The Journal of Thoracic and Cardiovascular Surgery, Vol 90, 29-34, Copyright © 1985 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
Surgical management of double-outlet right ventricle
A Mazzucco, G Faggian, G Stellin, U Bortolotti, U Livi, G Rizzoli and V Gallucci
From 1977 to 1983, 32 consecutive patients, ranging in age from 15 days to
24 years, underwent operations for double-outlet right ventricle. Twenty
patients had a palliative operation either to increase (12 cases) or to
reduce (eight cases) pulmonary blood flow: Ten of them have subsequently
undergone total repair, and in another six correction was delayed because
of possible incremental operative risk factors, such as multiple
ventricular septal defects or the need for an extracardiac conduit. Four
patients with multiple, complex associated intracardiac anomalies are
currently considered to have uncorrectable defects. A total of 22 patients
underwent correction either primarily (12) or after palliation (10).
Intraventricular tunneling was performed in 16 patients with a subaortic
ventricular septal defect and in one with a doubly committed ventricular
septal defect. Seven of these had pulmonary stenosis and five had
reconstruction of the right ventricular outflow by means of a patch (three)
or a conduit (two); among this group, five also had enlargement of the
ventricular septal defect. In three patients with a subpulmonary defect and
in one with a remote ventricular septal defect, all of them without
pulmonary stenosis, total repair was achieved by a Senning, a Mustard, or
an arterial switch operation. Finally, the only patient with
atrioventricular discordance and pulmonary stenosis had insertion of a left
ventricle- pulmonary artery conduit. No operative deaths were observed
after palliation, but one patient died of intrapulmonary hemorrhage after
total repair (4.5%). Major postoperative complications included detachment
of the ventricular septal defect patch in one patient and late progression
of pulmonary vascular obstructive disease in another. No late deaths have
been recorded. Surgical repair of double-outlet right ventricle can be
accomplished with gratifying early and late results, the risk of operative
death being below 5%. The outcome in patients with subaortic ventricular
septal defect appears particularly favorable, despite the extensive
intracardiac procedures required for total correction. An early
intervention is recommended to prevent development of pulmonary vascular
obstructive disease and to avoid massive cardiac hypertrophy and fibrosis,
which may cause late rhythm disturbances and impede the intracardiac
repair.
