Clinical anatomy of the normal pulmonary root compared with that in isolated pulmonary valvular stenosis.

OBJECTIVES This study aimed to clarify the clinical anatomy of the pulmonary root. BACKGROUND Many descriptions of valvular anatomy have focused on the annulus, leading to varied interpretations of abnormal valves. METHODS Twenty-two heart specimens with isolated pulmonary valvular stenosis were examined to analyze the gross structure of the pulmonary root. For comparison, we examined a normal series of a similar age range together with nine adult hearts. Serial histologic sections were prepared from five specimens. RESULTS The normal pulmonary valve is enclosed in a proximal sleeve of free-standing right ventricular infundibulum supporting the fibroelastic walls of the pulmonary sinuses at the anatomic ventriculoarterial junction. The valvular leaflets are attached in semilunar fashion across this junction, delimiting the extent of the valvular sinuses. The stenotic valves were separated into dome-shaped valves, dysplastic valves and a third group of less typical cases. In the dome-shaped valves, which had a relatively circular origin of their leaflets, three raphes were tethered to the arterial wall at the sinutubular junction, producing a waistlike narrowing. The leaflets of the dysplastic valves were attached in a relatively normal semilunar fashion, but stenosis was caused by thickening of the leaflets at their free edges. Serial histologic sections through normal and abnormal valves failed to demonstrate any well defined fibrous "annulus" that could be of clinical relevance. CONCLUSIONS Unlike the normal and the dysplastic valves, the dome-shaped valves have circular rather than semilunar lines of attachment of the valvular leaflets. Liberation of the fused zones of apposition of the leaflets within the dome is unlikely to restore such abnormal valves to normal structure, even if this procedure relieves the stenosis.