Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

acute pulmonary regurgitation

1st line – medical management of heart failure

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ACUTE
|
acute pulmonary regurgitation
|
nonsevere
1st line – 

medical management of heart failure

An almost unavoidable complication of balloon pulmonary valvuloplasty.

The severity is more dependent on symptoms than on imaging in this setting. Severe symptoms and signs include dyspnea on exertion, syncope, orthopnea, and lower extremity edema. However, in general, a jet width that occupies >65% of the right ventricular outflow tract is suggestive of severe pulmonary regurgitation.[8]

In nonsevere cases, management is directed at treating heart failure using standard therapies.[22]

1st line – medical management of heart failure

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ACUTE
|
acute pulmonary regurgitation
|
severe
1st line – 

medical management of heart failure

A complication of balloon pulmonary valvuloplasty; it is rare for severe disease to occur.

The severity is more dependent on symptoms than on imaging in this setting. Severe symptoms and signs include dyspnea on exertion, syncope, orthopnea, and lower extremity edema. However, in general, a jet width that occupies >65% of the right ventricular outflow tract is suggestive of severe pulmonary regurgitation.[8]​​

Most severe cases can be managed conservatively, directing treatment toward heart failure using standard therapies.[22]

Consider – inotropic support + pulmonary valve replacement

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ACUTE
|
acute pulmonary regurgitation
|
severe
Consider – 

inotropic support + pulmonary valve replacement

Treatment recommended for SOME patients in selected patient group

Fluid resuscitation and intravenous pressors such as dopamine or dobutamine may be necessary. However, in most cases, pulmonary valve replacement may be needed to treat severe pulmonary regurgitation. Transcatheter pulmonary valve replacement is a less invasive approach than surgery and may be considered in some patients.[15][16] Surgical valve replacement may be the only option in patients with large right ventricular outflow tracts and/or main pulmonary arteries, due to sizing of the available devices.[17] Transcatheter pulmonary valve replacement has been demonstrated to have comparable mortality and need for reintervention compared to surgical valve replacement, but with greater rates of infective endocarditis.[2][18]​​​​​

Traditionally bioprosthetic pulmonary valves have been implanted; however, they have the disadvantage of time-related structural valve failure.[14]​​ Mechanical pulmonary valves have the advantage of long-term durability, but they have been infrequently used due to concerns regarding thrombosis and difficulty performing invasive hemodynamic studies following mechanical valve replacement.

Reports on long-term outcomes of mechanical pulmonary valves are emerging; however, there is a selection bias for patients with prior operations in such reports.[25]​​

The appropriate valve used in pulmonary valve replacement needs to be tailored for the individual patient depending on age, multiple operations, and the need for long-term anticoagulation. All patients who receive a mechanical valve require lifelong anticoagulation. Anticoagulation may also be indicated due to the presence of other mechanical prostheses or as part of the treatment of comorbid conditions.[14]​​

Primary options

dopamine: 3 to 10 microgram/kg/min by intravenous infusion

OR

dobutamine: 1 to 10 microgram/kg/min by intravenous infusion

ONGOING

chronic pulmonary regurgitation

1st line – treatment of the underlying cause

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ONGOING
|
chronic pulmonary regurgitation
|
asymptomatic
1st line – 

treatment of the underlying cause

No specific treatment is required, and treatment is directed at the underlying cause. The only exception is in patients who develop pulmonary regurgitation following repair of tetralogy of Fallot or a Ross procedure (a procedure in which the diseased aortic valve is replaced with the patient's own pulmonary valve, and the pulmonary valve is then replaced with a cryopreserved cadaveric pulmonary valve), in whom valve replacement may be considered.

Consider – pulmonary valve replacement ± anticoagulation

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ONGOING
|
chronic pulmonary regurgitation
|
asymptomatic
Consider – 

pulmonary valve replacement ± anticoagulation

Treatment recommended for SOME patients in selected patient group

Replacement may be considered in patients who develop pulmonary regurgitation after repair of tetralogy of Fallot or a Ross procedure, as it may be unwise to wait until right ventricular (RV) function deteriorates with irreversible damage to ventricular performance.[20][21]​​​ In these patients, guidelines now recommend pulmonary valve replacement based on changes in objective exercise capacity, RV end-diastolic and end-systolic volumes, RV systolic dysfunction.[15][16]

Extrapolation of these criteria to those patients with isolated pulmonary regurgitation after intervention for pulmonary valve stenosis may not be appropriate.

Transcatheter pulmonary valve replacement is a less invasive approach than surgery and may be considered in some patients.[15][16]​ Surgical valve replacement may be the only option in patients with large RV outflow tracts and/or main pulmonary arteries, due to sizing of the available devices.[17]​ Transcatheter pulmonary valve replacement has been demonstrated to have comparable mortality and need for reintervention compared to surgical valve replacement, but with greater rates of infective endocarditis.[2][18]​​

The appropriate valve used in pulmonary valve replacement needs to be tailored for the individual patient depending on age, multiple operations, and the need for long-term anticoagulation. Traditionally bioprosthetic pulmonary valves have been implanted; however, they have the disadvantage of time-related structural valve failure.[14]​​ Mechanical pulmonary valves have the advantage of long-term durability, but they have been infrequently used due to concerns regarding thrombosis and difficulty performing invasive hemodynamic studies following mechanical valve replacement.​​

Reports on long-term outcomes of mechanical pulmonary valves are emerging; however, there is a selection bias for patients with prior operations in such reports.[25]

All patients who receive a mechanical valve require lifelong anticoagulation. Bioprosthetic valves do not require anticoagulation. Anticoagulation may also be indicated due to the presence of other mechanical prostheses or as part of the treatment of comorbid conditions.[14]​​

Primary options

warfarin: 2-5 mg orally once daily initially, adjust dose according to target international normalized ratio

More

1st line – treatment of the underlying cause + medical management of heart failure

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ONGOING
|
chronic pulmonary regurgitation
|
symptomatic with New York Heart Association (NYHA) class I
1st line – 

treatment of the underlying cause + medical management of heart failure

Longstanding pulmonary regurgitation can lead to severe right ventricular (RV) dilation and diminished RV systolic performance, which can lead to an inadequate ability to augment cardiac output with exercise and, in some cases, right-sided heart failure or congestive heart failure. Patients are classed as NYHA class I if they are in heart failure, there is no limitation of physical activity, and ordinary physical activity does not cause undue fatigue, palpitations, or dyspnea, but symptoms appear with more than ordinary activity.

No specific treatment is required. In general the underlying conditions such as left ventricular failure, mitral stenosis, and pulmonary hypertension need to be treated. Heart failure needs to be managed using standard therapies.[22]

Consider – pulmonary valve replacement ± anticoagulation

Back
ONGOING
|
chronic pulmonary regurgitation
|
symptomatic with New York Heart Association (NYHA) class I
Consider – 

pulmonary valve replacement ± anticoagulation

Treatment recommended for SOME patients in selected patient group

Patients who develop pulmonary regurgitation after repair of tetralogy of Fallot or a Ross procedure may be considered for early pulmonary valve replacement.[23] If pulmonary regurgitation is severe and associated with right-sided heart failure, then pulmonary valve replacement may also be necessary. There are, however, no definitive guidelines and the best course of action is unclear.

Transcatheter pulmonary valve replacement is a less invasive approach than surgery and may be considered in some patients.[15][16]​​ Surgical valve replacement may be the only option in patients with large right ventricular outflow tracts and/or main pulmonary arteries, due to sizing of the available devices.[17]​ Transcatheter pulmonary valve replacement has been demonstrated to have comparable mortality and need for reintervention compared to surgical valve replacement, but with greater rates of infective endocarditis.[2][18]​​​

The appropriate valve used in pulmonary valve replacement needs to be tailored for the individual patient depending on age, multiple operations, and the need for long-term anticoagulation. Traditionally bioprosthetic pulmonary valves have been implanted; however, they have the disadvantage of time-related structural valve failure.[14]​​ Mechanical pulmonary valves may be advantageous due to their structural stability, but they have been infrequently used due to concerns regarding thrombosis and difficulty performing invasive hemodynamic studies following mechanical valve replacement.​​

Reports on long-term outcomes of mechanical pulmonary valves are emerging; however, there is a selection bias for patients with prior operations in such reports.[25]

All patients who receive a mechanical valve require lifelong anticoagulation. Bioprosthetic valves do not require anticoagulation. Anticoagulation may also be indicated due to the presence of other mechanical prostheses or as part of the treatment of comorbid conditions.​[14]

Primary options

warfarin: 2-5 mg orally once daily initially, adjust dose according to target international normalized ratio

More

1st line – treatment of the underlying cause + medical management of heart failure

Back
ONGOING
|
chronic pulmonary regurgitation
|
symptomatic with New York Heart Association (NYHA) class II or III
1st line – 

treatment of the underlying cause + medical management of heart failure

Patients are classed as NYHA class II if they are in heart failure and have slight, mild limitation of activity. Patients are classed as NYHA class III if they are in heart failure, have marked limitation of activity, and are comfortable only at rest.

Pulmonary regurgitation commonly occurs after successful repair of tetralogy of Fallot or the Ross procedure.

In general, underlying and associated conditions such as left ventricular failure, mitral stenosis, and pulmonary hypertension need to be treated.

Heart failure needs to be managed using standard therapies.[22]

Plus – pulmonary valve replacement ± anticoagulation

Back
ONGOING
|
chronic pulmonary regurgitation
|
symptomatic with New York Heart Association (NYHA) class II or III
Plus – 

pulmonary valve replacement ± anticoagulation

Treatment recommended for ALL patients in selected patient group

Pulmonary valve replacement, usually with a homograft or xenograft, has been performed with low risk of complications.[21]​ Most specialists would perform pulmonary valve replacement in patients with NYHA class II or III symptoms and severe pulmonary regurgitation.[24]

Transcatheter pulmonary valve replacement is a less invasive approach than surgery and may be considered in some patients.[15][16]​​ Surgical valve replacement may be the only option in patients with large right ventricular outflow tracts and/or main pulmonary arteries, due to sizing of the available devices.[17]​ Transcatheter pulmonary valve replacement has been demonstrated to have comparable mortality and need for reintervention compared to surgical valve replacement, but with greater rates of infective endocarditis.[2][18]​​​

The appropriate valve used in pulmonary valve replacement needs to be tailored for the individual patient depending on age, multiple operations, and the need for long-term anticoagulation. Traditionally bioprosthetic pulmonary valves have been implanted; however, they have the disadvantage of time-related structural valve failure.[14]​​ Mechanical pulmonary valves may be advantageous due to their structural stability, but they have been infrequently used due to concerns regarding thrombosis and difficulty performing invasive hemodynamic studies following mechanical valve replacement.​​

Reports on long-term outcomes of mechanical pulmonary valves are emerging; however, there is a selection bias for patients with prior operations in such reports.[25]

All patients who receive a mechanical valve require lifelong anticoagulation. Bioprosthetic valves do not require anticoagulation. Anticoagulation may also be indicated due to the presence of other mechanical prostheses or as part of the treatment of comorbid conditions.[14]​​

Primary options

warfarin: 2-5 mg orally once daily initially, adjust dose according to target international normalized ratio

More

1st line – treatment of underlying cause + individualized medical and/or surgical treatment

Back
ONGOING
|
chronic pulmonary regurgitation
|
symptomatic with New York Heart Association (NYHA) class IV symptoms
1st line – 

treatment of underlying cause + individualized medical and/or surgical treatment

Patients are defined as NYHA class IV if they are confined to their bed or chair, any physical activity brings on discomfort, and symptoms occur at rest.

There are no guidelines or standard protocols on management of patients with NYHA class IV symptoms. Specialist referral for individualized management is advised in all circumstances.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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