Stenosis can occur proximal to the PV. Infundibular or subvalvular stenosis is typically caused by diffuse fibromuscular narrowing of the right ventricular outflow tract and is considered part of the spectrum of the double-chambered right ventricle.4 It can be an isolated finding or a component of complex congenital heart disease such as tetralogy of Fallot or D-transposition of the great arteries.3,21 It is well demonstrated on CT or MRI (Fig. 8). Consequences of subvalvular stenosis include right ventricular hypertrophy and eventual right heart failure. Infundibular stenosis is distinct from secondary outflow tract narrowing that occurs as a sequela of pulmonary hypertension or valvular pulmonary stenosis. Secondary outflow tract narrowing can be corrected by valvotomy or valvuloplasty, whereas infundibular stenosis is not corrected by repair of the PV.22
Treatment for PV stenosis includes balloon valvuloplasty and surgical valvulotomy. Endovascular or operative correction of pulmonary stenosis can lead to severe regurgitation. The decision to replace the PV with a mechanical or bioprosthetic device depends on the size and function of the right ventricle in addition to the extent of patient symptoms.22
Although the PV ordinarily comprise three equal-sized leaflets (anterior, left, and right) arranged in a semilunar pattern, developmental abnormalities of the valve occur, including unicuspid, bicuspid, or quadricuspid arrangements. Such idiosyncratic anatomy can be diagnosed incidentally with imaging or at autopsy, or lead to shortness of breath and jugular venous distention associated with inhibition of blood flow exiting the right ventricular outflow tract.23
Blood-borne infections that seed the PV leaflets leading to endocarditis can cause severe morbidity and mortality (Fig. 1C). Among the cardiac valves, endocarditis of the PV is least common.25 It occurs most often in intravenous drug users, in patients with congenital heart disease, in patients with automatic implantable cardiac defibrillators, and in those with central venous lines.26 Endocarditis of the valve typically results in friable vegetations that can be seen on echocardiography, CT, or MRI. Gross pathologic correlation reveals macroscopic deposits randomly situated on all valve leaflets. These vegetations cause abnormal flow jets and turbulence that are visible with color Doppler and the cine features available with cross-sectional imaging (Fig. 10). Vegetations can also dislodge from the valve leaflets and embolize to the pulmonary vasculature and lung parenchyma, causing ischemia or spread of infection. Vegetations <1-2 cm usually respond to medical treatment. Surgery for larger lesions may be necessary.
Both benign and malignant tumors can arise from or metastasize to the PV. Papillary fibroelastoma is the most common primary valvular tumor (Fig. 1D). It can originate from any endocardial surface, but 80% to 90% are situated on the valves themselves. Other endocardial surfaces account for 10% of papillary fibroelastomas.27 These locations include the left ventricular apex, chordae tendinae, and the right and left ventricular outflow tracts. It is rare for a patient to have multiple papillary fibroelastomas. The valvular frequency of fibroelastomas is aortic (35%), mitral (25%), tricuspid (17%), and pulmonary (13%).27 Most cases of valvular papillary fibroelastoma are discovered at autopsy (Fig. 12). Treatment is surgical excision and reconstitution or replacement of the valve to prevent postoperative stenosis or insufficiency. Fibroelastomas may show delayed enhancement on MRI because of the presence of fibrous tissue. Differential diagnosis includes bland thrombus, valvular vegetations, and tumor. Microscopically they have characteristic gelatinous, branching fronds.
Carcinoid syndrome can also affect the PV. A neoplasia arising from enterochromaffin cells, carcinoid tumors produce biologically active substances, of which serotonin is the most common. Vasoactive substances released from carcinoid metastases to the liver can reach the right side of the heart and result in fibrous deposition on endocardial surfaces (Fig. 13). Right-sided endocardial deposits can be seen in up to 50% of patients with liver-involved carcinoid syndrome.10 A PV that is affected by carcinoid deposits appears thickened, retracted, or stenotic on imaging, with narrowing of the annulus. Symptomatic patients may require surgical intervention. Because replacement with biological valve prosthesis can lead to recurrent carcinoid degeneration, use of mechanical prostheses is recommended. Patients who present late in the disease course are often treated with medical management only.
The PV receives less attention than the tricuspid or left-sided heart valves, but it can be affected by a wide variety of pathologic processes. Congenital abnormalities, most notably pulmonary stenosis, can cause symptoms even in adulthood. Acquired diseases such as endocarditis and tumors such as papillary fibroelastoma can also lead to significant morbidity and mortality.
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