Approach
Management of pulmonary regurgitation is different from that of aortic regurgitation and rarely requires surgery. Treatment should be directed at the underlying pathology (e.g., pulmonary hypertension, mitral stenosis, left ventricular (LV) dysfunction, and other underlying conditions). 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. While the likelihood of isolated right-sided heart failure secondary to pulmonary regurgitation is low, it should be considered in patients with lower extremity edema, elevated jugular venous pressure, hepatomegaly, or ascites. In severe cases where right-sided heart failure has occurred, diuretics may be used. Pulmonary valve replacement is required in patients post tetralogy of Fallot repair or Ross procedure who develop New York Heart Association (NYHA) class II or III symptoms, but may be considered earlier.
There are no specific criteria to grade severity of pulmonary regurgitation (PR). Severity of PR is usually assessed by the diameter of the jet at its origin immediately below the valve, in the right ventricular outflow tract (RVOT), on the parasternal short axis view. A jet width that occupies >65% of the RVOT is suggestive of severe PR.[8] Symptoms and signs of severe pulmonary regurgitation include dyspnea on exertion, syncope, orthopnea, and lower extremity edema. In many situations (e.g., acute pulmonary regurgitation post valvuloplasty, post-tetralogy of Fallot repair patients with NYHA class II symptoms or higher) the clinical judgment of severity is more dependent on symptoms than on imaging.
When valve replacement is indicated, transcatheter pulmonary valve replacement is a less invasive approach than surgery and may be considered in some select patients.[15][16] Initially transcatheter pulmonary valve replacement was applied to those patients with prior surgical pulmonary valve replacement or RV outflow tract conduits, but with new devices this technique is also being successfully applied to patients with native RV outflow tracts. In those patients with large RV outflow tracts and/or main pulmonary arteries, surgical pulmonary valve replacement may be the only option, due to sizing of the available devices.[17] Transcatheter pulmonary valve replacement has been demonstrated to have comparable mortality and need for reintervention when compared to surgical pulmonary valve replacement, but with greater rates of infective endocarditis.[2][18]
Acute pulmonary regurgitation
Acute pulmonary regurgitation is an almost unavoidable complication of balloon pulmonary valvuloplasty and can be managed conservatively in most cases. The clinical judgment of severity in these patients is more dependent on symptoms than on imaging. Severe pulmonary regurgitation post valvuloplasty is uncommon, affecting 17% of children in one study.[19] Treatment of even the most severe cases is directed at treating heart failure. Pulmonary valve replacement may be required, especially in a neonate with critical pulmonary stenosis following balloon dilation and a large patent ductus arteriosus. Fluid resuscitation and intravenous pressors such as dopamine or dobutamine may be necessary, acutely, for severe disease.
Chronic pulmonary regurgitation in asymptomatic patients
In most cases, no specific treatment is required for nonsevere pulmonary regurgitation, and treatment is directed at the underlying cause. There is growing recognition of the role of pulmonary valve replacement in the asymptomatic patient with evidence of hemodynamically significant pulmonary regurgitation.[20] In those patients with repaired tetralogy of Fallot, there are now guideline recommendations for pulmonary valve replacement based on changes in objective exercise capacity, RV end-diastolic and end-systolic volumes, and RV systolic dysfunction.[15][16] It is important to note that extrapolation of these criteria to those patients with isolated pulmonary regurgitation after intervention for pulmonary valve stenosis may not be appropriate. The typical situation in which pulmonary valve replacement may be considered is in patients who develop pulmonary regurgitation after repair of tetralogy of Fallot, patients with a dysfunctional RV to pulmonary valve conduit such as in repaired truncus arteriosus or following 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). Many would share the concern that it may be unwise to wait until RV function deteriorates in these patients, and that valve replacement should be considered before irreversible damage to ventricular performance occurs.[20][21]
Chronic pulmonary regurgitation in patients with NYHA class I symptoms
Patients are classed as NYHA class I if 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.
In most cases, no specific treatment is required. Underlying conditions such as LV failure, mitral stenosis, and pulmonary hypertension need to be treated. Heart failure should be managed using standard therapies.[22] Patients who develop pulmonary regurgitation after repair of tetralogy of Fallot or a Ross procedure may be considered for pulmonary valve replacement, as it may be better to perform valve replacement early in these patients.[23] If pulmonary regurgitation is severe and associated with right-sided heart failure, then pulmonary valve replacement may be necessary. There are, however, no definitive guidelines and the best course of action is unclear.
Chronic pulmonary regurgitation in patients with NYHA class II or III symptoms
Clinical judgment of disease severity is more dependent on symptoms than on imaging in this situation. Most patients with NYHA class II or III symptoms have developed pulmonary regurgitation as a complication of tetralogy of Fallot repair or a Ross procedure. Underlying and associated conditions such as LV failure, mitral stenosis, and pulmonary hypertension need to be treated. Pulmonary valve replacement, usually with a homograft or xenograft, has been performed with low risk of complications, and most specialists would perform pulmonary valve replacement in patients with NYHA class II or III symptoms.[21][24]
Chronic pulmonary regurgitation in patients with NYHA class IV symptoms
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 these patients. Specialist referral for individualized management is advised in all circumstances.
Choice of valve replacement
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 available; 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]
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