Pulmonary Valve Insufficiency

Pulmonary valve insufficiency patients are asymptomatic if disease severity is mild. However, one-third of them will become symptomatic before reaching the age of forty years [1]. Severe regurgitation leads to symptoms of right heart failure [2] like dyspnea, initially with physical effort and later at rest [3]; fatiguability, abdominal distention due to ascites, liver distention causing pain in the right hypochondrium, early satiety and peripheral edema. Non-specific signs include angina due to the inability of the coronary arteries to properly oxygenate a dilated right ventricle, palpitations caused by atrial [4] or ventricular arrhythmias, that have a potential to be life threatening [5] and syncope- an insufficient, dilated heart will not provide proper cerebral blood flow. Symptoms associated with the underlying disease causing secondary pulmonic regurgitation may also be encountered: fever in infective endocarditis, hemoptysis in pulmonary hypertension or articular symptoms in rheumatic heart disease. Furthermore, acute neonatal pulmonary valve insufficiency caused by a large patent ductus arteriosus in Ebstein's anomaly patients has also been described [6].

Physical examination reveals an accentuated and delayed pulmonary component of the second heart sound if pulmonary hypertension is present. The pulmonary insufficiency murmur is brief, early diastolic and decrescendo and increases with squatting and inspiration. Valsalva maneuver decreases its intensity. If pulmonary hypertension coexists, the murmur modifies its character by becoming high pitched and possibly holo- diastolic. A tricuspid regurgitation murmur may overlap, making auscultation more difficult. If the right ventricle is enlarged, an impulse may be palpated along the left sternal border. Signs of elevated jugular venous pressure signs may also be observed.

Blood workup should include brain natriuretic peptide plasma level that has proven useful in monitoring disease severity and right ventricular end-diastolic pressure [7].

Thoracic radiography is useful especially in pulmonary hypertension patients, where the typical aspect of enlarged central pulmonary and hilar vessels and diminished peripheral vascularization is observed. Cardiomegaly may also be present.

The electrocardiogram may show right ventricular hypertrophy (tall R or QR aspect in V1, negative T wave in V1-V3 and right axis deviation) or dilatation (complete or incomplete right bundle branch block and right axis deviation) and right atrial enlargement.

Echocardiography further characterizes chamber dimensions, wall thickness, and kinetics, as well as the aspect of the pulmonary valve, that may be dilated, perforated, ruptured or have attached vegetations. An immobile or abnormal motion of the interventricular septum is an indirect indicator of pulmonary hypertension and increased right ventricular pressures. Color flow Doppler offers a qualitative assessment of the regurgitant pulmonary jet in terms of dimensions and orientation. If regurgitation is holodiastolic or pulmonary acceleration time (normally 140 msec) is decreased, pulmonary hypertension must be suspected and demonstrated by calculating pulmonary artery systolic and diastolic pressures, both good predictors of cardiac status [8] [9]. Vena contracta has not yet been validated for pulmonary regurgitation. Regurgitant volume and regurgitant fraction can be calculated using pulsed Doppler [10] and stroke volume.

Pulmonary artery angiography is indicated when pulmonary embolism as a cause of pulmonary hypertension and pulmonary valve insufficiency is suspected. The same information can be obtained with less invasive methods, such as computer tomography angiography or ventilation/perfusion scanning, while cardiac magnetic resonance imaging offers high quality characterization of pulmonary valve anatomy and regurgitation mechanism.

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