The Annals of Thoracic Surgery, Vol 40, 409-413, Copyright © 1985 by The Society of Thoracic Surgeons
Comprehensive management of pulmonary atresia with intact ventricular septum
PS Rao
The prognosis for patients with pulmonary atresia with intact ventricular
septum is poor with or without conventional surgical intervention.
Therefore, a comprehensive program of medical and surgical treatment is
necessary to improve long-term outlook for these infants. Such a program
consists of management of the neonate at initial presentation with prompt
administration of prostaglandins and institution of a combination of
surgical procedures (isolated pulmonary valvotomy, valvotomy plus modified
Blalock-Taussig shunt, Blalock- Taussig shunt plus balloon atrial
septostomy, or Blalock-Taussig shunt alone) depending on the results of
morphological analysis of the right ventricle; this treatment regimen is
designed to relieve hypoxemia, encourage right ventricular growth, and
provide adequate egress of blood from the right atrium. Another important
element of management is to perform follow-up hemodynamic and angiographic
studies when the patient is between 6 and 12 months old to ensure that the
objectives of the comprehensive program are being met. Finally, a
definitive repair should be offered. This can be done by using or bypassing
the right ventricle, depending on whether it can support the pulmonary
circuit.
