Case Review

A 50-year-old woman with a history of a murmur was referred to the valve clinic at a large tertiary referral hospital. She denies any symptoms. Her exam is normal except for a blowing 3/6 holosystolic murmur at the apex that radiates to the base of the heart. Her transthoracic echocardiogram was of good quality and revealed a normal ejection fraction (EF; 68%) with normal left ventricular (LV) dimensions and severe eccentric anteriorly-directed mitral regurgitation (MR) secondary to posterior leaflet prolapse. Her predicted risk of mortality with surgical mitral valve (MV) repair or replacement is <1%.

Which of the following is the most appropriate next step in the management of this patient?

A.

Cardiac magnetic resonance imaging.

B.

Repeat transthoracic echocardiogram in 2 years.

C.

Transcatheter MV repair.

D.

Surgical MV replacement.

E.

Surgical MV repair.

A 50-year-old woman with a history of a murmur was referred to the valve clinic at a large tertiary referral hospital. She denies any symptoms. Her exam is normal except for a blowing 3/6 holosystolic murmur at the apex that radiates to the base of the heart. Her transthoracic echocardiogram was of good quality and revealed a normal ejection fraction (EF; 68%) with normal left ventricular (LV) dimensions and severe eccentric anteriorly-directed mitral regurgitation (MR) secondary to posterior leaflet prolapse. Her predicted risk of mortality with surgical mitral valve (MV) repair or replacement is <1%.

Which of the following is the most appropriate next step in the management of this patient?

A.

Cardiac magnetic resonance imaging.

B.

Repeat transthoracic echocardiogram in 2 years.

C.

Transcatheter MV repair.

D.

Surgical MV replacement.

E.

Surgical MV repair.

This patient has asymptomatic primary severe MR (stage C1) with a normal EF and normal LV dimensions. Patients with asymptomatic severe primary MR with a surgical risk of <1% and and a high likelihood of successful MV repair (>95%) may be considered for surgical MV repair to prevent long-term sequela and adverse remodeling (Class IIa). MV repair by an experienced surgeon is preferred over replacement. There is no indication for transcather repair as the patient is at a low risk for surgery. If observation is chosen, a repeat echocardiogram should be obtained in 6-12 months for severe MR. As the echocardiogram was of good diagnostic quality, cardiac magnetic resonance imaging is not indicated. 

51-year-old man is referred for evaluation of a chronic murmur. At 20 years of age, he underwent surgical repair of bicuspid aortic valve (BAV) stenosis. The patient has been followed intermittently and has no other significant medical history. He is physically active and asymptomatic, regularly running 5 miles without limitation.

On physical examination his heart rate is regular at 60 bpm and he has a blood pressure of 135/55 mm Hg. His lungs are clear to auscultation. Left ventricular (LV) apical impulse is enlarged and laterally displaced to the anterior axillary line. A grade 2/6 early-peaking systolic murmur and a decrescendo grade 2/4 diastolic murmur both are present along the left sternal border. The S2 is soft. There is no S3or S4. Peripheral examination demonstrates a pulsatile uvula and a collapsing water hammer radial pulse.

The patient undergoes a transthoracic echocardiogram that demonstrates BAV. LV end-diastolic dimension is 6.5 cm, LV end-systolic dimension is 4.2 cm, and LV ejection fraction is 57%. There is severe aortic regurgitation by color Doppler. Atrioventricular (AV) regurgitant volume is 70 ml, AV regurgitant fraction is 60%, and aortic root diameter is 4.2 cm.

Which of the following is the next best step in the management of this patient?

A.

Symptom-limited exercise stress test.

B.

Serial echocardiography.

C.

Surgical aortic valve replacement.

D.

Transcatheter aortic valve implantation.

51-year-old man is referred for evaluation of a chronic murmur. At 20 years of age, he underwent surgical repair of bicuspid aortic valve (BAV) stenosis. The patient has been followed intermittently and has no other significant medical history. He is physically active and asymptomatic, regularly running 5 miles without limitation.

On physical examination his heart rate is regular at 60 bpm and he has a blood pressure of 135/55 mm Hg. His lungs are clear to auscultation. Left ventricular (LV) apical impulse is enlarged and laterally displaced to the anterior axillary line. A grade 2/6 early-peaking systolic murmur and a decrescendo grade 2/4 diastolic murmur both are present along the left sternal border. The S2 is soft. There is no S3or S4. Peripheral examination demonstrates a pulsatile uvula and a collapsing water hammer radial pulse.

The patient undergoes a transthoracic echocardiogram that demonstrates BAV. LV end-diastolic dimension is 6.5 cm, LV end-systolic dimension is 4.2 cm, and LV ejection fraction is 57%. There is severe aortic regurgitation by color Doppler. Atrioventricular (AV) regurgitant volume is 70 ml, AV regurgitant fraction is 60%, and aortic root diameter is 4.2 cm.

Which of the following is the next best step in the management of this patient?

A.

Symptom-limited exercise stress test.

B.

Serial echocardiography.

C.

Surgical aortic valve replacement.

D.

Transcatheter aortic valve implantation.

51-year-old man is referred for evaluation of a chronic murmur. At 20 years of age, he underwent surgical repair of bicuspid aortic valve (BAV) stenosis. The patient has been followed intermittently and has no other significant medical history. He is physically active and asymptomatic, regularly running 5 miles without limitation.

On physical examination his heart rate is regular at 60 bpm and he has a blood pressure of 135/55 mm Hg. His lungs are clear to auscultation. Left ventricular (LV) apical impulse is enlarged and laterally displaced to the anterior axillary line. A grade 2/6 early-peaking systolic murmur and a decrescendo grade 2/4 diastolic murmur both are present along the left sternal border. The S2 is soft. There is no S3or S4. Peripheral examination demonstrates a pulsatile uvula and a collapsing water hammer radial pulse.

The patient undergoes a transthoracic echocardiogram that demonstrates BAV. LV end-diastolic dimension is 6.5 cm, LV end-systolic dimension is 4.2 cm, and LV ejection fraction is 57%. There is severe aortic regurgitation by color Doppler. Atrioventricular (AV) regurgitant volume is 70 ml, AV regurgitant fraction is 60%, and aortic root diameter is 4.2 cm.

Which of the following is the next best step in the management of this patient?

A.

Symptom-limited exercise stress test.

B.

Serial echocardiography.

C.

Surgical aortic valve replacement.

D.

Transcatheter aortic valve implantation.

This patient has asymptomatic severe aortic regurgitation (AR). Surgical aortic valve replacement (AVR) is a Class I indication if the LV ejection fraction is <55% or if the patient requires other cardiac surgery. (Figure 1). As intervention is not currently indicated for this patient, the most appropriate recommendation is to repeat the echocardiogram in 6-12 months. A symptom-limited exercise stress test is reasonable to clarify functional capacity in patients with equivocal symptoms, but is not indicated in a patient who can run 5 miles without limitations. Aortic valve replacement is not indicated in this asymptomatic patient. Transcatheter aortic valve implantation with currently available commercial devices is not indicated for management of AR.

54-year-old man presents for routine evaluation for known chronic aortic valve regurgitation due to bicuspid aortic valve. He remains asymptomatic. His vital signs include a heart rate of 84 bpm and blood pressure of 140/50 mm Hg. His physical examination reveals a grade 2/6 systolic ejection murmur along the left sternal border and a grade 3/4 diastolic flow murmur. His echocardiogram reveals severe aortic regurgitation.

Which of the following echocardiographic parameters would be an indication for surgical aortic valve replacement at this time?

A.

A left ventricular end-diastolic dimension of 6.0 cm.

B.

A vena contracta width of 0.7 cm.

C.

A left ventricular end-systolic dimension of 5.3 cm.

D.

A left ventricular ejection fraction of 55%.

E.

An aortic regurgitant fraction of 65%.

 

54-year-old man presents for routine evaluation for known chronic aortic valve regurgitation due to bicuspid aortic valve. He remains asymptomatic. His vital signs include a heart rate of 84 bpm and blood pressure of 140/50 mm Hg. His physical examination reveals a grade 2/6 systolic ejection murmur along the left sternal border and a grade 3/4 diastolic flow murmur. His echocardiogram reveals severe aortic regurgitation.

Which of the following echocardiographic parameters would be an indication for surgical aortic valve replacement at this time?

A.

A left ventricular end-diastolic dimension of 6.0 cm.

B.

A vena contracta width of 0.7 cm.

C.

A left ventricular end-systolic dimension of 5.3 cm.

D.

A left ventricular ejection fraction of 55%.

E.

An aortic regurgitant fraction of 65%.

 

Aortic valve replacement (AVR) is indicated for the asymptomatic patient with severe aortic regurgitation when there is evidence of left ventricular (LV) systolic dysfunction (LV ejection fraction <55%) or other cardiac surgery is planned (Class I indications; see Figure 1). AVR is also indicated if there is LVEF ≥55% but the left ventricular end-systolic dimension (LVESD) is >50 mm (Class IIa) or left ventricular end-diastolic dimension (LVEDD) is >65 mm and the patient is of low surgical risk (Class IIb). The other echocardiographic parameters (vena contracta width, regurgitant fraction) confirm severity of aortic regurgitation, but are not critical factors in the decision for surgery.

40-year-old man with a history of bicuspid aortic valve (BAV) presents to your office with complaints of exertional dyspnea. He recently joined a gym to help him lose weight. He has started jogging on a treadmill, however he notes shortness of breath when running at an incline. He denies chest pain and is able to run on level ground without difficulty. His physical exam includes a height of 70 inches, weight of 225 lbs, blood pressure of 112/73 mm Hg, and a heart rate of 82 bpm. His carotid pulses are prominent and lungs are clear. His heart exam is notable for a holodiastolic murmur. There is no lower extremity edema. A transthoracic echocardiogram is performed. His left ventricular ejection fraction is 60%. There is a BAV with aortic regurgitation (pressure half-time [PHT] 280 msec, vena contracta width 0.5 cm, and effective regurgitant oriface [ERO] 0.26 cm2). His ascending aorta measures 4.8 cm. These findings are similar to his echocardiogram performed last year.

What is the next best step in the management of this patient?

A.

Genetic testing.

B.

Echocardiogram in 6 months.

C.

Cardiac surgery referral.

D.

Hydralazine 10 mg three times a day.

E.

Computed tomography scan in 12 months.

40-year-old man with a history of bicuspid aortic valve (BAV) presents to your office with complaints of exertional dyspnea. He recently joined a gym to help him lose weight. He has started jogging on a treadmill, however he notes shortness of breath when running at an incline. He denies chest pain and is able to run on level ground without difficulty. His physical exam includes a height of 70 inches, weight of 225 lbs, blood pressure of 112/73 mm Hg, and a heart rate of 82 bpm. His carotid pulses are prominent and lungs are clear. His heart exam is notable for a holodiastolic murmur. There is no lower extremity edema. A transthoracic echocardiogram is performed. His left ventricular ejection fraction is 60%. There is a BAV with aortic regurgitation (pressure half-time [PHT] 280 msec, vena contracta width 0.5 cm, and effective regurgitant oriface [ERO] 0.26 cm2). His ascending aorta measures 4.8 cm. These findings are similar to his echocardiogram performed last year.

What is the next best step in the management of this patient?

A.

Genetic testing.

B.

Echocardiogram in 6 months.

C.

Cardiac surgery referral.

D.

Hydralazine 10 mg three times a day.

E.

Computed tomography scan in 12 months.

This patient has moderate aortic regurgitation (PHT 200-500 msec, vena contracta width 0.3-0.6 cm, and ERO 0.1-0.29 cm2). His exertional dyspnea is mild, only occurring when running at an incline and is likely due to deconditioning given that he has only recently started exercising and is overweight. The most notable finding in his presentation is the dilated ascending aorta at 4.8 cm. BAVs are frequently associated with aortic dilation either at the level of the sinuses of Valsalva or, more frequently, in the ascending aorta. The incidence of aortic dilation is higher in patients with fusion of the right and noncoronary cusps. Aortic imaging is recommended annually in patients with a BAV and significant aortic dilation (>4.5 cm), a rapid rate of change in aortic diameter (increase of >0.5 cm in a year, and in those with a family history of aortic dissection (Class I recommendation, Level of Evidence B-NR). Aortic imaging can be by echocardiogram if there is adequate image quality with visualization of the aorta up to 4 cm distal to the valve. Alternatively computed tomography or magnetic resonance imaging can provide better spatial resolution and is preferred in patients with poor echocardiographic windows.

This patient has no indication for cardiac surgery at this point. Surgical intervention is recommended at a dimension of 5.5 cm or at 5.1-5.5 cm in patients with rapid growth or a family history of aortic dissection. An echocardiogram at 6 months is too soon. Given his normal blood pressure, there is no benefit of adding hydralazine. At present, there are no proven drug therapies that have been shown to reduce the rate of progression of aortic dilation in patients with aortopathy associated with BAV. In patients with hypertension, control of blood pressure is warranted. A specific genetic cause has not been identified yet in patients with BAV and aortopathy and therefore genetic testing is not recommended.

A 34-year-old man is admitted with a fever. He has felt poorly for several months. He has had a 10-lb weight loss and recurring night sweats. In the past few weeks, he has experienced progressive dyspnea. He is on no medications at this time. His social history includes ongoing intravenous drug use.

His temperature is 38.5° Celcius, heart rate is 103 bpm, blood pressure is 95/50 mm Hg, and resting oxygen saturation on room air is 96%. His lungs reveal bibasilar fine rales. His jugular venous pressure is not elevated. His apex is hyperdynamic, and there is a grade 2/6 systolic murmur and grade 2/4 diastolic murmur along the left sternal border. The first heart sound (S1) is soft and there is an S3. He has no edema. There are splinter hemorrhages under the nailbeds on his left fingers.

His echocardiogram reveals a hyperdynamic left ventricle with evidence for severe aortic regurgitation (AR) and vegetations on his aortic valve (Figure 1).

(Figure 1)

In addition to broad spectrum antibiotics, which of the following is the most appropriate next step in the management of this patient?

A.

Aortic valve replacement.

B.

Intravenous esmolol 0.1 mg/kg/min.

C.

Intra-aortic balloon pump.

D.

Phenylephrine 40 mcg/min.

E.

Transesophageal echocardiogram.

A 34-year-old man is admitted with a fever. He has felt poorly for several months. He has had a 10-lb weight loss and recurring night sweats. In the past few weeks, he has experienced progressive dyspnea. He is on no medications at this time. His social history includes ongoing intravenous drug use.

His temperature is 38.5° Celcius, heart rate is 103 bpm, blood pressure is 95/50 mm Hg, and resting oxygen saturation on room air is 96%. His lungs reveal bibasilar fine rales. His jugular venous pressure is not elevated. His apex is hyperdynamic, and there is a grade 2/6 systolic murmur and grade 2/4 diastolic murmur along the left sternal border. The first heart sound (S1) is soft and there is an S3. He has no edema. There are splinter hemorrhages under the nailbeds on his left fingers.

His echocardiogram reveals a hyperdynamic left ventricle with evidence for severe aortic regurgitation (AR) and vegetations on his aortic valve (Figure 1).

(Figure 1)

In addition to broad spectrum antibiotics, which of the following is the most appropriate next step in the management of this patient?

A.

Aortic valve replacement.

B.

Intravenous esmolol 0.1 mg/kg/min.

C.

Intra-aortic balloon pump.

D.

Phenylephrine 40 mcg/min.

E.

Transesophageal echocardiogram.

This patient has evidence of infective endocarditis (IE) of the aortic valve resulting in severe AR and heart failure (HF). His exam is consistent with AR further supported by premature closure of the mitral valve (MV) on the m-mode echocardiogram shown. Early surgical intervention is a Class I indication for patients with IE and valve dysfunction causing HF.

His echocardiography reveals preclosure of the MV due to the rapidly rising left ventricular diastolic pressure as a result of his severe AR. This also results in the soft S1 observed. As opposed to chronic AR, the difference between the aortic and left ventricular pressures in diastole may be small in acute AR and there may be little diastolic murmur. Likewise the pulse pressure may not be wide, and there may be none of the classic hemodynamic findings of chronic AR. Schematic hemodynamic tracings of chronic versus acute AR are shown in Figure 2.

A transesophageal echocardiogram would be indicated in patients with aortic valve endocarditis to better assess the vegetation sizes and to help define the presence of an aortic abscess, but the procedure should not delay surgical intervention and can be performed intraoperatively.

Severe AR is a contraindication to the intra-aortic balloon pump. His tachycardia is appropriate for his serious hemodynamic state and beta-blockers therefore would not be appropriate. In addition, the reduced heart rate from a beta-blocker would increase diastolic time and the duration of AR per beat. Phenylephrine would increase afterload, which is contraindicated in severe AR.

45-year-old woman with mitral valve prolapse (MVP) for many years presents to your clinic for re-evaluation. She feels well. She teaches a spinning class at her gym. She takes no medications.

On exam her blood pressure is 100/60 mm Hg and pulse is 62 bpm. She has a mid systolic click followed by a loud murmur radiating to the axilla.

Her echocardiogram demonstrates a left ventricular end systolic diameter (LVESD) of 4.1 cm and left ventricular ejection fraction (LVEF) of 60%. There is posterior MVP resulting in severe mitral regurgitation (MR) with a calculated effective regurgitant orifice area of 0.45 cm2 and a regurgitant volume of 70 ml.

Which of the following is the best strategy for managing this patient's mitral valve disease?

A.

Bioprosthetic valve replacement.

B.

Mechanical valve replacement.

C.

Surgical valve repair.

D.

Watchful waiting.

E.

Transcatheter valve repair.

45-year-old woman with mitral valve prolapse (MVP) for many years presents to your clinic for re-evaluation. She feels well. She teaches a spinning class at her gym. She takes no medications.

On exam her blood pressure is 100/60 mm Hg and pulse is 62 bpm. She has a mid systolic click followed by a loud murmur radiating to the axilla.

Her echocardiogram demonstrates a left ventricular end systolic diameter (LVESD) of 4.1 cm and left ventricular ejection fraction (LVEF) of 60%. There is posterior MVP resulting in severe mitral regurgitation (MR) with a calculated effective regurgitant orifice area of 0.45 cm2 and a regurgitant volume of 70 ml.

Which of the following is the best strategy for managing this patient's mitral valve disease?

A.

Bioprosthetic valve replacement.

B.

Mechanical valve replacement.

C.

Surgical valve repair.

D.

Watchful waiting.

E.

Transcatheter valve repair.

Mitral valve (MV) surgery is recommended for asymptomatic patients with chronic severe primary MR and left ventricular (LV) dysfunction (LVEF of 30-60% and/or LVESD of 40 mm, stage C2).