Pulmonic regurgitation

INTRODUCTION — Physiologic trace to mild pulmonic valve regurgitation (also known as pulmonic regurgitation or PR) commonly occurs in normal individuals. Greater degrees of PR are caused by various disorders and can lead to right ventricular (RV) volume overload and right heart failure.

The causes, clinical manifestations, diagnosis, and treatment of PR are discussed here. Echocardiography of the pulmonic valve is discussed separately. (See "Echocardiographic evaluation of the pulmonic valve and pulmonary artery".)

CAUSES AND ASSOCIATED CONDITIONS — The etiologies of PR can be classified into physiologic, primary, and secondary causes. Primary causes include iatrogenic, infectious (infective endocarditis), immune-mediated (rheumatic heart disease), systemic (carcinoid disease), and congenital [1-3]. The epidemiology of primary PR is unknown, but it occurs in up to 30 percent of patients with certain congenital heart conditions such as after operation or intervention for tetralogy of Fallot and valvular pulmonic stenosis [4,5]. Secondary or functional PR occurs in patients with a morphologically normal pulmonic valve who have pulmonary artery dilatation and/or severe pulmonary arterial hypertension [6,7]. (See "Clinical features and diagnosis of pulmonary hypertension of unclear etiology in adults" and "Treatment and prognosis of pulmonary arterial hypertension in adults (group 1)".)

Physiologic trivial or mild PR is a common finding on color Doppler echocardiography of normal hearts [8-10].

The most common cause of severe PR is iatrogenic, due to surgical valvotomy/valvectomy or balloon pulmonary valvuloplasty performed for RV outflow tract obstruction as a component of Tetralogy of Fallot (or other conotruncal abnormality) repair [11-14] or for congenital pulmonic valve stenosis. Other causes of severe PR include endocarditis and carcinoid. (See "Tetralogy of Fallot (TOF): Pathophysiology, clinical features, and diagnosis" and "Tetralogy of Fallot (TOF): Management and outcome" and "Clinical manifestations and evaluation of adults with suspected left-sided native valve endocarditis" and "Clinical features of carcinoid syndrome" and "Clinical manifestations and diagnosis of pulmonic stenosis in adults".)

Congenital PR is rare and does not usually occur in isolation. It is seen in patients with tetralogy of Fallot and absent pulmonary valve syndrome, and in this setting, it usually presents as a mixed stenotic and regurgitant lesion [15,16].

PATHOPHYSIOLOGY — PR leads to RV volume overload, with subsequent RV enlargement, RV dysfunction, and functional tricuspid valve regurgitation. Chamber enlargement also gives rise to atrial and ventricular arrhythmias, and risk of morbidity and mortality if untreated [17-21].

PR is initially well tolerated, and patients are usually asymptomatic for many years [22]. However, with time, the RV dilates to compensate for the excess volume load in an effort to maintain cardiac output [23]. Progressive RV dilation eventually leads to RV dysfunction [21,24,25]. With further deterioration in RV function, cardiac output starts to decrease, and increased heart rate and oxygen extraction are required to maintain adequate tissue oxygenation. At that point, any increase in oxygen demand such as exercise is poorly tolerated [23].

CLINICAL MANIFESTATIONS

Symptoms and signs — Patients with PR are usually asymptomatic prior to onset of RV dysfunction.

In patients with severe PR developing RV dysfunction, initial symptoms consist of exertional dyspnea and fatigue owing to inability of the RV to increase its output in response to physical exertion [23]. With progression of RV dysfunction, and the onset of atrial and ventricular arrhythmias, patients may present with lightheadedness, palpitations, and rarely syncope. With enlargement of the RV and dilatation of the tricuspid valve annulus, tricuspid regurgitation may develop and when significant, can cause hepatic congestion, ascites, and pedal edema [22]. Chest pain is uncommon in patients with isolated severe PR.

Cardiac examination findings vary with the severity and cause of PR. The murmur of PR begins in early diastole and is generally best heard over the left second and third interspaces. The murmur may increase in intensity with inspiration.

In individuals with physiologic PR, cardiac exam findings are normal. An early diastolic murmur may be detected in thin individuals with mild PR. (See "Auscultation of cardiac murmurs in adults", section on 'Pulmonic regurgitation'.)

In patients with more significant PR, there may be a systolic ejection murmur at the left upper sternal border due to increased RV stroke volume. A third heart sound may be present but a fourth heart sound is unusual. In patients with severe PR a mildly accentuated RV impulse is generally present; however, the jugular venous pressure is usually normal. A prominent jugular venous "a" wave may be present in the setting of pulmonary artery hypertension, and a prominent "v" wave is noted in patients with severe tricuspid valve regurgitation. (See "Auscultation of cardiac murmurs in adults" and "Auscultation of heart sounds" and "Examination of the jugular venous pulse".)

The murmur of PR associated with pulmonary hypertension (Graham-Steell murmur) is high pitched and "blowing." It begins with an accentuated P2 component of the second heart sound. The duration of the murmur is variable and may occupy all of diastole if there is a pandiastolic gradient between the pulmonary artery and the RV diastolic pressure. The murmur has a decrescendo configuration and it may increase in intensity with inspiration. (See "Auscultation of cardiac murmurs in adults", section on 'Pulmonic regurgitation'.)

The murmur of PR differs in patients without pulmonary hypertension, such as most patients with PR after tetralogy of Fallot repair or other conditions requiring pulmonic valvotomy, idiopathic dilatation of the pulmonary artery, right-sided endocarditis, or congenital absence of the pulmonic valve. In these conditions, the pulmonary artery diastolic pressure is normal or low and there is a lower rate of regurgitant flow; the regurgitant murmur is of low to medium pitch and may be brief, in early diastole, because of early equalization of pulmonary artery and RV diastolic pressures. A to-and-fro murmur over the left second intercostal space may be noted or there may be no audible murmur with severe pulmonic regurgitation. In patients with normal pulmonary pressures, the murmur of PR may be inaudible.

In congenital absence of the pulmonic valve, P2 is absent and there is a silent interval between A2 and the onset of the regurgitant murmur. A loud to-and-fro murmur may be heard in these patients.

Test findings

Electrocardiogram — An electrocardiogram (ECG) is not required for the diagnosis of PR but is commonly obtained to identify associated conditions such as arrhythmias and findings suggestive of RV hypertrophy and congenital heart disease. The ECG usually demonstrates sinus rhythm, although arrhythmias may develop, as described above. Right bundle branch block with QRS prolongation is common in patients with repaired tetralogy of Fallot and longstanding severe PR (image 1). Right axis deviation and criteria for RV hypertrophy may be present in patients with associated RV outflow tract obstruction or pulmonary arterial hypertension [26].

Chest radiograph — A chest radiograph is not required for the diagnosis of PR but is commonly obtained in patients with dyspnea as an aid in evaluating pulmonary and cardiac causes. The most distinctive radiographic feature in patients with severe PR is RV enlargement. This is appreciated from a lateral chest radiograph as reduced retrosternal airspace. In addition, on the posteroanterior image, the left heart border demonstrates superior displacement of the apex. The main pulmonary trunk may be prominent but pulmonary vascularity is usually normal. The right atrium will be enlarged in patients with associated tricuspid regurgitation (image 2). Patients with prior operation will demonstrate features of prior sternotomy/thoracotomy and/or valve prosthesis.

DIAGNOSIS AND EVALUATION

Approach to diagnosis and evaluation — The diagnosis of pulmonic regurgitation (PR) should be considered in patients with an early diastolic murmur, in patients with incidentally detected RV enlargement, as well as in patients with a history of surgical valvotomy/valvectomy or balloon pulmonary valvuloplasty performed for RV outflow tract obstruction. Patients with repaired tetralogy of Fallot also frequently demonstrate severe PR due to patch enlargement of the RV outflow tract.

All patients with suspected valve disease should be evaluated by an echocardiogram, which generally confirms the diagnosis and also provides an evaluation of the mechanism, cause, and severity of valve disease, its hemodynamic effects, and assessment of associated disorders (such as pulmonary artery hypertension or tricuspid valve regurgitation).

Patients with moderate or greater PR should also undergo evaluation by cardiovascular magnetic resonance (CMR) imaging if available, which enables quantitative assessment of PR and of RV size and function, which may impact timing of intervention. Computed tomography (CT) is not generally required for the diagnosis and evaluation of PR but may be helpful in patients with suboptimal echocardiographic views who are not eligible for CMR testing.

Exercise testing is not generally required but may be helpful in patients with exertional symptoms that are greater than expected for the observed degree of valve disease and RV dysfunction. Cardiac catheterization is not generally required but is helpful in selected patients in the evaluation of pulmonary arterial hypertension. (See "Clinical features and diagnosis of pulmonary hypertension of unclear etiology in adults" and "Treatment and prognosis of pulmonary arterial hypertension in adults (group 1)".)

Echocardiogram — Two-dimensional and Doppler echocardiography are the most useful diagnostic tools in patients with PR. Echocardiography delineates the mechanism of regurgitation such as flail or dysplastic cusps, restricted cusp mobility (as seen in carcinoid heart disease), or pulmonary valve malcoaptation as seen in functional PR secondary to pulmonary arterial hypertension or idiopathic pulmonary artery dilatation [6,7,9,10]. In patients with infective endocarditis affecting the pulmonic valve, vegetations may be difficult to detect on transthoracic and transesophageal echocardiography; these imaging modalities are complementary [27]. (See "Echocardiographic evaluation of the pulmonic valve and pulmonary artery", section on 'Clinical considerations'.)

Parasternal short and long axis windows are the preferred views for assessing pulmonary valve anatomy and Doppler hemodynamics [28]. Apical and subcostal views can complement pulmonary valve anatomic and hemodynamic assessment. Color and spectral Doppler echocardiography are useful in quantifying the degree of PR [28,29].

PR severity is determined by jet width and duration (table 1). With mild PR, there is a thin regurgitant jet width. With moderate PR, the regurgitant jet widens but remains <50 percent of the pulmonary valve annulus width. With severe PR, the color jet fills the RV outflow tract (>50 percent of the pulmonary valve annulus dimension). PR is typically a holodiastolic jet; however, in severe PR, equalization of pulmonary artery and RV pressures occurs early- to mid-diastole, causing cessation of the jet. Severe PR is often underappreciated by color flow Doppler echocardiography due to the laminar signal and jet termination in early diastole (image 3A-B). (See "Echocardiographic evaluation of the pulmonic valve and pulmonary artery", section on 'Pulmonic valve regurgitation'.)

Vena contracta width, pulsed-wave Doppler flow measurement in the pulmonary arteries, and assessment of the continuous wave Doppler signal density and contour are important ancillary echocardiographic parameters used in the assessment of PR severity (table 1) [3,30]. With mild PR, the continuous wave Doppler signal density is light and the deceleration rate is slow. With moderate PR, the continuous wave Doppler signal density is moderate and the deceleration rate is variable. With severe PR, there is dense continuous wave Doppler signal, steep deceleration, and early termination of diastolic flow.

RV enlargement, systolic dysfunction, sequelae of severe PR can also be qualitatively assessed using two-dimensional Doppler echocardiography.

Due to the anterior location of the pulmonary valve, transesophageal echocardiography rarely provides incremental information about the severity of PR not detected on the transthoracic echocardiogram. Three-dimensional echocardiography has been reported to be useful in the assessment of PR in select patients [31,32]. (See "Echocardiographic evaluation of the pulmonic valve and pulmonary artery", section on 'Pulmonic valve regurgitation'.)

A comprehensive echocardiographic assessment should also include determination of left ventricular (LV) function, which is a prognostic factor for long-term survival [33-36], and associated anomalies such as tricuspid regurgitation, branch pulmonary artery stenosis, and intracardiac shunt.

Cardiovascular magnetic resonance — CMR imaging is the preferred modality for the assessment of RV enlargement and dysfunction, important sequelae of long-standing severe PR [19,29,37,38]. We suggest CMR in patients with moderate or greater PR. CMR can also be used for quantitative assessment of the severity of PR, including calculation of the regurgitant volume. The absence of ionizing radiation makes it ideal for serial assessment of disease progression (image 4). Exogenous contrast is not needed to quantify the severity of PR or assess RV size and function.

Computed tomography — CT can be used to evaluate the impact of PR on right heart size and function but is generally not used for serial assessment unless there is a contraindication to CMR such as an implanted cardiac pacemaker/device that is not MR compatible. Concerns about serial CT imaging include the use of ionizing radiation and iodinated contrast agent. In select cases, CT may be helpful prior to intervention to help determine whether percutaneous intervention is an option or before reoperation for surgical planning and assessment for coronary anatomy and atherosclerosis.

Exercise testing — Cardiopulmonary exercise testing provides prognostic information and may also be useful in deciding the timing of pulmonary valve replacement in patients with exertional symptoms out of proportion to disease severity and the degree of RV dysfunction [23,39]. Exercise testing is also useful in screening for exercise-induced arrhythmias and may be useful in risk stratification for sudden death [40].

Cardiac catheterization — Assessment of the mechanism, severity, and hemodynamic burden of PR can generally be reliably accomplished using non-invasive modalities. Cardiac catheterization, therefore, has a limited role in the diagnostic evaluation of patients with PR. It is primarily used in the evaluation of pulmonary vascular resistance in patients with pulmonary arterial hypertension, preoperative coronary artery assessment, or as part of comprehensive assessment for the patients being considered for percutaneous pulmonary valve replacement. Occasionally, preoperative invasive hemodynamic assessment helps to risk stratify patients with PR and associated lesions such as constrictive pericarditis, severe tricuspid regurgitation, and RV diastolic dysfunction. Cardiac catheterization may be helpful in differentiating the primary lesion. The hemodynamic findings of severe PR include low pulmonary artery end-diastolic pressure, resulting in wide pulse pressure; increased RV end-diastolic pressure; and in some cases, the pulmonary artery pressure tracing may become similar to the RV pressure tracing ("ventricularization") [41]. As compared with aortic root angiogram for assessment of aortic regurgitation, pulmonary artery angiogram is rarely performed in the current era.

DIFFERENTIAL DIAGNOSIS — An echocardiogram is used to distinguish pulmonic regurgitation (PR) from other causes of diastolic murmurs and right heart failure described here.

The differential diagnosis of a murmur consistent with PR includes other causes of diastolic murmurs, particularly aortic regurgitation (AR), which is associated with a similar decrescendo diastolic murmur starting in early diastole. The PR murmur can be distinguished from the murmur of AR by its increase in intensity during inspiration and its location (PR is best heard over the left second and third interspaces; AR is heard over left sternal border or over the right second interspace). A left anterior descending coronary artery stenosis is a rare cause of a diastolic murmur similar to that of PR. Mid- or late-diastolic murmurs caused by conditions such as mitral or tricuspid stenosis are less likely to be confused with the murmur of PR given differences in their timing, quality, and associated sounds. (See "Auscultation of cardiac murmurs in adults", section on 'Early diastolic murmurs'.)

The differential diagnoses of severe RV enlargement and dysfunction includes tricuspid regurgitation, left-to-right shunt resulting in RV volume overload, primary right heart myopathy such as arrhythmogenic RV cardiomyopathy, and the late phase of pulmonary hypertension.

In a patient with PR and symptoms of heart failure, a thorough cardiovascular evaluation (including history, physical examination, and echocardiography) is required to determine the cause of symptoms. Symptomatic right heart failure in a patient with PR with preserved RV function should prompt investigation for alternative diagnosis such as constrictive pericarditis and restrictive physiology. (See "Constrictive pericarditis: Diagnostic evaluation" and "Differentiating constrictive pericarditis and restrictive cardiomyopathy" and "Restrictive cardiomyopathies" and "Echocardiographic recognition of cardiomyopathies".)

MANAGEMENT

Monitoring — Patients with moderate or severe PR should be followed annually for clinical change by history, physical examination, and echocardiography. Earlier assessment is indicated if symptoms develop to determine optimal time for pulmonary valve replacement. CMR imaging should also be performed periodically.

Adults with repaired tetralogy of Fallot should generally have at least annual follow-up with a cardiologist with expertise in adult congenital heart disease, as recommended in the 2018 American College of Cardiology/American Heart Association (ACC/AHA) adult congenital heart disease guidelines [3]. (See "Tetralogy of Fallot (TOF): Management and outcome".)

Patients who have undergone surgical or percutaneous balloon pulmonary valvotomy should receive long-term follow-up by clinical examination and echocardiography to assess the severity of PR, RV size and function, tricuspid regurgitation, and pulmonary artery pressures [3]. The frequency of follow-up should vary according to the severity of disease, but should be at least every five years.

Medical therapy — Patients with asymptomatic, severe PR and normal RV function do not require any medical therapy.

For the patients with secondary PR, medical therapy should be directed at the underlying cause such as pulmonary arterial hypertension or carcinoid disease. (See "Treatment of pulmonary arterial hypertension (group 1) in adults: Pulmonary hypertension-specific therapy" and "Carcinoid heart disease".)

In patients with right heart failure and severe RV dysfunction who are not candidates for surgical or percutaneous intervention, medical therapy with diuretics, angiotensin converting enzyme inhibitors, and digoxin may be used but have not been demonstrated to provide survival benefit.

Endocarditis prophylaxis is not indicated for native PR unless there is a history of prior endocarditis. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)

Intervention

Surgical

Indications — Surgical pulmonary valve replacement or, in select situations, percutaneous pulmonary valve replacement is indicated in the following settings:

●For symptomatic, severe PR, surgical pulmonic valve replacement is recommended [3].

●For asymptomatic, severe PR when any two of the following four criteria are present, surgical pulmonic valve replacement is suggested [42-44]:

•Mild or moderate RV or LV systolic dysfunction.

•Severe RV dilation (RV end-diastolic volume index [RVEDVI] ≥160 mL/m², or RV end-systolic volume index [RVESVI] ≥80 mL/m², or RVEDV greater than or equal to two times the LV end-diastolic volume).

•RV systolic pressure due to RV outflow tract obstruction greater than or equal to two-thirds the systemic pressure.

•Progressive reduction in objective exercise tolerance.

●We also suggest pulmonary valve replacement for patients with asymptomatic severe PR and progressive tricuspid valve regurgitation, although evidence is more limited to support this approach. (See "Tetralogy of Fallot (TOF): Long-term complications and follow-up after repair", section on 'Pulmonary valve replacement (PVR)'.)

Simultaneous tricuspid annuloplasty is recommended in patients with moderate or worse tricuspid valve regurgitation due to tricuspid annular dilatation, while tricuspid valve replacement should be considered in cases of structurally abnormal valve due to damage from prior endocarditis or iatrogenic injury resulting in flail leaflet or leaflet perforation.

The first two indications above are similar to recommendations in the 2018 ACC/AHA adult congenital heart disease guidelines [3].

Indications for surgery for PR are based upon limited observational data, largely in patients with severe PR and repaired tetralogy of Fallot [45]. As described below, untreated severe PR is associated with right heart failure, and surgical treatment is associated with low risk and improved outcomes. The goal of pulmonary valve replacement is to eliminate volume overload of the RV and prevent irreversible ventricular dysfunction [46]. (See 'Outcomes' below.)

Valve choice — In patients undergoing surgical pulmonic valve replacement, bioprosthetic valves are generally preferred over mechanical valve prostheses with longevity up to approximately 15 years after implantation [37,47,48]. For patients that are at high risk for reoperation or already have a mechanical valve prosthesis in place (and thus already require anticoagulation), mechanical valve prosthesis may be appropriate [49,50].

Percutaneous — Patients with native PR are occasionally candidates for percutaneous pulmonary valve replacement [51-56]. The configuration of the native RV outflow tract is highly variable and depends on the type of initial intervention; thus, there is an increased risk of perivalvular regurgitation and device embolization. Although percutaneous pulmonic valve implantation has been performed in some cases of native PR, limited data are available to support this approach.

By contrast, percutaneous replacement of the pulmonary valve is an option for patients with prosthetic pulmonic valve regurgitation, including those with a pulmonary artery conduit with regurgitant prosthetic valve [57-60]. This procedure is discussed in detail separately. (See "Transcatheter pulmonary valve implantation".)

Follow-up after valve replacement — Following pulmonary valve replacement, all patients require an initial transthoracic echocardiogram to assess baseline valve hemodynamics for future comparison.

Meticulous anticoagulation management is required for all patients with mechanical valve prosthesis [47,49,61].

We now recommend temporary oral anticoagulation for three to six months after pulmonary valve replacement with a bioprosthesis, given our experience with bioprosthetic valve thrombosis [62]. For patients with bioprosthetic pulmonic valves, we suggest daily aspirin for the lifespan of the prosthesis. Long-term oral anticoagulation is indicated in patients with bioprosthetic pulmonic valves only when there are other indications for anticoagulation such as atrial arrhythmia, prior thromboembolic event, or features of premature prosthesis dysfunction concerning for bioprosthetic valve thrombosis [63,64].

All patients with a prosthetic pulmonic valve need life-long follow-up to assess valve and ventricular function [65,66]. In our practice, we check bioprosthetic valve function by echocardiographic imaging early postoperatively ("fingerprint") and then again within the first 12 to 18 months after implantation to monitor for development of changes related to bioprosthetic valve thrombosis. Unless an abnormality is found, further routine annual echocardiography generally is not required until 10 years after isolated-pulmonary valve implantation unless there is a change in clinical presentation. Given the high risk of infective endocarditis with a prosthetic heart valve, antimicrobial prophylaxis for bacterial endocarditis is recommended according to standard guidelines. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)

OUTCOMES — Untreated severe pulmonic valve regurgitation will result in RV enlargement, systolic dysfunction, arrhythmia, and death [17-21,46]. Surgical pulmonic valve replacement is a well-established treatment strategy with <1 percent periprocedural mortality, when performed by an experienced surgeon, and excellent long-term outcome with >60 percent freedom from reoperation at 10 years [37,47]. The timing of intervention is a crucial factor for long-term patient morbidity and survival. The freedom from reintervention after percutaneous pulmonary valve replacement is >90 percent at one year, but long-term data are lacking [58-60,67]. Cardiovascular magnetic resonance-derived preoperative RV volume and function are prognostic; preoperative RV end-diastolic volume index <160 mL/m² and end-systolic volume index <80 mL/m² are associated with normalization of ventricular dimensions during the first postoperative year [37,42,68-72].

Similarly, preoperative RV end-systolic volume index <80 mL/m² was strongly associated with mid-to-late (mean 6.3 years; interquartile range 4.9 to 9.5 years) RV normalization (ejection fraction >48 percent and RV end-diastolic volume <108 mL/m²) [73]. Conversely, a RV end-systolic volume >95 mL/m² was associated with increased risk for suboptimal hemodynamic outcome and adverse clinical events.

Structural valve failure and infective endocarditis are significant long-term complications after pulmonary valve replacement that require meticulous follow-up [37,47,58,74-77].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Cardiac valve disease".)

SUMMARY AND RECOMMENDATIONS

●Severe pulmonic regurgitation (PR) is most commonly iatrogenic, due to surgical or percutaneous procedures to treat right ventricular (RV) outflow obstruction. Other causes of severe PR include endocarditis and carcinoid heart disease. (See 'Causes and associated conditions' above.)

●Severe PR leads to RV volume overload, with subsequent RV enlargement, RV dysfunction, and functional tricuspid valve regurgitation. Chamber enlargement also gives rise to atrial and ventricular arrhythmias and risk of morbidity and mortality if untreated. (See 'Pathophysiology' above.)

●Patients with severe PR are usually asymptomatic prior to onset of RV dysfunction. In patients with severe PR developing RV dysfunction, initial symptoms consist of exertional dyspnea and fatigue owing to inability of the RV to increase its output in response to physical exertion. (See 'Symptoms and signs' above.)

●Cardiac examination findings vary with the severity and cause of PR. The murmur of PR begins in early diastole and is generally best heard over the left second and third interspaces. The murmur may increase in intensity with inspiration. (See 'Symptoms and signs' above.)

●The diagnosis of PR is generally confirmed by echocardiography, which also provides an evaluation of the mechanism, cause, and severity of valve disease, its hemodynamic effects, and assessment of associated disorders (such as pulmonary artery hypertension). (See 'Approach to diagnosis and evaluation' above.)

●Patients with moderate or severe PR should be followed annually for clinical change by history, physical examination, echocardiography, and cardiovascular magnetic resonance (CMR) imaging (if available). Earlier assessment is indicated if symptoms develop, to determine optimal time for pulmonary valve replacement. (See 'Monitoring' above.)

●The following are indications for surgical valve replacement for PR or, in select situations, percutaneous pulmonary valve replacement (see 'Indications' above):

•We recommend pulmonary valve replacement for symptomatic, severe PR.

•We suggest pulmonary valve replacement for asymptomatic, severe PR when any two of the following criteria are present:

-Mild or moderate RV or left ventricular (LV) systolic dysfunction.

-Severe RV dilation (RV end-diastolic volume index [RVEDVI] ≥160 mL/m², or RV end-systolic volume index [RVESVI] ≥80 mL/m², or RVEDV greater than or equal to two times the LV end-diastolic volume).

-RV systolic pressure due to RV outflow tract obstruction greater than or equal to two-thirds the systemic pressure.

-Progressive reduction in objective exercise tolerance.

•We suggest pulmonary valve replacement for patients with severe PR and progressive tricuspid valve regurgitation, although evidence is more limited to support this approach.

●Untreated severe PR will result in RV enlargement, systolic dysfunction, arrhythmia, and death. Surgical pulmonary valve replacement is a well-established treatment strategy with <1 percent periprocedural mortality when performed by an experienced surgeon and excellent long-term outcome with >60 percent freedom from reoperation at 10 years. (See 'Outcomes' above.)