 to 39). There is no role for intervention in those with gradients <30 mmHg who have no symptoms. A peak-to-peak gradient >40 mmHg by catheterization is a class I indication for intervention, even in an asymptomatic patient.

60.a. Percutaneous valvuloplasty. This is the preferred treatment for young adults with pulmonic stenosis.

61.b. Doppler echocardiography, using the continuity equation. With significant insufficiency, the Gorlin formula becomes less reliable. Pressure half-time is not used to calculate aortic valve area, but does give a clue to the severity of the AI.

62.c. Aortic homograft. Mechanical and bioprosthetic valves have a similar incidence of endocarditis, which is higher than that seen for homografts. In the setting of acute bacterial endocarditis of a prosthetic aortic valve, homografts are the valve of first choice when surgery is indicated.

63.d. Pulmonary hypertension resulting from primary left-sided disease. The most common cause of tricuspid insufficiency is pulmonary hypertension that results from primary pathology on the left side of the heart. This includes aortic and mitral valvular diseases, as well as LV dysfunction from coronary artery disease or other cardiomyopathies.

64.a. Streptococcus viridans accounts for up to 50% of cases. Staphylococcus aureus is the next most common pathogen.

65.c. Staphylococcus epidermidis. This patient presents with early prosthetic valve endocarditis (within 2 months of surgery). This is usually acquired during the operation, and the skin species Staphylococcus epidermidis is the most frequent pathogen encountered. Late prosthetic valve endocarditis is similar to native valve endocarditis in terms of the spectrum of pathogens involved.

SUGGESTED READINGS

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