Pulmonary stenosis refers to a narrowing of the valve between the right ventricle and the pulmonary artery. Occasionally, the obstruction occurs within the body of the right ventricle. As pulmonary stenosis becomes more severe, the thickness of the right ventricle increases and produces right ventricular hypertrophy.
Management of pulmonary stenosis is determined by the degree of obstruction. Patients with mild pulmonary stenosis usually have no cardiac symptoms and are followed medically. Patients with greater degrees of valvar pulmonary stenosis can often be treated with a balloon valvuloplasty. If the valve is not calcified, the leaflets can usually be opened with a balloon avoiding the need for pulmonary valve replacement. If balloon valvuloplasy is unsuccessful, surgery is highly effective in relieving the obstruction.
Patients with severe obstruction within the right ventricle require surgery to remove the excess muscle. These patients have either infundibular pulmonary stenosis or a double chambered right ventricle.
The long term issues for adults following repair of pulmonary stenosis include arrhythmias and the effect of a chronic leak of the pulmonary valve on right ventricular size and function. In some adults, the degree of right ventricular enlargement is enough to warrant pulmonary valve replacement. This occurs more frequently when the leak in the pulmonary valve is accompanied by a leak in the tricuspid valve. Echocardiography and cardiac MRI are useful ways to assess for these problems.
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