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Pulmonary valve stenosis (PS) is a common form of congenital heart disease that may present at any age, but often in infancy or early childhood in its more severe forms. The adult patient with PS tends to have more clinical symptoms than a child with a similar degree of PS; gradients can be progressive, often due to acquired right ventricular outflow tract (RVOT) infundibular muscle hypertrophy. The patient with an interatrial communication (patent foramen ovale/atrial septal defect [PFO/ASD]) may experience a right-to-left shunt as right ventricular hypertrophy progresses and compliance decreases. Percutaneous pulmonary balloon angioplasty (PBA) has been performed safely and effectively since 1982 with low morbidity and mortality. Acute success rates for PBA are reported to be between 80% and 90%, with potential for ongoing reduction in gradient due to spontaneous resolution of infundibular hypertrophy. However, the patient may require beta-blocker therapy as RVOT obstruction resolves.
Indications for intervention typically include the following: (1) pulmonary valve mean gradient by Doppler ≥35 to 40 mmHg and (2) symptomatic functional limitation. Favorable anatomy includes a normal pulmonary valve annulus with thin, doming pulmonary valve leaflets, and post-stenotic main pulmonary artery dilation. A thickened, dysplastic pulmonary valve with hypoplastic annulus and/or no evidence of main pulmonary artery dilation is much less likely to respond favorably to PBA, although some degree of reduction in valve gradient may provide symptomatic relief in the adult.
In adults with moderate or severe valvular pulmonary stenosis and otherwise unexplained symptoms of heart failure, cyanosis from interatrial right-to-left communication, and/or exercise intolerance, balloon valvuloplasty is recommended | I | B-NR |
In asymptomatic adults with severe valvular pulmonary stenosis, intervention is reasonable | IIa | C-EO |
Transthoracic echo is usually sufficient for diagnosis and can be reliable in determining the pulmonary valve gradient (mean Doppler gradient typically correlates best with peak-to-peak systolic gradient measured at cardiac catheterization). The nature of the pulmonary valve leaflets should be determined (i.e., thickened, dysplastic). The pulmonary valve annulus should be evaluated carefully and measured to provide guidance in balloon selection. In the adult patient with challenging acoustic windows, transesophageal echo or computed tomography can be considered (particularly to evaluate infundibular anatomy, anatomy of the main pulmonary artery, or pulmonary branch arteries if other stenotic lesions are suspected).
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