Pulmonic regurgitation

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Pulmonic regurgitation
ICD-10 I37.1, Q22.2
ICD-9 424.3, 746.09
DiseasesDB 11014
eMedicine med/1964 
MeSH D011665

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Synonyms and related terms: Pulmonary Insufficiency, Pulmonary Incompetence, Pulmonary Valve Regurgitation, Pulmonary Regurgitation, Pulmonary Valve Incompetence, Pulmonary Incompetence.

Pulmonary valve insufficiency (or incompetence, or regurgitation) is a condition where the pulmonary valve is not strong enough to prevent backflow into the right ventricle. Nearly all individuals have physiologic (trace-to-mild) pulmonic regurgitation, and the incidence increases with advancing age.

Pathophysiology

Differential diagnosis of underlying causes

Pulmonary hypertension (both primary and secondary pulmonary hypertension can cause insufficiency as a result of dilatation of the valve ring)

Infective endocarditis

Rheumatic heart disease

Congenital abnormalities (e. g., tetralogy of Fallot, ventricular septal defect, valvular Pulmonic stenosis)

Carcinoid heart disease (the majority of patients with metastatic disease will have both pulmonic stenosis and pulmonic regurgitation)

Marfan syndrome

Trauma from withdrawing a Swan-Ganz catheter with the balloon inflated.

Following valvuloplasty of pulmonary stenosis

Syphilis infection

Absence of the pulmonic valve

Fenestrations in or redundant leaflets of the pulmonic valve

Diagnosis

Symptoms

The condition may be asymptomatic or the patient may develop signs of right heart failure. Symptoms of right heart failure may develop when there is right ventricular enlargement and failure. Symptoms are right heart failure would include most commonly dyspnea on exertion, peripheral edema, chest pain and fatigue. Patients may also complain of palpitations, light-headedness, as well as frank syncope. Advanced or later symptoms include the development of ascites, right upper quadrant pain (as a result of hepatic distension), and early satiety.

Care should be paid to symptoms that might be associated with an underlying etiology of the pulmonic regurgitation.

Physical Examination

A low-pitched murmur is present which is usually best heard along the third or fourth intercostal spaces adjacent to the left sternal border. The murmur may be accentuated with inspiration. When the pulmonary artery systolic pressure exceeds 70 mm Hg, dilatation of the pulmonary artery ring may then result in Graham-Steell's murmur. This is a high-pitched, blowing decrescendo murmur heard best along the left parasternal region. Pulmonic regurgitation is associated with wide splitting of S2. A right-sided S3 may be audible and may also be accentuated with inspiration. Likewise, a right-sided S4 may also be audible and accentuated with inspiration.

Electrocardiogram

Both right ventricular hypertrophy and right bundle branch block may be present.

Chest X-Ray

Both an enlarged pulmonary artery and an enlarged right ventricle may be present.

Echocardiography

M-Mode echocardiography

Right ventricular enlargement is often present with a right ventricular volume overload pattern. Fine diastolic fluttering of the tricuspid valve may be observed. Premature opening of the pulmonic valve (defined as pulmonic valve opening on or before the QRS complex) may be observed as result of severe acute pulmonary insufficiency.

2-D echocardiography

Two-dimensional echocardiography may reveal the anatomic basis for the pulmonary insufficiency including causes such as infective endocarditis and valvular pulmonic stenosis. Dilatation of the right ventricle may be present, as well as a right ventricular volume overload pattern.

PW Doppler echocardiography

Care must be exercised in interpreting pulse wave Doppler echocardiography as up to 87% of normal patients may appear to have pulmonary insufficiency on examination. It is therefore critical to calculate the length and duration of the regurgitant jet to differentiate between true and physiologic insufficiency. In physiologic insufficiency the jet is < 1 cm in length and not holodiastolic in duration. The severity of pulmonary insufficiency should be assessed using mapping techniques.

CW Doppler echocardiography

In this analysis, a comparison is made between the regurgitant Doppler spectral display and the pulmonic outflow Doppler spectral display. The pulmonary artery end-diastolic pressure is assessed as well.

Color Flow Doppler echocardiography

In this analysis, the length and width of the pulmonary insufficiency is assessed. If there is a pattern of proximal acceleration (flow convergence), this is consistent with 3+ or 4+ pulmonary insufficiency.

Pulmonary Insufficiency Severity Scales

PW and Color flow Doppler
  • Physiologic : < 1 cm in length and not holodiastolic in duration
  • Borderline : 1 to 2 cm in length and holodiastolic in duration
  • Clinically significant : > 2 cm in length with a peak velocity > 1. 5 m/sec and holodiastolic in duration
CW Doppler Spectral Strength of Regurgitant Jet
  • Grade 1+ : Spectral tracing stains sufficiently for detection, but not enough for clear delineation
  • Grade 2+ : Complete spectral tracing can just be seen
  • Grade 3+ : Distinct darkening of spectral tracing is visible but density is less than antegrade flow
  • Grade 4+ : Dark-stained spectral tracing

Pulmonary Angiography

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Treatment

If the patient develops right-sided heart failure that is refractory to medical management, pulmonic valve repair or replacement (preferably with a bio prosthetic valve) may be necessary.

Patients with pulmonic regurgitation who become pregnant usually tolerate pulmonic regurgitation well. Exceptions to this rule include, those patients who have undergone surgical repairs for tetralogy of Fallot.

Antibiotic Prophylaxis

The American Heart Association Recommendations on Prevention of Bacterial Endocarditis indicate that antibiotic prophylaxis is not necessary for pulmonic regurgitation in those patients with otherwise structurally normal pulmonic valves, particularly if there is no diastolic murmur. It should be noted, though, that those patients with the following conditions may warrant antibiotic prophylaxis:

  1. Complex cyanotic heart disease
  2. Prosthetic heart valves
  3. Patients with congenital heart disease and pulmonic regurgitation
  4. Acquired pulmonic valve regurgitation as the result of rheumatic heart disease
  5. Patients with complex cyanotic heart disease
  6. In patients who have previously sustained bacterial endocarditis

Prognosis

Patient survival depends upon the underlying etiology of the pulmonic regurgitation. Among patients with pulmonary hypertension, the severity and duration of the pulmonary hypertension will be determinants of the ultimate prognosis. Patients with congenital absence of the pulmonic valve have severe regurgitation and this may limit their life expectancy if the valve is not replaced. Mild to moderate pulmonic regurgitation is not associated with shortened survival.

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