The Journal of Thoracic and Cardiovascular Surgery, Vol 83, 650-658, Copyright © 1982 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
Pulmonary vascular morphology in shunted and nonshunted patients with tetralogy of Fallot
N Shapira, A Rosenthal, K Heidelberger, R Badanowski and D Behrendt
The pulmonary vascular morphology of 20 patients with tetralogy of Fallot
(TOF), aged 2 months to 29 years, was studied by lung biopsy. In eight
patients, systemic-to-pulmonary anastomosis had been performed prior to the
lung biopsy and repair. The findings were compared to control data obtained
by biopsy (n = 8) or necropsy (n = 23). On comparison to control subjects,
patients with TOF displayed increased wall thickness of the intra-acinar
arteries, extensive extension of smooth muscle to the alveolar wall (AW)
and alveolar duct (AD) arteries, and increased diameter of the AW vessels.
Intimal proliferation and fibrosis was observed in two patients. Large
right-to- left shunt (Q p/Qs less than or equal to 0.75) was associated
with an increased number of arteries, whereas left-to-right shunt (Q p/Q s
greater than or equal to 1) was associated with a decreased number of
vessels. The severity of the pulmonary vascular changes were similar in the
shunted (N = 8) and nonshunted (n = 12) groups and in the ipsilateral and
contralateral lungs of four shunted patients having bilateral biopsy. We
conclude that some pulmonary morphologic features characteristic of
pulmonary vascular obstructive disease are present in all patients with TOF
surviving operation. An appropriate shunt causes no additional detrimental
effect on the pulmonary vasculature. Thus, although early repair may be
desirable for a variety of reasons, it is not preferable over a staged
repair with respect to the pulmonary vasculature.
