Pulmonic and Tricuspid Valvular Disease
Roger Byrne
Brian P. Griffin
1. In which of the following clinical scenarios is transesophageal echocardiography (TEE) usually indicated in addition to transthoracic echocardiography (TTE) in the diagnostic assessment?
A. Pulmonary artery (PA) pressure in primary pulmonary hypertension.
B. Inferior vena cava (IVC) thrombus.
C. Suspected pacemaker endocarditis.
D. Right ventricular (RV) function.
View Answer
1. Answer: C. Right-sided valve lesions are usually well identified by transthoracic imaging as these structures lie anterior in the chest and in the near field of the chest wall transducer. However, better resolution of pulmonary valve endocarditis or mass, embolus in transit in the right-sided heart, and of vegetations involving a pacer wire have been reported with TEE than with TTE alone. TEE is not usually indicated in the assessment of PA pressures in primary pulmonary hypertension. When the tricuspid regurgitant velocity is difficult to measure by TTE, injection of agitated saline contrast has been shown to improve the spectral display of the regurgitant jet and is more likely to be helpful than a TEE in this situation.
2. A patient is undergoing an examination of the heart following a heart transplant. Severe tricuspid regurgitation (TR) is present but no turbulence in the jet is appreciated. Right ventricular (RV) function is moderately reduced. The tricuspid regurgitant velocity is 1 m/s. What other finding is most likely to be evident in this patient?
A. McConnell sign.
B. Flail tricuspid valve leaflet.
C. Tricuspid stenosis.
D. Atrial septal defect (ASD).
E. Severe pulmonary insufficiency.
View Answer
2. Answer: B. A flail tricuspid valve is common with valvular (usually septal) leaflet/chordae damage due to repeated endomyocardial biopsies following transplantation and leads to severe TR often with laminar rather than turbulent flow. McConnell sign or apical sparing of RV function may be seen in acute pulmonary embolism but is not characteristic of this patient’s scenario and is neither very sensitive (70%) nor specific (33%) for the diagnosis of pulmonary embolism with positive and negative predictive values of 67% and 33%, respectively. Pulmonary insufficiency, tricuspid stenosis, and ASD are usually not seen in transplanted hearts.
3. In the patient in question 2, the estimated RV systolic pressure from the tricuspid regurgitant velocity is:
A. Low (<30 mm Hg).
B. Mildly increased (30-50 mm Hg).
C. Moderately increased (51-70 mm Hg).
D. High (>70 mm Hg).
E. Cannot be accurately estimated by this technique.
View Answer
3. Answer: E. In the presence of severe TR with laminar flow through the valve, the right atrium and right ventricle operate more as a common chamber and Bernoulli equation (4 × velocity2), which applies to turbulent flow through a narrow or restricted orifice, is not operational. Severe TR, such as that due to a flail valve, is one of the few instances where Bernoulli equation may not be used to accurately estimate the RV systolic pressure. If RV systolic pressure is required, invasive pressure recording may be needed. Alternatively, estimation of mean and diastolic PA pressures via peak- and end-diastolic pulmonary regurgitation velocities using CW Doppler may be used.
4. A young man with mild pulmonary valve stenosis is seen. He has a peak gradient across the pulmonary valve of 20 mm Hg. His tricuspid regurgitant velocity is 3 m/s and his right atrial size is normal. His IVC is not enlarged and decreases further on sniffing. Which of the following is true?
A. His PA systolic pressure is normal.
B. His PA systolic pressure is moderately elevated.
C. His PA systolic pressure cannot be estimated when pulmonic stenosis is present.
D. He has severe PA hypertension that will require treatment.
E. None of the above.
View Answer
4. Answer: A. His PA systolic pressure is normal. The estimated RV systolic pressure from his tricuspid regurgitant velocity is 36 mm Hg + 5 mm Hg = 41 mm Hg assuming a normal right atrial (RA) pressure (a reasonable assumption given normal RA size and IVC size). The peak systolic pressure across the pulmonary valve is 20 mm Hg. Therefore, the PA systolic pressure is approximately 41 mm Hg – 20 mm Hg = 21 mm Hg or normal. PA systolic pressure can be estimated in pulmonary stenosis as long as the pressure gradient across the pulmonary valve is known.
5. The tricuspid valve consists of the following leaflets:
A. Anterior, lateral, medial.
B. Anterior, posterior, septal.
C. Left, right, posterior.
D. Moderator, anterior, posterior.
E. Moderator, posterior, septal.
View Answer
5. Answer: B. Using TTE, in the parasternal long-axis view (RV inflow), the anterior leaflet is imaged superiorly and to the right, adjacent to the RV free wall; and the septal leaflet is imaged inferiorly and to the left, adjacent to the interventricular septum. In the parasternal short-axis view (at the level of the aortic valve) the posterior leaflet is imaged adjacent to the RV free wall, and either the anterior or septal leaflet is imaged adjacent to the aortic root. The anterior and septal leaflets are also imaged in the apical 4-chamber view, with the anterior leaflet adjacent to the RV free wall and the septal leaflet adjacent to the interventricular septum. See Figure 18-20 (used with permission from Badano LP).
6. A Gerbode defect is:
A. Atrialization of the right ventricle in Ebstein anomaly.
B. An unroofed coronary sinus with resultant bidirectional ASD at the level of the AV groove.
C. A left-sided superior vena cava entering and enlarged coronary sinus.
D. A communication between the right atrium and the left ventricle that may encompass the tricuspid valve leaflets.
E. A sinus of Valsalva aneurysm that communicates with the right atrium.
View Answer
6. Answer: D. A Gerbode defect is a communication between the right atrium and left ventricle, often iatrogenic after surgery on the AV valves or following endocarditis of these valves. As the tricuspid valve is more apically situated under normal conditions in the heart than the mitral valve, the right atrium abuts the left ventricle over a small area. If a defect develops in this area, communication occurs between the right atrium and the left ventricle.
7. A patient with prior rheumatic disease is seen. An echocardiogram is obtained. Which of the following is true about the Doppler echocardiographic assessment of tricuspid stenosis in this condition?
A. Doming and thickening of the valve in systole are seen.
B. The mean pressure gradient is at least 10 mm Hg in severe stenosis.
C. The valve area may be estimated by dividing 190 by the pressure halftime.
D. Planimetry of the valve area is readily obtained.
E. Tricuspid stenosis is clinically significant in 25% of patients with rheumatic mitral stenosis.
View Answer
7. Answer: C. The constant used to estimate the valve area in tricuspid stenosis by pressure half time is 190 not 220 (used in mitral stenosis). Doming of the valve is seen in tricuspid stenosis but this is seen in diastole not systole. The mean gradient expected across the tricuspid valve in severe stenosis may be 5 mm Hg. Planimetry of the valve is difficult in tricuspid stenosis as it is difficult to get a true short-axis view of the valve; however, 3D TTE or 3D TEE may be useful for this. Although rheumatic involvement of the tricuspid valve occurs with some frequency, hemodynamically significant stenosis is relatively uncommon and is reported in about 5% of patients with rheumatic involvement of the mitral valve.
8. Pulmonary artery pressures cannot be estimated by measurement of a:
A. Tricuspid regurgitation (TR) jet.
B. Pulmonic insufficiency jet.
C. Ventricular septal defect (VSD) jet.
D. Atrial septal defect jet.
E. Right ventricular outflow tract jet.
View Answer
8. Answer: D. Pulmonary artery systolic pressure can be estimated using the TR jet using CW Doppler: 4(TR Vmax)2 plus estimated right atrial pressure (RAP). Mean and diastolic pulmonary artery pressures can be estimated using the pulmonic insufficiency jet using CW Doppler: 4(PI Vmax)2 + RAP and 4(PI end-diastolic velocity)2 + RAP, respectively. Mean pulmonary artery pressure may also be estimated using the right ventricular outflow tract PW Doppler acceleration time (ms): 79 – (0.45 × RVOT AT). A VSD may also be used to estimate right ventricular systolic pressure if a CW Doppler signal can be obtained coaxial with the direction of flow: systolic blood pressure – 4(VSD velocity)2. An atrial septal defect cannot be utilized to estimate pulmonary artery pressures.
9. The most common cause of significant TR is:
A. Myxomatous change or prolapse.
B. Rheumatic disease.
C. Endocarditis.
D. Secondary to pulmonary hypertension and/or RV dilatation.
E. Trauma.
View Answer
9. Answer: D. Secondary pulmonary hypertension and/or RV dilatation is the most common cause of significant TR. All of the other conditions may also lead to significant TR.
10. Which of the following is consistent with the diagnosis of severe pulmonic stenosis?
A. Peak velocity of >4 m/s across the pulmonic valve.
B. Normal RV systolic pressure.
C. RV wall thickness of 0.3 cm.
D. Normal size of the PA.
View Answer
10. Answer: A. Normal RV systolic pressure should not occur with severe pulmonic stenosis as the RV systolic pressure must exceed the PA systolic pressure by the gradient across the pulmonary valve. RV hypertrophy (wall thickness of >0.4 cm) and post-stenotic dilatation are common in severe pulmonic stenosis. Severe pulmonic stenosis is defined by Doppler echocardiography as a peak velocity across the valve of ≥4 m/s, moderate 3-4 m/s, and mild <3 m/s. Of note: the PA systolic pressure is usually normal in the setting of severe pulmonic stenosis.
11. A 25-year-old asymptomatic man presents with a systolic murmur at the second left interspace. An echocardiogram is obtained and he is found to have pulmonic stenosis. A peak pressure gradient is measured and is 20 mm Hg. Which of the following statements about his condition is most likely to be true?
A. He is likely to require surgical or balloon valvuloplasty in the next decade.
B. He should undergo yearly examination and echocardiography and a baseline transesophageal echocardiogram.
C. Cardiac catheterization is indicated to more accurately determine his pulmonic pressure gradient.
D. Systolic doming of the pulmonary valve is present.
View Answer
11. Answer: D. Mild stenosis is considered present when the Doppler velocity is <3 m/s or a pressure of <36 mm Hg. The prognosis is excellent and intervention is rarely necessary. It is appropriate to follow with yearly echocardiography, but cardiac catheterization or TEE is not indicated. Doming of the valve in systole is a common echocardiographic feature of pulmonic stenosis.
12. Which of the following statements about pulmonary insufficiency is correct?
A. Pulmonary insufficiency detected by Doppler of any degree is abnormal.
B. Severe pulmonary insufficiency leads to a highly turbulent jet on color flow Doppler.
C. Severe pulmonary insufficiency most commonly occurs in the setting of prior treatment of congenital heart disease.
D. Pulmonary insufficiency may be used to measure the PA systolic pressure.
View Answer
12. Answer: C. Severe pulmonary insufficiency is usually seen in the setting of prior surgery on the RV outflow tract or pulmonary valve as part of the treatment of a congenital heart lesion. Pulmonary insufficiency is detected to be trivial or mild normally. Severe pulmonary insufficiency is associated with a high end-diastolic pressure and a reduced pressure gradient across the pulmonic valve; thus, it is more often associated with laminar rather than turbulent velocity. The pulmonary insufficiency end-diastolic velocity (V) may be used to estimate the PA diastolic pressure as 4V2 + estimated RA pressure but is not used to estimate the PA systolic pressure. Pulmonary insufficiency may be graded using a number of parameters, at a Nyquist limit of 50-60 cm/s. Semiquantitative parameters and quantitative parameters such as vena contracta width, effective regurgitant orifice area, and regurgitant volume have not been well defined for PI. Qualitative parameters include an abnormal pulmonic valve morphology, a large jet with a wide origin, a dense jet by CW with steep deceleration/early termination of diastolic flow, and pulmonic flow > aortic flow by PW Doppler. Other suggestive findings include a dilated right ventricle.
13. Which of the following is the most likely cause of a mobile tricuspid valve mass?
A. Sarcoma.
B. Fibroelastoma.
C. Myxoma.
D. Chiari network.
E. Carcinoid syndrome.
View Answer
13. Answer: B. A fibroelastoma is the most common cause of a mobile mass on the tricuspid valve among the choices provided. Myxoma and sarcoma of the valve are much less common. A Chiari network is a fenestrated membranous structure, which originates at the orifice of the IVC and is an embryologic remnant. It may rarely float through the tricuspid valve but is usually confined to the right atrium, and is not attached to the tricuspid valve. Carcinoid syndrome causes immobility of the valve leaflets such that they may remain in a partially open condition throughout the cardiac cycle.
14. Which of the following conditions is not likely to cause hemodynamically significant lesions at both the tricuspid and pulmonary valve?
A. Carcinoid syndrome.
B. Staphylococcal infection.
C. Rheumatic fever.
D. Ebstein anomaly.
View Answer
14. Answer: D. Ebstein anomaly involves the apical displacement of the septal leaflet of the tricuspid valve but does not involve the pulmonary valve. Carcinoid syndrome, staphylococcal endocarditis, and rheumatic involvement may involve both the tricuspid and pulmonic valves. Rheumatic involvement may be primary or more commonly secondary to pulmonary hypertension from left-sided valve lesions that produce tricuspid and pulmonary regurgitation.
15. Which of the following statements about infundibular pulmonic stenosis is correct?
A. Infundibular pulmonic stenosis is always part of a congenital syndrome.
B. Infundibular stenosis may cause a high-velocity jet that impinges on the pulmonary valve causing pulmonary insufficiency.
C. The site of stenosis is usually discrete.
D. Doppler estimation of the pressure gradient across the infundibular stenosis is inaccurate except when valvular stenosis coexists.
E. Infundibular stenosis is most easily assessed from a parasternal short-axis imaging plane.
View Answer
15. Answer: B. Infundibular stenosis may give rise to a high-velocity jet that causes damage to the pulmonary valve leaflets and pulmonary insufficiency in a manner similar to subaortic stenosis. Infundibular stenosis may be either congenital or acquired. It occurs not only in congenital heart disease syndromes such as tetralogy of Fallot but also in hypertrophic cardiomyopathy, in tumors of the RV outflow tract, or in infiltrative disorders. It may be discrete or consist of a more extensive region of fibromuscular thickening. It is often best imaged and evaluated from a parasternal short-axis view or from the subcostal window. Pressure gradients measured by Doppler across the infundibular stenosis are reasonably accurate. When concomitant pulmonic valvular stenosis is present, it is usually impossible to isolate the precise contribution of the pulmonic valve and infundibulum to the total gradient measured by continuous-wave Doppler across the RV outflow tract.
16. A young man presents with fatigue and a history of occasional near syncope with onset of a fast heart rhythm. Based on the apical 4-chamber image in Figure 18-1, which is the least likely finding in this patient?
A. Wolff-Parkinson-White pattern on electrocardiogram.
B. Intracardiac shunt.
C. Parchment-like RV wall.
D. Severe TR.
View Answer
16. Answer: C. The image is characteristic of Ebstein anomaly of the tricuspid valve with displacement of the septal leaflet into the right ventricle so that a portion of the right ventricle is “atrialized.” Ebstein anomaly is associated with accelerated conduction via accessory pathways (Wolff-Parkinson-White), and severe TR but not with a parchment-like RV wall. This is seen in dysplastic RV or Uhl syndrome, which may be associated with ventricular arrhythmias.
17. You see a young man with prior open heart surgery for the first time. He is unaware of what surgery he had performed in the past. He has significant RV dilatation and some RV dysfunction. Based on the accompanying parasternal short-axis image of the pulmonary valve in Figure 18-2, which of the following statements is most likely to be correct?
A. Mild pulmonary regurgitation is present. No further workup is indicated.
B. He likely has an ASD with RV overload and high flow through the pulmonary circuit.
C. He has severe pulmonary regurgitation likely as a result of prior surgery on his pulmonary valve or RV outflow tract.
D. This represents a patent ductus arteriosus and requires reoperation.
E. If replacement of the pulmonic valve is required, a mechanical valve should be contemplated.
View Answer
17. Answer: C. This is severe pulmonary regurgitation with evidence of a proximal flow convergence on the PA side of the valve and a flail leaflet. It is consistent with RV dilation and RV dysfunction. The most common cause of severe pulmonary regurgitation is prior surgery for congenital heart disease involving the pulmonary valve or RV outflow tract. Patent ductus arteriosus will give rise to continuous flow into the PA above, not below, the valve. A homograft is usually the valve replacement of choice at the pulmonic position. Mechanical valves are associated with higher rates of thrombosis at right-sided valve positions because of the lesser pressure gradient across them and are usually avoided. Transcatheter pulmonic valve replacement is a growing field, and a potential option in selected patients.
18. A 57-year-old man is undergoing mitral valve repair for severe mitral regurgitation from mitral valve prolapse. He has an intraoperative TEE before the surgical repair and you are asked to consult regarding the image of the TR and tricuspid valve in the midesophageal 4-chamber view (Fig. 18-3). Which of the following is correct?
A. The degree of TR detected intraoperatively will likely overestimate that detected on routine ambulatory examination and should not be used in the decision making regarding concomitant tricuspid valve surgery.
B. Surgical intervention on the tricuspid valve is rarely required in this situation as it always improves after surgical correction of the mitral valve.
C. Surgical correction of the tricuspid valve should be considered as the regurgitation appears severe with a significant flow convergence area, and TR is more likely underestimated in the operative setting.
D. The most likely cause of severe TR in this situation is a flail tricuspid valve.
View Answer
18. Answer: C. There is severe TR present with a flow convergence area and dilation of the right atrium. Intraoperative TEE is more likely to underestimate the degree of regurgitation compared with the ambulatory setting due to decreased intravascular volume and change in loading consequent to anesthesia and mechanical ventilation. Severe TR occurs in mitral valve prolapse as a result of prolapse of the tricuspid valve or secondary to pulmonary hypertension from severe mitral regurgitation but rarely due to a concomitant flail of the tricuspid valve leaflet. Severe pulmonary hypertension is not necessary to cause this degree of TR; prolapse or secondary changes in the tricuspid annulus or valve may alone cause it. TR may improve after surgical repair of the mitral valve, especially if the pulmonary pressures fall, but this is less likely when the TR is severe preoperatively as in this case and therefore concomitant tricuspid valve repair should be considered. This adds relatively little to the operative risk or the duration of the case in experienced centers.
19. A 35-year-old woman has a history of recurrent fever of unknown origin and fleeting pleuritic chest pain. Multiple blood cultures have been negative, and a transthoracic echocardiogram of reasonable quality has been unremarkable. A chest x-ray film has shown a small pleural effusion but an ultrasound has shown that this is too small to aspirate. She undergoes TEE. A representative image of the RV outflow tract is shown in Figure 18-4 and constitutes the only abnormality detected. Which of the following would be the most appropriate next step in managing this patient?
A. Hypercoagulability workup.
B. Venous duplex of lower limbs to exclude venous thrombosis.
C. CT scan with contrast of the chest to exclude pulmonary emboli.
D. Noncontrast chest CT.
View Answer
19. Answer: D. This patient has a thickened pulmonary valve and pleuritic symptoms. Either endocarditis of the pulmonary valve or a pulmonary fibroelastoma is possible. The pleuritic symptoms and small pleural effusion suggest embolization to the lungs. A chest CT scan to look for septic emboli is the test of choice now. A chest CT scan was performed in this patient and showed abscess formation in the pulmonary parenchyma. Blood cultures were negative as the patient took oral antibiotics early in the course of treatment. Endocarditis was diagnosed and it transpired that the patient had an occult IV drug abuse habit.
20. The hepatic vein pulsed-wave Doppler profile shown in Figure 18-5 is most likely associated with which of the following clinical profiles?
A. Large “v” waves in the jugular venous profile.
B. Pulsus paradoxus.
C. Kussmaul sign.
D. Pulsus alternans.
View Answer
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20. Answer: A. High-velocity systolic reversal in the hepatic veins is seen in severe TR which also gives rise to large “v” waves in the jugular venous profile (Fig. 18-21). Pulsus paradoxus, an abnormally large reduction in systolic blood pressure by ≥10 mm Hg during inspiration, is most characteristic of cardiac tamponade (with a pericardial effusion the sensitivity is >80%), although is typically absent in occult (low pressure) or regional tamponade. Kussmaul sign with an increase in the venous pressure on inspiration is most characteristic of constrictive pericarditis. Pulsus alternans, alternating strong and weak peripheral pulses, is seen in end-stage LV systolic dysfunction. Pulsus bisferiens is a characteristic pulse felt in the setting of both significant aortic stenosis and regurgitation, and hypertrophic obstructive cardiomyopathy.
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