Chapter 285 - Tricuspid and Pulmonic Valve

Question 1

What is the most frequent cause of tricuspid stenosis?

Answer 1

Rheumatic disease.

Question 2

Does tricuspid stenosis occur as an isolated phenomenon?

Answer 2

“It does not occur as an isolated lesion and is usually asssociated with mitral stenosis.”

Question 3

How frequently is there mitral stenosis with hemodinamically significant tricuspid stenosis?

Answer 3

10-15%.

Question 4

Does rheumatic stenosis occur with any other valvulopathy?

Answer 4

Yes. Besides its correlation with mitral stenosis (due to rheumatic fever), “rheumatic tricuspid stenosis is commonly associated with some degree of tricuspid regurgitation.”

Question 5

Besides rheumatic fever as the cause of tricuspid stenosis (TS), are there any other common causes?

Answer 5

No.

“Nonrheymatic causes of TS are rare.”

Question 6

How does one define echocardiographically tricuspid stenosis (TS)? Is there any maneuvers that might help one define it?

Answer 6

“A diastolic pressure gradient between the right atrium and right ventricle defines TS. It is augmented when the transvalvular blood flow increases during inspiration and declines during expiration.”

Question 7

What is the mean diastolic pressure gradient in tricuspid stenosis that is usually sufficient to result in systemic venous congestion?

Answer 7

4mmHg.

Question 8

Which findings would you expect to find in tricuspid stenosis regardind: (i) physical findings; (ii) right atrium (RA) a wave; (iii) y descent; (iv) cardiac output at rest and during exercise.

Answer 8

(i) “Unless sodium intake has been restricted and diuretics administered, this venous congestion is associated with hepatomegaly, ascites, and edema, sometimes severe.”

For patients in sinus rhythm:

(ii) “the RA a wave may be extremely tall and may even approach the level of the right ventricle systolic pressure.”
(iii) “The y descent is prolonged.”

(iv) “The cardiac output (CO) at rest is usually depressed, and it fails to rise during exercise.”

Question 9

How does one explain the fact that tricuspid stenosis can mask the hemodynamic and clinical features of any associated mitral stenosis?

Answer 9

“The low cardiac output is responsible for the normal or only slightly elevated left atrial, pulmonary artery, and right ventricle systolic pressures despite the presence of mitral stenosis.”

Question 10

How come tricuspid stenosis is associated with pulmonary congestion initially but later in the disease, patients have little dyspnea complaints for the degree of hepatomegaly, ascites and edema?

Answer 10

“Because the development of MS generally precedes that of tricuspid stenosis (TS), many patients initially have symptoms of pulmonary congestion and fatigue. Characteristically, patients with severe TS complain of relatively little dyspnea for the degree of hepatomegaly, ascites, and edema that they have. However, fatigue secondary to a low cardiac output and discomfort due to refractory edema, ascites, and marked hepatomegaly are common in patients with advanced TS and/or tricuspid regurgitation.”

Question 11

Tricuspid stenosis may be suspected for the first time when symptoms of right-sided failure persist after adequate mitral valvulotomy.
True or False?

Answer 11

True.

Question 12

What are the physical findings (except auscultation) of severe tricuspid stenosis (TS)? Explain the pathophysiollogy associated with each finding.

Answer 12

“Because TS usually occurs in the presence of other obvious valvular dissease, the diagnosis may be missed unless it is considered. Severe TS is associated with marked hepatic congestion, often resulting in cirrhosis, jaundice, serious malnutrition, anasarca, and ascites. Congestive hepatomegaly and, in cases of severe tricuspid valve disease, splenomegaly are present. The jugular veins are distended, and in patients with sinus rhythm, there may be giant a waves. The v waves are less conspicuous and because tricuspid obstruction impedes right atrium empying during diastole, there is a slow y descent. In patients with sinus rhythm, there may be prominent presystolic pulsations of the enlarged liver as well.”

Question 13

What do you expect to auscultate in a patient with tricuspid stenosis? Is there any other murmur with similar characteristics? If so, how do you differentiate them?

Answer 13

“On auscultation, an opening snap (OS) of the tricuspid valve may rarely be heard approximately 0,06 s after pulmonic valve closure. The diastolic murmur of TS has many of the qualities of the diastolic murmur of MS, and because TS almost always occurs in the presence of MS, it may be missed. However, the tricuspid murmur is generally heard best along the left lower sternal border ad ove the xiphoid process, and is most prominent during presystole in patients with sinus rhythm. The murmur of TS is augmeneted during inspiration, and it is reduced during expiration and particularly during the strain phase of the Valsalva maneuver, when tricuspid transvalvular flow is reduced.”

Question 14

What is the ECG finding in mitral stenosis that should raise the suspicion of concomitant tricuspid valve disease?

Answer 14

Absence of signs of right ventricle hypertrophy.

Question 15

How do you expect to find P-waves in the ECG of a patient with tricuspid stenosis? How do you explain these fidings?

Answer 15

“The electrocardiogram features of right atrium enlargement include tall, peaked P waves in lead II, as well as prominent, upright P waves in lead V1.”

Question 16

What are the chest x-ray findings in tricuspid stenosis (TS)?

Answer 16

“The chest x-ray in patients with combined TS and mitral stenosis shows particular prominence of the right atrium and superior vena cava without much enlargement of the pulmonary artery and with less evidence of pulmonary vascular congestion than occurs in patients with isolated mitral stenosis.”

Question 17

What are the echocardiographic findings in tricuspid stenosis (TS)?

Answer 17

“On echocardiographic examination, the tricuspid valve is usually thickened and domes in diastole; the transvalvular gradient can be estimated by continuous wave Doppler echocardiography. Severe TS is characterized by a valve area equal or less than 1cm2 or pressure half-time of ≥190ms. The right atrium and inferior vena cava are enlarged.”

Question 18

Cardiac catheterization is mandatory in tricuspid stenosis (TS).
True or False?

Answer 18

False.

“Cardiac catheterization is not routinely necessary for assessment of TS.”

Question 19

Why is it that the decreasing of hepatic congestion is an important therapeutic in tricuspid stenosis (TS) preoperative patients?

Answer 19

“Patients with TS generally exhibit marked systemic venous congestion; salt restriction, bed rest, and diuretic therapy are required during the preoperative period. Such a preparatory period may dimish hepatic congestion and thereby improve hepatic function sufficiently so that the risks of operation, particularly bleeding, are dimished.”

Question 20

What are the indications for surgical repair of a tricuspid stenosis (TS)?

Answer 20

“Surgical relif of the TS should be carried out, preferably at the time of surgical mitral valvotomy or mitral valve replacement for mitral valve disease, in patients with moderate or severe TS who have mean diastolic pressure gradients exceeding ~4mmHg and tricuspid orifice areas

Question 21

Tricuspid stenosis surgical repair may permit substantial improvement of the tricuspid valve function.
True or False?

Answer 21

True.

Question 22

Meta-analysis have shown no difference in overall survival between mechanical and tissue valve replacement.
True or False?

Answer 22

True.

Question 23

Is there any complication more frequent in mechanical valves in tricuspid position, in comparison to other position?

Answer 23

Yes, this position is more prone to thromboembolic complications.

Question 24

Besides surgical repair and replacement, is there any other approach to tricuspid stenosis (TS)?

Answer 24

“Percutaneous tricuspid balloon valvuloplasty for isolated severe TS without significant tricuspid regurgation is very rarely performed.”

Question 25

What is the most common cause of tricuspid regurgitaton (TR)?

Answer 25

“In at least 80% of cases, TR is secondary to marked dilation of the tricuspid annulus from right ventricle enlargement due to pulmonary artery hypertension.”

Question 26

Functional tricuspid regurgitation (TR) only occurs secondarily to pulmonary hypertension.
True or False?

Answer 26

False.
“Functional TR may complicate right ventricle (RV) enlargement of any cause, however, including an inferior myocardial infarction that involves the RV.”

Question 27

Name the functional and “organic” (primary) causes of TR.

Answer 27

• Functional cause: pulmonary arterial (PA) hypertension (due to any cause) - “It is commonly seen in the late stages of heart failure due to rheuymatic or congenital heart disease with severe PA hypertension (PA systolic pressure >55mmHg), as well as in ichemic and idiopathic dilated cardiomypathies.”
• “Organic” causes: rhematic fever (associated with tricuspid stenosis); infarction of the right ventricle papillary muscles, tricuspid valve prolapse, carcinoide heart disease, endomyocardial fibrosis, radiation, infective endocarditis, and leaflet trauma; congenital (with defects of the atrioventricular canal as well as in Ebstein’s malformation of the tricuspid valve).

Question 28

What are the variables that determine the regurgitation volume in tricuspid regurgitaton (TR)?

Answer 28

“The incompetent tricuspid valve allows blood to flow backward from the RV into the RA, the volume of which is dependent on the driving pressure (i.e., RV systolic pressure) and the size of the regurgitant orifice.”

Question 29

The severity and physical signs of tricuspid regurgitaton (TR) might change due to different variables. Name them.

Answer 29

“The severity and physical sigs of TR can vary as a function of pulmonary artery systolic pressure (in the absence of RV outflow tract stenosis), the dimension of the tricuspid valve annulus, the respiratory cycle-dependent changes in RV preload, and RA compliance.”

Question 30

Why can one say that right atrium (RA) is “ventricularized” with the progression of tricuspid regurgitaton (TR)?

Answer 30

“Progressively severe TR can lead to “ventricularization” of the RA wave form.”

Question 31

What are the most early and common signs in severe isolated tricuspid regurgitaion (TR)?

Answer 31

Fatigue and exertional dyspnea.

Question 32

With progressive tricuspid regurgitaton (TR) patients complain of muscular wasting and fatigue but also weight gain. How does one explain this symptoms? Which other symptoms are common later in the course of the disease?

Answer 32

“As the disease progresses and Rv function declines, patients may report cervical pulsations, abdominal fullness/bloating, dimished appetite, and muscle wasting, although with progressive weight gain and painful swelling of the lower extremities.”

Question 33

How do you expect to find y descent in patients with: (i) tricuscpid stenosis; (ii) tricuspid regurgitation; (iii) both.

Answer 33

(i) prolonged y descent
(ii) rapid y descent
(iii) it depends which ones predominantes

Question 34

Tricuspid regurgitation (TR) is usually diagnosed during heart auscultation.
True or False?

Answer 34

False.

“TR is more often diagnosed by examination of the neck veins than by auscultation of the heart sounds.”

Question 35

What are the physical findings of tricuspid regurgitaton (TR)?

Answer 35

“The neck veins in patients with severe TR are distended with prominent c-v waves and rapid y descents (in the absence of TS). (…) Other findings may include marked hepatomegaly with systolic pulsations, ascites, pleural effusions, edema and a positive hepatojugular reflex. A prominent RV pulsation along the left parasternal region and a blowing holosystolic murmur along the lower left sternal margin, which may be intensified during inspiration (Carvall’s sign) and reduced during expiration or the strain phase of the Valsalva maneuver, are characteristic findings.”

Question 36

Atrial fibrillation is usually present in the chronic phase of tricuspid regurgitaiton.
True or False?

Answer 36

True.

Question 37

How does one differentiate a murmur due to regurgitation of the tricuspid valve from that of the mitral valve?

Answer 37

“The murmur of tricuspid regurgitation may sometimes be confused with that of mitral regurgitation unless attention is paid to its variation during the respiratory cycle and the extent of RV enlargement is appreciated.”

Question 38

What is the ECG alteration that one might see in tricuspid regurgitaton (TR) with Ebstein’s anomaly?

Answer 38

Bizarre right bundle branch block type pattern.

Question 39

What do you expect to find in a chest x-ray from a patient with tricuspid regurgitaton (TR)?

Answer 39

“The chest x-ray may show RA and RV enlargement, depending on the chronicity and severity of TR.”

Question 40

Accurate assessment of TR severity, PA pressures, and RV size and systolic function with TTE can be quite challenging in many patients.
True or False?

Answer 40

True.

Question 41

X descent might be absent in severe tricuspid regurgitaton (TR).
True or False?

Answer 41

True.

Question 42

Although exercise testing can be used to assess functional capacity in patients with asymptomatic severe TR, its prognostic significance is not well studied.
True or False?

Answer 42

True.

Question 43

Why is it that aldosterone antagonists might be useful in tricuspid regurgitation?

Answer 43

“Diuretics can be useful for patients with severe TR and signs of right heart failure. An aldosterone antagonist may be particularly helpful because many patients have secondary huperaldosteronim from marked hepatic congestion.”

Question 44

In functional tricuspid regurgitaton (with pulmonary artery hypertension) which therapies might one consider?

Answer 44

Therapies to reduced elevate pulmonary artery pressure, including those targeted at left-sided heart disease.

Question 45

What are the surgical indications for tricuspid regurgitation (TR)?

Answer 45

“Tricuspide valve surgery is recommmended for patients with severe TR who are undergoing left-sided valve surgery and is also undertaken frequently for treatment of even moderate TR in patients undergoing left-sided valve surgery who have tricuspid annular dilation (>40mm), a history of right heart failure, or PA hypertension. (…) Surgery may also infrequently be required for treatment of severe, primary TR with right heart failure not responsive to standard medical therapy or because of progressively declining Rv systolic function.”

Question 46

How high is the mortality rate for repair or replacement of isolated tricuspid regurgitation?

Answer 46

~8-9%.

Question 47

Which type of surgery is mostly used for tricuspid regurgitaton - repair or replacement?

Answer 47

Repair.

Question 48

What is the most frequent cause of pulmonic stenosis (PS)? Do you know any other causes for PS?

Answer 48

Congenital pulmonic stenosis.
“Dysplastic pulmonic valves are seen as part of the Noonan’s syndrome, which maps to chromosome 12. Much less common etiologies include carcinoid and obstructing tumors or bulky vegetations.”

Question 49

Rheumatic fever commonly involves the pulmonic valve.

True or False?

Answer 49

False.

“The pulmonic valve is only very rarely affected by the rheumatic process.”

Question 50

Compare pathophysiologically and the natural history of aortic stenosis (AS) to pulmonic stenosis (PS).

Answer 50

“Compared with the ability of the LV to compensate for the pressure overload imposed by aortic stenosis (AS), RV dysfunction from afterload mismatch occurs earlier in the course of PS and at lower peak systolic pressures, because the RV adapts less well to this type of hemodynamic burden.”

Question 51

What are the values of peak systolic gradient indicative of severe and moderate pulmonic stenosis (PS)?

Answer 51

“With normal systolic function and cardiac output, severe PS is defined by a peak systolic gradient across the pulmonic valve of >50mmHg; moderate PS correlates with a peak gradient of 30-50mmHg.”

Question 52

A peak systolic gradient less than 30mmHg never worsens in pulmonic stenosis.
True or False?

Answer 52

False.
“OPS rarely progresses in patients with peak gradients less than 30 mmHg, but may worsen in those with moderate disease due to valve thieckening and calcification with age.”

Question 53

Cardiac output is maintained until late in the course of pulmonic stenosis.
True or False?

Answer 53

True.

Question 54

Do right atrium a waves elevate in pulmonic stenosis?

Answer 54

Yes, since atrial systole occurs to a hypertrophied and noncomplacent right ventricle.

Question 55

Which symptoms and signs do you expect to find in pulmonic stenosis (PS)? Explain them regardind the severity of this valvulopathy.

Answer 55

“Patients with mild or even moderate PS are usually asymptomatic and first come to medical attention because of heart murmur that leads to echocardiography. With severe PS, patients may report exertional dyspnea or early-onset fatigue. Anginal chest pain from RV oxygen supply-demand mismatch and syncope may occur with very severe forms of obstruction, particularly in the presence of a destabilizing trigger such as atrial fibrillation, fever, infection, or anemia.”

Question 56

Which valvulopathy is more frequently associated with syncope?

Answer 56

Aortic stenosis.

Question 57

The pulmonic ejection sound is the only right-sided sound that decreases its intensity with inspiration.
True or False?

Answer 57

True.

Question 58

What do you expect to find as you auscultate a patient with pulmonic stenosis?

Answer 58

Meso-systolic murmur, ejection sound (if the valve is pliable, especially in young adults) and, eventually, an S4 due to an hypertrophied right ventricle.

Question 59

As the pulmonic stenosis aggravates, what do you expect to happen to the ejection sound, P2 component of the second heart sound, a wave and congestion signs?

Answer 59

“With progressively severe PS, the ejection sound moves closer to the first heart sound and eventually becomes inaudible. A right-sided fourth heart sound may emerge. The systolic murmur peaks later and may persist through the aortic component of the second heart sound (A2). Pulmonic valve closure is delayed and pulmonic component of the second heart sound (P2) is reduced or absent. A prominent a wave, indicative of the higher atrial pressure necessary to fill the noncompliant RV, may be seen in the jugular venous pulse. A parasternal or RV lift can be felt with significant pressure overload. Signs of right heart failure, such as hepatomegaly, ascites, and edema, are uncommon but may appear very late in the disease.”

Question 60

What do you expect to find in the ECG and chest x-ray of a patient with pulmonic stenosis (PS)?

Answer 60

“The ECG will show right axis deviation, right ventricle hypertrophy, and RA enlargement in adult patients with severe PS. Chest x-ray findings include poststenotic dilation of the main PA in the frontal plane projection and filling of the retrosternal airspace due to RV enlargement on the lateral film. In some patients with RVH, the cardiac apex appers to be lifted off the left hemidiaphragm. The RA may also be enlarged.”

Question 61

Is there any utility for transesophageal echocardiography (TEE) in pulmonic stenosis?

Answer 61

Yes.
“TEE may be useful in some patients for improved delineation of the RV outflow tract (RVOT) and assessment of infundibular hypertrophy.”

Question 62

How strong is the correlation between Doppler assessment of peak instantaneous gradient and catheterization-measued peak-to-peak gradient?

Answer 62

The correlation is weak.

“The latter may correlate better with the Doppler mean gradient.”

Question 63

How does one treat pulmonic stenosis?

Answer 63

Diuretics for symptoms and signs of right heart failure, pulmonic balloon valvotomy and surgical repair.

Question 64

What are the indications for pulmonic balloon valvotomy and surgical repair of a stenotic pulmonic valve?

Answer 64

“Provided there is less than moderate pulmonic regurgitation, pulmonic balloon valvotomy is recommended for symptomatic patients with a domed valve and a peak gradient >50mmHg (or a mean gradient >30mmHg) and for asymptomatic patients with a peak gradient >60mmHg (or mean gradient >40mmHg). Surgery may be required when the valve is dysplastic (as seen in patients with Noonan’s syndrome and other disorders).”

Question 65

Name the causes for pulmonic regurgitation.

Answer 65

“Pulmonic regurgitatiopn (PR) may develop as a consequence of primary valve pathology, annular enlargement, or their combinations; after surgical treatment of right ventricle outflow tract obstruction in children with such disorders as tetralogy of Fallot; or after pulmonic balloon valvotomy. Carcinoid usually causes mixed pulminic valve disease with PR and PS. Long-standing severe PA hyeprtension from any cause can result in dilation of the pulmonic valve ring and PR.”

Question 66

The duration of the murmur in pulmonic regurgitation increases with aggravating regurgitation.
True or False?

Answer 66

False.
As the disease progresses, the gradient between the pulmonary artery and right ventricle decreases, which leads to a shorter murmur.

Question 67

Which symptoms do you expect to find in pulmonic regurgitation (PR)? Explain the correlation with the severity of this valvulopathy.

Answer 67

“Mild to moderate degrees of PR do not, by themselves, result in symptoms. Other problems, such as PA hypertension, may dominant the clinical picture. With progressively severe Pr and RV dysfunction, fatigue, exertional dyspnea, abdominal fullness/bloating, and lower extremity swelling may be reported.”

Question 68

Is there any characteristic features of the pulmonic murmur in pulmonic regurgitation (PR) due to a congenital cause?

Answer 68

Yes.
“Survivors of childhood surgery for tetralogy of Fallot or PS/pulmonary atresia may have an RV-PA conduit that is freely regurgitant because it does not contain a valve. PA pressures in these individuals are not elevated and the diastolic murmur can be misleadingly low pitched and of short duration despite significant degrees of PR and RV volume overload.”

Question 69

Cardiac magnetic ressonance provides a greater anatomic detail, particularly in patients with repiared congenital heart disease, and more precise assessment of RV volumes.
True or False?

Answer 69

True.

Question 70

Pulmonic valve replacement due to carcinoid or endocarditis is frequently undertaken.
True or False?

Answer 70

False.

Question 71

Aortic valve replacement was clinically introduced before pulmonic valve replacement via transcatheter.
True or False?

Answer 71

False.