The Annals of Thoracic Surgery, Vol 37, 12-24, Copyright © 1984 by The Society of Thoracic Surgeons
Surgical management of hypoplastic right ventricle with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum
CS Weldon, AF Hartmann Jr and RC McKnight
Our experience with the surgical management of hypoplastic right ventricle
with intact ventricular septum includes 26 patients with pulmonary atresia
and 4 with critical pulmonary stenosis. Group 1 consisted of 8 neonates
managed initially by transventricular valvotomy; 6 later required a
secondary procedure, with 100% survival. Group 2 had 11 neonates managed by
aorta-pulmonary artery shunting without operative death. However, only 3
have survived over the long term and 1 has required an additional shunt
procedure. Group 3 had 9 infants who underwent concomitant valvotomy and
shunting. There were 4 operative deaths and 1 late death. Finally, Group 4
included 2 infants managed by primary repair at 3 days and 6 days old with
prosthetic enlargement of the right ventricle; 1 required the addition of a
shunt. Both are alive. Seven of the 15 patients in Groups 1, 2, and 3 who
survived neonatal palliative procedures have undergone reparative
operations. Two had no growth of the right ventricle and underwent repair
after conversion to tricuspid atresia, by a Fontan procedure. Five had
prosthetic enlargement of the right ventricle in childhood with 1 late
death. Findings of this review were as follows: (1) effective palliation of
pulmonary atresia and intact ventricular septum or critical pulmonary
stenosis with cavitary hypoplasia of the right ventricle is rare unless
transventricular flow can be established; (2) establishment of
transventricular flow produces a high incidence of cavitary "growth," which
permits later repair; (3) the Fontan operation is available for repair in
patients who have no cavitary growth; and (4) when all three portions of
the right ventricular cavity can be identified by angiography, a primary
repair can be performed in the neonatal period with a good long-term
prognosis.
