Abstract
Congenital absence of one pulmonary valve cusp with normal morphology of
the other two cusps is a very rare finding. In this unusual case we
present the first images of 3-dimensional pulmonary valve reconstruction
as a useful tool for preoperative evaluation of pulmonary valve anatomy.
A 46 years old male patient presented pulmonary valve (PV) regurgitation
and left pulmonary artery (PA) aneurysm. Preoperative computed
tomography (CT) confirmed a 50x65 mm left PA aneurysm, normal pulmonary
annulus (25 mm) and normal pulmonary trunk (35 mm) (Figure 1 A-B).
Multiplanar reformation (Figure 2A,arrow), virtual angioscopy (Figure
2B), and volume rendering reconstruction (Figure 2 C-D) showed the
complete absence of the left-posterior pulmonary valve cusp. The other
two cusps appeared normal. At surgical inspection radiological findings
were confirmed. Insufficient PV was excised (Figure 3A) and replaced
with a 23 mm Inspiris Resilia bioprosthesis (Edwards Lifesciences,CA,
USA). Pulmonary reductive elliptical left arterioplasty was also
performed. Microscopic examination showed only a mild focal myxoid
degeneration of two pulmonary cusps, otherwise normal (Figure 3B).
Postoperative course was uneventful. Agenesis of one PV cusp with normal
morphology of the other two cusps, is a very rare
finding.1,2 In the only two cases previously reported,
patients became symptomatic and were operated at a younger age (21 and
16 years old) than our patient. Differently from our case, both previous
patients presented also aneurysm of pulmonary trunk. In the current era,
CT 3-dimensional reconstructions could be determinant for diagnosis and
may represent a very useful technique for optimal preoperative
evaluation of PV morphology.