The Annals of Thoracic Surgery, Vol 42, 264-268, Copyright © 1986 by The Society of Thoracic Surgeons
Surgical management of patients with pulmonary valve dysplasia
WH Merrill, JR Stewart, JW Hammon Jr, RJ Boucek Jr and HW Bender Jr
Pulmonary valve dysplasia is a distinct pathological entity consisting of
markedly thickened, deformed, and largely immobile pulmonary valve
leaflets. The clinical features and surgical management of 21 consecutive
patients operated upon between 1975 and 1985 were reviewed. Mean age at
operation was 26.7 months (range: 1 month to 9.5 years). Initial surgical
intervention in 3 patients consisted of closed valvotomy or systemic
pulmonary artery shunt. One of these patients subsequently had a partial
pulmonary valvectomy; the other 2 had total valvectomy and pulmonary
annuloplasty. Two patients had partial valvectomy as a primary procedure,
and 1 has subsequently required total valvectomy and annuloplasty. Sixteen
patients initially had total pulmonary valvectomy, 4 with a simultaneous
transannular outflow tract patch. One patient subsequently required a
transannular outflow tract patch. Therefore, definitive repair consisted of
partial valvectomy in 2 patients, total valvectomy in 19, and a
transannular outflow tract patch in 9 patients with a hypoplastic pulmonary
annulus. There were no operative deaths, and all patients are asymptomatic
at a mean follow-up of 37.9 months. Five patients who underwent total
valvectomy were catheterized postoperatively. The mean residual gradient
was 25 mm Hg. Pulmonary valvectomy appears to provide both effective relief
of stenosis and long-term asymptomatic status in patients with pulmonary
valve dysplasia. Total excision of all valve leaflets is recommended.
Careful consideration should be given to a simultaneous transannular
outflow tract patch if the annulus is hypoplastic.
